Microcystic adnexal carcinoma

2018-02-26T15:01:59Z

Zlatica: /* IHC */

{{ Infobox diagnosis
| Name = {{PAGENAME}}
| Image =
| Width =
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| Micro = small basaloid cells - often forming small cystic spaces, usu. very bland cytology, fibrotic stroma
| Subtypes =
| LMDDx = [[sclerosing basal cell carcinoma]], desmoplastic [[trichoepithelioma]], infiltrative [[squamous cell carcinoma]]
| Stains =
| IHC = [[Ber-EP4]] -ve, CD15 -ve
| EM =
| Molecular =
| IF =
| Gross =
| Grossing =
| Site = [[skin]] - usually head and neck
| Assdx =
| Syndromes =
| Clinicalhx = adults
| Signs =
| Symptoms =
| Prevalence = very rare
| Bloodwork =
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| Endoscopy =
| Prognosis = good
| Other =
| ClinDDx =
}}
'''Microcystic adnexal carcinoma''', also known as ''syringomatous carcinoma'' and ''sclerosing sweat duct carcinoma'',<ref name=dermaamin>URL: [http://www.dermaamin.com/site/histopathology-of-the-skin/65-m/1926-microcystic-adnexal-carcinoma-.html http://www.dermaamin.com/site/histopathology-of-the-skin/65-m/1926-microcystic-adnexal-carcinoma-.html]. Accessed on: 16 September 2011.</ref> is rare malignant [[dermatologic neoplasm|tumour of the skin]] that can have a deceptively bland appearance.
==General==
*Low-grade tumour.
*Adults.
*Very rare<ref name=pmid21606063>{{Cite journal | last1 = Tawfik | first1 = AM. | last2 = Kreft | first2 = A. | last3 = Wagner | first3 = W. | last4 = Vogl | first4 = TJ. | title = MRI of a microcystic adnexal carcinoma of the skin mimicking a fibrous tumour: case report and literature review. | journal = Br J Radiol | volume = 84 | issue = 1002 | pages = e114-7 | month = Jun | year = 2011 | doi = 10.1259/bjr/44068180 | PMID = 21606063 }}</ref> ~ 150 cases in world literature in 2001.<ref name=pmid11298716/>
*Uncommonly metastases.<ref name=pmid11298716/>
==Gross==
*Typical location: head and neck.<ref name=pmid11298716/><ref name=pmid16549917>{{Cite journal | last1 = Gabillot-Carré | first1 = M. | last2 = Weill | first2 = F. | last3 = Mamelle | first3 = G. | last4 = Kolb | first4 = F. | last5 = Boitier | first5 = F. | last6 = Petrow | first6 = P. | last7 = Ortoli | first7 = JC. | last8 = Margulis | first8 = A. | last9 = Souteyrand | first9 = P. | title = Microcystic adnexal carcinoma: report of seven cases including one with lung metastasis. | journal = Dermatology | volume = 212 | issue = 3 | pages = 221-8 | month = | year = 2006 | doi = 10.1159/000091248 | PMID = 16549917 }}</ref>
*Usu. a firm subcutaneous nodule.<ref name=pmid11298716>{{Cite journal | last1 = Snow | first1 = S. | last2 = Madjar | first2 = DD. | last3 = Hardy | first3 = S. | last4 = Bentz | first4 = M. | last5 = Lucarelli | first5 = MJ. | last6 = Bechard | first6 = R. | last7 = Aughenbaugh | first7 = W. | last8 = McFadden | first8 = T. | last9 = Sharata | first9 = H. | title = Microcystic adnexal carcinoma: report of 13 cases and review of the literature. | journal = Dermatol Surg | volume = 27 | issue = 4 | pages = 401-8 | month = Apr | year = 2001 | doi = | PMID = 11298716 }}</ref>

==Microscopic==
Features:<ref>{{Ref Derm|412}}</ref>
*Small basaloid cells - often forming small cystic spaces - '''key feature'''.
**Very bland cytology.
*Fibrotic stroma.

Notes:
*Margins typically positive - as MAC typically extends well beyond what is suspected clinically.<ref name=pmid11298716/>

DDx:
*[[Sclerosing basal cell carcinoma]].
*Desmoplastic [[trichoepithelioma]] (DTE).<ref name=pmid22366669>{{Cite journal | last1 = Kirzhner | first1 = M. | last2 = Jakobiec | first2 = FA. | last3 = Borodic | first3 = G. | title = Desmoplastic trichoepithelioma: report of a unique periocular case. | journal = Ophthal Plast Reconstr Surg | volume = 28 | issue = 5 | pages = e121-3 | month = | year = | doi = 10.1097/IOP.0b013e318245535a | PMID = 22366669 }}</ref>
*Infiltrative [[squamous cell carcinoma]].

===Images===
www:
*[http://www.dermaamin.com/site/images/histo-pic/m/microcystic-adnexal-carcinoma/microcystic-adnexal-carcinoma2.jpg Microcystic adnexal carcinoma (dermaamin.com)].<ref name=dermaamin>URL: [http://www.dermaamin.com/site/histopathology-of-the-skin/65-m/1926-microcystic-adnexal-carcinoma-.html http://www.dermaamin.com/site/histopathology-of-the-skin/65-m/1926-microcystic-adnexal-carcinoma-.html]. Accessed on: 16 September 2011.</ref>
*[http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3473631/figure/bjr-84-e114-g004/ MAC (nih.gov).<ref name=pmid21606063/>

==IHC==
Features:<ref name=pmid23398472 >{{Cite journal | last1 = Sellheyer | first1 = K. | last2 = Nelson | first2 = P. | last3 = Kutzner | first3 = H. | last4 = Patel | first4 = RM. | title = The immunohistochemical differential diagnosis of microcystic adnexal carcinoma, desmoplastic trichoepithelioma and morpheaform basal cell carcinoma using BerEP4 and stem cell markers. | journal = J Cutan Pathol | volume = 40 | issue = 4 | pages = 363-70 | month = Apr | year = 2013 | doi = 10.1111/cup.12085 | PMID = 23398472 }}</ref>
*[[BerEP4]] -ve.
**Positive in [[sclerosing basal cell carcinoma]].
*CK15 -ve.
**Positive in DTE.
**Negative in sclerosing BCC.
*EMA +ve
**Negative in sclerosing BCC and desmoplastic trichoepithelioma

==See also==
*[[Dermatologic neoplasms]].

==References==
{{Reflist|2}}

[[Category:Diagnosis]]
[[Category:Dermatologic neoplasms]]

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Microcystic adnexal carcinoma