PMID- 18520436
OWN - NLM
STAT- MEDLINE
DCOM- 20080717
LR  - 20151119
IS  - 1532-0979 (Electronic)
IS  - 0147-5185 (Linking)
VI  - 32
IP  - 7
DP  - 2008 Jul
TI  - Primary lymph node gastrinoma or occult duodenal microgastrinoma with lymph node 
      metastases in a MEN1 patient: the need for a systematic search for the primary 
      tumor.
PG  - 1101-5
LID - 10.1097/PAS.0b013e3181655811 [doi]
AB  - Gastrinoma tissue has been found frequently in lymph nodes located near the 
      duodenum without a known primary tumor. Therefore, it has been suggested that a 
      primary lymph node gastrinoma exists. We report on a 38-year-old woman suffering 
      from multiple endocrine neoplasia type 1 (MEN1) confirmed by menin gene mutation 
      analysis. MEN1 disease started with primary hyperparathyroidism followed by 
      Cushing disease, the detection of tumors of the pituitary, adrenal cortex, and 
      the pancreas and also an elevated serum gastrin level. An octreotide scan 
      revealed 4 tumors in the upper abdomen. A selective arterial calcium stimulation 
      test located the source of the hypergastrinemia to the area of the gastroduodenal 
      and the superior mesenteric arteries. Total pancreatoduodenectomy was performed 
      and conventional histopathologic examination revealed a well-differentiated 
      cystic neuroendocrine tumor of the pancreas expressing glucagon and accompanied 
      by several microadenomas. In addition, 3 suprapancreatic lymph nodes with 
      gastrin-positive endocrine tissue were found. None of the pancreatic 
      microadenomas expressed gastrin and no duodenal endocrine tumor was found despite 
      careful macroscopic examination. Only after complete embedding of the duodenal 
      and pancreatic tissue in 65 paraffin blocks, 2 microgastrinomas (0.45 and 0.8 mm 
      in diameter) were identified in the duodenum. It is concluded that duodenal 
      gastrinomas that give rise to lymph node metastases may be so tiny that they are 
      easily overlooked in a routine examination and that systematic tissue monitoring 
      is required to identify them.
FAU - Anlauf, Martin
AU  - Anlauf M
AD  - Department of Pathology, University of Kiel, Kiel, Germany. 
      manlauf@path.uni-kiel.de
FAU - Enosawa, Tetsuji
AU  - Enosawa T
FAU - Henopp, Tobias
AU  - Henopp T
FAU - Schmitt, Anja
AU  - Schmitt A
FAU - Gimm, Oliver
AU  - Gimm O
FAU - Brauckhoff, Michael
AU  - Brauckhoff M
FAU - Dralle, Henning
AU  - Dralle H
FAU - Musil, Anette
AU  - Musil A
FAU - Hauptmann, Steffen
AU  - Hauptmann S
FAU - Perren, Aurel
AU  - Perren A
FAU - Kloppel, Gunter
AU  - Kloppel G
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 (Biomarkers, Tumor)
RN  - 0 (MEN1 protein, human)
RN  - 0 (Proto-Oncogene Proteins)
SB  - IM
MH  - Adult
MH  - Biomarkers, Tumor/metabolism
MH  - Duodenal Neoplasms/metabolism/*secondary/surgery
MH  - Duodenum/pathology/surgery
MH  - Female
MH  - Gastrinoma/metabolism/*secondary/surgery
MH  - Humans
MH  - Lymph Nodes/*pathology
MH  - Lymphatic Metastasis
MH  - Multiple Endocrine Neoplasia Type 1/genetics/metabolism/*pathology
MH  - *Neoplasms, Unknown Primary
MH  - Pancreas/pathology/surgery
MH  - Pancreaticoduodenectomy
MH  - Proto-Oncogene Proteins/genetics/metabolism
EDAT- 2008/06/04 09:00
MHDA- 2008/07/18 09:00
CRDT- 2008/06/04 09:00
PHST- 2008/06/04 09:00 [pubmed]
PHST- 2008/07/18 09:00 [medline]
PHST- 2008/06/04 09:00 [entrez]
AID - 10.1097/PAS.0b013e3181655811 [doi]
PST - ppublish
SO  - Am J Surg Pathol. 2008 Jul;32(7):1101-5. doi: 10.1097/PAS.0b013e3181655811.