Giant multicystic malignant pheochromocytoma

Abstract

Pheochromocytoma is a rare tumor originating from the embryonic neural crest and secreting high levels of catecholamines. The average tumor size is approximately 7 cm, and the average weight is approximately 200 g in the previous publications. Sometimes these tumors may be bigger. In this report, a case of multicystic malignant pheochromocytoma with a huge size is presented, which is seldom reported in the past. A 37-year-old male patient was referred to our hospital for etiological investigation of his recently diagnosed hypertension. Contrast-enhanced computed tomography (CT) examination was performed for further evaluation of the lesion and surrounding tissues. The lesion was 18×8×13 cm in size. It had lobulated margins, large cystic components, and peripheral and septal contrast enhancement. The levels of metanephrine, normetanephrine, adrenaline, noradrenaline, vanilmandelic acid, and dopamine were significantly elevated. The patient was prepared for surgery. In the pathological evaluation, the mass weighed 1018 g and was 18×8x13 cm in size. He was diagnosed with malignant pheochromocytoma. After eight months, a CT examination showed a recurrent mass, liver metastasis, and distant metastasis. The patient received chemotherapy and radiotherapy. Multicystic malignant pheochromocytoma may reach huge sizes without causing any symptoms.

Giant Malignant Pheochromocytoma: A Unique Case Report from Turkey

“…In the literature search covering summaries of all giant (>10 cm) pheochromocytomas (13), the largest tumor was reported as 45x20 cm (14). Although, previously rare cases of average size have been reported in Turkey (15), this remarkable case showcases one of the largest pheochromocytomas ever reported in Turkey.…”

Incidental giant cystic pheochromocytoma: a case report and review of the literature

“…Including both open and laparoscopic cases, a total of 33 cystic pheochromocytoma !10 cm had sufficient detail regarding perioperative outcomes for inclusion (including current case). Of all patients, 19 had a preoperative diagnosis [13][14][15][16][17][18][19][20][21][22][23][24][25][26][27] and 14 had no preoperative diagnosis. [28][29][30][31][32][33][34][35][36][37][38][39][40] A summary of characteristics of these two groups is shown in Table 3 and demonstrates a striking difference in number and severity of complications, suggesting that careful anaesthetic and surgical planning can have a positive impact on outcomes.…”

A huge asymptomatic pheochromocytoma

“…Due to their evolution in the retroperitoneal space, pheochromocytomas may grow significantly in size and remain asymptomatic for a long period of time . Moreover, contrary to solid, cystic pheochromocytomas may not present typical clinical symptomatology or urine values of catecholamine metabolites may be found within normal range .…”