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- W2912249770 abstract "Secondary causes of mast cell activation syndrome (MCAS) include multiple entities. Tryptase is a biomarker for mast cell disease. Incidence of elevated tryptase is unknown in patients with tumors of neuroectodermal origin. Here we present an adult with elevated tryptase with evidence of MCAS and a diagnosis of Pancreatic Neuroendocrine Tumor (PanNET). Serum Tryptase, Endoscopic Ultrasound(EUS) biopsy and octreotide scan A 69-year-old male was evaluated for episodes of hives, diaphoresis, hypertension and tachycardia requiring hospitalizations. Triggers included stings from wasps and fire ants. SPT was positive and subcutaneous immunotherapy was started. However, during build-up, patient reported dizziness, mind fogginess, hypertension and tachycardia with improvement after IM epinephrine. Other episodes were similar but no clear triggers were identified. Physical examinations were normal except anxious appearance. Tryptase was 27 ng/mL during symptoms, no change from baseline. These episodes occurred for a few years, but his reactions started to include flushing, abdominal cramping with diarrhea. Further investigation showed a negative bone marrow, elevated urine HIAA, 12.7mg/24 h (ref:<6 mg/24h), and elevated chromogranin A, 572 A ng/ml (ref range 25-140 ng/ml). Octreotide scan showed focal area of uptake in the tail of the pancreas consistent with MRI findings of a mass. EUS biopsy of lesion confirmed pancreatic neuroendocrine tumor. Elevated tryptase in the peripheral blood may be associated with PanNET or MCAS may be triggered by the presence of PanNET. In addition, episodes showed improvement with IM epinephrine over many years. Once the PanNET is resected and we will reassess his tryptase." @default.
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- W2912249770 date "2019-02-01" @default.
- W2912249770 modified "2023-09-27" @default.
- W2912249770 title "An Usual Presentation of Mast Cell Activation Syndrome and Pancreatic Neuroendocrine Tumor (PanNET)" @default.
- W2912249770 doi "https://doi.org/10.1016/j.jaci.2018.12.555" @default.
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