This information is found on M.D. Anderson’s necervix.com website.

“Neuroendocrine tumors (NETs) are neoplasms that are composed of cells which have features of both the endocrine (hormonal) as well as the nervous system [1]. They can be classified as benign or malignant (cancer). These tumors can originate from many different sites in the body, including the uterine cervix. The following discussion will be limited to malignant neuroendocrine carcinoma (NEC) of the cervix.

The cervix is the narrow, lower segment of the uterus (womb) that connects with the upper vagina. Tumors can arise from the outer (ectocervical) or inner (endocervical) portion of the cervix. Approximately 12,000 women in the United States will be diagnosed with cervical cancer in 2012 [2]. That means that approximately 1 in 147 women will develop cervical cancer in their lifetime [3].

There are multiple different types of cervical cancer, named after the appearance of the cells under the microscope. The most common type is squamous cell cancer, accounting for 70% of all cervical cancers. The second most common is adenocarcinoma, which accounts for 20-25% of all cervical cancer [4]. Neuroendocrine tumors account for only 2% of all cervical cancers [5]. Therefore, approximately 250 women are diagnosed annually with NEC of the cervix in the United States.

Four subtypes of NEC have been delineated:
• Small cell neuroendocrine carcinoma
• Large cell neuroendocrine carcinoma
• Typical carcinoid tumor
• Atypical carcinoid tumor

Of these four types, carcinoid tumors, although malignant, are considered to be well differentiated and therefore have a more indolent course and favorable prognosis [1]. Poorly differentiated, or high grade, NEC includes small cell neuroendocrine carcinoma (SCNEC) and large cell neuroendocrine carcinoma (LCNEC). Of the four subtypes, SCNEC is most common and LCNEC second most common of NEC arising from the cervix [6]. Since these two subtypes represent the majority of NEC of the cervix as well as the most aggressive phenotypes, the remaining discussion below will be limited to these two subtypes. In fact, small cell and large cell subtypes behave and are therefore treated similarly, and will be grouped together in the following statements.”

1. Klimstra, D.S., et al., The pathologic classification of neuroendocrine tumors: a review of nomenclature, grading, and staging systems. Pancreas, 2010. 39(6): p. 707-12.

2. Siegel, R., D. Naishadham, and A. Jemal, Cancer Statistics, 2012. CA Cancer J Clin, 2012. 62(1): p. 10-29.

3. American Cancer Society, Cancer Facts and Figures 2012. Available from http://www.cancer.org/research/cancerfactsfigures/cancerfactsfigures/cancer-facts-figures-2012.

4. Alfsen, G.C., et al., Histopathologic subtyping of adenocarcinoma reveals increasing incidence rates of endometrioid tumors in all age groups: a population based study with review of all nonsquamous cervical carcinomas in Norway from 1966 to 1970, 1976 to 1980, and 1986 to 1990. Cancer, 2000. 89(6): p. 1291-9.

5. Albores-Saavedra, J., et al., Terminology of endocrine tumors of the uterine cervix: results of a workshop sponsored by the College of American Pathologists and the National Cancer Institute. Arch Pathol Lab Med, 1997. 121(1): p. 34-9.

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