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Cutaneous Adnexal Tumors – What's New?

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Cutaneous Adnexal Tumors – What's New? 3/27/2017 Disclosure of Relevant Financial Relationships Cutaneous adnexal USCAP requires that all planners (Education Committee) in a position to influence or control the content of CME disclose any relevant financial tumors – What’s new? relationship WITH COMMERCIAL INTERESTS which they or their spouse/partner have, or have had, within the past 12 months, which relates Meera Mahalingam, MD, PhD, FRCPath to the content of this educational activity and creates a conflict of interest. Dr. Mahalingam has nothing to disclose. Age-adjusted IR 5.1/million person years 37.3 0.37 IR increased 100 fold with age 1 3/27/2017 Part I New Entities New“ish” Entities . Endocrine mucin-producing sweat gland carcinoma . Primary cutaneous cribriform carcinoma . Squamoid eccrine ductal carcinoma “Monocle” tumor Am J Surg Path, 2005 2 3/27/2017 3 3/27/2017 Defining histopathology . Well-circumscribed single or multiple tumor lobules in the dermis with solid, cystic and papillary growth patterns . Bland cytomorphology . Extracellular mucin 4 3/27/2017 EMSGC Clarification of nomenclature . Rare, low-grade sweat gland carcinoma, which is morphologically analogous to solid papillary carcinoma of the breast. Considered to be in the spectrum of cutaneous mucinous neoplasms and a precursor of primary cutaneous mucinous carcinoma Arch Path Lab Med, 2012 IHC Calponin ER Synaptophysin CK5/6 EMSGC Clarification of nomenclature . Rare, low-grade sweat gland carcinoma, which is morphologically analogous to solid papillary carcinoma of the breast. Considered to be in the spectrum of cutaneous mucinous neoplasms and a precursor of primary cutaneous mucinous carcinoma 5 3/27/2017 Mimics Mimic 1 - BCC 6 3/27/2017 Mimic 2 - Spiradenoma Mimic 3 – Chondroid syringoma 7 3/27/2017 EMPSGC Take home points New “ish” Entities . 50% of endocrine mucin-producing sweat gland . Endocrine mucin-producing sweat gland carcinoma carcinomas in one series was associated with mucinous carcinoma suggesting that it is a precursor . Primary cutaneous cribriform carcinoma to some mucinous carcinomas . Squamoid eccrine ductal carcinoma . A limited biopsy showing only the cystic component may very easily be mistaken for something else…. 8 3/27/2017 More recent series……. Biologic behavior PCCC - IHC S100 C-KIT 9 3/27/2017 Mimics Mimic 1 – Tubular adenoma PCCC 10 3/27/2017 PCCC - Calponin Tubular adenoma Calponin Mimic 2 – Adenoid cystic carcinoma 11 3/27/2017 New “ish” Entities . Endocrine mucin-producing sweat gland carcinoma . Primary cutaneous cribriform carcinoma . Squamoid eccrine ductal carcinoma “ ” Other names 2003 . Adenosquamous carcinoma . Ductal eccrine carcinoma with squamous differentiation . ?squamoid variant of microcystic adnexal carcinoma 12 3/27/2017 2004 2005 2005 - Excisional 13 3/27/2017 Am J Surg Path, 2016 Last week….. 14 3/27/2017 Mimics Sclerosing neoplasms MAC (18) SC (5) SEDC (7) Age in years 56 47 81 Localization Periocular area Cheek Cheek Histopathology Cysts Strands Keratinization Strands PNI LVI PNI PNI IHC PHLDA1+ PHLDA1+ PHLDA1+ only K77- K77+ only in in deeper ducts portion K77+ Adnexal neoplasms and IHC Part II What to use and when? 15 3/27/2017 BCC vs trichoepithelioma BCC? Trichoepithelioma? BCC - Androgen receptor BCC - CK20 MAC? Other sclerosing neoplasm? BCC vs trichoepithelioma Recommended panel 16 3/27/2017 MAC vs other sclerosing neoplasms Porocarcinoma? SCC? Recommended panel Porocarcinoma SCC 49% 25% 64% 23% 46% 12% 64% 28% 17 3/27/2017 Porocarcinoma SCC Porocarcinoma vs SCC Recommended panel ?Sebaceous carcinoma ?SCC Sebaceous carcinoma BCC SCC 18 3/27/2017 Sebaceous carcinoma vs SCC Recommended panel Sebaceous carcinoma, PD Renal cell carcinoma BerEP4 Adipophilin Perforin expression in eyelid sebaceous carcinomas: a useful and specific Perforin expression in eyelid sebaceous carcinomas: a useful and specific immunomarker for the differential diagnosis of eyelid carcinomas immunomarker for the differential diagnosis of eyelid carcinomas Perforin Adipophilin EMA BerEP4 Acta Ophthalmologica Acta Ophthalmologica Volume 94, Issue 5, pages e325-e330, 4 FEB 2016 DOI: 10.1111/aos.12972 Volume 94, Issue 5, pages e325-e330, 4 FEB 2016 DOI: 10.1111/aos.12972 http://onlinelibrary.wiley.com/doi/10.1111/aos.12972/full#aos12972-fig-0001 http://onlinelibrary.wiley.com/doi/10.1111/aos.12972/full#aos12972-fig-0003 19 3/27/2017 Pts with unselected sebaceous neoplasms Sebaceous adenoma, epithelioma, or carcinoma Site: Below head and neck Sebaceous neoplasms & Age <50 years Muir-Torre syndrome Immunohistochemical analysis (MSH6, MSH2, MLH1) Positive (lack of one/more Negative MMRs) (No lack of MMRs) Positive MSI Negative MSI analyses Negative MSI analyses analyses Negative Negative Positive family Mutational Positive family family history history analysis history family history No further work- No further work- up required up required Strict cancer surveillance for proband and family members at risk Mismatch repair proteins MSH2 MLH1 Modern Pathology, 2008 20 3/27/2017 ?Primary adnexal ?Epidermotropic metastases Eccrine carcinoma Metastatatic carcinoma Primary adnexal carcinoma vs cutaneous metastases Recommended panel Primary Met Human Pathology, 2014 21 3/27/2017 Primary adnexal carcinoma vs cutaneous metastases Recommended panel Part III P40 Controversies 22 3/27/2017 Apocrine vs. eccrine ? Role for EM Benign adnexal tumors CK15 Apocrine vs. eccrine ? Role of IHC Mixed tumor HAP Apocrine cystadenoma Cylindroma Malignant adnexal tumors CK15 MAC Porocarcinoma Eccrine carcinoma 23 3/27/2017 The end! J Cut Pathol, 1984 24.
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