Endocrine mucin-producing sweat gland carcinoma and associated primary cutaneous mucinous carcinoma: Review of the literature.

BACKGROUND

Endocrine mucin-producing sweat gland carcinoma (EMPSGC) is a rare, low-grade, cutaneous adnexal carcinoma with neuroendocrine differentiation. It is considered to be a precursor of invasive neuroendocrine type primary cutaneous mucinous carcinoma (PCMC).

OBJECTIVE

To review clinicopathological literature summary of EMPSGC and associated neuroendocrine PCMC from all reported cases and compare its behavior vs non-neuroendocrine PCMC data reported in the literature.

METHODS

A review of English literature of all EMPSGC cases with and without associated PCMC was carried out.

RESULTS

EMPSGC was associated with invasive neuroendocrine type PCMC in 35.7% of cases. We found the recurrence rate of PCMC associated with EMPSGC of about 12.3%, which is much less than the 30% recurrence rate reported for the non-neuroendocrine subtype of PCMC. The non-neuroendocrine subtype of PCMC shows a 4% and 11% rate of distant and lymph node metastasis, respectively, vs EMPSGC-associated neuroendocrine type of PCMC, which is very indolent and only one recent case of locoregional metastasis out of 190 EMPSGC cases has been reported so far.

LIMITATION: Limitation of our study includes data derivation from case reports and case series in the literature.

CONCLUSION

The prognostic benefits for this cohort of patients may be considered in their overall management.