Primary vulvar adenocarcinomas are rare, and tumours arising from anogenital mammary-like glands (A-AMG) are exceptionally uncommon. These glands share morphological and immunophenotypic features with breast tissue. We report the case of a woman in her 90s with no previous gynaecological follow-up or breast cancer screening, admitted with septic shock and multiorgan dysfunction. As part of the investigation for the source of infection, an abdominopelvic CT scan revealed a perineal mass. Gynaecological examination confirmed a firm 5 cm lesion involving the right labia majora and extending to the lower third of the vagina. Histological examination demonstrated an adenocarcinoma arising from A-AMG. Further investigation showed no evidence of a breast primary or distant metastases, supporting the diagnosis of a primary vulvar tumour. The patient declined surgical management and was managed conservatively. This case highlights the diagnostic challenges posed by this rare entity and emphasises the importance of careful histopathological evaluation.
Aguiar et al. (2026) studied this question.