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腹部血管滤泡性淋巴结增生症的临床病理行为及外科治疗。

The clinicopathological behaviour and surgical treatment of abdominal Castleman's disease.

机构信息

Department of General Surgery, The First Affiliated Hospital of Wenzhou Medical College, Wenzhou City 325000, Zhejiang Province, China.

出版信息

Singapore Med J. 2010 Oct;51(10):813-6.

Abstract

INTRODUCTION

Castleman's disease, a rare atypical lymphoproliferative disorder of the lymphoid tissue with unknown cause, remains a diagnostic challenge. This study was conducted to analyse the clinicopathological behaviour and reasonable surgical treatment for patients with abdominal Castleman's disease.

METHODS

The medical records of seven patients with abdominal Castleman's disease were reviewed.

RESULTS

The patients comprised two men and five women, and their median age was 42.3 (range 29-53) years. The main clinical manifestation was an abdominal mass or an enlargement of the retroperitoneal lymph node. Other clinical manifestations included anaemia, loss of body weight and hypoalbuminaemia. The mean size of the tumour was 5.5 (range 4.0-8.0) cm. Postoperatively, all the patients were diagnosed with a hyaline vascular type of the disease, and had localised manifestations of the disease. All seven patients underwent complete surgical resection, two of whom also received adjuvant irregular chemotherapy (CHOP) and steroids postoperatively. All the patients survived, with no evidence of recurrence. One patient had survived for more than two years, four patients for more than three years, and two patients for more than five years.

CONCLUSION

Abdominal Castleman's disease is difficult to diagnose preoperatively, and surgical excision remains the treatment of choice, especially for localised disease.

摘要

简介

卡斯特曼病是一种罕见的淋巴组织非典型性淋巴增生性疾病,病因不明,诊断仍具有挑战性。本研究旨在分析腹部卡斯特曼病患者的临床病理行为及合理的外科治疗方法。

方法

回顾了 7 例腹部卡斯特曼病患者的病历。

结果

患者包括 2 名男性和 5 名女性,中位年龄为 42.3(范围 29-53)岁。主要临床表现为腹部肿块或腹膜后淋巴结肿大。其他临床表现包括贫血、体重减轻和低蛋白血症。肿瘤的平均大小为 5.5(范围 4.0-8.0)cm。术后所有患者均被诊断为透明血管型疾病,且均为局限性病变。7 例患者均行完全手术切除,其中 2 例术后还接受了不规则 CHOP 化疗和类固醇辅助治疗。所有患者均存活,无复发证据。1 例患者存活超过 2 年,4 例患者存活超过 3 年,2 例患者存活超过 5 年。

结论

腹部卡斯特曼病术前难以诊断,手术切除仍然是主要治疗方法,尤其是对于局限性病变。

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