Neurofibromatosis Type 1: A Rare Predisposition for Gastrinomas and Other Neuroendocrine Tumors.
Pancreas
; 51(5): 559-562, 2022 05 01.
Article
in En
| MEDLINE
| ID: mdl-35973017
ABSTRACT: Neurofibromatosis type (NF-1) is an autosomal dominant disorder characterized predominantly by neurocutaneous manifestations. Involvement of the gastrointestinal tract is uncommon but is associated with a significant risk of malignancy. There are a handful of case reports linking NF-1 with pancreatic neuroendocrine tumors; these include gastrin-secreting variants with the attendant Zollinger-Ellison syndrome. We present the case of a 52-year-old lady who presented with recurrent peptic ulceration and diarrhea. Serum gastrin levels were elevated and magnetic resonance imaging demonstrated the presence of a pancreatic lesion with multiple liver metastases. The lesion was moderately fludeoxyglucose avid on positron emission tomography-computed tomography. Endoscopic ultrasonography-guided sampling revealed the presence of synaptophysin positive neuroendocrine cells with positive gastrin immunostaining. A conservative approach was adopted, and the patient's symptoms improved on proton pump inhibitors. Zollinger-Ellison syndrome is an important condition, which should be kept in mind in the patient with NF-1 who presents with recurrent peptic ulceration and diarrhea. The emerging association between these 2 conditions is being examined on a cellular and immunohistochemical level.
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Pancreatic Neoplasms
/
Peptic Ulcer
/
Gastrinoma
/
Zollinger-Ellison Syndrome
/
Neurofibromatosis 1
/
Neuroendocrine Tumors
Type of study:
Diagnostic_studies
Limits:
Female
/
Humans
/
Middle aged
Language:
En
Journal:
Pancreas
Journal subject:
GASTROENTEROLOGIA
Year:
2022
Document type:
Article
Country of publication:
United States