1亞東紀念醫院 外科部 一般外科；2亞東紀念醫院 外科部 泌尿科
A huge cystic pheochromocytoma presenting with hypertensive crisis and treated with laparoscopic surgery :
A rare and challenging case report
Shang-Ju Hsieh2, Kuo-Hsin Chen1, Shiu- Dong Chung2, Wei-Che Wu2, Shyi-Chun Yii2
Division of General surgery1, Department of Surgery, Far Eastern Memorial Hospital, New Taipei City, Taiwan; Division of Urology2 Department of Surgery, Far Eastern Memorial Hospital, New Taipei City, Taiwan
Purpose: Pheochromocytomas (PCCs) are tumors arising from chromaffin cells of the adrenal medulla which may cause adrenergic symptoms such as paroxysmal high blood pressure, palpitation and headaches. Although PCC is usually a solid tumor, it can present as a cystic mass. Comparing with solid PCC, cystic PCC is extremely rare with only few cases reported worldwide and it can be easily confused with other intra-abdominal cystic lesions. Gold standard to treat a large cystic PCC is en-block resection via open surgery. In this article, we present a case with 12cm cystic PCC treated with laparoscopic surgery.
Materials and Methods: A 62 years old woman without systemic disease including hypertension, diabetes mellitus, heart disease before was sent to emergency department presenting with acute respiratory failure and hypertensive crisis. Acute pulmonary edema was impressed from chest plain film and abdomen computed tomography disclosed a 12*10.2cm cystic mass between liver and right kidney. Further computed tomography angiography showed engorged arterial branches on the walls of tumor arising from right adrenal arteries. Right adrenal PCC was confirmed subsequently by excessive serum and 24 hours urine catecholamine levels.
Results: Laparoscopic adrenalectomy and partial hepatectomy were performed uneventfully after resuscitation and adequate alpha blockade in cardiac intensive care unit. Histopathology finding revealed a multi-cystic, hemorrhagic tumor with focal solid component at the cystic wall. The tumor is compatible with a PCC. No tumor invasion to liver but only fibrous adhesion was confirmed in liver specimen.
Conclusions: Cystic PCCs are mostly asymptomatic and can grow into large tumors silently. Sudden excessive catecholamine released from the tumor can be life threatening and the only curative treatment for such tumor is surgical resection. Although open surgery is recommended on huge cystic PCCs to prevent tumor rupture, we showed that laparoscopic surgery is also feasible in experienced and skillful surgeon.