J Pathol Transl Med.  2018 Jul;52(4):238-242. 10.4132/jptm.2017.11.21.

Primary Cutaneous Mucinous Carcinoma with Extramammary Paget’s Disease: Eccrine or Apocrine?

• Oh SJ
• Kim YO

• KMID: 2417808
• DOI: http://doi.org/10.4132/jptm.2017.11.21

Abstract

Primary cutaneous mucinous carcinoma (PCMC) is an uncommon tumor of the sweat gland origin. The occurrence of PCMC is mostly in middle-aged and older patients, with a slight male predominance. Most cases of PCMC arise on the head, with a preference for eyelids. The histogenesis of PCMC, whether eccrine or apocrine, remains controversial. We report a rare case of PCMC with secondary extramammary Paget's disease in the groin of a 75-year-old man, which favored an apocrine origin. Furthermore, based on a review of the literature, we provide several histologic clues that can be used to differentiate PCMC from metastatic mucinous carcinoma.

Keyword

Cutaneous mucinous carcinoma; Skin adnexal tumor; Apocrine carcinoma; Paget's disease, extramammary

MeSH Terms

Adenocarcinoma, Mucinous*
Aged
Eyelids
Groin
Head
Humans
Male
Mucins*
Paget Disease, Extramammary
Sweat Glands
Mucins

Figure

• 

  Fig. 1. (A) Erythematous plaque of the left groin with extension to the scrotum and penis. (B) Histology of preoperative skin biopsy revealing extensive involvement of the epidermis by large pagetoid cells with clear or eosinophilic cytoplasm.

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  Fig. 2. (A) Moderately differentiated adenocarcinoma of the colon with dirty necrosis (arrows) diagnosed 13 years earlier. (B) Low magnification of the mass in the dermis with epidermal acanthosis due to pagetoid spread of the tumor cells. (C) Mucinous lakes with free-floating tumor nests (left) are juxtaposed with a cellular apocrine carcinoma (right), of which high magnification (D) reveals solid nests of tumor cells with round nuclei and abundant eosinophilic cytoplasm. (E) Comedo-type in situ component is depicted with normal apocrine glands (arrow). (F) Colonization of tumor cells in the hair follicle.

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  Fig. 3. Immunohistochemical findings of tumor cells showing positivity for cytokeratin (CK) 7 (A), CK20 (B), Gross cystic disease fluid protein 15 (C), human epidermal growth factor receptor 2 (D), and estrogen receptor (E). Myoepithelial cells of in situ components and normal apocrine glands highlighted by immunostaining for CK5/6 (F).

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