PMID- 27292293
OWN - NLM
STAT- MEDLINE
DCOM- 20161213
LR  - 20190609
IS  - 1941-5923 (Electronic)
IS  - 1941-5923 (Linking)
VI  - 17
DP  - 2016 Jun 13
TI  - Zollinger-Ellison Syndrome Associated with von Recklinghausen Disease: Case 
      Report and Literature Review.
PG  - 398-405
AB  - BACKGROUND: Pancreatic endocrine tumors (PETs) are rare and can occur as part of 
      neurofibromatosis type 1 (NF1). Gastrinomas are functional PETs that are rarely 
      associated with NF1. Only two cases of their occurrence have been reported in the 
      literature. CASE REPORT: A 28-year-old woman was admitted for further evaluation 
      of epigastric soreness, heartburn, nausea, vomiting, diarrhea, and a significant 
      weight loss. Physical examination was remarkable for cutaneous findings (axillary 
      freckling and multiple cafe-au-lait spots) as well as neurofibromas (dermal, 
      plexiform). A diagnosis of NF1 was confirmed. Esophagogastroduodenoscopy (EGD) 
      revealed multiple ulcers in the duodenum and the upper jejunum. A fasting gastrin 
      level exceeded ten times the normal limit. An abdominal multi-slice 128 computed 
      tomography (CT) scan revealed an oval mass of 26 mm in diameter adjacent to the 
      second section of the duodenum. The patient was examined carefully to rule out 
      multiple endocrine neoplasia type 1 (MEN1). Surgical resection was performed and 
      a gastrinoma, causing Zollinger-Ellison syndrome (ZES), was diagnosed by 
      histological examinations of the extirpated mass. The serum gastrin level 
      decreased to normal limits shortly after surgery. Continuous follow-up revealed 
      that the symptoms and the EGD findings completely resolved without recurrences. 
      CONCLUSIONS: Although NF1 has common skeletal, visual, neurological, and 
      cardiovascular complications, it also has a rare association with duodenal or 
      pancreatic gastrinomas. Vigilance for this possible association is important to 
      promote timely and careful management to help eliminate serious and potentially 
      life-threatening complications.
FAU - Alshikho, Mohamad J
AU  - Alshikho MJ
AD  - Department of Neurology, Massachusetts General Hospital, Harvard University, 
      Boston, MA, USA.
FAU - Noureldine, Salem I
AU  - Noureldine SI
AD  - Department of Otolaryngology - Head and Neck Surgery, Johns Hopkins University, 
      Baltimore, MD, USA.
FAU - Talas, Joud M
AU  - Talas JM
AD  - Department of Dermatology, Aleppo University, Aleppo, Syrian Arab Republic.
FAU - Nasimian, Antoine
AU  - Nasimian A
AD  - Department of Radiology, Aleppo University, Aleppo, Syrian Arab Republic.
FAU - Zazou, Safi
AU  - Zazou S
AD  - Department of General Internal Medicine, Aleppo University, Aleppo, Syrian Arab 
      Republic.
FAU - Mobaed, Bashir
AU  - Mobaed B
AD  - Department of Gastroenterology, Aleppo University, Aleppo, Syrian Arab Republic.
FAU - Nasser, Mahmoud
AU  - Nasser M
AD  - Department of Gastroenterology, Aleppo University, Aleppo, Syrian Arab Republic.
LA  - eng
PT  - Case Reports
PT  - Journal Article
PT  - Review
DEP - 20160613
PL  - United States
TA  - Am J Case Rep
JT  - The American journal of case reports
JID - 101489566
SB  - IM
MH  - Adult
MH  - Female
MH  - Humans
MH  - Neurofibromatosis 1/*complications/diagnosis
MH  - Zollinger-Ellison Syndrome/*complications/diagnosis
PMC - PMC4917078
EDAT- 2016/06/14 06:00
MHDA- 2016/12/15 06:00
PMCR- 2016/06/13
CRDT- 2016/06/14 06:00
PHST- 2016/06/14 06:00 [entrez]
PHST- 2016/06/14 06:00 [pubmed]
PHST- 2016/12/15 06:00 [medline]
PHST- 2016/06/13 00:00 [pmc-release]
AID - 898472 [pii]
AID - 10.12659/ajcr.898472 [doi]
PST - epublish
SO  - Am J Case Rep. 2016 Jun 13;17:398-405. doi: 10.12659/ajcr.898472.