Adel K. Younoszai, MD

• Assistant Professor of Pediatrics
• Director of Cardiac Imaging
• The Children? Hospital
• Denver, Colorado

Finding other areas of conventional squamous cell carcinoma resolves this differential diagnosis arthritis mutilans feet buy discount indocin 75mg online. A chronic inflammatory cell infiltrate is usually present arthritis panel buy indocin 25 mg overnight delivery, as are thick-walled reactive arthritis in dogs discount 25 mg indocin amex, peripheral blood vessels arthritis in feet diagnosis purchase indocin 25 mg fast delivery. B arthritis treatment by ayurveda purchase discount indocin on line, Slit-like vascular spaces lined by vaguely myoid-appearing spindled cells in Kaposi sarcoma arthritis quinine cheap indocin 25 mg. The majority of leiomyomas of the head and neck arise in the dermis, from pilar smooth muscle, with a smaller number arising from vascular smooth muscle in the subcutis and within the oropharyngeal cavity, ear, nose, and larynx. Pilar leiomyomas may be multiple and painful, whereas more deeply seated leiomyomas are usually solitary and present with symptoms related to the site of origin. Solitary dermal leiomyomas tend not to recur, whereas recurrence is frequent in patients with multiple leiomyomas. Occasional leiomyomas lack significant desmin expression; for this reason smooth muscle actin is a better screening marker for leiomyomas in all locations. Heterozygous loss of function germline mutations in fumarate hydratase, located on chromosome 1q42. B, Hypocellular fascicles of eosinophilic, normochromatic, mitotically inactive spindle cells in pilar leiomyoma. D, Deeply seated leiomyomas consist of bland, mature-appearing, amitotic smooth muscle. Although the peripheral zones in myofibroma may be indistinguishable from leiomyoma, they also display more central zones of primitive-appearing rounded cells, arranged about hemangiopericytoma-like vessels. The term atypical dermal smooth muscle tumor is now considered to be preferable to pilar leiomyosarcoma, as the natural history of these lesions is extremely favorable. Atypical dermal smooth muscle tumors may be extremely well differentiated, closely mimicking leiomyomas. As noted earlier, atypical dermal smooth muscle tumors can be difficult to distinguish from leiomyomas. Features that should strongly suggest this diagnosis include extension into the subcutis, size >2 cm, mitotic activity of greater than 1/10 high-powered fields, and the presence of hyperchromatic tumor cells. Fibromatoses grow in much longer fascicles and contain small, dilated blood vessels, as well as patchy chronic inflammation. Rhabdomyomatous mesenchymal hamartomas is an extremely rare tumor of the face and neck in newborns, which typically presents as a very small papule or pedunculated lesion. Almost all reported cases have occurred in males, and an association with congenital abnormalities has been described. Rhabdomyomatous mesenchymal hamartomas is adequately treated with local excision, and does not appear to recur. Histologically, rhabdomyomatous hamartomas consist of an intradermal and subcutaneous collection of mature skeletal muscle, arranged singly and in small groups. These skeletal muscle fibers are surrounded by a collagenized and variably myxoid stroma, with admixed mature adipose tissue, dermal adnexal structures, nerves, and blood vessels. Neuromuscular choristoma (benign Triton tumor) arises in association with a peripheral nerve and consists of an intimate admixture of nerves and muscle, without fat. Fibrous hamartoma of infancy is a triphasic tumor, consisting of primitive mesenchyme, fat, and mature fibrous tissue, without skeletal muscle. Embryonal rhabdomyosarcoma is a much more cellular tumor consisting of mitotically active immature skeletal muscle and rhabdomyoblasts. Fetal rhabdomyomas typically occur as a solitary mass in the subcutaneous tissue of the head and neck region of boys less than 5 years of age. Predominantly, myxoid fetal rhabdomyomas (myxoid type fetal rhabdomyoma) most often occur in the pre- and postauricular regions of infants; less myxoid, more cellular tumors (intermediate type fetal rhabdomyoma) occur more often in adults, and tend to involve the orbit, tongue, nasopharynx, and soft palate. Most fetal rhabdomyomas are 2 to 6 cm in size, and are well circumscribed but not encapsulated. Recurrent tumors should be very carefully reexamined for evidence that they may have represented subtle embryonal rhabdomyosarcomas. The myxoid type of fetal rhabdomyoma shows an abundant myxoid matrix, within which are found bland, elongate spindle cells and rare immature skeletal muscle fibers. C, Many leiomyosarcomas of somatic soft tissue arise from blood vessels, which may impart a deceptive appearance of encapsulation. D, Well-differentiated leiomyosarcoma, consisting of intersecting fascicles of mitotically active eosinophilic spindled cells, with blunt ended nuclei and perinuclear vacuoles. F, When leiomyosarcomas hyalinize, they may be very difficult to recognize as tumors of smooth muscle. Ganglion-like rhabdomyoblasts, matureappearing skeletal muscle cells with prominent cross-striations, and vacuolated cells are frequently present. Embryonal rhabdomyosarcoma is by far and away the most important entity in the differential diagnosis of fetal rhabdomyoma. Unlike fetal rhabdomyomas, embryonal rhabdomyosarcomas are poorly circumscribed, mitotically active, contain hyperchromatic cells, and typically show a reversed pattern of zonation, with the least mature cells present at the periphery of the lesion. Rhabdomyomatous mesenchymal hamartoma consists of mature skeletal muscle with associated nerves, collagen, fat, and adnexal structures. Adult rhabdomyomas usually occur in the head and neck of males older than 40 years of age (median 60 years of age), most often in the pharynx, oral cavity, tongue base, and larynx. Occasional spider cells, with clear cytoplasm and thin strands of material extending from the nucleus to the cytoplasmic membrane, are present. In our experience, these lesions tend to be negative for myogenin and MyoD1, suggesting an advanced degree of maturation. Adult rhabdomyomas may be confused with granular cell tumors, hibernomas, paragangliomas, and reticulohistiocytomas, as well as with rhabdomyosarcomas. Granular cell tumors, hibernomas, paragangliomas, and reticulohistiocytomas lack cross-striations and do not express skeletal muscle markers. Rhabdomyosarcomas are much more infiltrative lesions that display prominent pleomorphism, mitotic activity and often necrosis, and which usually occur in much younger patients. In the head and neck, the nose and paranasal sinuses are relatively frequently involved. Multinucleated tumor giant cells, with brightly eosinophilic cytoplasm, are occasionally identified within these nests, are foci of clear cell change. Rhabdomyoblastic differentiation is difficult to discern by light microscopy, with only rare, scattered rhabdomyoblasts. B, Alveolar rhabdomyosarcomas may consist entirely of primitive round cells, as in this example, or may show rhabdomyoblastic differentiation in the form of multinucleated tumor giant cells. C, Solid variant of alveolar rhabdomyosarcoma of the nose, mimicking other small cell malignant neoplasms. As with other high-grade pleomorphic sarcomas, it most often shows complex structural and numerical chromosomal abnormalities. Identification of such tumors as rhabdomyosarcoma requires the use of ancillary immunohistochemistry for myogenin and MyoD1. C, Sclerosing rhabdomyosarcoma, consisting of highly malignant round cells in an abundant, vaguely chondroosseous-appearing matrix. D, Microalveolar pattern and primitive round cells in sclerosing rhabdomyosarcoma. E, Desmin expression may be limited in extent in spindle cell/sclerosing rhabdomyosarcoma. B, Higher-power view of bizarre-appearing rhabdomyoblasts in pleomorphic rhabdomyosarcoma. Alveolar soft part sarcomas are composed of large, eosinophilic cells, rather than small, round cells. Predominantly spindled tumors may resemble a variety of other low- and high-grade spindle cell sarcomas. The presence of pleomorphic lipoblasts is definitional of pleomorphic liposarcoma. Many previously reported cases have behaved in an aggressive fashion, with metastatic disease and death from disease. It grows as fascicles of monotonous spindled cells with bland, hypochromatic nuclei (B). The tumor is consistently positive for both S100 protein (C) and smooth muscle actin (D). Superficial angiomyxoma presents as a small, slow-growing dermal or subcutaneous nodule, usually in the head and neck of adults. Although most angiomyxomas are not associated with Carney syndrome, one should always raise this possibility when confronted with this lesion. Angiomyxomas are typically poorly circumscribed, typically multinodular, and often entrap dermal adnexal structures, with secondary epithelial proliferation, often in the form of cysts. The presence of neutrophils is particularly characteristic of angiomyxoma and assists in its distinction from other low-grade myxoid tumors. Angiomyxoma is typically confused with other low-grade myxoid tumors, including myxofibrosarcoma and myxoid liposarcoma. Both myxofibrosarcoma and myxoid liposarcoma are rare in the head and neck, and typically present as much larger, more deeply situated tumors. Angiomyxomas lack the hyperchromatic spindled cells and the arborizing thick-walled vessels seen in myxofibrosarcomas, and may contain neutrophils, which are not typically a feature of myxofibrosarcomas. Myxoid liposarcomas usually show a much more fully developed, arborizing capillary network, show accentuated peripheral cellularity, and contain uni- and multivacuolated lipoblasts, often found in association with capillaries. Clinically, these lesions typically present as ulcerated, firm, single or multiple nodules that mimic nonspecific ulcers or "infected warts. Fusion of individual tumor nodules and extension along tendons may create a garlandlike or scalloped appearance. This pattern of necrosis and the relatively bland nuclear features of the neoplastic cells may closely mimic a granulomatous process, such as a necrobiotic granuloma. B, Arborizing small blood vessels, bland fibroblastic cells, and stromal neutrophils in superficial angiomyxoma. The cells usually have distinctly eosinophilic cytoplasm and relatively uniform appearing, but hyperchromatic nuclei. B, A characteristic feature of epithelioid sarcoma is this admixture of epithelioid and spindled cells, with imperceptible transition between the two forms. Highly spindled epithelioid sarcomas may be easily mistaken for cellular fibrous histiocytomas. C, Despite their aggressive behavior, epithelioid sarcomas are usually composed of deceptively bland cells. This is of particular concern in unusual locations, such as the head and neck, where clinical suspicion for epithelioid sarcoma may be extremely low. Keratin immunostains are often critical in making this crucial distinction, and one should have a very low threshold for performing such immunostains on any suspicious lesion, particularly in a child. The largest series to date has reported survival rates of 77% at 2 years, 60% at 5 years, 38% at 10 years, and only 15% at 20 years. Tumors occurring in younger patients and in confined locations, such as the tongue, often show very small nests of cells, closely mimicking paragangliomas. Nonspecific markers such as neuronspecific enolase and vimentin may be present in roughly 30% to 50% of cases. B, Nests of dishesive eosinophilic cells, with prominent nucleoli typify alveolar soft part sarcoma. B, Typical ossifying fibromyxoid tumor, with fibromyxoid-appearing matrix and small, bland round cells arranged in vague rosette-like structures, with uniform cell-cell spacing. C, Malignant ossifying fibromyxoid tumor, showing high cellularity and nuclear grade. Bone production may either be absent or may be increased, sometimes within the center of the lesion. Extraskeletal myxoid chondrosarcomas are extremely rare in the head and neck, and contain distinctly eosinophilic cells that grow in nests, cords and chains, often with abundant associated hemorrhage and hemosiderin staining. Anomalous desmin expression may be seen in a very small minority of cases (<3%), typically limited to only a few cells. B, the cells of Ewing sarcoma are usually uniform and small, with finely dispersed chromatin and small nucleoli. C, Adamantinoma-like Ewing sarcoma, consisting of a distinctly nested proliferation of primitive round blue cells. E, Strong expression of keratins, including high-molecular-weight keratins, is typically seen in adamantinoma-like Ewing sarcoma. Mesenchymal chondrosarcomas and small cell osteosarcomas contain chondroid or osteoid, respectively. Synovial sarcomas account for approximately 10% of soft-tissue sarcomas generally,156 and between 2% and 29% of soft-tissue sarcomas of the head and neck. Symptoms of head and neck synovial sarcoma are nonspecific, typically relating to the site of origin. B, Monomorphic, hyperchromatic spindled cells, with nonoverlapping nuclei in monophasic synovial sarcoma. D, Higher-power view of biphasic synovial sarcoma, with an admixture of spindled zones identical to monophasic synovial sarcoma, and malignant glands, lined by cuboidal epithelial cells. E, Poorly differentiated monophasic synovial sarcoma, with a prominent hemangiopericytoma-like vascular pattern, marked hypercellularity, and round cell change. Cytogenetic or molecular genetic evidence of the t(X;18) should be considered the "gold standard" for this distinction. Paragangliomas are relatively common tumors in the head and neck, most commonly arising from the carotid body and vagal paraganglia, but also from the paraganglia of the jugulotympanic region, larynx, and other locations. The carotid body, for example, serves to monitor arterial blood gas partial pressure of oxygen (pO2) and pH, responding to acute hypoxia with signals that result in increased ventilation, and to chronic hypoxia with hyperplasia and attenuation of its sensitivity to hypoxia.

Salivary fistulas arising from ectopic salivary gland tissue may also clinically mimic a branchial cleft fistula i have arthritis in my back what do i do order indocin 25mg mastercard. The fourth branchial anomalies treatment for arthritis in dogs nz order indocin 50 mg otc, similar to the third arthritis in back and legs generic indocin 75mg, are usually found on the left side of the neck but may also involve the right side arthritis of the knee surgery video order indocin american express. Their clinical presentation varies from no symptoms to acute suppurative thyroiditis arthritis lupus order indocin overnight, a neck abscess arthritis pain worse in the morning indocin 50mg visa, a cutaneous fistula and rarely a neck mass which may have suddenly enlarged. Complete surgical excision is the only satisfactory method of treatment for branchial cleft anomalies. The lesions are prone to recurrent infection and scarring, rendering dissection tedious and difficult. Any infection should be treated with antibiotics and the area drained before surgical excision is attempted. Aspiration of an uninfected cyst is not indicated because this may predispose the patient to infection and make dissection more hazardous and increase recurrence rates. The wall of the cyst and the tract may be extremely adherent to adjacent nerves and vessels. The surgical principles are similar, regardless of whether one is dealing with a first, second, third or fourth cleft remnant, although the approaches are different. Injection of dye intraoperatively to delineate the course of the embryonal tract, as much as possible, and careful microscopic dissection and removal of the tract, along with surrounding tissue, is very important in preventing recurrences. All first branchial cleft abnormalities appear to be associated with external ear canal abnormalities (all 41 patients with type I had an abnormality in the posterior wall of the external ear canal, and 96. Other series have reported an incidence ranging from <1% to 2% of branchial arch malformations. This lesion most commonly affects white females and usually presents at birth with a ventral midline vertical defect of the skin of the neck. Often there is a nipple-like projection at the cephalic end of the fissure and an associated sinus tract at the caudal aspect that may discharge mucoid material. Other associated defects include a cleft lower lip, tongue, and mandible; cleft sternum; ectopia cordis with intracardiac anomalies; thyroglossal duct cyst; ectopic bronchial cysts; a midline cleft from mandible to sternum; an atrophic kidney; and one patient with undescended testes. Histologically, the most consistent finding is a thin atrophic epidermis with absence of skin appendages in the dermis of the cleft tissue. The cephalic skin tag shows normal epidermis, and the central core exhibits bundles of skeletal muscle abnormally arranged and occasionally fibrofatty tissues. A sinus tract lined with both nonkeratinizing squamous epithelium and pseudostratified ciliated columnar epithelium is often present, as are seromucinous glands. If left untreated, it can result in neck webbing with contractures and mandibular deformities. If portions of the cleft remain, subcutaneous inclusion cysts will promptly recur. Few, if any, of the purported examples of this entity fulfill the four criteria that Martin and colleagues52 considered necessary to establish the diagnosis. The cervical tumor occurs along the line extending from a point just anterior to the tragus to the clavicle, along the anterior border of the sternocleidomastoid muscle. The histologic appearance is consistent with an origin from tissue known to be present in branchial vestigia. The patient has survived and has been monitored by periodic examinations for at least 5 years without development of any other lesion that could possibly have been the primary tumor. The cancer is histologically demonstrated to arise in the wall of an epithelium-lined cyst situated in the lateral aspect of the neck. Martin and colleagues52 stated that the fourth criterion was most important in confirmation of primary branchial cleft cyst carcinoma. They argued that the third criterion often cannot be satisfied because patients die of an unrelated cause before this period has elapsed. Moreover, many patients receive postoperative radiation therapy, which may control an occult primary tumor. So they proposed two additional criteria: (1) the absence of an identifiable primary cancer by a thorough evaluation, including endoscopy, computed tomography scan of the head and neck, and appropriate biopsies; and (2) histologic identification of a cystic structure partially lined with normal squamous or pseudostratified columnar epithelium with gradual transition to invasive squamous cell carcinoma. Reports indicate a significant incidence of local recurrence, frequent node metastases, and occasionally death because of disease. There is no doubt that most, if not all, of these lesions are cervical node metastases with a cystic pattern. Beginning during the fourth week as an endodermal thickening just caudal to the median tongue bud (tuberculum impar), the thyroid primordium elongates forming a prominent downgrowth called the thyroid diverticulum. The pathway of the bilobed thyroid diverticulum extends caudally as determined by the pattern of cervical arteries and continues to extend during pharyngeal development. As it migrates caudally, the tip of the thyroid diverticulum expands and bifurcates forming the thyroid gland, consisting of its two main lobes with a connecting isthmus. For a while, the thyroid remains connected to its original site of origin (foramen cecum) by a narrow thyroglossal duct. Nevertheless, in almost half the population, the distal portion of the thyroglossal duct persists as the pyramidal lobe of the thyroid. The original site of the thyroid primordium persists as the foramen cecum, a small pit at the base of the tongue. In the literature review,60 children younger than 10 years of age accounted for 31. In the largest community-based practice series, 26% of patients presented in the first decade, 12% in the second decade, 9% in the third decade, 13% in the fourth decade, 15% in the fifth decade, and 24% of patients were 50 years of age or older. Seventy-six percent of patients had an infrahyoid presentation and 24% were suprahyoid. Five patients had a fixed mass, of which three were associated with thyroid carcinomas and 23% had pain or tenderness. Often there are no symptoms except the presence of the mass, unless the cyst becomes infected. Fistulas may occur spontaneously or may be secondary to trauma, infection, drainage, or inadequate surgery. Despite the fact that a small amount of functioning thyroid tissue is associated with the tract in 30% of cases, it rarely, if ever, represents the only functioning thyroid tissue, as is often true with lingual thyroid cyst. The treatment of thyroglossal duct remnants, whether cyst, sinus, or fistula, is complete surgical excision using the Sistrunk operation. If the central hyoid bone is not removed, a recurrence rate as high as 45% may be expected. Eighty percent to 92% of such neoplasms are papillary thyroid carcinomas with the remaining neoplasms being predominantly follicular carcinomas or squamous cell carcinomas with a rare adenosquamous or anaplastic carcinoma. Because of the paucity of cases and the fact that the malignancy is not recognized until after complete pathologic examination of the remnant, it is difficult to delineate treatment and prognosis. Most researchers agree, however, that: (1) total thyroidectomy is not routinely indicated, as long as there are no palpable abnormalities in the gland, the carcinoma is not high grade or aggressive and no significant radiographic findings are found; and (2) that the Sistrunk operation offers a reasonable chance of cure. Cervical thymic cysts represent less than 1% of cystic lateral neck masses; approximately 50% have thoracic extension. Originating in third pharyngeal pouch, the paired endodermal thymic primordia begin to migrate during the sixth week. Lymphocyte precursors first invade the thymic primordia during migration before they have become vascularized. This is followed by a refractory period, which persists until the thymic primordia have completed their migration and have become vascularized. The most common presenting symptom is a slowly enlarging mass that may or may not be painful; rarely a patient may present with increasing swelling, while phonating. They can be found anywhere from the angle of the mandible to the sternum, paralleling the sternocleidomastoid muscle and the normal embryologic pathway of the thymus. The cysts are round to tubular, are unilocular or multilocular, and can measure more than 9 cm in greatest dimension. In some areas, it may be replaced by granulation or fibrous tissue, and occasionally cholesterol clefts are present. In addition, the cyst may have a fibrous cord tracking inferiorly to the superior mediastinum. Moran and colleagues78 reported three patients with carcinoma arising in a multilocular cervical thymic cyst. Bronchial cysts are derived from small buds of diverticula that separate from the foregut during formation of the tracheobronchial tree. When they occur outside the thoracic cavity, the cysts presumably arise from erratic migration of sequestered primordial cells. Most cervical bronchial cysts are present in the skin and the subcutaneous tissue of the suprasternal notch. Rarely, they are found in the lower anterior neck, chin, shoulder, intraorally, posterior pharyngeal wall, thyroid gland, and cervical spinal canal. Draining sinuses that exude mucoid material are present in approximately one-third of cases. Noninfected cysts are grossly tubular rather than of an ovoid configuration and are filled with either clear serous or thick mucoid material. Squamous stratified epithelium often makes up the lining of the sinus, but this epithelium rarely lines the cyst, unless the cyst is infected. A bronchial cyst can be distinguished from a teratoma by the complete absence of tissues, other than those that can be explained based on a malformation of the respiratory tract. A dermoid cyst can be excluded by the lack of hair and skin appendages and the absence of squamous epithelium. The presence of smooth muscle, mucoserous glands, and cartilage (should it be found), and paucity of lymphoid tissue, eliminates the possibility of a branchial cleft cyst. Complete surgical excision of the cyst along with its sinus tract is usually curative. The parathyroid/thymus primordium develops early from the third pouch endoderm; by 5 weeks of gestation, cells identifiable as parathyroid tissue can be recognized in the endoderm. The primordia of the thymus and the parathyroid glands lose their connection with the third pharyngeal pouch and they migrate caudally. The suggestion that the cysts are embryologic remnants of the third or fourth branchial cleft or result from coalescence of multiple microscopic cysts, degeneration of a parathyroid adenoma, and retention of glandular secretions has been put forth. It may be that those cysts with clear, colorless fluid are developmental in origin, whereas the cysts with bloody or straw-colored fluid may result from infarction or cystic degeneration of a parathyroid adenoma. Most patients with parathyroid cysts present with an asymptomatic low anterior neck mass. Tracheal and esophageal compression, hoarseness secondary to recurrent laryngeal nerve compression, and pain secondary to hemorrhage into the cyst have been reported. Aspiration of clear fluid with an elevated parathyroid hormone level is a definite indication of a parathyroid cyst. Parathyroid cysts vary from <1 to 10 cm in diameter, but the mean diameter is approximately 3 to 5 cm. Even in these cases, however, a diagnosis can be established by testing the cyst fluid94,96 or doing immunohistochemistry stains for parathyroid hormone on the cyst lining cells. The presence of lymphoid tissue and the stratified squamous epithelium, lining the cyst, help distinguish those unusual branchial cleft cysts with parathyroid tissue from parathyroid cysts. Aspiration may be curative, but persistence or recurrence of the cyst is common and a sign that surgical removal is needed. Dermoid cysts of the head and neck are located most often in subcutaneous tissues. The positions of these dermoid cysts at the midline and along the lines of embryonic fusion of the facial processes are consistent with origin by inclusion of ectodermal tissue. Such inclusions would take place along lines of closure at junctions of bone, soft tissue, and embryonic membranes. More than 50% are detected by the time the patient is 6 years old, and approximately one-third are present at birth. Dermoid cysts in the neck are rare but can account for close to one-fourth (22%) of midline or near-midline neck lesions. They may occasionally present in the lateral neck and also arise in the submandibular and parotid glands. Microscopic examination reveals that this developmental cyst is lined with keratinizing stratified squamous epithelium supported by a fibrous connective tissue wall. Complete surgical excision is required, after which there is little risk of recurrence. Because they mimic true cysts histopathologically, as well as clinically or radiographically, it is reasonable and convenient to include them in a general discussion of cystic lesions. Plunging or Cervical Ranula A plunging or cervical ranula appears as a soft, usually asymptomatic swelling in the submandibular or sublingual triangle, with or without evidence of a cystic lesion in the floor of the mouth. Thus the ranula often has ready access to the neck through the floor of the mouth. Usually located above the hyoid bone and in the submental or submandibular region, these lesions may extend deeply into the neck to the supraclavicular region and upper mediastinum or posteriorly to the skull base. The etiology of plunging ranula is still unknown, but an association with trauma, congenital anomalies, such as an imperforate salivary gland duct or ostial stenosis, and sublingual glandular diseases have been described. Most ranulas, whether oral or cervical, are pseudocysts with no epithelial lining. Mucus extravasated from the sublingual glands enters the soft tissues and dissects fascial planes. Histiocytes and so-called mucocytes lie in the cyst wall and may be dominant in some areas, as seen in histologic sections.

The differential diagnosis includes parathyroid adenoma arthritis video discount 25 mg indocin with amex, clear cell thyroid tumors rheumatoid arthritis diagnosis order discount indocin line, and metastases of clear cell carcinoma rheumatoid arthritis vs osteoarthritis purchase indocin australia, particularly of renal origin vinegar arthritis pain relieve purchase indocin 75 mg otc. The treatment of choice for patients with clear cell hyperplasia is subtotal parathyroidectomy mouse for arthritic fingers quality 50 mg indocin. The rate of recurrence and long-term prognosis are not well established because of the rarity of the disease best treatment for arthritis in feet discount 75 mg indocin with mastercard. Secondary hyperparathyroidism refers to an adaptive increase in parathyroid parenchymal mass resulting from a proliferation of chief cells, oncocytes, and transitional oncocytes in multiple parathyroid glands in the presence of a known stimulus of parathyroid hormone secretion, most commonly low levels of ionized calcium in the blood. Other causes include dietary deficiency of vitamin D, other abnormalities of vitamin D metabolism, and pseudohypoparathyroidism. Once the process of parathyroid hyperplasia begins, the set point for the control of parathyroid hormone secretion by ionic calcium increases, and this leads to further parathyroid hyperplasia and hypersecretion of parathyroid hormone. Although the parathyroid changes that occur in patients with secondary hyperparathyroidism are classified as hyperplasias, molecular studies indicate that some of the proliferations are monoclonal. The gross appearance of the glands is generally similar to that seen in patients with primary chief cell hyperplasia. With prolongation of the stimulus for parathyroid hormone hypersecretion, there is a tendency for a greater degree of variation in gland size. In a large series of cases reported by Roth and Marshall,575 the weight of the glands varied from 120 mg to 6 g. The earliest change in the glands is a decreased number of fat cells and their replacement by widened cords and nests of chief cells. Typically, the proliferating chief cells are present in diffuse sheets, but other areas may show cordlike, acinar, or trabecular growth patterns. In some instances, the foci of nodular proliferation may be surrounded by a fibrous capsule. Areas of hemorrhage, calcification, chronic inflammation, and cyst formation may be evident, particularly in large glands. Parathyromatosis may be responsible for the recurrence of hyperparathyroidism after parathyroidectomy in patients with chronic renal failure. Parathyromatosis may also occur as a result of developmental dispersion of parathyroid tissue in the neck or mediastinum (ontogenous parathyromatosis). The differential diagnosis includes primary chief cell hyperplasia, parathyroid adenoma, and parathyroid carcinoma. Primary and secondary hyperplasia cannot be distinguished based on gross or microscopic examination. As noted in previous sections, parathyroid adenoma typically involves a single gland, whereas secondary hyperplasia involves multiple glands. Glands from patients with long-standing secondary hyperplasia may, on occasion, be difficult to distinguish from those with parathyroid carcinoma. The glands from both conditions may exhibit extensive fibrosis, hemorrhage, and mitotic activity. Hyperplastic glands, however, lack the infiltrative properties of parathyroid carcinomas. Approximately 50 mg of parathyroid tissue is left in situ in the neck or is autotransplanted into the forearm. Recurrent hyperparathyroidism is generally treated by excision of the parathyroid remnant or the engrafted tissue. In some instances, the proliferation of autografted tissue may be extreme enough to simulate malignancy. According to this hypothesis, the cellular response function is shifted away from normal toward higher calcium concentrations. Parathyroid chief cells, which have higher set points of suppression, increase their biosynthetic and secretory activities and are stimulated to divide even at normal calcium concentrations. In the series of cases studied by Krause and Hedinger,579 only 5% of the patients had adenomas. The hyperplasia was predominantly diffuse in 44%, and the remaining patients had nodular hyperplasia. Patients with tertiary hyperparathyroidism are generally treated by subtotal parathyroidectomy. Historically metastases to the parathyroid glands were found in 6% to 12% of autopsy studies. Hypoparathyroidism resulting from tumorous involvement of these parathyroids is rare. Cysts containing thymus and parathyroid are sometimes referred to as third pharyngeal pouch cysts. Clinically, hypoparathyroidism is characterized by increased neuromuscular excitability, mental changes, calcifications in the basal ganglia, and lens and cardiac conduction abnormalities. The most common cause of hypoparathyroidism is inadvertent excision or devascularization of the glands during thyroid or parathyroid surgery. Congenital abnormalities involving maldevelopment of the third and fourth pharyngeal pouches (DiGeorge syndrome) may also be associated with neonatal hypoparathyroidism. In patients with type I polyglandular autoimmune disease, there is evidence of hypoparathyroidism, Addison disease, and mucocutaneous candidiasis. Histologically, autoimmune parathyroid disease is characterized by glandular atrophy and lymphocytic infiltration. Infiltrative processes such as amyloidosis may also involve the parathyroid glands and may give rise to hypoparathyroidism. Some of these disorders have been traced to mutations in the gene encoding parathyroid hormone. The term pseudohypoparathyroidism is used to describe patients with hypocalcemia, hyperphosphatemia, increased plasma levels of parathyroid hormone, and unresponsiveness of target tissues to the effects of parathyroid hormone. The term pseudo-pseudohypoparathyroidism refers to patients with the phenotype of Albright hereditary osteodystrophy but with normal biochemical parameters. The parathyroid glands and lateral thyroid in man: their morphogenesis, histogenesis, topographic anatomy and prenatal growth. The embryology of the human thyroid gland including ultimobranchial body and others related. 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The histological appearance of hyperfunctioning thyroids following various pre-operative treatments. American Thyroid Association Guidelines on the Management of Thyroid Nodules and Differentiated Thyroid Cancer Task Force Review and Recommendation on the Proposed Renaming of Encapsulated Follicular Variant Papillary Thyroid Carcinoma Without Invasion to Noninvasive Follicular Thyroid Neoplasm with Papillary-Like Nuclear Features. Thyroid ultrasound reporting lexicon: white paper of the acr thyroid imaging, reporting and data system (tirads) committee. The role of fine needle aspiration biopsy in the rapid diagnosis and management of thyroid neoplasm. Fine needle aspiration biopsy of thyroid nodules: advantages, limitations and effect. Molecular testing of thyroid nodules: a review of current available tests for fine-needle aspiration specimens. Analytical performance of the ThyroSeq v3 genomic classifier for cancer diagnosis in thyroid nodules. Comparison between preoperative cytology and intraoperative frozen section biopsy in the diagnosis of thyroid nodules. Influence of fine needle aspiration biopsy and frozen section examination on the management of thyroid cancer. Thyroid transcription factor-1, thyroglobulin, cytokeratin 7, and cytokeratin 20 in thyroid neoplasms. Cytogenetic investigations of 340 thyroid hyperplasias and adenomas revealing correlations between cytogenetic findings and histology. Diversity and prevalence of somatic mutations in the thyrotropin receptor and Gs alpha genes as a cause of toxic thyroid adenomas. Detection of thyroid-stimulating hormone receptor and Gs mutations: in 75 toxic thyroid nodules by denaturing gradient gel electrophoresis. Spindle cell metaplasia of the thyroid arising in association with papillary carcinoma and follicular adenoma. Hyalinizing trabecular adenoma of the thyroid: a report of three cases with immunohistochemical and ultrastructural studies. Hyalinizing trabecular tumor of the thyroid: a variant of papillary carcinoma proved by molecular genetics. Nomenclature revision for encapsulated follicular variant of papillary thyroid carcinoma: a paradigm shift to reduce overtreatment of indolent tumors. Pathological definition and clinical significance of vascular invasion in thyroid carcinomas of follicular epithelial derivation. Young investigator challenge: the morphologic analysis of noninvasive follicular thyroid neoplasm with papillary-like nuclear features on liquid-based cytology: some insights into their identification. Preoperative cytologic diagnosis of noninvasive follicular thyroid neoplasm with papillary-like nuclear features: a prospective analysis. Thyroid tumours of uncertain malignant potential: frequency and diagnostic reproducibility. Encapsulated follicular thyroid tumor with equivocal nuclear changes, so-called welldifferentiated tumor of uncertain malignant potential: a morphological, immunohistochemical, and molecular appraisal. Encapsulated welldifferentiated follicular-patterned thyroid carcinomas do not play a significant role in the fatality rates from thyroid carcinoma. Noninvasive follicular thyroid neoplasm with papillary-like nuclear features: a review for pathologists. Impact of reclassifying noninvasive follicular variant of papillary thyroid carcinoma on the risk of malignancy in the Bethesda System for Reporting Thyroid Cytopathology. Outcome of large noninvasive follicular thyroid neoplasm with papillary-like nuclear features. Should subcentimeter non-invasive encapsulated, follicular variant of papillary thyroid carcinoma be included in the noninvasive follicular thyroid neoplasm with papillary-like nuclear features category Initial results from a prospective cohort study of 5583 cases of thyroid carcinoma treated in the United States during 1996. An American College of Surgeons Commission on Cancer Patient Care Evaluation study. Papillary thyroid cancer treated at the Mayo Clinic, 1946 through 1970: initial manifestations, pathologic findings, therapy and outcome. Growing incidence of thyroid carcinoma in recent years: factors underlying overdiagnosis. Familial adenomatous polyposisassociated thyroid carcinoma: a distinct type of follicular cell neoplasm. Nuclear crease as a cytodiagnostic feature of papillary thyroid carcinoma in fine needle aspiration biopsies. A histopathological, immunohistochemical and ultrastructural study of intranuclear cytoplasmic inclusions in thyroid papillary carcinoma. The optically clear nucleus: a reliable sign of papillary carcinoma of the thyroid Independent clonal origins of distinct tumor foci in multifocal papillary thyroid carcinoma.

Atypical fibroxanthoma - histological diagnosis arthritis pain spreading discount 50 mg indocin with mastercard, immunohistochemical markers and concepts of therapy arthritis headache back head purchase genuine indocin. Immunohistochemical characterization of atypical fibroxanthoma and dermatofibrosarcoma protuberans healing arthritis in feet generic 75mg indocin free shipping. The expression of p63 in actinic keratoses bursitis vs arthritis pain order indocin 25mg amex, seborrheic keratoses arthritis in neck chiropractic treatment order indocin 25 mg fast delivery, and cutaneous squamous cell carcinomas rheumatoid arthritis what causes it buy indocin 75 mg mastercard. Postoperative/posttraumatic spindle cell nodule of the skin: the dermal analogue of nodular fasciitis. Cutaneous manifestations of opportunistic infections in patients infected with human immunodeficiency virus. Intravascular papillary endothelial hyperplasia: a clinicopathologic study of 91 cases. Meningoceles, meningomyeloceles, and encephaloceles: a neurodermatopathologic study of 132 cases. Lentiginous melanoma: a histologic pattern of melanoma to be distinguished from lentiginous nevus. Pigmented birthmarks and precursor melanocytic lesions of cutaneous melanoma identifiable in childhood. Malignant melanoma simulants arising in congenital melanocytic nevi do not show experimental evidence for a malignant phenotype. Genetic changes in neoplasms arising in congenital melanocytic nevi: differences between nodular proliferations and melanomas. Spindle and epithelioid cell nevi in children and adults: a review of 211 cases of the Spitz cell nevus. Desmoplastic nevus: a distinct histologic variant of mixed spindle cell and epithelioid cell nevus. Spindle cell and epithelioid cell nevi with atypia and metastasis (malignant Spitz nevus). Recurrent pigmented melanocytic nevus: a benign lesion, not to be mistaken for malignant melanoma. Origin of familial malignant melanoma from heritable melanocytic lesions: the B-K mole syndrome. Melanoma of the lentigo maligna subtype: diagnostic challenges and current treatment paradigms. Malignant melanoma of the head and neck: a multi-institutional retrospective analysis of cases in northern Japan. Desmoplastic melanoma of the head and neck: histopathologic and immunohistochemical study of 28 cases. Perineural spread of malignant melanoma of the head and neck: clinical and imaging features. The spectrum of minimal deviation melanoma: a clinicopathologic study of 21 cases. The major causes of conjunctival, orbital, and intraocular diseases, the histopathological findings, and major differential diagnoses are described in this chapter. A discussion on the normal histopathology of the ocular tissues will allow the reader to better understand the different pathological processes. The first section describes the most common benign and malignant pathologies of the conjunctiva. The second section is dedicated to pathological processes affecting the orbital tissues and lacrimal gland, and the third section describes the most common intraocular tumors. Understanding the most common pathologies affecting the eye, knowledge of the nomenclature used to best communicate the findings to ophthalmologists, and recognizing challenging differential diagnoses are the key to the accurate diagnosis and treatment of ocular diseases. Pigmented Lesions of the Conjunctiva Racial Melanosis Conjunctival Nevi Primary Acquired Melanosis Conjunctival Melanoma V. The conjunctiva can be subdivided into palpebral (or tarsal), forniceal, and bulbar (or covering the surface of the globe). Nasally, a fold of loose conjunctiva forms the plica semilunaris, where smooth muscle cells and cartilage may occasionally be found. The limbal epithelium might be slightly pigmented with branching dendritic melanocytes. The stroma of the bulbar conjunctiva is composed of collagen fibers, loosely intermixed fibroblasts, blood vessels, lymphatic vessels, and chronic inflammatory cells, including mast cells, macrophages, lymphocytes, plasma cells, polymorphonuclear leukocytes, and eosinophils. The stroma of the palpebral conjunctiva is more uniform and closely attached to the tarsus, where the meibomian sebaceous glands of the eyelid are located. The goal is to aid in the diagnosis of usual but challenging conjunctival lesions encountered in surgical pathology practice. Review of the normal histologic features of the conjunctival epithelium and caruncle is included to contrast the histologic findings of benign and malignant stromal, epithelial, and melanocytic lesions of the conjunctiva (see section outline later). The slight majority (53%) of conjunctival lesions are non-melanocytic, which include tumor-like proliferations; epithelial lesions; developmental conditions; hematolymphoid proliferations; vascular lesions; and an array of soft tissue tumors (Table 15. Subepithelial Conjunctival Lesions Pinguecula and Pterygium Chalazion 1098 15 Pathology of the Conjunctiva, Orbit, Lacrimal Gland, and Intraocular Tumors 1099 of Wolfring and Krause are present in the subepithelial tissues of the palpebral conjunctiva at the upper edge of the tarsus and close to the fornices. In children, papillomas are most commonly multiple and pedunculated, involving the fornix, caruncle, or eyelid margin, rarely involving the cornea. In adults, they are usually single and sessile, usually occurring at the limbus or bulbar conjunctiva. Typical findings show a benign papillomatous lesion composed of fibrovascular cores lined by squamous epithelium. Some papillomas have numerous melanocytes that impart a darker color to the lesion clinically. Lymphoid Vascular Congenital/choristomatous Subepithelial soft tissue Fibrous Neural Myxomatous Lipomatous Xanthomatous Lacrimal gland origin Leukemic Metastatic Secondary tumors Melanocytic Tumors Nevi Melanoma Primary acquired melanosis Data from Shields C. B, Full-thickness eyelid lined by the conjunctiva (bottom of the eyelid), the skin (top of the eyelid), and the margin, where both epithelia meet. Notice the prominent meibomian glands in the tarsus of the conjunctiva and the levator muscle located under the dermis of the eyelid and right above the tarsus of the conjunctiva. The main consideration, especially in adults, is a well-differentiated in situ or invasive papillary squamous cell carcinoma. Surgical excision of the lesion and cryotherapy to the adjacent conjunctiva is the treatment of choice. Clinically, lesions can appear focal and well circumscribed or diffuse and poorly demarcated. The lesion is located in the limbus, involving both the conjunctival and corneal epithelia appearing as a fleshy, well-vascularized arborizing mass. Histopathology features comprise intraepithelial dysplasia originating in the basal layers extending toward the surface epithelium overlying areas of solar elastosis. These lesions can show different growth patterns, mainly keratinizing (leukoplakic), nonkeratinizing, or spindle cell, with actinic or papillomatous features. However, it can be challenging to accurately discern the degree of dysplasia in keratinizing lesions. A variant of squamous cell carcinoma, conjunctival keratoacanthoma, shows an acanthotic epithelial proliferation with cytologic atypia, an intact basement membrane, increased mitotic figures, and a central crater filled with keratin. Ki-67 stain is helpful in demonstrating the increased cells in cycle beyond the basal epithelial cell layer. Intraepithelial involvement by sebaceous carcinoma of the conjunctiva can be especially challenging because if the tumor involves all the layers of the conjunctiva it can appear similar to squamous cell carcinoma in situ. In conjunctival sebaceous carcinoma the basal layer of the conjunctiva shows a normal population of basal cells that appear smaller than the larger, very atypical, malignant sebaceous cells with foamy cytoplasm and atypical mitoses found in the mid and upper layers of the epithelium. Oil Red O stain and/or adipophilin immunohistochemical stain to demonstrate intraepithelial fat globules is very helpful in the differential diagnosis of these cases. Mucoepidermoid (adenosquamous) carcinoma is comprised of atypical squamous cells with eosinophilic cytoplasm, arranged in sheets and cords, with interspersed mucous cells. Less commonly, primary acquired melanosis with atypia without pigmentation may be confused with a dysplastic or squamous lesion. Excision of the affected epithelium with cryotherapy of the margins is the standard treatment. Deep invasion of the cornea or sclera and intraocular spread are uncommon complications. It can be locally invasive into the orbit, globe, and cornea, causing glaucoma and pain requiring enucleation. Squamous cell carcinoma is typically a well-differentiated neoplasm composed of abnormal squamous epithelial cells with increased mitoses and keratin production. Squamous cells might appear less differentiated with large, pleomorphic cells, giant cells, dyskeratosis, and abnormal mitoses. Squamous cell carcinoma should be differentiated from mucoepidermoid (adenosquamous) carcinoma arising in the conjunctiva or originating from the paranasal sinuses. Mucicarmine, Alcian blue, colloidal iron, and mucin-1 stains are useful in highlighting the mucus-secreting cells present in mucoepidermoid carcinoma. The spindle cell variant of squamous cell carcinoma may be indistinguishable from malignant fibroblastic proliferations on light microscopy alone. Immunohistochemical studies may be helpful in differentiating spindle cell carcinoma from sarcomas. Complete surgical excision with alcohol corneal epitheliectomy, partial lamellar sclerokeratoconjunctivectomy, and double freeze-thaw cryotherapy to the surrounding conjunctival margins is advised. However, tumors larger than 5 mm in diameter, tumors extending more than 2 mm onto the cornea, and tumors with local invasion (corneal, scleral, intraocular, or orbital invasion) are associated with a higher risk of recurrence. Histopathologically, mucoepidermoid carcinoma shows sheets and cords of large squamous epithelial cells with eosinophilic cytoplasm, and mucin-containing cells. Other diagnostic possibilities include basal cell carcinoma involving the conjunctival epithelium and adnexal malignancies with clear cell change. It has been reported that fusion-positive cases show significantly better survival than fusion-negative cases. In the majority of cases a secondary intraepithelial pagetoid spread from an occult eyelid sebaceous carcinoma is present. The hyperchromatic cells have enlarged nuclei and are significantly larger than the surrounding normal basal keratinocytes. The malignant cells may spread to replace the entire epithelial thickness, and atypical mitotic figures are often present. If the tumor cells replace the entire conjunctival epithelium, then differentiating it from a nonkeratinizing squamous cell carcinoma in situ can be challenging. The main differential diagnosis of intraepithelial sebaceous carcinoma includes in situ squamous cell carcinoma and basal cell carcinoma. Pterygium is more common with increasing age and among nonwhite persons, particularly those of African origin. Radiation causes mutations in the p53 tumor suppressor gene, thus facilitating the abnormal proliferation of limbal epithelial cells. Pinguecula is a small, yellowish gray nodule, often bilateral, situated at the nasal or temporal limbus of the conjunctiva. On examination a pterygium appears as a pinkish triangular wedge of vascular conjunctiva on the surface of the cornea, often growing from the limbus on the nasal side measuring approximately 1 to 3 mm. It is painless, but is often associated with dryness and foreign body sensation in the eye and may obstruct vision if left untreated. The lesion involves the conjunctival limbal epithelium with involvement of the cornea. The epithelium may be hyperplastic or atrophic, and may harbor dysplasia, microcalcifications, and benign racial melanosis. If pterygium recurs, elastotic degeneration is less prominent with more abundant tissue fibrosis. Pterygium should be clinically and histologically distinguished from pseudopterygium, a conjunctival fold often secondary to corneal disease.

Their chromatin is usually dense and pyknotic arthritis in dogs how to tell purchase genuine indocin on line, but enlarged nucleoli may be found arthritis in neck and back symptoms 75 mg indocin visa. Myxoid/round cell liposarcoma is characterized by a stromal background that is loose how to relief arthritis pain purchase indocin with mastercard, myxoid arthritis pain or bone cancer order indocin overnight, and perforated by a fine chicken-wire meshwork of arborizing vessels arthritis in your neck and back discount 50 mg indocin mastercard. The lipoblasts appear as univacuolated signet-ring arthritis relief for legs purchase indocin 75mg without prescription, round, and multivacuolated cells. The lipoblasts may be scarce, congregated at the periphery of the expanding tumor lobules. Pleomorphic liposarcomas are characterized by densely packed malignant spindle cells and bizarre, highly pleomorphic forms; the lipoblastic component (lipoblasts and signet-ring cells) may be minimal. Liposarcoma with high-grade transformation is a well-differentiated liposarcoma that, after single or multiple recurrences, gives rise to a (usually) high-grade, nonlipogenic sarcoma. Rarely described in the larynx,877 liposarcoma with high-grade transformation is commonly seen in the retroperitoneum. B, Another laryngeal liposarcoma, presenting as a recurring endoluminal polypoid mass. This tumor demonstrates a collagenized and fibroblastic background within a lipomatous tumor, which should hint at the diagnosis of lowgrade liposarcoma. The immunohistochemical profile of liposarcomas is nonspecific; strong staining with vimentin and S100 can be expected. Formalin-fixed, paraffin-embedded tissue is suboptimal for identification of fat droplets by fat stains. However, adipophilin immunohistochemical staining is helpful to identify intracellular lipid material in formalin-fixed tissue. This has been shown to be useful when one needs to demonstrate intracellular lipid material in a liposarcoma. However, this is no longer necessary and can be replaced with immunohistochemistry. Low-grade liposarcomas may be diagnosed as fibroadipose polyps, lipomas, or fibromas when lipoblasts are a focal finding or absent from the biopsy sample. Equally, higher-grade tumors may not be recognized as adipose in nature on a limited biopsy sample. Adipophilin staining may be helpful in this context to demonstrate areas with intracellular lipid material. Pleomorphic liposarcomas may be confused with other pleomorphic sarcomas, such as undifferentiated pleomorphic sarcoma, which may have lipid droplets as a degenerative change; extensive sectioning of the resection specimen will aid in confirming the adipose nature of a tumor. Wenig and colleagues argue that although tumors of similar low-grade histology may be called atypical lipomatous tumors of the subcutaneous or intramuscular tissues, this might encourage inadequate removal in the upper aerodigestive tract, leading to multiple recurrences. The 5-year survival rate for laryngeal liposarcomas varies according the histological subtype. For well-differentiated liposarcoma, the prognosis is excellent, with 100% survival rate at 5 years. The less-differentiated forms have a survival rate of 89%, which is significantly better than for some other head and neck sites, such as the soft tissue of the neck (60%), pharynx (59%), and oral cavity (50%). It is highly likely that many monophasic and poorly differentiated synovial sarcomas of the head and neck have been reported as "fibrosarcoma" or "hemangiopericytoma" in older series (see Chapter 9). Nine percent (31 cases) of 345 Armed Forces Institute of Pathology cases of synovial sarcoma occurred in head and neck sites: soft tissue of the neck (12 cases), pharynx (seven cases), and larynx (seven cases). Smaller interposed epithelial nests may be apparent only after immunohistochemical studies (see later discussion). The epithelial elements may have a characteristic cleft-like glandular pattern, mimicking papillary synovial proliferation. The spindled elements form whorls and fascicles of closely packed, somewhat uniform-appearing tumor cells. Solitary fibrous tumor-like areas of increased vascularity with perivascular hyalinization and staghorn-type vessels may be seen. The epithelial elements, as well as the spindle cells, may express low- and highmolecular weight cytokeratin and epithelial membrane antigen. Extensive histologic sampling of the tumor and immunohistochemistry will usually allow proper classification. Epithelioid sarcoma usually affects the superficial tissues of the distal extremities; supraclavicular involvement is usually limited to the scalp. Locoregional recurrence may develop within the first few years, but late recurrences (>5 years after diagnosis) are also possible. Polypoid exophytic tumors are anecdotally noted to be associated with long disease-free survivals, which probably relates to degree of infiltration and resectability. These tumors appear as polypoid or exophytic soft-tissue tumors and cause nonspecific vocal and airway symptoms. Whorls and fascicles of malignant fibroblastic spindle cells forming a "rush-mat" or radiating star-like (storiform) pattern characterize these tumors. This is obviously an important distinction as the latter entity is treated more conservatively. An exception to this would be pediatric laryngeal rhabdomyosarcoma, which might require neoadjuvant chemotherapy. Laryngeal factors, such as tumor stage, degree of infiltration into the larynx, initial resectability, and tumor grade are probably more important prognosticators. Hematogenous metastatic disease, usually pulmonary, has been reported; survival is short after the development of metastases. Conversely, many of the patients collected by Ferlito and colleagues896 were disease free after surgery (with or without adjuvant radiotherapy); 10 patients were disease free 8 months to 10 years after treatment (mean, 55 months), compared with five patients with metastasis, who died 13 to 44 months after treatment (mean, 22 months). Teratoma (Greek, monstrous tumor) is a tumor of variable maturity and organization; its elements represent differentiation from all three embryonic germ layers. The term teratoma is also loosely applied to dermoid cysts or tumors, even though they show only bivergent differentiation into ectoderm and mesodermal tissues. Teratomas involve midline structures (skull base, sinonasal cavity, neck, mediastinum, and sacrococcygeus) and gonads. They vary greatly in maturity and oncogenic potential: teratomas may be mature and benign. A generalization that usually holds true for the head and neck is that pediatric teratomas tend to be histologically immature but oncologically benign, with few instances of metastases, and fewer instances still of tumor-related deaths. Primary laryngeal teratomas are extremely rare, with only four primary laryngeal cases having been identified, one in a child and three in adults. Teratomas have a mixture of immature and some maturing elements of ecto-, meso-, and endodermal origin. Concerning the four laryngeal cases, three tumors were composed of mature tissues; one was predominantly composed of immature, yet oncologically benign, fetal tissue, including cartilage and epithelial, neuronal, and retinal tissue. The ectodermal structures include fetaltype squamous cysts, with clear cell change and sebaceous elements. The mesodermal elements may be sparse and include immature yet bland loose myxoid stroma, as well as maturing cartilage (although the latter may often be absent) and occasional muscle differentiation. The endodermal elements include cysts, lined with ciliated cells or gastrointestinal-type epithelium with goblet cells. Metastatic elements in adult teratomas can arise from either immature elements or histologically malignant elements. The latter appear in areas with necrosis, a significant mitotic rate, and sufficient nuclear atypia. Evidence of germ cell tumors (yolk sac tumors, germinomas, embryonal carcinoma, choriocarcinoma) is usually not seen in head and neck teratomas. A broad differential diagnosis exists with these tumors when initially evaluated on small biopsy samples. Attention to the nondescript mesenchymal background and heterogeneity of elements encountered provide clues for this rare diagnosis. However, the ectodermal elements of mature teratomas (squamous cysts) have a decidedly fetal, "cleared out" appearance. Sebaceous elements, if present, are nonindigenous and therefore inconsistent with the diagnosis of hamartoma. Unlike a laryn- 5 Nonsquamous Pathologic Diseases of the Hypopharynx, Larynx, and Trachea 413 geal teratoma, a choristoma would not have areas with a fetallike appearance. All three patients were disease free after primary excision (two patients were known to have 15 months and 5-years follow-up time). A blastoma (embryoma) is a malignant neoplasm of mixed mesenchymal, epithelial, and nondifferentiated blastemous elements that mimics embryonic development of the particular organ. They can occur in both children and adults; pulmonary blastomas occur with a male predominance, usually in adults; hepatoblastomas and pancreaticoblastomas occur mostly in children. Eble and colleagues901 reported an exophytic tumor of the piriform sinus that developed in a 65-year-old man, an employee of a glass factory and smoker, which microscopically was analogous to a pulmonary blastoma. The three elements, mesenchymal, epithelial, and blastemous, exist in varying proportions; transformation can be seen from one element to another. The mesenchymal element forms primitive-appearing spindle cell, chondroid, and myxoid elements. The epithelial elements form strands of primitive-appearing cells, glandular elements, and squamous elements. The blastemous elements are small, nondifferentiated primitive-appearing cells that may be poorly cohesive and lack definite epithelial or mesenchymal characteristics. Blastomas of other sites (lungs, liver, and pancreas) are malignant tumors with the potential to metastasize. The patient reported by Eble and colleagues901 was disease free 13 months after total laryngectomy. Secondary Tumors (Intratracheal Thyroid Ectopia, Metastatic Tumors) Not uncommonly, cancers invade the larynx by contiguous extension. This is a familiar scenario for large carcinomas of the tongue base; they may insinuate into the soft tissues of the preepiglottic space, destroy the epiglottis, and invade supraglottic soft tissue. Piriform sinus carcinomas invariably involve soft tissues lateral to the thyroid lamina and may extend over and around it to involve the supraglottis. Unlike the fenestrated elastic cartilage of the epiglottis, nonossified hyaline cartilage is relatively resistant to tumor invasion. Secondary tumors-intratracheal thyroid ectopia-metastatic tumors are rare and equally rare, are reported cases. Thyroid carcinomas, either papillary or anaplastic, may directly invade the trachea via intratracheal ring spaces. Because the trachea is posterior to the path of the descending thyroid, the mechanism of intratracheal ectopia is not immediately obvious. Two nonexclusive theories prevail: (1) the malformation theory holds that thyroidal descent is completed before tracheal cartilage formation, allowing thyroid tissue to become entrapped and displaced by the developing trachea. By contrast, the lung bud of the putative trachea is seen at 3 weeks, differentiation of the primitive cartilaginous tissue is seen after 8 weeks, cartilaginous rings are delineated by 9 weeks, and the development of the major tracheal topography is completed at 5 months. The purported left-sided propensity of the intratracheal thyroid has been thought to relate to migratory differences between the left and right lateral ultimobranchial contribution to the thyroid anlage. The left lateral ultimobranchial tissue lies more rostral than the right-sided tissue and so may be less enveloped by the left thyroid lobe than on the right side. Thus unmerged left-sided thyroid tissue may be more available for displacement by the developing trachea; and (2) the invasion theory holds that thyroid tissue continues to migrate, albeit along an aberrant pathway, and becomes situated in the trachea as a result of direct, yet oncologically benign, invasion, as is seen quite frequently with mediastinal thyroid tissue. The invasion theory is supported by the microscopic confirmation of pseudoinfiltration of thyroid tissue around but not through normal firm structures and along soft-tissue planes. Intratracheal thyroid ectopia may be histologically benign and hyperplastic, may give rise to malignancy (usually papillary carcinoma), or may be an incidental finding in the setting of thyroid carcinoma. The latter situation may lead to some confusion, with regard to tumor staging, because a papillary thyroid carcinoma can be erroneously upstaged as a T4 tumor because of incidental intratracheal rests. In a series of 900 laryngeal malignancies over a three-decade period, one case was seen. Metastatic cutaneous melanomas and renal cell carcinomas were the two leading metastatic diagnoses913,914 further observed in most recent reports. Because of the rarity of primary laryngeal melanomas, metastatic laryngeal melanomas are a more likely occurrence. No cases of secondary laryngeal melanoma have been noted in which an occult skin primary was discovered after the laryngeal tumor. Thus, despite its rarity, a laryngeal melanoma diagnosed in the absence of a previous skin malignancy is more likely to be a laryngeal primary. However, the clinician should be urged to investigate all previous skin biopsy results. In the case of renal cell carcinoma, the laryngeal metastasis will usually occur with a known primary site and after a long interval of time. However, laryngeal metastasis may occasionally herald the onset of disseminated disease. The presence of in situ disease will be consistent with a laryngeal primary, whereas its absence does not rule out that possibility. Comparison of the genetic profiles of the laryngeal and pulmonary carcinoma can be helpful. Concordant loss of heterozygosity patterns for both tumors is consistent with metastatic disease. However, a gain in the number of losses of heterozygosity loci in the pulmonary neoplasm cannot exclude the possibility of metastasis because this is consistent with overall genetic instability and disease progression. A clinic-epidemiological study of head and neck tuberculosis - a single-center experience.

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