Au Rebecca Tian Mei, Bundele Manish M
Department of Pathology, Tan Tock Seng Hospital, Singapore, Singapore.
J Cutan Pathol. 2021 Sep;48(9):1156-1165. doi: 10.1111/cup.13983. Epub 2021 Mar 5.
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).
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.
A review of English literature of all EMPSGC cases with and without associated PCMC was carried out.
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 of our study includes data derivation from case reports and case series in the literature.
The prognostic benefits for this cohort of patients may be considered in their overall management.
内分泌性黏液生成汗腺癌（EMPSGC）是一种罕见的、低级别、具有神经内分泌分化的皮肤附属器癌。它被认为是侵袭性神经内分泌型原发性皮肤黏液癌（PCMC）的前驱病变。
回顾所有已报道病例中EMPSGC及相关神经内分泌型PCMC的临床病理文献综述，并将其行为与文献中报道的非神经内分泌型PCMC数据进行比较。
对所有伴有或不伴有相关PCMC的EMPSGC病例的英文文献进行综述。
35.7%的病例中EMPSGC与侵袭性神经内分泌型PCMC相关。我们发现与EMPSGC相关的PCMC复发率约为12.3%，远低于非神经内分泌型PCMC报道的30%的复发率。非神经内分泌型PCMC的远处转移率和淋巴结转移率分别为4%和11%，而与EMPSGC相关的神经内分泌型PCMC生长非常缓慢，迄今为止，在190例EMPSGC病例中仅报道了1例局部区域转移的近期病例。
我们研究的局限性包括数据来源于文献中的病例报告和病例系列。
在对这组患者的整体管理中可考虑其预后优势。
