Neuroendocrine Neoplasms of the Gynecologic Tract

Abstract

Gynecological tract neuroendocrine neoplasms (NEN) are rare, aggressive tumors from endocrine cells derived from the neuroectoderm, neural crest, and endoderm. The primary gynecologic NENs constitute 2% of gynecologic malignancies, and the cervix is the most common site of NEN in the gynecologic tract. The updated WHO classification of gynecologic NEN is based on the Ki-67 index, mitotic index, and tumor characteristics such as necrosis, and brings more uniformity in the terminology of NENs like other disease sites. Imaging plays a crucial role in the staging, triaging, restaging, and surveillance of NENs. The expression of the somatostatin receptors on the surface of neuroendocrine cells forms the basis of increasing evaluation with functional imaging modalities using traditional and new tracers, including 68Ga-DOTA-Somatostatin Analog-PET/CT. Management of NENs involves a multidisciplinary approach. New targeted therapies could improve the paradigm of care for these rare malignancies. This article focuses on the updated staging classifications, clinicopathological characteristics, imaging, and management of gynecologic NENs of the cervix, ovary, endometrium, vagina, and vulva, emphasizing the relatively common cervical neuroendocrine carcinomas among these entities.

Neuroendocrine Carcinomas of the Uterine Cervix, Endometrium, and Ovary Show Higher Tendencies for Bone, Brain, and Liver Organotrophic Metastases

“…All NECs included in this study showed significantly higher overall metastatic rates than non-NECs of the same primary sites. These findings are consistent with previous reports on NECs in the female genital tract [ 2 , 7 , 8 ].…”

Combined Large-Cell Neuroendocrine and Squamous Cell Carcinoma of the Uterine Cervix with a Personal History of the Primary Breast Duct Carcinoma in situ: A Clinicopathological Characteristic and Outcome

“…Therefore, the regimen given is similar to NEC alone. Although there are no optimal treatment guidelines for this subgroup, surgery is recommended and followed by adjuvant therapy in the early stage [ 4 ]. Our patient, with an initial diagnosis of FIGO IB2, underwent surgery.…”

“…Most of them present a pure form, and only 4% of this type of tumor is associated with adenocarcinoma [ 2 , 3 ], much less common with squamous cell carcinoma. Overall, NENs of the gynecologic tract are uncommon and aggressive, with high recurrence rates and dismal prognosis, making their treatment challenging [ 4 ]. In a study by Crane et al [ 5 ], the origin of these neoplasms in the cervix is highest, at 54%.…”

“…High-grade NECs are expressed with a high Ki67 index (more than 20%) and >20/10 HPF mitotic indices and include small- and large-cell NECs [ 1 ]. Large-cell NECs account for only about 12% of cervical NENs [ 4 ]. High-grade NEC of large cell has the diffuse organoid, trabecular or cord-like patterns.…”

Neuroendocrine carcinoma of vagina with prolonged survival

“…It commonly arises in the lung and only 2.5%–5% are extrapulmonary 1–3. Primary SCNC of the female genital tract constitutes less than 2% of all gynaecological cancers 4–6. It has been reported in the cervix, endometrium, ovary, vagina and vulva.…”