PMID- 9298884
OWN - NLM
STAT- MEDLINE
DCOM- 19971006
LR  - 20190822
IS  - 0147-5185 (Print)
IS  - 0147-5185 (Linking)
VI  - 21
IP  - 9
DP  - 1997 Sep
TI  - Aggressive forms of gastric neuroendocrine tumors in multiple endocrine neoplasia 
      type I.
PG  - 1075-82
AB  - In recent classifications of gastric endocrine tumors, tumors arising in patients 
      with multiple endocrine neoplasia type 1 (MEN-1) are regarded to be regulated by 
      the concomitant hypergastrinemia resulting from to pancreatic or, most commonly, 
      duodenal gastrinomas and to have a benign behavior. In this article, we report on 
      two cases of MEN-1 gastric neuroendocrine tumors having a fatal course. Case 1 
      was a young male with hyperparathyroidism and Zollinger-Ellison syndrome and with 
      florid development of multiple gastric carcinoids and multiple duodenal 
      gastrinomas. Metastases occurred in the liver, of exclusive gastric origin, in 
      periduodenal lymph nodes, of exclusive duodenal origin, and in perigastric lymph 
      nodes, of mixed origin. The patient died 48 months after diagnosis. Case 2 was an 
      adult female patient with hyperparathyroidism, adrenocortical disorders, and 
      gastric tumors but no hypergastrinemia. The patient died 3 months after tumor 
      diagnosis. At autopsy, the stomach showed multiple benign carcinoids and two 
      independent neuroendocrine carcinomas not reported before in MEN-1 and massively 
      metastatizing to lymph nodes, liver, and peritoneum. Multiple islet cell tumors 
      mostly producing pancreatic polypeptide were found, whereas gastrinomas were seen 
      in neither the pancreas nor the duodenum. Allelic losses at the MEN-1 gene locus 
      in chromosome 11q13, the mechanism responsible for tumor development in MEN-1 
      syndrome, were demonstrated in the carcinoid tumors of case 1 and in the 
      neuroendocrine carcinoma of case 2. We conclude that gastric neuroendocrine 
      tumors in patients with MEN-1 may have a poor outcome, they have the same genetic 
      mechanism as MEN-1 tumors in other organs, and they may be independent of the 
      trophic effect of hypergastrinemia.
FAU - Bordi, C
AU  - Bordi C
AD  - Institute of Anatomic Pathology, University of Parma, Italy.
FAU - Falchetti, A
AU  - Falchetti A
FAU - Azzoni, C
AU  - Azzoni C
FAU - D'Adda, T
AU  - D'Adda T
FAU - Canavese, G
AU  - Canavese G
FAU - Guariglia, A
AU  - Guariglia A
FAU - Santini, D
AU  - Santini D
FAU - Tomassetti, P
AU  - Tomassetti P
FAU - Brandi, M L
AU  - Brandi ML
LA  - eng
PT  - Case Reports
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PL  - United States
TA  - Am J Surg Pathol
JT  - The American journal of surgical pathology
JID - 7707904
RN  - 0 (DNA, Neoplasm)
RN  - 0 (DNA, Satellite)
SB  - IM
MH  - Adult
MH  - Alleles
MH  - Carcinoid Tumor/pathology
MH  - Chromosomes, Human, Pair 11
MH  - DNA, Neoplasm/genetics
MH  - DNA, Satellite/genetics
MH  - Disease Progression
MH  - Duodenal Neoplasms/genetics/pathology
MH  - Female
MH  - Gastrinoma/genetics/pathology
MH  - Humans
MH  - Male
MH  - Middle Aged
MH  - Multiple Endocrine Neoplasia Type 1/genetics/mortality/*pathology
MH  - Neuroendocrine Tumors/genetics/mortality/*pathology
MH  - Polymerase Chain Reaction/methods
MH  - Prognosis
MH  - Stomach Neoplasms/genetics/mortality/*pathology
EDAT- 1997/09/23 00:00
MHDA- 1997/09/23 00:01
CRDT- 1997/09/23 00:00
PHST- 1997/09/23 00:00 [pubmed]
PHST- 1997/09/23 00:01 [medline]
PHST- 1997/09/23 00:00 [entrez]
AID - 10.1097/00000478-199709000-00012 [doi]
PST - ppublish
SO  - Am J Surg Pathol. 1997 Sep;21(9):1075-82. doi: 10.1097/00000478-199709000-00012.