Malignant Neuroendocrine Neoplasms
John Howard, M.D.
Administrator,
World Trade Center Health Program
I. Introduction
Neuroendocrine cells are nerve cells that respond to signals from other nerve cells by releasing
hormones into the blood. They are distributed widely throughout the body and may undergo
malignant transformation to give rise to neuroendocrine neoplasms.
Neuroendocrine neoplasms are commonly defined as epithelial neoplasms with a predominant
presence of scattered neuroendocrine cells singly or in small nests (neuroendocrine
differentiation).
Many neuroendocrine neoplasms traditionally have been called "carcinoids,"
but this term does not accurately account for their variable biology, histologic differentiation,
and secretory potential.
The WTC Health Program uses the term “malignant neuroendocrine
neoplasm” to refer to the family of solid malignant tumors that are believed to originate from
neuroendocrine cells found throughout the body, including “carcinoid tumors.”
II. Anatomic Distribution
Neuroendocrine neoplasms arise in a variety of anatomic locations, including the lung, stomach,
small intestine, pancreas, colon, rectum, breast, prostate and ovary.3,4
1
The International Classification of Diseases, Ninth Revision, Clinical Modification (ICD-9-CM) is based on the
World Health Organization's Ninth Revision, International Classification of Diseases (ICD-9). ICD-9-CM is the official
system of assigning codes to diagnoses and procedures associated with hospital utilization in the United States.
2
Klimstra DS, Modlin IR, Coppola D, Lloyd RV, Suster S [2012]. The pathologic classification of neuroendocrine
tumors: a review of nomenclature, grading and staging systems. Pancreas 39(6):707-712.
3 Modlin IM, Oberg K, Chung DC, Jensen RT, deHerder WW, Thakker RV, Caplin M, Delle Fave G, Kaltsas GA,
Krenning EP, Moss SF, Nilsson O, Rindi G, Salazar R, Ruszniewski, Sundin A [2008]. Gastroenteropancreatic
neuroendocrine tumours. Lancet Oncol 9:61-72.
4
Ramage JK, Ahmed A, Ardill J, Bax N, Breen DJ, Caplin ME, Corrie P, Davar J, Davies AH, Lewington V, Meyer T,
Newell-Price J, Poston G, Reed N, Rockall A, Steward W, Thakker RV, Toubanakis C, Valle J, Verbeke C, Grossman
The majority of neuroendocrine tumors occur in the gastrointestinal tract (67.5%) and the
bronchopulmonary tree (25.3%).5
Within the gastrointestinal tract, most neuroendocrine tumors occur in the small intestine
(41.8%), rectum (27.4%), and stomach (8.7%), and less than 1% of neuroendocrine neoplasms
occur in the pancreas.6
Other anatomical sites where neuroendocrine neoplasms also arise—
but very rarely—include the uterus, ovary, testis, breast and larynx.7
III. Clinical and Pathologic Features
Some of the clinical and pathologic features of neuroendocrine neoplasms are
characteristic of the anatomic site of origin, but, more frequently, neuroendocrine
neoplasms have more in common with each other than they do with the anatomic site
where they arise. For example, some functioning neuroendocrine neoplasms produce
polypeptide hormones that are not commonly produced by normal cells within the
same anatomic site, such as gastrin, vasoactive intestinal polypeptide, or
adrenocorticotropic hormone production by neuroendocrine neoplasms in the
pancreas.8
A hypersecretory syndrome may be the first indication of the presence of a
neuroendocrine neoplasm.4 Other neuroendocrine neoplasms may be non-functioning
and exhibit no hormone-related clinical features. Neuroendocrine neoplasms, then, are
said to “involve” a particular organ, but they are not “specific” to that particular organ.
IV. Classification
Neuroendocrine neoplasms do not have a single unified system of nomenclature,
grading or staging. However, both the World Health Organization (WHO), and the
International Classification of Diseases (ICD) coding system, classify neuroendocrine
neoplasms as a unified group, distinct from other neoplasms.9,10,11
AB [2012] . Guidelines for the management of gastroenteropancreatic neuroendocrine (including carcinoid)
tumours (NETs). Gut 61:6-32. “Gastroenteropancreatic NETs may be classified into non-functioning tumours, which
have no hormone-related clinical features, and functioning tumours, which cause symptoms due to peptide and
hormone release.”
Reference
Obtained Obtober 5, 2016
prevalence of neuroendocrine tumors as a group meets the current WTC Health Program’s definition for
“rare cancers” found at 42 C.F.R. § 88.1.(4)(Table 1)—“any type of cancer affecting populations smaller than
200,000 individuals in the United States, i.e., occurring at an incidence rate less 0.08 percent of the U.S.
population.”
16 WTC Health Program. Minimum Latency & Types or Categories of Cancer (May 1, 2013).
http://www.cdc.gov/wtc/pdfs/wtchpminlatcancer2013-05-01.pdf
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