Endocrine Abstracts

Primary vaginal malignancy is uncommon, accounting for about 2% of all gynecological tract cancers, with small-cell neuroendocrine carcinoma of the vagina being exceptionally rare and aggressive. They display similar histological and immunohistochemical features to small-cell carcinomas of different origin. Few cases are reported in the literature so far and therefore, there is no current treatment protocol consensus. We report the case of a 34-year-old nulliparous patient, who presented for evaluation in May 2022 after palpatory detection of a vaginal mass during ovule treatment, following an annual Pap smear, that showed nonspecific inflammation and was negative for squamous intraepithelial lesion. She was otherwise asymptomatic and had a medical history of two surgically removed polypoid uterine adenomyomas. Family history was also positive for genital and gastrointestinal malignancy. Upon gynecological examination, a highly vascularized, friable, 5 cm mass was identified, located on the posterior vaginal wall, occupying almost the entire vagina. The histopathological biopsy report showed a poorly differentiated carcinoma and immunohistochemistry confirmed a neuroendocrine carcinoma, with SSTR2 expression. The following imagistic screening revealed secondary pulmonary determinations and metastatic adenopathies, and also a solid 6 cm mass on the left ovary. PET-CT scan also showed some metabolically active bone lesion, one on the tenth costal arch. The multidisciplinary team decided initiation of cisplatin and etoposide combination chemotherapy. After 8 cycles, the patient had a good response to the treatment, with normalization of tumoral markers (initially high CA125, CA15-3, CEA) and dimensional regression of the primary tumor, some metastases and of the ovarian tumor. The patient underwent whole-body octreotide scintigraphy with SPECT/CT and is currently being evaluated for surgical excision and radiotherapy of bone determinations. In conclusion, we present the unique case of advanced, metastatic neuroendocrine carcinoma of the vagina in a young patient with associated genital malignancy family history, emphasizing the necessity of a multidisciplinary approach, genetic screening and further study of the disease. Small-cell neuroendocrine carcinoma of the vagina is extremely rare, with aggressive behavior and poor prognosis. Histological recognition of this separate entity is important and multimodality treatment could improve patient outcomes.