Digital Papillary Adenocarcinoma in Nonacral Skin : Clinicopathologic and Genetic Characterization of 5 Cases
(2023) In American Journal of Surgical Pathology 47(10). p.1077-1084- Abstract
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.... (More)
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 rarely present in nonacral locations mainly in the female anogenital area, usually with a more solid pattern as compared with those cases presenting on the digits and it is also associated with HPV42.
(Less)
- author
- organization
- publishing date
- 2023-10-01
- type
- Contribution to journal
- publication status
- published
- subject
- keywords
- digital papillary adenocarcinoma, genital, HPV42
- in
- American Journal of Surgical Pathology
- volume
- 47
- issue
- 10
- pages
- 8 pages
- publisher
- Lippincott Williams & Wilkins
- external identifiers
-
- pmid:37505796
- scopus:85171600196
- ISSN
- 0147-5185
- DOI
- 10.1097/PAS.0000000000002096
- language
- English
- LU publication?
- yes
- id
- 6ab32f47-29a5-496e-b269-683c09a6e7a5
- date added to LUP
- 2023-12-06 13:47:34
- date last changed
- 2024-04-19 08:21:48
@article{6ab32f47-29a5-496e-b269-683c09a6e7a5,
abstract = {{<p>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 rarely present in nonacral locations mainly in the female anogenital area, usually with a more solid pattern as compared with those cases presenting on the digits and it is also associated with HPV42.</p>}},
author = {{Kervarrec, Thibault and Imbeaud, Sandrine and Veyer, David and Pere, Helene and Puech, Julien and Pekár-Lukacs, Agnes and Markiewicz, Dorota and Coutts, Michael and Tallet, Anne and Collin, Christine and Berthon, Patricia and Bravo, Ignacio G. and Seris, Alice and Jouary, Thomas and Macagno, Nicolas and Touzé, Antoine and Cribier, Bernard and Battistella, Maxime and Calonje, Eduardo}},
issn = {{0147-5185}},
keywords = {{digital papillary adenocarcinoma; genital; HPV42}},
language = {{eng}},
month = {{10}},
number = {{10}},
pages = {{1077--1084}},
publisher = {{Lippincott Williams & Wilkins}},
series = {{American Journal of Surgical Pathology}},
title = {{Digital Papillary Adenocarcinoma in Nonacral Skin : Clinicopathologic and Genetic Characterization of 5 Cases}},
url = {{http://dx.doi.org/10.1097/PAS.0000000000002096}},
doi = {{10.1097/PAS.0000000000002096}},
volume = {{47}},
year = {{2023}},
}