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Difference between revisions of "Microcystic adnexal carcinoma"
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Microcystic adnexal carcinoma (view source)
Revision as of 01:54, 16 October 2013
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{{ Infobox diagnosis | |||
| Name = {{PAGENAME}} | |||
| Image = | |||
| Width = | |||
| Caption = | |||
| 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] - usu. head and neck | |||
| Assdx = | |||
| Syndromes = | |||
| Clinicalhx = adults | |||
| Signs = | |||
| Symptoms = | |||
| Prevalence = very rare | |||
| Bloodwork = | |||
| Rads = | |||
| 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. | |||
==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]]. | |||
Image: | |||
*[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. | |||
==See also== | |||
*[[Dermatologic neoplasms]]. | |||
==References== | |||
{{Reflist|2}} | |||
[[Category:Diagnosis]] | [[Category:Diagnosis]] | ||
[[Dermatologic neoplasms]] | |||