The management of unicentric and multicentric Castleman's disease - A report of 16 cases and a review of the literature
The management of unicentric and multicentric Castleman's disease - A report of 16 cases and a review of the literature
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DOI：
10.1002/(sici)1097-0142(19990201)85:3
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发表时间：
1999-02-01
期刊：
影响因子：
6.2
通讯作者：
Brennan, MF
中科院分区：
文献类型：
--
作者：
Bowne, WB;Lewis, JJ;Brennan, MF
背景资料。Castleman病(CD)，或血管滤泡性淋巴结增生，给大多数医生带来了诊断和治疗的两难境地。对于这种罕见且知之甚少的患者，最佳治疗方案尚不清楚。作者报告了他们在1986-1997年间这种不常见的临床病理实体的经验。方法：在病理数据库中确定了16例CD的组织学诊断患者。单中心性疾病定义为孤立性肿块。多中心性疾病损害了广泛淋巴清扫的患者。分析临床、放射逻辑和实验室数据以评估治疗反应。结果：研究组由16名患者组成，分为3个临床病理组：透明血管组、浆细胞组和混合组。在那些接受了单中心性透明血管CD肿块完全手术切除的患者(n=8)中，所有患者都保持了无症状，没有临床或放射学复发。对于不能切除的单中心性透明血管CD肿块，两名患者在部分切除或放射治疗后仍无症状。两名患有多中心透明血管CD的患者目前在辅助治疗后完全缓解。单个患者存在多中心浆细胞CD。这位患者(接受了手术和系统治疗)在发病后4个月内死于疾病。3例单中心性透明-血管/浆细胞-CD患者在完全切除或观察后仍无症状。结论：作者建议对单中心性CD变异型患者进行手术切除。手术切除单中心性透明血管或透明血管/浆细胞型肿块是治愈的。部分切除、放射治疗或单独观察可以避免过度激进的治疗。患有多中心疾病的患者，无论是透明血管型还是浆细胞型，都不能从手术治疗中受益，应该是多模式治疗的候选者，其性质尚未确定。癌症1999；85：706-17，(C)1999美国癌症协会。
BACKGROUND. Castleman's disease (CD), or angiofollicular lymph node hyperplasia, creates both diagnostic and therapeutic dilemmas for most physicians. For patients with this rare and poorly understood disease, the optimal therapy is unknown. The authors report their experience during the years 1986-1997 with this uncommon clinicopathologic entity.METHODS. Sixteen patients with a histologic diagnosis of CD were identified in the pathology database. Unicentric disease was defined as a solitary mass. Multicentric disease compromised patients with widespread lymphadenectomy. Clinical, radio logic, and laboratory data were analyzed to evaluate treatment response.RESULTS. The study group consisted of 16 patients classified into 3 clinicopathologic groups: hyaline-vascular, plasma cell, and "mixed." Of those patients who underwent complete surgical excision of a unicentric hyaline-vascular CD mass (n = 8), all remain symptom free without clinical or radiographic recurrence. Two patients remain asymptomatic following partial resection or radiation therapy for an unresectable unicentric hyaline-vascular CD mass. Two patients with multicentric hyaline-vascular CD are currently in complete remission following adjuvant therapy. Multicentric plasma cell CD was present in a single patient. This patient (who underwent surgical and systemic therapy) died of disease within 4 months of presentation. Three patients with unicentric hyaline-vascular/plasma cell-CD remain symptom free following either complete resection or observation.CONCLUSIONS. The authors recommend surgical resection for patients with the unicentric variant of CD. Surgical removal of a unicentric mass of hyaline-vascular or hyaline-vascular/plasma cell type is curative. Partial resection, radiotherapy, or observation alone may avoid the need for excessively aggressive therapy. Patients with multicentric disease, either hyaline-vascular or plasma cell type, do not benefit from surgical management and should be candidates for multimodality therapy, the nature of which has yet to be defined. Cancer 1999;85:706-17, (C) 1999 American Cancer Society.