Difference between revisions of "Microcystic adnexal carcinoma"

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#redirect [[Dermatologic_neoplasms#Microcystic_adnexal_carcinoma]]
{{ 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]]

Revision as of 01:54, 16 October 2013

{{ Infobox diagnosis | Name = Microcystic adnexal carcinoma | 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,[1] is rare malignant tumour of the skin that can have a deceptively bland appearance.

General

  • Low-grade tumour.
  • Adults.
  • Very rare[2] ~ 150 cases in world literature in 2001.[3]
  • Uncommonly metastases.

Gross

  • Typical location: head and neck.[3][4]
  • Usu. a firm subcutaneous nodule.[3]

Microscopic

Features:[5]

  • 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.[3]

DDx:

Image:

IHC

Features:[7]

See also

References

  1. ↑ 1.0 1.1 URL: http://www.dermaamin.com/site/histopathology-of-the-skin/65-m/1926-microcystic-adnexal-carcinoma-.html. Accessed on: 16 September 2011.
  2. ↑ 2.0 2.1 Tawfik, AM.; Kreft, A.; Wagner, W.; Vogl, TJ. (Jun 2011). "MRI of a microcystic adnexal carcinoma of the skin mimicking a fibrous tumour: case report and literature review.". Br J Radiol 84 (1002): e114-7. doi:10.1259/bjr/44068180. PMID 21606063.
  3. ↑ 3.0 3.1 3.2 3.3 Snow, S.; Madjar, DD.; Hardy, S.; Bentz, M.; Lucarelli, MJ.; Bechard, R.; Aughenbaugh, W.; McFadden, T. et al. (Apr 2001). "Microcystic adnexal carcinoma: report of 13 cases and review of the literature.". Dermatol Surg 27 (4): 401-8. PMID 11298716.
  4. ↑ Gabillot-Carré, M.; Weill, F.; Mamelle, G.; Kolb, F.; Boitier, F.; Petrow, P.; Ortoli, JC.; Margulis, A. et al. (2006). "Microcystic adnexal carcinoma: report of seven cases including one with lung metastasis.". Dermatology 212 (3): 221-8. doi:10.1159/000091248. PMID 16549917.
  5. ↑ Busam, Klaus J. (2009). Dermatopathology: A Volume in the Foundations in Diagnostic Pathology Series (1st ed.). Saunders. pp. 412. ISBN 978-0443066542.
  6. ↑ Kirzhner, M.; Jakobiec, FA.; Borodic, G.. "Desmoplastic trichoepithelioma: report of a unique periocular case.". Ophthal Plast Reconstr Surg 28 (5): e121-3. doi:10.1097/IOP.0b013e318245535a. PMID 22366669.
  7. ↑ Sellheyer, K.; Nelson, P.; Kutzner, H.; Patel, RM. (Apr 2013). "The immunohistochemical differential diagnosis of microcystic adnexal carcinoma, desmoplastic trichoepithelioma and morpheaform basal cell carcinoma using BerEP4 and stem cell markers.". J Cutan Pathol 40 (4): 363-70. doi:10.1111/cup.12085. PMID 23398472.

Dermatologic neoplasms

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