Department of Pathology, Portuguese Institute of Oncology-Porto, Porto, Portugal.
Department of Endocrinology, Portuguese Institute of Oncology-Porto, Porto, Portugal.
Virchows Arch. 2023 Aug;483(2):267-275. doi: 10.1007/s00428-023-03492-y. Epub 2023 Jan 16.
Composite pheochromocytoma (CP) is a rare adrenal tumor, composed of ordinary pheochromocytoma and neuroblastic components. There is a paucity of information in the literature regarding this entity. We report the case of a 56-year-old woman with a CP of the left adrenal gland with a ganglioneuroma component. A review of the published literature found 110 cases of CP. The median age was 51.5 (5.86) years, and 59/110 (53.6%) were female. Association with genetic predisposition syndromes was found in 22/110 (20%), the most common of which was neurofibromatosis type 1, in 15/110 (13.6%). The most common histologic type of the neuroblastic component was ganglioneuroma in 83/110 (75.5%). Twenty-seven cases reported SDHB immunohistochemistry results; none of which was positive. Nine patients (8.2%) presented/developed metastatic disease, and 9 patients (8.2%) died from disease. To our knowledge, this is the largest review describing clinical, histopathological, molecular, and prognostic features of CP.
复合嗜铬细胞瘤（CP）是一种罕见的肾上腺肿瘤，由普通嗜铬细胞瘤和神经母细胞成分组成。关于这种肿瘤的文献资料很少。我们报告了一例 56 岁女性左肾上腺 CP 伴神经节瘤成分的病例。对已发表文献的回顾发现了 110 例 CP。中位年龄为 51.5（5.86）岁，59/110（53.6%）为女性。22/110（20%）与遗传易感性综合征有关，其中最常见的是神经纤维瘤病 1 型，15/110（13.6%）。神经母细胞成分最常见的组织学类型是神经节瘤，83/110（75.5%）。27 例报告了 SDHB 免疫组化结果；均为阴性。9 例（8.2%）患者出现/发生转移性疾病，9 例（8.2%）患者死于疾病。据我们所知，这是描述 CP 的临床、组织病理学、分子和预后特征的最大综述。
