Digital Papillary Adenocarcinoma in Nonacral Skin

作者：Thibault Kervarrec, Sandrine Imbeaud, David Veyer, Hélène Péré, Julien Puech, Ágnes Pekár-Lukacs, D Markiewicz, Michael Coutts, Anne Tallet, Christine Collin, Patricia Berthon, Ignacio G. Bravo, Alice Seris, Thomas Jouary, Nicolas Macagno, Antoine Touzé, B. Cribier, Maxime Battistella, Eduardo Calonje · 发表于：The American Journal of Surgical Pathology · 年份：2023 · DOI：10.1097/pas.0000000000002096 · 被引用次数：22 · 研究领域：Cancer and Skin Lesions、Nonmelanoma Skin Cancer Studies、Tumors and Oncological Cases

Digital papillary adenocarcinoma (DPA) is a rare sweat gland neoplasm that has exceptionally been reported outside acral locations. Recently, human papillomavirus 42 was identified as the main oncogenic driver of DPA. Herein, we report 5 tumors arising in extra-acral locations predominantly in the female anogenital skin. Four patients were female and 1 patient was male. The mean age at the diagnosis time was 65 years (range: 55 to 82 y). Tumors were located on the vulva (n=3), perianal area (n=1), and forearm (n=1). Histologically, all tumors were lobular and mainly solid and composed of sheets of cells with rare focal papillae and frequent glandular structures in a "back-to-back" pattern and lined by atypical basophilic cells. Immunohistochemistry showed diffuse positivity for SOX10. Epithelial membrane antigen and carcinoembryonic antigen highlighted the luminal cells and staining for p63 and p40 revealed a consistent and continuous myoepithelial component around glandular structures. Follow-up was available in 3 cases (mean duration: 12 mo [range: 8 to 16 mo]). One patient developed local recurrence and 1 experienced regional lymph node metastases. HPV Capture Next-generation sequencing revealed the presence of the HPV42 genome in all samples. Viral reads distributions were compatible in the 5 cases with an episomal nature of the viral genome, with a recurrent deletion in the E1 and/or E2 open reading frames. In conclusion, this study demonstrates that digital DPA may rare...