腹膜后局限性Castleman's病的临床特点及治疗策略

Clinical characteristics and treatment of unicentric retroperitoneal Castleman's disease

  • 摘要:
      目的  探讨腹膜后局限性Castleman's病的临床特点及治疗策略,提高对该疾病的诊治水平。
      方法  回顾性分析2002年1月至2016年12月天津医科大学肿瘤医院收治的18例腹膜后局限性Castleman's病患者的临床资料,所有组织标本均经病理确诊为Castleman's病,16例为透明血管型,2例为混合型。分析其人口学特点、临床表现、病理、治疗和预后特征。
      结果  18例患者中男性4例、女性14例,平均年龄为44.1(24~70)岁。肿瘤位于肾脏1例,肾上腺区域8例,肾上腺外腹膜后9例。具有临床不典型腹痛症状7例,中度贫血患者1例。患者均行手术治疗,手术时间平均为153.3(60~260)min,术中出血量为447.2(10~3 000)mL;2例患者术后输血,1例术后出现尿瘘并发症,保守治疗后好转。中位随访时间为25个月,1例患者术后2年肿瘤复发死亡。
      结论  腹膜后局限性Castleman's病临床罕见,术前诊断困难,确诊依赖于病理检查。目前手术切除为主要治疗方法,预后较好。

     

    Abstract:
      Objective  To investigate the clinical characteristics and treatment analysis of unicentric retroperitoneal Castleman's disease (CD) and to improve its levels of diagnosis and treatment.
      Methods  The clinical data of 18 patients with unicentric retroperitoneal CD were retrospectively reviewed from January 2002 to December 2016. We analyzed the demographic characteristics, clinical features, histological diagnosis, treatment, and prognostic characteristics of these patients.
      Results  The patients comprised 4 males and 14 females with a median age of 44.1 years old (ranging from 24 years old to 70 years old). The tumor was located in the kidney in one case, while it was located in the adrenal region in eight cases. In the other cases, the tumor was in the retroperitoneal region. Seven patients had a clinical atypical abdominal pain, and one patient had moderate anemia. All patients underwent surgical resection. The mean operation time was 153.3 min with a range of 60 min to 260 min. The mean blood loss was 447.2 mL (ranging from 10 mL to 3000 mL). Two cases had blood transfusion after operation, and one had urinary fistula complication. CD was confirmed by histopathology. Hyaline vascular type of CD was observed in 16 cases, and mixed type of CD was observed in 2 cases. The median follow-up was 25 months. One patient had tumor recurrence after 2 years of operation and died. Other patients remained alive without recurrence.
      Conclusion  Unicentric retroperitoneal CD is a rare disease that is often misdiagnosed because of the absence of specific clinical manifestations. The final diagnosis depends on the results of a pathologic examination. Complete surgical resection offers a favorable result for unicentric retroperitoneal CD.

     

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