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无明显原发部位的骨与骨髓神经母细胞瘤:4例长期随访报告

Neuroblastoma of the Bone and Bone Marrow Without an Apparent Primary Site: Report of 4 Cases With Long-term Follow-up.

作者信息

Rainusso Nino, Seghers Victor, Egler Rachel, Hicks John, Russell Heidi V

机构信息

1 Division of Hematology-Oncology, Department of Pediatrics, Texas Children's Cancer & Hematology Centers, Baylor College of Medicine, Houston, Texas.

2 Department of Radiology, Texas Children's Hospital, Baylor College of Medicine, Houston, Texas.

出版信息

Pediatr Dev Pathol. 2019 Jul-Aug;22(4):329-333. doi: 10.1177/1093526618822597. Epub 2019 Jan 2.

Abstract

Children with neuroblastoma rarely present with metastatic disease without identifiable primary tumors. We describe the clinical and histopathologic characteristics of 4 patients aged 1, 7, 7, and 11 years with neuroblastoma involving bone or bone marrow without an apparent primary site. One patient presented with a periorbital bone lesion, 1 presented with a distal femoral lesion, and 2 presented with diffuse bone marrow involvement. All tumors were negative for amplification. All patients were alive without evidence of disease 5 years after completion of multimodality therapy. Patients with neuroblastoma of the bone and bone marrow without an apparent primary site may constitute a unique group characterized by older age at diagnosis, nonamplified tumors, and good response to treatment.

摘要

神经母细胞瘤患儿很少在没有可识别的原发性肿瘤的情况下出现转移性疾病。我们描述了4例年龄分别为1岁、7岁、7岁和11岁的神经母细胞瘤患者的临床和组织病理学特征,这些患者的神经母细胞瘤累及骨骼或骨髓,但没有明显的原发部位。1例患者出现眶周骨病变,1例出现股骨远端病变,2例出现弥漫性骨髓受累。所有肿瘤均未检测到 扩增。所有患者在完成多模式治疗5年后均存活,且无疾病证据。骨骼和骨髓神经母细胞瘤且无明显原发部位的患者可能构成一个独特的群体,其特征为诊断时年龄较大、肿瘤无扩增且对治疗反应良好。

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