Diagnosis and management of primary vulvar adenocarcinoma of mammary gland type: report of two distinct cases

Abstract

Adenocarcinoma of mammary gland type (AMGT) of the vulva is extremely rare and its aetiopathogenesis is not fully understood. Low incidence is partly responsible for the lack of guidelines for patient management. Here, two cases of postmenopausal patients diagnosed with AMGT with different therapeutical approaches are reported. Histopathological patterns are considered essential for diagnosis. The triple-negative immunohistochemical (IHC) profile of one of the cases represented a diagnostic challenge. Interestingly, it presented an immunophenotypical profile similar to triple-negative breast cancers, supporting the molecular similarities between vulvar AMGT and breast carcinomas. Surgical procedures include radical vulvectomy or radical local excision. Lymphatic involvement may be assessed by sentinel lymph node biopsy or lymphadenectomy. Adjuvant treatment was dependent on the IHC profile and disease staging. Although both cases had similar features on clinical examination, pathological and molecular characteristics and treatment approach were distinct. That illustrates the diagnostic and therapeutical challenge of this rare entity.