Pancreatic neuroendocrine tumor presenting in indolent systemic mastocytosis: A case report

Keith Sacco; Tahsin M. Khan; Monica Passi; Jonathan M. Hernandez; Hirsh Komarow

doi:10.1016/j.ijscr.2020.11.028

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Pancreatic neuroendocrine tumor presenting in indolent systemic mastocytosis: A case report

Journal article

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Pancreatic neuroendocrine tumor presenting in indolent systemic mastocytosis: A case report

Keith Sacco, Tahsin M. Khan, Monica Passi, Jonathan M. Hernandez and Hirsh Komarow

International journal of surgery case reports, Vol.77(C), pp.397-402

01/01/2020

DOI: 10.1016/j.ijscr.2020.11.028

PMCID: PMC7688994

PMID: 33220657

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Abstract

•Systemic mastocytosis and neuroendocrine tumors may present with similar symptoms.•Co-occurrence of both diseases may be due to activating mutations in KIT.•Recognition of these distinct diseases is necessary to ensure timely treatment. Systemic mastocystosis, a disorder of clonal mast cell expansion presents with symptoms of flushing, pruritus, musculoskeletal pain, gastrointestinal cramping and vascular instability. Patients with neuroendocrine tumors may present with similar symptoms due to the release of vasoactive mediators in both diseases. We report the co-occurrence of systemic mastocytosis and a neuroendocrine pancreatic tumor for which the patient received disease-specific treatment. A 58-year-old woman with a history of indolent systemic mastocytosis and a serum tryptase of 51 ng/mL was diagnosed with a solid pancreatic lesion on ultrasound when assessing for organomegaly. Lesional biopsy was consistent with a pancreatic neuroendocrine tumor which was successfully resected. Presenting symptoms such as skin rashes, flushing, fatigue and diarrhea, are similar for systemic mastocytosis and neuroendocrine tumors. The co-occurrence of both diseases has not been previously reported. Activating mutations in KIT, which are a hallmark of systemic mastocytosis, may drive neoplastic proliferation in neuroendocrine tumors. Furthermore, mast cells infiltrating pancreatic tissue may have a trophic effect on the development of pancreatic neuroendocrine tumors. While challenging to diagnose both diseases presenting with similar symptoms, recognition of these distinct diseases is necessary to ensure timely treatment.

Case report

Chromogranin A

Neuroendocrine tumor

Pancreas

Systemic mastocytosis

Tryptase

Details

Title: Subtitle: Pancreatic neuroendocrine tumor presenting in indolent systemic mastocytosis: A case report
Creators: Keith Sacco - National Institute of Allergy and Infectious Diseases
Tahsin M. Khan - National Cancer Institute
Monica Passi - National Institute of Diabetes and Digestive and Kidney Diseases
Jonathan M. Hernandez - National Cancer Institute
Hirsh Komarow - National Institute of Allergy and Infectious Diseases
Resource Type: Journal article
Publication Details: International journal of surgery case reports, Vol.77(C), pp.397-402
DOI: 10.1016/j.ijscr.2020.11.028
PMID: 33220657
PMCID: PMC7688994
NLM abbreviation: Int J Surg Case Rep
ISSN: 2210-2612
eISSN: 2210-2612
Publisher: Elsevier Ltd
Number of pages: 6
Language: English
Date published: 01/01/2020
Academic Unit: Surgery
Record Identifier: 9985224321002771

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