Simple adrenal cyst masquerading clinically silent giant cystic pheochromocytoma

Abstract

Pheochromocytoma (PCC) may present as a cystic or solid tumour. Cystic PCCs are difficult to differentiate from simple cysts in the absence of classic symptoms of PCCs. Cystic nature develops due to intralesional bleeding and necrosis. We present a case of young man without any comorbidity who was diagnosed as a simple adrenal cyst and planned for laparoscopic excision but found to be functional PCC during the time of surgery only. The patient was managed with extensive monitoring and use of multiple drugs to control blood pressure. Surgery was completed without any complication laparoscopically and postoperative period was also uneventful. Histopathology confirmed the diagnosis of cystic PCC. Our case also shows the importance of functional imaging like metaiodobenzylguanidine (MIBG) scan in doubtful cases of adrenal cysts when other biochemical markers are unremarkable to diagnosis. We emphasise the importance of meticulous preparation for any intraoperative disasters even for apparently simple adrenal cyst.

Massive Biochemically Silent Pheochromocytoma Masquerading as Nonfunctioning Adrenocortical Cancer

“…In 29 cases of biochemically silent pheochromocytomas reporting tumor characteristics in the previously mentioned systematic review, 31% had cystic changes and 38% had evidence of hemorrhage and/or necrosis [ 2 ]. It has been postulated that extensive necrosis and hemorrhagic and cystic changes may cause significantly decreased catecholamine-producing cells in pheochromocytoma tissue [ 7 ]; this may have contributed to nonsecretory pheochromocytoma in our case.…”

Case report: Significant liver atrophy due to giant cystic pheochromocytoma

“…Since serious complications such as myocardial infarction, arrhythmias, dissection aortic aneurysms, heart failure, malignant hypertension, and sudden death can be caused by pheochromocytoma, it might give rise to premature mortality if overlooked ( 10 , 11 ). In addition to this, cases of misdiagnosis of cystic pheochromocytoma such as pancreatic cystic tumor ( 12 , 13 ), hepatic cystic tumor ( 14 ), simple adrenal cyst ( 15 ), and liver abscess ( 16 ) under CT scan have also been previously documented. Hence, it is essential to investigate the characteristics of this tumor.…”

Diagnostic and therapeutic challenges in the management of silent and functional giant pheochromocytoma

“…Retroperitoneal sarcoma, cystic pancreatic neoplasm, lymphoma, and adrenal incidentaloma are typical differential diagnoses. Failure to detect them before resection may result in intraoperative hypertension, postoperative hypotension, and an increased risk of cardiac events such as arrhythmia [4].…”

“…Thorough blood pressure monitoring in the postoperative period is also recommended to minimize complications caused by hypotension episodes. 2,4,8 CONCLUSION "Clinical and biochemically," a silent large pheochromocytoma is an exceptionally unusual tumor; the conventional triad of symptoms may not be present owing to cystic degeneration of the tumor, which results in the minimal functioning of sympathetic cells with catecholamine release. In patients presenting with leftsided retroperitoneal tumor characteristics, a high index of suspicion for a giant pheochromocytoma should be maintained.…”