Giant Malignant Cystic Pheochromocytoma: A Case Report

Abstract

Cystic malignant pheochromocytomas are uncommon. Differing from solid pheochromocytomas, which produce catecholamines and present adrenergic syndrome, cystic pheochromocytomas, may not produce these. Preoperative diagnosis may be difficult. Ct scan is useful for preoperative management. In this report, we describe a case of a giant malignant cystic pheochromocytoma in a young woman (17 years old) which presented as giant abdominal mass. The malignancy was confirmed by the presence of liver metastasis. Two years after curative resection, the patient is in good health with no recurrence.

Adrenal giant cystic pheochromocytoma treated by posterior retroperitoneoscopic adrenalectomy

“…In the general population the incidence of pheochromocytoma is about 0,005–0,1% [3], [4]. Cystic pheochromocytoma is even more rare with only a few cases reported worldwide [4], [8]. Although the cystic pheochromocytoma is a rare entity, its possibility must be considered for any adrenal mass because of the high risk of surgery on unsuspected patients.…”

“…Although the cystic pheochromocytoma is a rare entity, its possibility must be considered for any adrenal mass because of the high risk of surgery on unsuspected patients. Clinical manifestations of a cystic pheochromocytoma are varied, and it may not demonstrate the clinical (tachycardia, headache, sweating), radiological and biochemical features of a pheochromocytoma [2], [6], [7], [8]. Although abdominal pain as been reported in several case reports, most of the times cystic pheochromocytomas are asymptomatic, and yield normal plasma and urinalysis because secreted cathecolamines are metabolized within the neoplasm, with only a small amount being release into circulation [2], [3], [4], [8].…”

“…Surgery is the curative treatment for a pheochromocytoma because if left untreated it can lead to several complications like myocardial infarction, cardiac arrhythmias, heart failure, hypertensive encephalopathy, cerebrovascular accident and sudden death [2], [4]. It is very difficult to distinguish a benign pheochromocytoma from a malignant one from histopathological evaluation [4], [5], [8]. Malignancy can be indicated by radiological and intraoperative findings like invasion of adjacent organs or structures, regional adenopathy, or the presence of metastasis [5], [6], [8], [9].…”

“…It is very difficult to distinguish a benign pheochromocytoma from a malignant one from histopathological evaluation [4], [5], [8]. Malignancy can be indicated by radiological and intraoperative findings like invasion of adjacent organs or structures, regional adenopathy, or the presence of metastasis [5], [6], [8], [9]. Although malignant tumours tend to be larger, size is not an absolute criterion for malignancy [5].…”

“…Preoperative suspicion and intraoperative crisis management are the key factors for a successful treatment [6]. However the preoperative diagnosis of a cystic pheochromocytoma is very difficult because many times the clinical, biochemical and radiologic finds are not consistent with a pheochromocytoma [2], [7], [8].…”