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Resident PGY 1
Henry Ford Hospital
Introduction: Neuroendocrine tumors (NETs) are rare endocrine neoplasms with myriad of clinical manifestations. We present a case of two different NETs in a patient to increase physician awareness and highlight the importance of prompt multidisciplinary approach to avoid catastrophic complications.
Case Presentation: A 58-year-old female with history of hypertension, diabetes mellitus and gastroesophageal reflux was referred to an oncology office for systemic therapy regarding suspected metastatic pancreatic neuroendocrine tumor based on abdominal imaging demonstrating a 1.6 cm pancreatic lesion and a 1.2 cm hypervascular right adrenal nodule. Endoscopic ultrasound with fine needle aspiration/biopsy of the pancreatic lesion was consistent with well differentiated NET WHO grade 1. Ga-68 Dotetate scan showed focal radiotracer uptake in the pancreatic tail and the right adrenal gland compatible with metastatic disease. The patient endorsed flushing, headache, gastroesophageal reflux and cough in addition to recent worsening in glycemic control, but denied any diarrhea or rashes. Vital signs were notable for elevated blood pressure to 188/90 mm of Hg. Physical exam was unremarkable. Further investigations revealed Chromogranin A 205 ng/ml, Plasma free metanephrines 552 pg/ml, a.m. cortisol 1 ug/dl following dexamethasone suppression test. Non-suppressibility in plasma free metanephrines was demonstrated with clonidine suppression test, concerning for pheochromocytoma. Multidisciplinary approach was initiated, she was placed on Doxazosin with improvement in her blood pressure. Staged surgical resection of pheochromocytoma followed by pancreatic NET management is currently planned.
Discussion: Pancreatic NETs, also known as islet cell tumors, arise in the endocrine tissues of the pancreas and may be non-functional or secrete a variety of hormones including insulin, glucagon, gastrin and vasoactive intestinal peptide. In general, these are rare neoplasms, representing one to two percent of all pancreatic tumors (Manuel-Vasquez et al., 2018). Most commonly pancreatic NETs arise sporadically, however they can be associated with genetic syndromes including multiple endocrine neoplasia type I (MEN1), von Hippel-Lindau syndrome, neurofibromatosis type I or tuberous sclerosis (Metz & Jensen, 2008). Pancreatic neuroendocrine tumors may be associated with pheochromocytomas as seen in the patient represented in our report as in patients with the above-mentioned genetic predispositions. Pheochromocytomas are rare catecholamine secreting tumor with malignant potential that arises from chromaffin cells of the adrenal medulla. The most common clinical sign of pheochromocytoma is sustained hypertension, occasionally accompanied by excessive sweating, headache & heart palpitations (Pacak et al., 2001). However, pheochromocytomas are incredible rare with the incidence being cited as 0.8 per 100,000 person years in one study (Beard et al., 1983). Biochemical confirmation of the diagnosis using total fractionated urine metadrenalines or plasma-free metadrenalines should be followed by radiological evaluation to locate the tumor (Davison et al., 2017). Treatment of pheochromocytomas involves resection of the pheochromocytoma following appropriate medical preparation with alpha-adrenoreceptor blockade, calcium channel antagonists or alpha-metyrosine (Naranjo et al., 2017). Prompt diagnosis and proper multidisciplinary management is important to avoid delay in management and potential catastrophic cardiovascular and neurologic complications. To our knowledge this is the first reported case of a pancreatic NET and pheochromocytoma in an adult patient. Discovering two different NETs dramatically changed treatment strategy and offered hope for curative resection.
Kudirka, Adam A. and Balanchivadze, Nino, "A Tale of Two NETs: A Pheochromocytoma Masquerading as a Pancreatic NET" (2020). Case Reports. 61.