Cribriform apocrine carcinoma of the skin

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 24.05.2025

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Synonym(s)

cribriform carcinoma (engl.); cutaneous cribriform carcinoma (engl.); primary cutaneous cribriform apocrine carcinoma; Syringoides eccrine carcinoma

History
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Requena 1998

Definition
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Carcinoma classified as low-risk carcinoma, which is considered a histopathological variant of apocrine carcinomas.

Manifestation
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Young adults (mean age of onset: 47.8 years) with a clear preference for females

Localization
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Proximal parts of the upper and lower extremities

Clinic
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1.0-2.0 cm in size, slowly developing over a period of 1-2 years, usually flesh-colored, surface-smooth, painless (slight tenderness may be present) firm plaques or flat protuberant nodules. Clinically, the tumors are classified as dermatofibromas or cicatricial keloids.

Histology
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In the middle and deep dermis, spreading to the subcutis, there are moderately sharply defined, solid, CEA- and pancytokeratin positive, and S 100 and cytokeratin 20 negative tumor aggregates which are characterized by tubular strands embedded in a fibrillar stroma when magnified. The epithelial proliferates are characterized by small and larger holes, which cause the sieve-like (cribriform) aspect. The lumina are optically empty. Individual tubules are lined by cubic or cylindrical cells. Occasional mitoses.

Differential diagnosis
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adenoid basal cell carcinoma, metastasis of a breast carcinoma (atypical localization, lack of expression of estrogen or progesterone receptors), metastases of visceral carcinomas, primary cutaneous adenoid-cystic carcinoma (pronounced infiltrative growth with perineural infiltration)

Operative therapie
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Excision with a small (0.5 cm on all sides) safety margin. Mohs micrographic surgery is recommended (Kim MJ et al. 2024), as the tumor margins are difficult to delineate and there is a risk of local recurrence.

Progression/forecast
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After excised tumour on all sides in a healthy person healing. In a larger study of 26 cases none of the patients who were excised in sano developed a recurrence.

Literature
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  1. Adamski H et al.(2005) Primary cutaneous cribriform carcinoma: a rare apocrine tumor. J Cutan Pathol 32:577-580.
  2. Innocenti S et al. (2012) Primary cutaneous cribriform carcinoma of the neck: a case report. Pathologica 104:190-192.
  3. Kim MJ et al. (2024) Mohs micrographic surgery for the management of primary cutaneous cribriform carcinoma of the back. J Dtsch Dermatol Ges 22:584-586.
  4. Lestouquet FR et al. (20139 Primary cutaneous adenoid cystic carcinoma: an unusual case. Dermatol Online J 19:5.
  5. Requena D et al. (1998) Neoplasms with apo crine differentiations. Philadelphia: Lippincott-Raven
  6. Rütten A et al.(2009) Primary cutaneous cribriform apocrine carcinoma: a clinicopathologic and immunohistochemical study of 26 cases of an under-recognized cutaneous adnexal neoplasm. J Am Acad Dermatol 61:644-651.
  7. Udvardi A et al. (2016) Primary cutaneous cribriform apocrine carcinoma of the skin. Dermatol 67:750-752.
  8. Weinreb I et al..(2014) Novel PRKD gene rearrangements and variant fusions in cribriform adenocarcinoma of salivary gland origin. Genes Chromosomes Cancer 53:845-856
  9. Worrall DM et al,(2015) Cribriform adenocarcinoma of the tongue and minor salivary gland: transoral robotic surgical resection. ORL J Otorhinolaryngol Relat Spec 77:87-92.

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Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

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Last updated on: 24.05.2025