Non-human-papillomavirus-related malignancies of the vulva: A clinicopathological study

J Cutan Pathol. 2020 Oct;47(10):917-922. doi: 10.1111/cup.13768. Epub 2020 Jul 29.

Abstract

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.

Keywords: Vulvar EMPD; vulvar BCC; vulvar DFSP; vulvar melanoma; vulvar metastasis.

Publication types

  • Review

MeSH terms

  • Adult
  • Aged
  • Aged, 80 and over
  • B7-H1 Antigen / metabolism
  • Carcinoma, Basal Cell / diagnosis
  • Carcinoma, Basal Cell / epidemiology
  • Databases, Factual
  • Dermatofibrosarcoma / diagnosis
  • Dermatofibrosarcoma / epidemiology
  • Diagnosis, Differential
  • Female
  • Humans
  • Melanoma / diagnosis
  • Melanoma / epidemiology
  • Melanoma / metabolism
  • Middle Aged
  • Neoplasm Invasiveness / pathology
  • Neoplasm Metastasis / pathology
  • Neoplasms / epidemiology*
  • Neoplasms / pathology*
  • Neoplasms / virology
  • Neoplasms, Second Primary / epidemiology
  • Neoplasms, Second Primary / pathology
  • Paget Disease, Extramammary / complications
  • Paget Disease, Extramammary / diagnosis
  • Paget Disease, Extramammary / epidemiology
  • Paget Disease, Extramammary / metabolism
  • Papillomavirus Infections / complications*
  • Papillomavirus Infections / virology
  • Prevalence
  • Receptor, ErbB-2 / metabolism
  • Retrospective Studies
  • Skin Neoplasms / metabolism
  • Skin Neoplasms / pathology*
  • Vulvar Neoplasms / ethnology
  • Vulvar Neoplasms / pathology*
  • Vulvar Neoplasms / secondary

Substances

  • B7-H1 Antigen
  • CD274 protein, human
  • ERBB2 protein, human
  • Receptor, ErbB-2