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*Multiple lesions, early in life suggest a genetic syndrome. | *Multiple lesions, early in life suggest a genetic syndrome. | ||
**Brooke-Spiegler syndrome - spiradenomas, cylindromas and trichoepitheliomas | **Brooke-Spiegler syndrome - spiradenomas, cylindromas and trichoepitheliomas | ||
*Generally considered to be an 'eccrine' tumor but some hypothesize a pilar origin <ref>{{Cite journal | last1 = Kazakov | first1 = DV. | last2 = Soukup | first2 = R. | last3 = Mukensnabl | first3 = P. | last4 = Boudova | first4 = L. | last5 = Michal | first5 = M. | title = Brooke-Spiegler syndrome: report of a case with combined lesions containing cylindromatous, spiradenomatous, trichoblastomatous, and sebaceous differentiation. | journal = Am J Dermatopathol | volume = 27 | issue = 1 | pages = 27-33 | month = Feb | year = 2005 | doi = | PMID = 15677973 }}</ref> | |||
==Microscopic== | ==Microscopic== | ||
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*Biphasic cell population: | *Biphasic cell population: | ||
**outer dark cells with small hyperchromatic nuclei and minimal cytoplasm. | **outer dark cells with small hyperchromatic nuclei and minimal cytoplasm. | ||
**inner larger cells with vesicular nuclei and more cytoplasm. | **inner larger cells with [[vesicular nuclei]] and more cytoplasm. | ||
**In some areas the two cell types mix together with dispersed hyaline droplets. | **In some areas the two cell types mix together with dispersed hyaline droplets. | ||
*Ductal differentiation. | *Ductal differentiation. | ||
*These cells form lobules that are surrounded by hyaline or reticulin sheath | *These cells form lobules that are surrounded by a hyaline or reticulin sheath. | ||
*+/-Lymphocytes. | *+/-Lymphocytes. | ||
*Vascular component - large and small blood vessels. | *Vascular component - large and small blood vessels. | ||
DDx: | DDx: | ||
*[[Dermal cylindroma]] | *[[Dermal cylindroma]]. | ||
**These two tumors are very closely related and overlap. | **These two tumors are very closely related and overlap. | ||
**Many tumors have areas of both spiradenoma and cylindroma. | **Many tumors have areas of both spiradenoma and cylindroma. | ||
**The lobules of cylindroma are more consistently discrete and surrounded by prominent hyaline material. | **The lobules of cylindroma are small | ||
***more consistently discrete, | |||
***show more a more consistent arrangement of the light and dark cells and are | |||
***often surrounded by prominent hyaline material. | |||
**Individual lobules of cylindroma fit together like pieces of a puzzle. | **Individual lobules of cylindroma fit together like pieces of a puzzle. | ||
**Lobules of spiradenoma are larger | |||
***may run together and fuse, | |||
***may not have a prominent hyaline surround and | |||
***may show more disorganization and mixing of the two cell types | |||
*[[Trichoepithelioma]]. | *[[Trichoepithelioma]]. | ||
**Trichoepithelioma will show | |||
***attempts at hair bulbs, | |||
***areas with more eosinophilic cytoplasm and | |||
***characteristic peritumoral stroma | |||
*[[Glomus tumour]], hemangioma or hemangiopericytoma (vascular spiradenomas). | *[[Glomus tumour]], hemangioma or hemangiopericytoma (vascular spiradenomas). | ||
**The location often speaks against glomus tumour. | **The location often speaks against glomus tumour. | ||
*Basal cell carcinoma | |||
**Spiradenoma is deeper, without connection to the epidermis | |||
**Spiradenoma lacks clefting artefact. | |||
**Spiradenoma lacks mitoses and prominent apoptosis. | |||
**BCC lacks ducts | |||
**BCC lobules lack a prominent surrounding hyaline membrane | |||
*Lymphoid aggregate (spiradenoma will be cytokeratin positive) | *Lymphoid aggregate (spiradenoma will be cytokeratin positive) | ||
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*S100 +ve. | *S100 +ve. | ||
*Keratins 7, 8, and 18 +ve. | *Keratins 7, 8, and 18 +ve. | ||
*Ductules are EMA and CEA positive. | *Ductules are [[EMA]] and [[CEA]] positive. | ||
Notes: | Notes: | ||
*IHC profile essentially identical to dermal cylindroma.<ref name=pmid9129700/> | *IHC profile essentially identical to dermal cylindroma.<ref name=pmid9129700/> |
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