小儿腹膜后非嗜铬性副神经节瘤6例

Retroperitoneal nonchromaffin paraganglioma: a report of 6 cases

目的 探讨腹膜后非嗜铬副神经节瘤 (副节瘤 )的诊断与治疗方法。方法 1992年 2月～ 1998年 8月收治 6例腹膜后非嗜铬副神经节瘤 ,其中 ,男 4例 ,女 2例。年龄 5～ 14岁 ,平均9.5岁。均采用手术切除治疗 ,3例取患侧 11肋间切口 ,2例 12肋缘下切口 ,1例经上腹切口。术中见肿瘤与腹主动脉、下腔静脉等粘连紧密 ,分离切除相当困难。 1例下腔静脉撕裂行吻合术。病理诊断 :良性副节瘤 5例 ,恶性副节瘤 1例。结果 所有病例均完整切除 ,肿瘤最大直径 6～ 7cm。 4例随访 1～ 4年 ,1例死于肿瘤转移。结论 手术治疗是唯一有效的治疗方法 ,但手术切除的危险性高 ,应做好充分的准备 :①备足血源 ;②做好紧急处理高血压、心跳骤停的准备 ;③做好吻合血管的准备等。

Objective To explore the diagnosis and treatment for retroperitoneal nonchromaffin paraganglioma. Methods From Feb.1992 to Aug.1998, 6 cases (male 4, female 2) with retroperitoneal nonchromaffin paraganglioma were treated. Their age ranged from 5 to 14 years (mean 9.5yrs ). The operation was performed through 11th intercostal incision (3), subcostal incision (2) and abdominal incision (1). The tumours were adherent to the abdominal aorta and inferior vena cava but resected completely except one case had a tear in inferior vena cava. Results The maximum diameter of the tumour was 6-7?cm. The pathological examination were benign in 5 and malignant in 1. The followed up of 4 cases from 1-4 years showed one died of tumor metastasis. Conclusions Surgical excision is the only therapeutic option. Adequate intravenous access, blood loss, sudden hypertension and avoid damaging adjacent main vessels are important to the resection of retroperitoneal nonchromaffin paraganglioma.