A case report of HPV negative small cell neuroendocrine carcinoma and squamous cell carcinoma of the cervix : a rare but fatal mix

Abstract

Malignant neoplasms that show divergent differentiation, like squamous cell carcinoma (SCC) and small cell neuroendocrine carcinoma (SNEC), occur very rarely in the cervix. Neuroendocrine tumors of the female genital tract tend to occur in combination with other types of tumors although they have been also described to occur as solitary neoplasms. Here, we present a case of a 51-year-old woman with a one-month history of vaginal bleeding and one week history of persistent lower abdominal pain. On vaginal examination a large irregular fixed cylindrical mass in mid-vagina, extending to the cervix, was felt. A computerized tomographic scan showed a uterine mass with retroperitoneal and pelvic lymphadenopathy together with multiple bilateral lung metastases. Cytological analysis via a cervical pap smear reported a high-grade intraepithelial lesion and atypical glandular cells of undetermined significance. Histological analysis of the cervical biopsies showed a necrotic biphasic neoplasm. The morphological and immunohistochemical findings were those of a poorly differentiated carcinoma with squamous and high grade neuroendocrine (small cell) differentiation. Polymerase chain reaction analysis for Human Papilloma Virus (HPV) performed on shavings from the paraffin-embedded tissue showed no evidence of HPV DNA. The patient was planned to receive primary chemotherapy but passed away within 3 weeks of her diagnosis. In conclusion, tumors showing SNEC differentiation, together with rare cases of primary cervical SNEC exhibit a different disease profile when compared with pure cervical SCC, in that the former are highly aggressive and has a greater propensity for nodal and distant organ metastasis. These tumors are associated with a poor prognosis.