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Endocrine mucin-producing sweat gland carcinoma (EMPSGC) is a rare, low-grade adnexal neoplasm with predilection for the periorbital skin of older women. Histologically and immunophenotypically, EMPSGC is analogous to another neoplasm with neuroendocrine differentiation, solid papillary carcinoma of the breast. Both lesions are spatially associated with neuroendocrine mucinous adenocarcinomas of the skin and breast, respectively. EMPSGC is ostensibly a precursor of neuroendocrine-type mucinous sweat gland adenocarcinoma (MSC), a lesion of uncertain prognosis. Non-neuroendocrine MSC has been deemed locally aggressive with metastatic potential, and previous works speculated that EMPSGC-associated [neuroendocrine-type] MSC had similar recurrence and metastatic potential with implications for patient follow-up. Only 96 cases of EMPSGC have been reported (12 cases in the largest case series). Herein, we present 63 cases diagnosed as ‘EMPSGC’ in comparison with aggregated results from known published EMPSGC cases. We aim to clarify the clinicopathologic features and prognostic significance of the neuroendocrine differentiation of EMPSGC and its associated adenocarcinoma and to determine the nosological relevance of EMPSGC-association in the spectrum of MSC histopathogenesis. Results established an overall female predominance (66.7%) and average presenting age of 64 years. EMPSGC lesions were associated with adjacent MSC in 33.3% of cases. The recurrence rate for neuroendocrine-type MSC was approximately 21%, less than the reported 30% for non-neuroendocrine MSC. There were no cases of metastasis. EMPSGC and neuroendocrine-type MSC are distinct entities with more indolent behavior than previously reported, supporting a favorable prognosis for patients.

Objective Acral lentiginous melanoma is the most common subtype of cutaneous melanoma in Asian countries. This study aims to clarify the associations between certain histologic and immunohistochemical parameters, and identify their prognostic values. Methods We assessed several histologic features and conducted immunohistochemical study of programmed cell death ligand 1 (clone 22C3) and CD8/CD4 in 61 Taiwanese patients with Stage III/IV, non-BRAF acral lentiginous melanomas. Results A total of 41 males and 20 females were included, with a median age of 74 years. The majority of tumors occurred at nonungual locations (86.9%), with ‘foot’ being the most frequently affected site (85.2%). Positive programmed cell death ligand 1 staining (combined positive score ≥ 10) was significantly associated with the status of tumor-infiltrating lymphocytes (P = 0.036). Lack of skin ulceration was linked to the immunoexpression of CD8/CD4-high (P = 0.004). A superior clinical outcome was found in the tumor-infiltrating lymphocytes-present group (P = 0.011), and among which, CD8/CD4-high was significantly correlated with better survival (P Conclusion For advanced, non-BRAF acral lentiginous melanomas, comprehensive assessments of these microscopic traits, along with CD8/CD4 and PD-L1 immunoprofiles, may help guide the clinicians and patients through treatment decisions.