Cazzato, G.; Bellitti, E.; Trilli, I.; Colagrande, A.; Sgarro, N.; Scarcella, V.S.; Lettini, T.; Ingravallo, G.; Piscitelli, D.; Resta, L.; Lospalluti, L. Endocrine Mucin-Producing Sweat Gland Carcinoma: Case Presentation with a Comprehensive Review of the Literature. Dermatopathology2023, 10, 266-280.
Cazzato, G.; Bellitti, E.; Trilli, I.; Colagrande, A.; Sgarro, N.; Scarcella, V.S.; Lettini, T.; Ingravallo, G.; Piscitelli, D.; Resta, L.; Lospalluti, L. Endocrine Mucin-Producing Sweat Gland Carcinoma: Case Presentation with a Comprehensive Review of the Literature. Dermatopathology 2023, 10, 266-280.
Cazzato, G.; Bellitti, E.; Trilli, I.; Colagrande, A.; Sgarro, N.; Scarcella, V.S.; Lettini, T.; Ingravallo, G.; Piscitelli, D.; Resta, L.; Lospalluti, L. Endocrine Mucin-Producing Sweat Gland Carcinoma: Case Presentation with a Comprehensive Review of the Literature. Dermatopathology2023, 10, 266-280.
Cazzato, G.; Bellitti, E.; Trilli, I.; Colagrande, A.; Sgarro, N.; Scarcella, V.S.; Lettini, T.; Ingravallo, G.; Piscitelli, D.; Resta, L.; Lospalluti, L. Endocrine Mucin-Producing Sweat Gland Carcinoma: Case Presentation with a Comprehensive Review of the Literature. Dermatopathology 2023, 10, 266-280.
Abstract
(1) Background: Endocrine Mucin-Producing Sweat Gland Carcinoma (EMPSGC) is a rare low-grade, neuroendocrine-differentiated, cutaneous adnexal tumour, officially recognized by the World Health Organization (WHO) Skin Tumours Classification in 2018 as a separate entity and homologue of endocrine ductal carcinoma in situ (eDCIS)/solid papillary carcinoma of the breast. Although it is more frequent in the female sex, between the sixth and seventh decade, in the peri-orbital region, EMPSGC has also been described in the male sex, in subjects under 60 and over 80, and in extra-eyelid localizations (cheek, temple, scalp), but also in extra-facial localizations (chest and scrotum). (2) Methods: We present the clinical case of a 71-year-old woman with an undated lesion of the scalp, which presented as a nodule, skin-coloured, 2.5 cm in maximum diameter. We also conduct a comprehensive literature review from 1997 to the end of 2022, consulting PubMed, Scopus and Web of Science (WoS), using the following keywords: "Endocrine mucin-producing sweat gland carcinoma" and/or "EMPSGC" and/or "skin" AND "cutaneous neoplasms", and following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. A total of 248 patients were recorded with the majority, 146 females (58,8%) and 102 males (41,1%). The vast majority of the lesions were in the elides (peri-ocular region) and only a minority of cases involved the cheeks, supra-auricular, retro-auricular and occipital region, with very rare cases in the scalp, to which the present is also added (4) Conclusions: The morphological and immunophenotypical features are essential both for the correct diagnosis and to be able to classify this lesion among the corresponding eDCIS/solid papillary carcinoma of the breast, with neuroendocrine differentiation. Recent papers have attempted to shed light on the molecular features of EMPSGC and much remains to be done in the attempt to subtype the molecular profiles of these entities. Future studies with large case series, and especially with molecular biology techniques, will be needed to further add information about EMPSGC and its relationship in the PCMC spectrum.
Medicine and Pharmacology, Pathology and Pathobiology
Copyright:
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