Service de Chirurgie Endocrinienne, Université Lille Nord, CHU, 59037 Lille Cedex, France.
World J Surg. 2012 Jun;36(6):1382-8. doi: 10.1007/s00268-012-1488-1.
Ectopic hormone-secreting pheochromocytomas are rare; only case reports exist in the literature. This condition has been linked with increased malignancy, familial syndromes, and ACTH secretion. We wanted to test these hypotheses and shed light on the nature of ectopic hormone-secreting pheochromocytomas.
This is a multicenter (francophone) observational study. Inclusion was based upon abnormal preoperative hormone tests in patients with pheochromocytoma that normalized after removal of the tumor. Where possible, immunohistochemistry was performed to confirm that ectopic secretion came from the tumor.
Sixteen cases were found: nine female and seven male patients. Median age was 50.5 (range 31-89) years. Most presented with hypertension, diabetes, or cushingoid features. Ten patients had specific symptoms from the ectopic hormone secretion. Two had a familial syndrome. Of eight patients with excess cortisol secretion, three died as a result of the tumor resection: two had pheochromocytomas >15 cm and their associated cortisol hypersecretion complicated their postoperative course. The other died from a torn subhepatic vein. The 13 survivors did not develop any evidence of malignancy during follow-up (median 50 months). Symptoms from the ectopic secretion resolved after removal of the tumor. Immunohistochemistry was performed and was positive in eight tumors: five ACTH, three calcitonins, and one VIP.
Most pheochromocytomas with ectopic secretion are neither malignant nor familial. Most ectopic hormone-secreting pheochromocytoma cause hypercortisolemia. Patients with a pheochromocytoma should be worked up for ectopic hormones, because removal of the pheochromocytoma resolves those symptoms. Associated cortisol secretion needs careful attention.
异位激素分泌性嗜铬细胞瘤罕见，文献中仅有病例报告。这种情况与恶性程度增加、家族综合征和 ACTH 分泌有关。我们希望验证这些假说，并阐明异位激素分泌性嗜铬细胞瘤的本质。
这是一项多中心（法语区）观察性研究。纳入标准为术前激素检测异常的嗜铬细胞瘤患者，肿瘤切除后激素恢复正常。在可能的情况下，进行免疫组织化学检查以确认异位分泌来自肿瘤。
共发现 16 例患者：9 名女性，7 名男性。中位年龄为 50.5 岁（范围 31-89 岁）。大多数患者表现为高血压、糖尿病或库欣样特征。10 例患者有异位激素分泌的特异性症状。2 例患者存在家族综合征。8 例皮质醇分泌过多的患者中，有 3 例因肿瘤切除而死亡：2 例肿瘤>15cm，其相关皮质醇过度分泌使术后过程复杂化。另 1 例死亡原因是肝下静脉撕裂。13 例存活患者在随访期间未发现任何恶性肿瘤证据（中位时间 50 个月）。肿瘤切除后，异位分泌引起的症状得到缓解。进行了免疫组织化学检查，8 个肿瘤呈阳性：5 个 ACTH、3 个降钙素和 1 个 VIP。
大多数有异位分泌的嗜铬细胞瘤既非恶性也非家族性。大多数异位激素分泌性嗜铬细胞瘤引起皮质醇增多症。患有嗜铬细胞瘤的患者应进行异位激素检查，因为肿瘤切除可缓解这些症状。应密切注意相关皮质醇分泌。
