adnexal tumors of the skin

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Nodular or multinodular tumor of the dermis, prototypically without any connection to the epidermis as seen in poromas; some observers

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adnexal tumors of the skin

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1. Adnexal Tumors of the Skin

2. Nodular or multinodular tumor of the dermis, prototypically without any connection to the epidermis as seen in poromas; some observers “allow” this finding Clinically presents as a slowly-growing reddish-tan nodule in virtually any skin field, although trunk and extremities are favored; all ages and both genders Micronodular groups of polyhedral cells with variably eosinophilic, clear, or squamoid features, often with microcyst formation and tubular differentiation Mitoses may be numerous but are not important alone “Budding” of tumor cell nests into stroma correlates with low-grade malignant behavior (local recurrence) Eccrine Acrospiroma (Hidradenoma)

5. Almost exclusively seen in genital skin of women or perianal skin of either gender; nodular, reddish, often-fluctuant lesion up to several cm. in greatest dimension Central cystic space into which elaborately arborizing papillary profiles project; peripheral aspects of lesion show tubular differentiation or are more solid Cells of papillae and in tubules show apocrine “snouts” Mild cytologic atypia and mitotic activity may be seen, with no apparent impact on biology HP is often mistaken for a carcinoma by general pathologists Hidradenoma Papilliferum

7. MALIGNANT ECCRINE TUMORS Ductal eccrine carcinoma Mucinous eccrine carcinoma Eccrine porocarcinoma Clear cell hidradenocarcinoma Adenoid cystic carcinoma Papillary digital adenocarcinoma Sweat gland carcinoma ex eccrine spiradenoma Mucoepidermoid carcinoma

8. REASONS FOR DIFFICULTY IN DIAGNOSIS OF ECCRINE CARCINOMA 1. Rarity-- <0.005% of all skin tumors 2. Diversity of morphologic patterns 3. Mimicry of metastatic carcinomas that may involve the skin

9. HISTOLOGIC DISTINCTIONSBETWEEN BENIGN AND MALIGNANTECCRINE TUMORS: CLUES 1. Irregularly-permeating growth pattern on low-power microscopic examination 2. Nuclear atypia (hyperchromasia; nucleoli) 3. Perineural/vascular invasion by tumor cells 4. Histologic foci of spontaneous tumor necrosis 5. Mitotic activity is NOT helpful diagnostically


11. MUCINOUS ECCRINE CARCINOMA -- Resembles "colloid" carcinoma of the breast and alimentary tract -- Favors facial and proximal truncal skin -- Adults; males > females by 2:1 -- Has "gelatinous" cut surface -- Cords and clusters of polygonal tumor cells embedded in abundant mucinous stroma (+ with PAS-D/Mucicarmine)

13. MUCINOUS ECCRINE CARCINOMA:BEHAVIOR & PROGNOSIS -- Indolent and relatively innocuous form of sweat gland carcinoma -- Recurrence seen in approximately 30% of all cases after excision -- Distant metastases very rare

14. MUCINOUS ECCRINE CARCINOMA:DIFFERENTIAL DIAGNOSIS -- Metastatic "colloid" carcinoma from breast or GI tract (largely an academic inclusion) -- Mixed tumor of the skin with abundant myxomucinous stromal change

15. PAPILLARY DIGITAL ECCRINEADENOCARCINOMA -- First described by Kao et al. in series from the AFIP in 1987 -- Arises on distal extremities in adult men; particularly digits -- Enlarging painless mass of several months or years duration

16. PAPILLARY DIGITAL ECCRINECARCINOMA VS. PAPILLARY ECCRINE ADENOMA -- PDEC seen almost exclusively on digits, whereas PEA is not -- PDEC affects middle-aged or elderly pts., where PEA has broad age distribution -- PDEC features macropapillae of tumor cells projecting into microcysts, whereas PEA resembles intraductal hyperplasia of the mammary glands

19. PAPILLARY DIGITAL ECCRINECARCINOMA: DIFFERENTIAL DX -- Metastatic papillary carcinomas of mammary, pulmonary, thyroid, or gonadal origin (academic inclusion) -- Papillary eccrine adenoma (tubulo- papillary hidradenoma)

20. PAPILLARY DIGITAL ECCRINECARCINOMA: BEHAVIOR -- Two histologic grades of PDEC -- Low grade tumor lacks pleomorphism and necrosis, with modest mitotic activity -- High grade PDEC shows marked nuclear atypia, cellular pleomorphism, necrosis, and abundant mitoses -- Low grade PDEC recurs but usually does not show distant metastasis -- High grade PDEC recurs and metastasizes

21. CUTANEOUS ADENOID CYSTICCARCINOMA -- Cutaneous ACC is virtually identical to similar tumors in salivary glands, breast, and tracheobronchial tree -- Perineural invasion commonly seen -- Favors skin of face and scalp in elderly individuals (no gender predilection) -- Flesh-colored nodules or plaques

23. CUTANEOUS ADENOID CYSTICCARCINOMA: BEHAVIOR -- Relatively innocuous, low grade eccrine carcinoma -- Recurs in approximately 20% of cases, but very rare examples of metastasis

24. CUTANEOUS ADENOID CYSTICCARCINOMA: DIFFERENTIAL DX -- Metastases from more deeply-situated adenoid cystic carcinomas are possible on theoretical grounds, but do not seem to involve the skin by embolic spread -- ADENOID BASAL CELL CARCINOMA is the most frequent misdiagnosis in ACC cases, and the reverse also is true

25. CARCINOMA EX ECCRINESPIRADENOMA -- Rare tumor, with less than 100 cases reported in the literature to date -- Exception to the rule that sweat gland carcinomas arise de novo -- Manifests as rapid growth in long- standing cutaneous nodule; patients also may complain of pain in lesion

26. CARCINOMA EX ECCRINESPIRADENOMA: HISTOLOGY -- By definition, remnants of the "parent" spiradenoma must be seen histologically -- Malignant component typically separated from parent adenoma by sharp interface -- Carcinomatous elements may resemble ductal breast cancer, "carcinosarcoma," spindle-cell tumors, or anaplastic but "uncommitted' looking polygonal cell tumors

28. CARCINOMA EX ECCRINESPIRADENOMA: BEHAVIOR -- CAEES has a "bark" that is often worse than its "bite"; less than 10% of these tumors have metastasized distantly, despite high-grade histologic appearances -- Recurrence seen in approximately 20 to 30% of cases post-excision

29. MICROCYSTIC ADNEXALCARCINOMA -- Described by Goldstein et al. in 1982 as a tumor of mixed lineage (eccrine-pilar) that arose in the facial skin of middle aged or elderly patients (Females > Males) -- Marked tendency to recur after excision; may pursue inexorable and mutilating course and necessitate heroic surgical removal if neglected -- Should NOT be removed by Mohs' surgery

30. MICROCYSTIC ADNEXALCARCINOMA: HISTOLOGY -- Bland cytology but extremely infiltrative growth, with perineural/perivascular permeation by tumor cells -- Tumor cell nests tend to assume an internally-concentric, squamoid profile -- Keratinous pilar-type microcysts are seen in type I MAC -- Narrow tubular profiles of tumor cells in MAC type II ("syringoid" carcinoma)

32. MICROCYSTIC ADNEXALCARCINOMA: DIFFERENTIAL DX -- Morpheaform basal cell carcinoma -- Syringoma -- Desmoplastic trichoepithelioma -- Adenosquamous carcinoma of skin

33. ECCRINE POROCARCINOMA -- Considered to be part of the spectrum of "malignant acrospiromas" by some authors -- Favors extremities, particularly legs/feet -- Seen in all age groups with no gender predilection; most often a tumor of adults -- Distant metastases and mortality from tumor in approximately 30-35% of cases

34. ECCRINE POROCARCINOMA:HISTOLOGY -- Cords and lobules of polygonal cell tumor in the dermis, some of which have squamoid features and central necrosis -- Overt nuclear atypia with nucleoli -- Permeative peripheral growth -- Intraepidermal tumor cells in "lakes," often centered on acrosyringial pores

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