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儿童胸腺瘤:来自单一机构的11例病例

Thymomas in childhood: 11 cases from a single institution.

作者信息

Yalçin Bilgehan, Demir Haci Ahmet, Ciftçi Arbay Ozden, Orhan Diclehan, Varan Ali, Akyüz Canan, Kutluk Tezer, Büyükpamukçu Münevver

机构信息

Department of Pediatric Oncology, Institute of Oncology, School of Medicine, Hacettepe University, Ankara, Turkey.

出版信息

J Pediatr Hematol Oncol. 2012 Nov;34(8):601-5. doi: 10.1097/MPH.0b013e31825808e9.

Abstract

AIM

Thymomas are rare in children. Our aim was to review clinical and pathologic characteristics and outcome in children with thymomas.

PATIENTS AND METHODS

Between 1979 and 2011, 11 children with thymomas were diagnosed. Hospital files were reviewed for presenting complaints, clinical, radiologic, and other laboratory data, surgical practices, chemotherapy and radiotherapy outcomes.

RESULTS

Median age was 8 years (range, 1 to 13 y). Male to female ratio was 9/2. Most common initial complaints were dyspnea, cough, chest pain, and fever. Median time from onset of symptoms was 1 month (range, 0.23 to 3 mo). Associated conditions including hyper-IgE syndrome, hypogammaglobulinemia, and systemic lupus erythematosus plus idiopathic thrombocytopenic purpura were present in 3 patients. Chest x-rays and/or thoracic computed tomographies displayed thymic hyperplasia and/or masses in anterior mediastinum accompanied by pleural (n = 2) and pericardial effusions (n = 1), pulmonary metastases (n = 1), and cervical lymph node metastasis (n = 1). Compression or invasion of trachea or vessels was documented in 5 cases. Seven cases underwent initial tumor resection; others experienced open or trucut biopsies. Histopathologically, 5 cases had invasive and 6 had benign thymomas. Benign thymomas did not receive any postoperative treatment; all cases are disease free at a median follow-up of 211 months. Three of 5 cases with invasive thymomas underwent surgery, 4/5 received chemotherapy and external radiotherapy (3600 to 4500 cGy). Two invasive thymomas died of disease. Three cases with invasive thymomas are disease free at a median follow-up of 209 months.

CONCLUSIONS

Benign thymomas have excellent prognosis. For invasive thymomas with or without metastasis, radiotherapy, and chemotherapy offers survival advantage. Complete surgical resection may increase chances for cure.

摘要

目的

胸腺瘤在儿童中较为罕见。我们的目的是回顾儿童胸腺瘤的临床和病理特征及预后情况。

患者与方法

1979年至2011年间,共诊断出11例儿童胸腺瘤。查阅医院病历,了解患者的主诉、临床、放射学及其他实验室数据、手术情况、化疗和放疗结果。

结果

中位年龄为8岁(范围1至13岁)。男女比例为9比2。最常见的初始主诉为呼吸困难、咳嗽、胸痛和发热。症状出现至诊断的中位时间为1个月(范围0.23至3个月)。3例患者伴有高IgE综合征、低丙种球蛋白血症、系统性红斑狼疮合并特发性血小板减少性紫癜等相关疾病。胸部X线和/或胸部计算机断层扫描显示胸腺增生和/或前纵隔肿块,伴有胸腔积液(n = 2)和心包积液(n = 1)、肺转移(n = 1)及颈部淋巴结转移(n = 1)。5例记录有气管或血管受压或侵犯。7例患者最初接受了肿瘤切除术;其他患者进行了开放活检或粗针活检。组织病理学检查显示,5例为侵袭性胸腺瘤,6例为良性胸腺瘤。良性胸腺瘤术后未接受任何治疗;中位随访211个月时,所有病例均无疾病复发。5例侵袭性胸腺瘤中有3例接受了手术,5例中有4例接受了化疗和外照射放疗(3600至4500 cGy)。2例侵袭性胸腺瘤患者死于疾病。3例侵袭性胸腺瘤患者在中位随访209个月时无疾病复发。

结论

良性胸腺瘤预后良好。对于有或无转移的侵袭性胸腺瘤,放疗和化疗可提高生存率。完整的手术切除可能增加治愈机会。

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