Microcystic adnexal carcinoma

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Microcystic adnexal carcinoma
Diagnosis in short

LM small basaloid cells - often forming small cystic spaces, usu. very bland cytology, fibrotic stroma
LM DDx sclerosing basal cell carcinoma, desmoplastic trichoepithelioma, infiltrative squamous cell carcinoma
IHC Ber-EP4 -ve, CD15 -ve
Site skin - usually head and neck

Clinical history adults
Prevalence very rare
Prognosis good

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.[3]

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:

Images

www:

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 3.4 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.