Elsevier

Gynecologic Oncology

Volume 122, Issue 1, July 2011, Pages 190-198
Gynecologic Oncology

Review
Neuroendocrine tumors of the gynecologic tract: A Society of Gynecologic Oncology (SGO) clinical document

https://doi.org/10.1016/j.ygyno.2011.04.011Get rights and content

Abstract

Objective

Neuroendocrine tumors of the gynecologic tract are rare, and pose a significant clinical challenge because of the tumor heterogeneity and lack of standardized guidelines for treatment. This manuscript summarizes the available literature concerning these tumors in an effort to provide the clinician a framework from which to guide patient management.

Methods

MEDLINE was searched for all research articles published in English between January 1, 1966 and March 1, 2011 in which the studied population included women diagnosed with neuroendocrine tumors of the gynecologic tract. Although preference was given to prospective studies, studies were not limited by design or by numbers of subjects given the limited availability of reports.

Results

Most, but not all, neuroendocrine tumors of the gynecologic tract have an aggressive clinical course and those of the cervix histologically and clinically share similarities with small cell lung cancer. Cumulative data supports a multi-modality therapeutic strategy. A proposed management algorithm for neuroendocrine carcinomas of the cervix is outlined. For less frequent disease sites including the adnexa, uterus, vagina and vulva, as well as well differentiated carcinoid tumors, surgical resection is appropriate in selected cases. Etoposide/platinum based chemotherapy is used for neuroendocrine carcinomas but not for well differentiated carcinoid tumors. Well differentiated carcinoid and atypical carcinoid tumors should be managed similar to gastroenteropancreatic NETs (GEP-NETs).

Conclusions

Most neuroendocrine tumors of the gynecologic tract require a multi-modality therapeutic approach, determined by extent of disease and primary organ of involvement. Pathologic diagnosis is critical to guide therapy.

Research highlights

► Etoposide/cisplatin should be considered for early stage cervical SCC. ► Fertility sparing surgery can be considered for ovarian NETs. ► Gynecologic NETs are rare and data is limited.

Section snippets

SGO clinical document

The Society of Gynecologic Oncology (SGO) working through the Clinical Practice Committee (CPC) has developed a series of Clinical Documents designed in part to improve the overall quality of women's cancer care; reduce the use of unnecessary, ineffective or harmful interventions; as well as facilitate the optimal treatment of patients with a goal to maximize the therapeutic benefit, and minimize the risk of harm, at acceptable cost. SGO Clinical Documents remain strictly confidential and are

Methods

In 2007, at the 38th Annual Meeting on Women's Cancer, sponsored by the SGO, a subcommittee of the CPC was formed to begin development of clinical papers for subject areas where consensus was perceived as lacking. A MEDLINE search of English literature published between January 1966 and March 2011 was performed. All publications with the keywords “cervix”, “ovary”, “uterus”, and “vagina” were combined and then searched for the keyword “neuroendocrine, small cell, carcinoid” in order to develop

Small cell and large cell NECs of the cervix

In 1997, a workshop sponsored by the College of American Pathologists and the NCI proposed standardized terminology for neuroendocrine tumors of the uterine cervix [3]. The nomenclature was created as a parallel to that used for pulmonary endocrine tumors, and four categories were identified: Small cell carcinoma, large cell neuroendocrine carcinomas, typical carcinoid tumors, and atypical carcinoid tumors. Each of the four histologic categories can be associated with an in situ or invasive

Well differentiated neuroendocrine tumors (NETs): Typical and atypical carcinoids

Well differentiated NETs are thought to arise from the neural crest and have enzymatic potential. Similar to the poorly differentiated small and large cell neuroendocrine carcinomas, immunohistochemistry with well differentiated neuroendocrine markers, such as chromogranin, synaptophysin, and neuron specific enolase, can facilitate a histologic diagnosis.

Tumors that develop from the neuroendocrine tissues of the aerodigestive, gynecologic, and genitourinary tracts are called carcinoid tumors,

Summary and recommendations

Questions

  • 1.

    Given the high rate of metastatic spread, what is the role of surgery in the management of small cell cervical cancer? Hysterectomy is sometimes required to secure the diagnosis of small cell carcinoma of the cervix. In some cases, the limited tissue available on cervical biopsy yields only the diagnosis of a poorly differentiated carcinoma. For those patients in whom the diagnosis is known, hysterectomy can be considered for early stage disease as part of a multi-modality therapeutic

Conflict of interest statement

The authors of this review have no conflict of interests to declare.

Acknowledgments

The authors would like to thank the members of the Clinical Practice Committee with specific recognition of Dr. David Moore, Chair, Dr. Randall Gibb, Dr. Mitchell Edelson, Mary Eiken (SGO administration) and Melissa Finnegan (SGO administration).

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