Cushing Syndrome due to a Pancreatic Neuroendocrine Tumor Treated With Radiofrequency Ablation.
Aristea Sideri GuggerJiali FangKavel H VisrodiaGabrielle Page-WilsonPublished in: JCEM case reports (2023)
Delayed diagnosis of Cushing syndrome (CS) results in advanced disease, treatment delays, and poor outcomes. We present a patient with ectopic ACTH syndrome (EAS) from a pancreatic neuroendocrine tumor (NET) whose care posed diagnostic and therapeutic challenges. A 59-year-old female with classic Cushing stigmata, biochemical evidence of ACTH-dependent hypercortisolism, and a 5-mm pituitary lesion presented for inferior petrosal sinus sampling, which was contraindicated due to non-ST elevation myocardial infarction and acute/subacute strokes. Whole-body computed tomography (CT) scan was unrevealing, but elevations in chromogranin A and proopiomelanocortin (POMC) concentrations suggested EAS. Positron emission tomography-CT with gallium 68-DOTATATE demonstrated a 7-mm pancreatic tail lesion, suspicious for a pancreatic NET. The patient was not a surgical candidate and treatment with ketoconazole was complicated by hepatoxicity. Endoscopic ultrasound-guided biopsy and radiofrequency ablation of the lesion was pursued. Pathology confirmed ACTH immunoreactive low-grade pancreatic NET. Post procedure, sustained normalization of ACTH and cortisol was achieved. This case supports the utility of POMC measurements in the differential diagnosis of CS and the use of advanced nuclear imaging for tumor localization. For patients with functional pancreatic NET who are poor surgical candidates or intolerant of pharmacotherapy, novel endoscopic ablation may offer a low-risk therapeutic option and should be further investigated.
Keyphrases
- computed tomography
- radiofrequency ablation
- positron emission tomography
- ultrasound guided
- low grade
- case report
- dual energy
- st elevation myocardial infarction
- pet ct
- image quality
- fine needle aspiration
- magnetic resonance imaging
- percutaneous coronary intervention
- contrast enhanced
- healthcare
- high grade
- type diabetes
- minimally invasive
- pet imaging
- smoking cessation
- acute respiratory distress syndrome
- liver failure
- magnetic resonance
- atrial fibrillation
- hepatitis b virus
- photodynamic therapy
- coronary artery disease
- fluorescence imaging
- respiratory failure
- glycemic control
- high speed