Giant cystic malignant pheochromocytoma invading right hepatic lobe: report on two cases

Abstract

CONTEXT: Cystic pheochromocytomas are uncommon neuroendocrine tumors that originate from the adrenal medulla. Differing from the more frequent solid pheochromocytomas, which produce catecholamines and present adrenergic syndrome, cystic pheochromocytomas may not produce these. Their symptoms are generally associated with an abdominal mass or even pain, particularly if the mass attains large dimensions. Similarly, radiological diagnosis may also be difficult. Right-side lesions may be confounded with cystic hepatic tumors or even retroperitoneal sarcomas with cystic areas, using radiological methods. Sometimes, there may be a preoperative diagnosis of malignancy. Invasion of organs in this region (i.e. liver or kidney), or even the presence of a large retroperitoneal mass (of uncertain origin) with which multiple organs are involved, may be indicative of malignant origin. CASE REPORT: Two cases of giant cystic pheochromocytoma that invaded the right hepatic lobe are described. These presented as abdominal masses. Both cases were malignant. They were treated by radical right nephrectomy plus right hepatectomy.

Malignant pheochromocytomas and paragangliomas: a diagnostic challenge

“…In the case of large cystic PCCs, many patients present without hypertension [52]. Patients with malignant PCCs may even lack clinical signs until the late stage [53].…”

Giant multicystic malignant pheochromocytoma

“…The average tumor size is approximately 7 cm (2). There are only few reports of huge multicystic malignant pheochromocytoma above 18 cm, and to our knowledge, the pheochromocytoma in this case is the largest one reported from our country (1,(3)(4)(5)(6)(7)(8). Seven giant pheochromocytomas larger than 16 cm in current literature have been documented (Table 1).…”

“…The average tumor size is approximately 7 cm, and the average weight is approximately 200 g in the previous publications (2). Sometimes these tumors may be bigger (1,(3)(4)(5)(6)(7)(8). In this report, a case of multicystic malignant pheochromocytoma with a huge size is presented, which is seldom reported in the past.…”

Undiagnosed giant cystic pheochromocytoma: A case report

“…Therefore, blocking the effects of released catecholamines are recommended for all patients with biochemically positive PCCs (14). However, in the case of silent PCCs which are not diagnosed until the tumor is further investigated, surgeons may prefer to complete the resection immediately following achieving control over the hypertension during surgery (3,7,10,15,16) compared with postponing the surgery until later (17), despite the evidence that this may result in a hypertensive crisis (3,7), ventricular ectopic rhythms and cardiac arrest (10), or acute myocardial damage as in the present report. When unexpected hypertension occurs during resection of an abdominal tumor, the anesthesiologist and surgeon must consider the diagnosis of pheochromocytoma and clearly realize that it is an anesthetic challenge.…”