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The archaic distinction between functioning and nonfunctioning neuroendocrine neoplasms is no longer clinically
Irvin M Modlin1, Steven F Moss, Bjorn I Gustafsson
1Gastrointestinal Pathobiology Group, Yale University School of Medicine, 310 Cedar Street, New Haven, CT, USA. imodlin@optonline.net
Langenbeck'S Archives of Surgery
|April 28, 2011
Summary
Nonfunctional neuroendocrine neoplasms (NENs) are biologically identical to functional NENs and should be treated the same. Discarding the functional/nonfunctional distinction improves diagnosis and management of these growing tumors.
Area of Science:
- Oncology
- Endocrinology
- Pathology
Background:
- Neuroendocrine neoplasms (NENs) are increasing in incidence and prevalence due to improved detection.
- Clinical neuroendocrine syndromes are well-defined, but asymptomatic NENs present diagnostic challenges.
Purpose of the Study:
- To evaluate the distinction between functional and nonfunctional neuroendocrine neoplasms (NENs).
- To advocate for a unified approach to the diagnosis and treatment of all NENs.
Main Methods:
- Histopathological analysis comparing functional and nonfunctional NENs.
- Biochemical and imaging diagnostic modalities (e.g., CgA, CT, MRI, somatostatin receptor scintigraphy).
- Assessment of treatment response to somatostatin analogs, tyrosine kinase inhibitors, antiangiogenics, and cytotoxics based on grade.
Main Results:
- Nonfunctional NENs are often diagnosed incidentally at later stages.
- Histopathology, somatostatin receptor expression, and neuroendocrine markers are indistinguishable between functional and nonfunctional NENs.
- Growth and metastatic behavior correlate with staging and grading (mitotic rate, Ki67) irrespective of function.
Conclusions:
- Nonfunctional NENs should be managed identically to symptomatic NENs due to indistinguishable tumor characteristics.
- The distinction between functional and nonfunctional NENs is archaic and should be abandoned.
- Unified diagnostic and treatment principles for all NENs are supported by current evidence.
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