Neuroendrocrine tumors of the uterine cervix: A therapeutic challenge for gynecologic oncologists

医学 神经内分泌肿瘤 子宫颈 淋巴结 嗜铬粒蛋白A 肿瘤科 宫颈癌 化疗 病理 内科学 癌症 免疫组织化学
作者
Angiolo Gadducci,Silvestro Carinelli,Giovanni Aletti
出处
期刊:Gynecologic Oncology [Elsevier BV]
卷期号:144 (3): 637-646 被引量:138
标识
DOI:10.1016/j.ygyno.2016.12.003
摘要

Neuroendocrine tumors (NETs) are aggressive diseases developing from neuroendocrine cells that most frequently involve the gastro-entero-pancreatic tract and the lung, but more rarely are found in almost all body tissues. Limited biological and clinical data are currently available for NETs in uncommon sites, such as female genital tract. NETs represent 0.9% to 1.5% of the tumors of the uterine cervix. They are more likely to have lymph-vascular space invasion and lymph node involvement, and to develop local and distant relapses when compared with the mostly common cervical squamous cell carcinomas or adenocarcinomas. Positive immunostaining for synaptophysin, chromogranin, CD56, and neuron-specific enolase is often detected in cervical NETs . The most recent editions of the World Health Organization Classification of Gynecologic Tract tumors grouped cervical carcinoid tumor and atypical carcinoid tumor into low-grade NETs and cervical small cell neuroendocrine carcinoma and large cell neuroendocrine carcinoma into high-grade NETs. High-risk HPV DNA is detected in almost all cervical high-grade NETs. No treatment guidelines, based on prospective, well-designed clinical trials, are currently available due to the rarity of these tumors. Many authors have reported different multimodality approaches, mainly derived from NETs of the lung. These usually consist in radical hysterectomy followed by adjuvant chemotherapy or concurrent chemoradiation for early stage disease, definitive concurrent chemoradiation sometimes preceded by neoadjuvant chemotherapy and followed by adjuvant chemotherapy for locally advanced disease, and palliative chemotherapy for metastatic disease. In this systematic review, we address the histologic classification of cervical NETs, analyze their pathogenesis and overall prognosis, and evaluate the different treatment modalities described in the literature, in order to offer a possible algorithm that may help the clinicians in diagnosing and treating patients with these uncommon and aggressive malignancies.
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