Poorly Differentiated Scrotal Carcinoma with Apocrine Immunophenotype

• Type of Material: Article
• Authors: Sonia Kamanda, Johns Hopkins Medical Institutions, Baltimore; Jonathan I Epstein, Johns Hopkins Medical Institutions, Baltimore; Adeboye Osunkoya, Emory University; Adeboye Cimino-Mathews, Johns Hopkins Medical Institutions, Baltimore; Argani Pedram, The Johns Hopkins Medical Institutions; Pedram Argani, The Johns Hopkins Medical Institutions; Martin Sangüeza, Hospital Obrero Nro.1 CNS, La Paz, Bolivia; Jose Antonio Plaza, The Ohio State University; Andres Matoso, The Johns Hopkins Medical Institutions, Baltimore
• Language: English
• Date: 2022-04-01
• Publisher: Wolters Kluwer Health, Inc.
• Publication Version: Author Accepted Manuscript (After Peer Review)
• Copyright Statement: © 2021 Wolters Kluwer Health, Inc. All rights reserved.
• Final Published Version (URL): http://dx.doi.org/10.1097/DAD.0000000000002100
• Title of Journal or Parent Work: American Journal of Dermatopathology
• Volume: 44
• Issue: 4
• Start Page: 260
• End Page: 266
• Keywords: Apocrine Glands; Humans; Immunohistochemistry; Middle Aged; Male; Carcinoma, Squamous Cell; Scrotum; Immunophenotyping; Skin Neoplasms; Adult; Aged, 80 and over; Combined Modality Therapy; Aged

Abstract

Cutaneous carcinoma of the scrotum is rare with the most common type being squamous cell carcinoma. Here, we report 6 cases of poorly differentiated carcinoma with apocrine immunophenotype. Mean age at presentation was 68 years (range: 31-91 years). Clinical presentation included eczematous rash over mass, scrotal cyst, ulcerated mass, and mass. Tumor size ranged from 1.2 to 5.5 cm (average 2.5 cm). The tumors were solid with involvement of the dermis/hypodermis and composed of cords and nests of eosinophilic cells displaying nuclei with prominent nucleoli and surrounded by desmoplastic stroma. Focal squamous differentiation was evident in one case (17%). An intraductal component was seen in one case (17%). Pagetoid spread in the epidermis was seen in 3 cases. There was no morphologic evidence of apocrine differentiation. By immunohistochemistry, the tumor cells were positive for GCDFP-15 (n = 6/6), GATA3 (n = 6/6), CK7 (n = 5/5), AR (n = 4/4), and mammaglobin (n = 3/5). Five (83%) patients had metastases at diagnosis. Treatment included wide local excisions and inguinal lymph node dissection, followed by chemotherapy (gemcitabine, carboplatin; n = 3), trastuzumab/Lupron (n = 1), tamoxifen/Arimidex (n = 1), and radiotherapy (n = 1). Two patients (40%) were dead of disease, less than 2 years from diagnosis. Four patients developed metastases to lymph nodes, liver, bones, and lungs. Molecular analysis (n = 2) detected a HER-2 mutation in one and microsatellite instability in another. Although the presence of an intraepidermal pagetoid component could hint toward the diagnosis of invasive extramammary Paget disease, tumors without an intraepidermal component could be diagnostically challenging given the lack of morphologic evidence of apocrine differentiation.