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Endocr Pathol. 2018 Dec;29(4):369-373. doi: 10.1007/s12022-018-9541-8.

Grade 3 Neuroendocrine Tumor (G3 NET) in a Background of Multiple Serotonin Cell Neoplasms of the Ileum Associated with Carcinoid Syndrome and Aggressive Behavior.

Author information

1
Anatomic Pathology Unit, Department of Molecular Medicine, University of Pavia and Fondazione IRCCS Policlinico San Matteo, Via Forlanini 14, 27100, Pavia, Italia.
2
Unit of General Surgery 2, Department of Clinical-Surgical, Diagnostic and Pediatric Sciences, University of Pavia and Fondazione IRCCS Policlinico San Matteo, Pavia, Italy.
3
Anatomic Pathology Unit, Department of Molecular Medicine, University of Pavia and Fondazione IRCCS Policlinico San Matteo, Via Forlanini 14, 27100, Pavia, Italia. alessandro.vanoli@unipv.it.

Abstract

Grade 3 well-differentiated neuroendocrine tumors (G3 NETs) have been characterized in the pancreas and stomach and distinguished from low-to-intermediate grade (G1-G2) NETs, as well as from highly malignant, poorly differentiated neuroendocrine carcinomas (NECs). Up to now, no G3 NET has been thoroughly described in the distal small intestine. We herein report a case of a 61-year-old man presenting with carcinoid syndrome. The surgical specimen showed, in a background of small G1 ileal NETs, a larger, grade 3 NET, which retained the nesting pattern and the expression of serotonin, chromogranin-A, and type 2A somatostatin receptors, typical of well-differentiated jejuno-ileal NETs. The patient had G3 NET metastasis to the liver and he died 14 months after surgery, due to deterioration in his clinical conditions.

KEYWORDS:

G3 neuroendocrine tumor; Grading; Prognosis; Small intestine neuroendocrine tumors

PMID:
30076568
DOI:
10.1007/s12022-018-9541-8
[Indexed for MEDLINE]

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