Costa Sergio Renato Pais, Cabral Nivaldo Marques, Abhrão Ademir Torres, Costa Ricardo Borges da, Silva Lilian Mary da, Lupinacci Renato Arioni
General Surgery and Surgical Oncology Service, Hospital do Servidor Público de São Paulo, São Paulo, Brazil.
Sao Paulo Med J. 2008 Jul;126(4):229-31. doi: 10.1590/s1516-31802008000400008.
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.
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.
囊性嗜铬细胞瘤是起源于肾上腺髓质的罕见神经内分泌肿瘤。与更常见的实性嗜铬细胞瘤不同，实性嗜铬细胞瘤产生儿茶酚胺并表现出肾上腺素能综合征，而囊性嗜铬细胞瘤可能不产生这些物质。其症状通常与腹部肿块甚至疼痛有关，特别是当肿块较大时。同样，放射学诊断也可能困难。使用放射学方法时，右侧病变可能与肝囊性肿瘤甚至伴有囊性区域的腹膜后肉瘤相混淆。有时，术前可能诊断为恶性。该区域器官的侵犯（即肝脏或肾脏），甚至存在涉及多个器官的大的腹膜后肿块（起源不明），可能提示恶性起源。
描述了两例侵犯右肝叶的巨大囊性嗜铬细胞瘤。这些病例表现为腹部肿块。两例均为恶性。均接受了根治性右肾切除术加右肝切除术。
