Zafad S, Madani A, Harif M, Quessar A, Trachli A, Benchekroun S
Service d'hématologie et d'oncologie pédiatriques, hôpital 20 Août 1953, Casablanca, Maroc.
Arch Pediatr. 2000 Oct;7(10):1077-80. doi: 10.1016/s0929-693x(00)00316-x.
Multifocal osteosarcoma is an uncommon and aggressive presentation of osteosarcoma.
We report a case of multifocal osteosarcoma in a 12-year-old boy who was admitted for a tumor of the skull, associated with pleural and pulmonary nodules. A computed tomography scan of the head showed a calcified lesion with intra- and extracranial extent. A biopsy of the skull tumor showed an osteoblastic osteosarcoma. Three weeks later, the patient developed two other tumors of the skull, a tumor of the left shoulder and a pelvic pain. Radiographic studies confirmed the presence of further lytic lesions at these sites. The patient was unresponsive to chemotherapy (high dose methotrexate and doxorubicin) and died nine months later.
Multifocal osteosarcoma, or osteosarcomatosis, is a highly aggressive form of osteosarcoma. The multiple lesions can all arise synchronously as primary tumors, or appear to be one dominant site with early and rapid appearance of secondary lesions. The incidence is reported to be between 1 and 10% of osteosarcoma. The histology shows an osteosarcoma of osteoblastic type in most cases.
Despite intensive chemotherapy, the prognosis remains poor. The debate remains as to whether or not this clinical presentation represents true multifocality of the disease or a relatively unusual metastatic pattern of osteosarcoma.
多灶性骨肉瘤是骨肉瘤一种罕见且侵袭性的表现形式。
我们报告一例12岁男孩的多灶性骨肉瘤病例,该男孩因颅骨肿瘤入院,伴有胸膜和肺结节。头部计算机断层扫描显示一个有颅内和颅外范围的钙化病变。颅骨肿瘤活检显示为成骨性骨肉瘤。三周后,患者又出现另外两处颅骨肿瘤、一处左肩肿瘤和骨盆疼痛。影像学研究证实这些部位存在更多溶骨性病变。该患者对化疗(高剂量甲氨蝶呤和阿霉素)无反应,九个月后死亡。
多灶性骨肉瘤,或骨肉瘤病,是骨肉瘤一种高度侵袭性的形式。多个病变可全部作为原发性肿瘤同时出现,或表现为一个主要部位伴继发性病变早期快速出现。据报道,其发病率占骨肉瘤的1%至10%。大多数情况下,组织学显示为成骨型骨肉瘤。
尽管进行了强化化疗,预后仍然很差。关于这种临床表现是代表疾病真正的多灶性还是骨肉瘤相对不寻常的转移模式,仍存在争议。