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
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The end!
J Cut Pathol, 1984
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