EP200 - ECE_1398 - A calm before the catecholamine storm: case of normotensive pheochromocytoma

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Abstract Background Pheochromocytomas are functional tumors that originate from the adrenal medulla and extramedullary sympathetic ganglia, arising from chromaffin cells that secrete catecholamines. It is estimated that 5%-6.5% of all adrenal incidentalomas are pheochromocytomas, which are associated with a diverse array of clinical symptoms. Notably, around 8% of these cases may involve patients who are asymptomatic, with their diagnosis occurring incidentally or prompted by discomfort due to tumor mass growth. Case Presentation An 18-year-old female patient presented at the endocrinology outpatient clinic with a complaint of recurrent pain in the left lumbar region, accompanied by episodes of vomiting over the past two months. Prior imaging at another institution, specifically an abdominal ultrasound, indicated a notable displacement of the left adrenal gland, measuring 76 mm × 50 mm × 56 mm. A subsequent CT scan of the abdomen and pelvis confirmed this finding, confirming the presence of a heterogeneous ill-defined tumor mass in the area of the right adrenal gland, which also led to a comprehensive hormonal evaluation with the same measurements. The hormonal assessment revealed significant results: VMA levels were measured at 112.7 umol/dU (7.0-68.0 umol/dU), Metanephrines at 26.2 umol/dU (<5 umol/dU), Cortisol at 897 nmol/L (55-690 nmol/L), and ACTH at 28.7 pg/mL (7.2-63.3 pg/mL). These findings suggested a diagnosis of pheochromocytoma. Management She opted to commence treatment with a minimal dose of prazosin and planned to titrate to the optimal dosage while maintaining careful monitoring to prevent hypotensive episodes. During the laparoscopic surgical procedure, several hypertensive crises were encountered and subsequently managed by the anesthesiology team. Following this, the tumor was successfully excised using a traditional surgical approach, resulting in the removal of the tumor-altered adrenal gland, which weighed 99 grams and measured 9 cm × 6 cm × 2.7 cm. Pathological and immunohistochemical analyses indicated that the tumor was positive for Chromogranin and Synaptophysin, and S100 was positive in the sustentacular cells. Post-operative evaluations of VMA and metanephrine levels were conducted to verify normalization of both cortisol and ACTH levels. Conclusions Pheochromocytoma is a rare condition that presents significant challenges regarding timely diagnosis, particularly in cases involving normotensive patients, who may remain asymptomatic for extended periods until a compressive effect manifests. Therefore, it is essential to conduct hormonal evaluations for all adrenal incidentalomas, irrespective of clinical symptoms. This approach facilitates timely diagnosis and ensures appropriate preoperative preparation for patients, thereby minimizing potential complications during surgical intervention.

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