Introduction. Pheochromocytomas are rare neuroendocrine tumors that arise from sympathetic adrenomedullary chromaffin tissue. Depending on the amount of catecholamines they secrete, they have variable presentations. There have been reported cases of adrenocorticotrophic (ACTH) secreting pheochromocytomas that present with severe Cushing syndrome. Here, we present a pheochromocytoma with adrenocorticotrophic hormone (ACTH) cosecretion, which due to its rarity and variable presentation, may be a diagnostic challenge. Presentation. A 64-year-old woman with history of colon cancer presented with new-onset diabetes, worsening hot flashes, and hypertension. On CT imaging she had an enlarging right adrenal nodule (1.7 cm) with 60 Hounsfield units of attenuation and no PET avidity. Biochemical evaluation showed elevated urinary and plasma metanephrines, elevated plasma cortisol levels despite dexamethasone suppression, elevated late-night salivary cortisol, and high-normal adrenocorticotrophic hormone. The patient underwent laproscopic right adrenalectomy, and pathology confirmed pheochromocytoma. Her lab abnormalities and symptoms of hot flashes and hypertension improved postoperatively. Conclusion. This case demonstrates an unusual ACTH-secreting pheochromocytoma with subtle presentation and highlights the importance of obtaining a complete biochemical evaluation of incidental adrenal adenomas.

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Background: Pheochromocytoma rupture is a rare endocrine emergency. There have been approximately 85 documented cases, and the mortality rate for patients who require emergent surgery is as high as 34% (1). Clinical Case: A 30-year-old man with no past medical history presented to the Emergency Department with abdominal pain and vomiting. He was hypertensive, tachycardic, and hypoxic. CT Chest Angiogram revealed bilateral pulmonary emboli and a 6.5 x 5.5 x 6.4 cm left heterogenous adrenal mass. His plasma free metanephrines were elevated to 31,173 pg/mL (n<62 pg/mL) with normetanephrines >50,000 pg/mL (n<145 pg/mL). His pulmonary emboli was treated with continuous intravenous heparin, and he was also treated with intravenous fluids and phentolamine due to concern for pheochromocytoma. Within hours, he became hypotensive, and his hemoglobin downtrended from 16.4 g/L to 7.3 g/L (n 14–18 g/L). Repeat CT showed that the adrenal mass had ruptured, enlarging to 15.4 x 9.7 x 10.0 cm. He was taken for an emergent laparotomy with piecemeal resection of the mass; surgical pathology confirmed a pheochromocytoma. Fortunately, the patient recovered well after surgery. After 6 months, his plasma free metanephrines normalized to 15 pg/mL with normetanephrines of 80 pg/mL. His DOTATATE scan was negative for malignancy and his genetic testing was negative. Conclusion: The patient’s systemic anticoagulation and critical illness likely increased his risk for pheochromocytoma rupture. Standard preoperative pheochromocytoma management, to include aggressive fluid resuscitation and alpha blockade, is important in reducing risk of morbidity and mortality. Biochemical follow up is required, as there is a 16% risk of recurrence. Reference: (1) Kobayashi T, Iwai A, Takahashi R, Ide Y, Nishizawa K, Mitsumori K. Spontaneous rupture of adrenal pheochromocytoma: Review and analysis of prognostic factors. Journal of Surgical Oncology. 2005;90(1).

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