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.