Spigland N, Di Lorenzo M, Youssef S, Russo P, Brandt M
Ste-Justine Hospital, Montreal, Quebec, Canada.
J Pediatr Surg. 1990 Nov;25(11):1143-6. doi: 10.1016/0022-3468(90)90750-4.
Malignant thymomas are extremely rare in children, with only 27 cases reported thus far in the pediatric surgical literature. We report four additional cases diagnosed at this institution over the past 20 years (ages 3 to 14 years). Clinical presentations included superior vena cava syndrome, cough, dyspnea, cyanosis, enlarging mediastinal mass, spontaneous pneumothorax, and pleuropericardial effusion. Three patients underwent incomplete resection of the mass or biopsy because of "unresectability" and were treated with radiotherapy and adjunctive chemotherapy. One patient underwent near complete macroscopic resection as well as radiotherapy and chemotherapy. All patients died at intervals ranging from 6 months to 2 1/2 years after diagnosis. Three patients were found to have metastatic disease prior to death or at autopsy. In one case, the initial pathological diagnosis was lymphocytic thymoma. After ultrastructural studies were performed, the diagnosis was changed to thymic T-cell lymphoma. This patient subsequently developed acute lymphoblastic leukemia 3 months after surgical resection followed by radiotherapy. Malignant thymomas are highly aggressive tumors in children. A radical surgical approach with complete excision of the tumor and contiguous structures in continuity, with adjunctive radiotherapy and chemotherapy remains the only hope for survival in children with these rare lesions.
恶性胸腺瘤在儿童中极为罕见，迄今为止儿科外科文献中仅报道了27例。我们报告了本机构在过去20年中诊断出的另外4例（年龄3至14岁）。临床表现包括上腔静脉综合征、咳嗽、呼吸困难、发绀、纵隔肿块增大、自发性气胸和胸膜心包积液。3例患者因“无法切除”而接受了肿块的不完全切除或活检，并接受了放疗和辅助化疗。1例患者接受了近乎完全的肉眼切除以及放疗和化疗。所有患者在诊断后6个月至2年半内死亡。3例患者在死亡前或尸检时发现有转移性疾病。1例患者最初的病理诊断为淋巴细胞性胸腺瘤。在进行超微结构研究后，诊断改为胸腺T细胞淋巴瘤。该患者在手术切除并放疗后3个月随后发生急性淋巴细胞白血病。恶性胸腺瘤在儿童中是高度侵袭性肿瘤。采用根治性手术方法，连续完整切除肿瘤及相邻结构，并辅以放疗和化疗，仍然是患有这些罕见病变的儿童生存的唯一希望。
