Remission of Longstanding Insulin-Treated Diabetes Mellitus Following Surgical Resection of Pheochromocytoma

ABSTRACT Objective: To describe the case of a woman with a longstanding misdiagnosis of type 1 diabetes mellitus (T1DM), in whom diabetes remission was achieved following surgical resection of adrenal pheochromocytoma. Methods: We present a case report with accompanying biochemical, histologic, and genetic investigation results. Results: We report the case of a 57-year-old woman with a 13-year history of apparent T1DM, and her presentation with intermittent exertional nausea. An abdominal ultrasound scan identified a rounded heterogeneous mass measuring 120 × 110 × 104 mm superior to her left kidney. Plasma metanephrine levels were markedly elevated with normetaneprhine at 17,856 pmol/L (reference range is 120 to 1,180 pmol/L), metanephrine >25,000 pmol/L (reference range is 80 to 510 pmol/L), 3-methoxytyramine at 1,393 pmol/L (reference range is 0 to 180 pmol/L). The mass demonstrated marked uptake on an iodine-123-meta-iodobenzylguanidine scan. The patient's hypertension was managed with phenoxybenzamine, and she underwent adrenalectomy. Following surgery, she was able to discontinue all diabetes medications and has maintained a hemoglobin A1c value <48 mmol/mol for over 12 months. Conclusion: The case illustrates that remission of T1DM is possible even after prolonged insulin treatment upon surgical resection of pheochromocytoma. It highlights the potential benefits of reevaluating the diagnostic classification of T1DM in the light of new clinical information. Abbreviations: PCC = pheochromocytoma;T1DM = type 1 diabetes mellitus