Malignant vascular neoplasms of the distal female genital tract: a series of 19 cases emphasizing unusual features and diagnostic challenges

Aims Scarce literature exists regarding the spectrum of vascular neoplasms of the distal female genital tract. Herein, we describe the clinicopathologic features of a cohort of intermediate to malignant endothelial neoplasms of the vulva and vagina. Methods and results Specimens were identified retrospectively from in‐house and consultation files at our institution. Clinicopathologic data was obtained from review of the chart and available histologic material. Nineteen cases were identified in total. Kaposi sarcoma ( n = 2) was of the enigmatic ‘sporadic’ form in elderly non‐ HIV females. Kaposiform haemangioendothelioma ( n = 1) presented at an older (rather than paediatric) age and was negative for HHV ‐8 by IHC ; both Kaposi sarcomas were positive. Pseudomyogenic haemangioendothelioma ( n = 2) presented in elderly females (rather than young males) with positivity for both FOSB and AE1 / AE3 . Epithelioid haemangioendothelioma ( n = 8), showed variably well‐developed chondromyxoid matrix and consistent positivity for CAMTA1 IHC . Angiosarcomas were disproportionately of the epithelioid subtype, with high‐grade cytologic atypia, destructive infiltration and rare EMA positivity. Conclusions While many morphologic features of vascular lesions at this site are shared with those of extra‐genital soft tissues, pathologists must be aware that these tumours may occur in the vulvovaginal area and display unconventional clinical profiles. As such, a broad differential for epithelioid and spindle cell neoplasms at this site is prudent.