An elderly lady presented with a 2-year history of intermittent vaginal bleeding and later the development of a vulvovaginal mass. A core biopsy histology specimen from the mass and the left inguinal lymph node was suggestive of metastatic adenocarcinoma of breast origin. No breast lesion was detected on mammography, and axillary nodes were negative. The histopathologic features and the expression of GATA3, cytokeratin (CK)7, mammaglobin staining and estrogen and progesterone receptors led to a diagnosis of breast cancer originating from the ectopic mammary tissue in the vulva. Given the rarity of these lesions, and the lack of standard treatment guidelines, the management of the patient was extrapolated from the established breast cancer treatment guidelines. Radiotherapy and chemotherapy followed by hormone therapy with aromatase inhibitor were administered to this patient in the metastatic setting with good palliation.

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We present a 70-year old woman with metastatic gastrointestinal stromal tumor (GIST) and her partner, a 79-year old man with multiple gastric GIST tumors. This tumor is considered a rare malignancy with a reported incidence of 6-13 new cases per million. Our patients were found to have different genetic mutations in the C-KIT gene as the cause of their disease but, given the rarity of this tumor, it raises a question about their possible exposure to carcinogens or another shared mechanism that might have been involved in the pathogenesis of this cancer.