Neuroendocrine Carcinoma of Female Genital Tract: Series of Nine Cases

Abstract

Introduction Neuroendocrine carcinoma of female genital tract is a rare, aggressive disease, with incidence of less than 2%. Optimal therapy is poorly understood; hence, more case reports and studies are required to formulate appropriate therapy. Methods Retrospective analysis by chart review of histopathology- and immunohistochemistry-confirmed neuroendocrine carcinoma between October 2014 and August 2019 in a tertiary care hospital. Results Female genital tract neuroendocrine carcinoma was diagnosed in nine patients: Out of nine, six women were diagnosed with neuroendocrine carcinoma of the cervix, two with ovarian neuroendocrine carcinoma and one with neuroendocrine carcinoma of endometrium. Among the patients with neuroendocrine carcinoma cervix, vaginal bleeding and discharge were common and all were small-cell neuroendocrine carcinoma. Two of them were stage IIB and IIIC1r and received chemo-radiation and etoposide–platinum-based chemotherapy with progression-free survival of 53\xa0months and 18\xa0months, respectively. Four stage IVB patients received only chemotherapy and progressed or died few months after completion of treatment. Out of two with ovarian neuroendocrine carcinoma, one was small cell and another large cell. Large-cell case was in stage IIb, diagnosed postoperatively and lost to follow-up. Small-cell case was young and in stage IVB who progressed and succumbed after the second cycle of chemotherapy. One large-cell neuroendocrine carcinoma of endometrium stage IVB presented with extensive pelvic disease and skeletal and lung metastasis and defaulted after one cycle of chemotherapy. Conclusions Neuroendocrine carcinoma patients, presented in advanced stage, are resistant to therapy and have an early demise. Although diagnosis is made, therapy has to be optimized by multicentric protocols.