Epic battles in endocrinology — malignant pheochromocytoma: a case report

Abstract

The rarity of malignant pheochromocytoma coupled with the lack of definitive predictors of malignancy and the variability of clinical course, poses a significant diagnostic and therapeutic challenge. Since data on treatment is so scarce, case reports are a valuable source of knowledge for clinicians. This case report describes the medical history of a woman, aged 51 at the time of initial diagnosis and adrenalectomy. Within over 5 years she presented with recurrent relapse of tumour in adrenal gland bed and multiple distant metastases to descending colon, abdominal wall, postoperative scars, and the peritoneum. Neither before diagnosis nor during the whole follow-up were symptoms associated with pheochromocytoma present. The treatment administered to our patient consisted of numerous debulking surgeries along with administration of both hot and cold somatostatin analogues. We believe that debulking surgeries played a substantial role in enabling the patient to survive nearly 6 years despite aggressive clinical course of pheochromocytoma. She passed away in 2012 as a result of postlaparotomy complications. We stress the role of debulking surgery in the treatment of malignant pheochromocytoma and summarise current literature.