Department of Medicine, Section of Endocrinology, Tulane University, New Orleans, Louisiana 70112, USA.
Endocr Pract. 2010 Mar-Apr;16(2):291-9. doi: 10.4158/EP09205.RA.
To present a rare case of composite pheochromocytoma-ganglioneuroma (Pheo-GN) of the adrenal medulla, review the related literature, and discuss the clinical features, pathologic findings, behavior, and management of such tumors.
A case report of a patient with composite Pheo-GN of the adrenal gland is presented. Using the online database PUBMED, we searched and analyzed all cases of composite pheochromocytoma reported in the English-language literature during the past 70 years.
On computed tomography, a 61-year-old man was incidentally found to have a 3.8-cm nonadenomatous right adrenal lesion. Adrenalectomy revealed a 5-cm mass consistent with composite Pheo-GN. To date, 45 cases of composite pheochromocytomas have been reported during the past 70 years, 71% of which coexisted with ganglioneuromas. These tumors occurred with approximately equal frequency in male and female patients, the majority of whom were from 40 to 60 years old. Only 14 cases have been reported in the United States. Bilateral tumors were found in 3 cases. The mean size was 4 to 6 cm. Preoperatively, functional evidence was found in 76.3% of all composite pheochromocytomas (and in 67% of Pheo-GN). Only one Pheo-GN was found to have liver metastatic lesions at the time of autopsy; the rest were not aggressive.
To our knowledge, this is the first literature review describing the characteristics and behavior of all reported cases of composite pheochromocytomas, with an emphasis on those with ganglioneuromas. Composite pheochromocytoma is a rare variant of a relatively uncommon disease diagnosed by pathologists only. Fortunately, the treatment of such an entity remains the same as for any pheochromocytoma.
介绍一例罕见的肾上腺髓质嗜铬细胞瘤-神经节瘤（Pheo-GN）复合瘤病例，复习相关文献，并讨论此类肿瘤的临床特征、病理表现、行为和治疗。
报告一例肾上腺复合 Pheo-GN 患者。我们利用在线数据库 PUBMED 搜索并分析了过去 70 年英语文献中报道的所有复合嗜铬细胞瘤病例。
计算机断层扫描发现 1 例 61 岁男性右侧肾上腺 3.8cm 无腺瘤性病变。肾上腺切除术显示 5cm 肿块符合复合 Pheo-GN。迄今为止，过去 70 年共报道了 45 例复合嗜铬细胞瘤，其中 71%与神经节瘤共存。这些肿瘤在男性和女性中的发生频率大致相同，大多数患者年龄在 40 至 60 岁之间。仅在美国报道了 14 例。3 例发现双侧肿瘤。平均大小为 4 至 6cm。术前所有复合嗜铬细胞瘤中有 76.3%（Pheo-GN 中为 67%）发现功能性证据。尸检时仅发现 1 例 Pheo-GN 有肝转移病灶，其余均无侵袭性。
据我们所知，这是第一篇描述所有报道的复合嗜铬细胞瘤病例特征和行为的文献综述，重点介绍了那些伴有神经节瘤的病例。复合嗜铬细胞瘤是一种罕见的嗜铬细胞瘤变体，仅由病理学家诊断。幸运的是，此类实体的治疗与任何嗜铬细胞瘤相同。
