A rare case of functional Pancreatic Neuroendocrine tumor with multi-organ involvement in a 25-year old female.
- Author:
Jester I. Mendoza
1
;
Sarah Jean C. Bellido
2
Author Information
- Publication Type:Journal Article
- From: Journal of the Philippine Medical Association 2023;101(2):24-28
- CountryPhilippines
- Language:English
-
Abstract:
SIGNIFICANCE:Pancreatic neuroendocrine tumors (pNET) account for 1-10% of tumors arising in the oancreas, with functional pNETs reported less commonly than their non-functional counterpart. Glucagonoma is an even rarer form of functional pNET which has a reported annual incidence of 0.01-0.1 per 100,000.
CLINICAL PRESENTATION:This is a case of a 25 year old female presenting with a two-year history of palpable epigastric mass, abdominal pain and weight loss. She came in at our institution with worsened signs and symptoms in which she already had chronic diarrhea, anemia, glossitis, ad dermatitis eventually leading to development of early onset diabetes, stroke and cardiomyopathy.
MANAGEMENT:Initial contrast-enhanced CT scan revealed a pancreatic mass and was confirmed by endoscropic sonography as a large solid encapsulated mass at the head of the body of the pancreas measuring 7.7x5.5 cm. Histopathologic and immunohistochemical tests to tissue specimen obtained by fine needle aspiration biopsy of the mass revealed a well-differentiated pancreatic neuroendocrine tumor confirmed as glucagonoma by a remarkably elevated plasma glucagon level. The mass was deemed non-resectable at the time of diagnosis, hence the patient was started on octreotide LAR depot injection. Multi-systemic complications caused by the functional tumor were also managed through multidiscliplinary approach. Medical management resulted to marked improvement of the signs and symptoms and lead to a better quality of life.
RECOMMENDATION:In rare cases such as this, diagnosis is often a dilemma and causes delay in treatment. Prompt diagnosis will lead to early intervention preventing life-altering complications, disease progression, and mortality. - Full text:101(2)_4.pdf