Adult neoplasms with neuroendocrine differentiation and a known primary site

Indolent biology

Aggressive biology

Well-differentiated neuroendocrine tumor (carcinoid, atypical carcinoid, many primary sites)

Well-differentiated pancreatic neuroendocrine tumor (islet cell tumor)

Medullary carcinoma of the thyroid

Paraganglioma

Pheochromocytoma

Small cell and large cell neuroendocrine lung cancer

High-grade poorly-differentiated neuroendocrine carcinoma (small cell and large cell types, many primary sites)*

Extrapulmonary small cell carcinoma (many primary sites)

Merkel cell tumor of the skin

Neuroblastoma

* A subset of high-grade neuroendocrine carcinomas has morphology consistent with a well-differentiated tumor but high proliferative activity (high-grade well-differentiated neuroendocrine tumor). These neoplasms have biologic behavior that is intermediate between an indolent and aggressive natural history.

Reproduced with modifications from: Greco FA, Hainsworth JD. Cancer of Unknown Primary site. In: Cancer: Principles and Practice of Oncology 8th edition, DeVita VT, Lawrence TS, Rosenberg SA (Eds), Lippincott Williams & Wilkins, Philadelphia 2008. Copyright © 2008 Lippincott Williams & Wilkins.

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Adult neoplasms with neuroendocrine differentiation and a known primary site