Background: Extra-adrenal pheochromocytoma, or paraganglioma, is a rare tumour arising from paraganglion chromaffin cells of the sympathetic nervous system. In adults, pheochromocytomas are often called the "10% tumor" because approximately 10% occur above the diaphragm, 10% of intraabdominal pheochromocytomas are extra-adrenal, 10% are bilateral, 10% are multiple, 10% are familial, 10% are malignant, and 10% recur postoperatively. In children, instead, this tumor occurs in ectopic sites in 30-40% of the cases. This paper reports the successful laparoscopic resection of an extra-adrenal pheochromocytoma, simulating an ovarian tumor, combined with a laparoscopic cholecystectomy for gallstones.
Case report: The case of a 48-year-old woman affected by an extra-adrenal pheochromocytoma that had been unsuspected for a long time is presented. The patient had some clinical symptoms that had been taken for a climacteric syndrome given her premenopausal age. The atypical and rare location of the pheochromocytoma (parauterine) had contributed to misdiagnosing it as an ovarian tumor. Laparoscopic surgery was chosen for the removal of the tumor because it is a safe technique requiring a shorter hospital stay; a concomitant cholecystectomy was performed due to the presence of gallstones.
Conclusion: Surgical resection is the only treatment option for extra-adrenal pheochromocytomas. With adequate preoperative adrenergic receptor blockers and vascular filling, the laparoscopic approach appears to be a valid alternative to open surgery for paragangliomas. Gynecologists should consider the possibility, although rare, of an extra-adrenal pheocromocytoma when preparing to surgically remove a pelvic mass.