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Non‐human‐papillomavirus‐related malignancies of the vulva: A clinicopathological study
Author(s) -
Furlan Karina,
Rohra Prih,
Mir Fatima,
Gattuso Paolo
Publication year - 2020
Publication title -
journal of cutaneous pathology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.597
H-Index - 75
eISSN - 1600-0560
pISSN - 0303-6987
DOI - 10.1111/cup.13768
Subject(s) - vulva , medicine , malignancy , extramammary paget's disease , pathology , disease , dermatology , dermatofibrosarcoma protuberans , vulvar neoplasm , differential diagnosis , oncology
Background Malignant tumor of the vulva is the fourth gynecological malignancy in frequency. Close to 70% of all vulvar malignancies are related to high‐risk human papillomavirus (HPV) infection. Methods A search for non‐HPV‐related malignant tumors of the vulva was performed in the last 20 years (2000‐2020) in the pathology database of a single tertiary institution. We aim to estimate the prevalence of non‐HPV‐related malignancies in our population, describe clinicopathological features of these tumors and investigate the expression of some potential therapeutic targets. Results A total of 71 patients were recovered; 26 patients (36%) had the diagnosis of extramammary Paget disease, 17 patients (24%) had basal cell carcinomas, 17 patients (24%) had primary melanomas, 10 patients (14%) had metastatic disease to the vulva and one patient (1%) had a primary dermatofibrosarcoma protuberans. Fifty‐four percent of patients with extramammary Paget disease had a secondary malignancy and 12.5% had invasive disease. Programmed death‐ligand 1 (PDL‐1) was positive in seven out of nine primary melanomas and Her2/neu was overexpressed in six out of seven extramammary Paget disease. Conclusion Non‐HPV‐related malignancies are important differential diagnoses in patient with vulvar lesions. Additional research is necessary to further understand these complex malignancies and potential new therapeutic targets.

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