Clinical, radiological, and pathological features of extraskeletal osteosarcoma
Clinical, radiological, and pathological features of extraskeletal osteosarcoma
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DOI：
10.1007/s00256-018-2908-6
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发表时间：
2018-09-01
影响因子：
2.1
通讯作者：
Chang，Connie Y.
中科院分区：
文献类型：
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作者：
Roller，Lauren A.;Chebib，Ivan;Chang，Connie Y.
目的评价经病理证实的骨外骨肉瘤的临床和放射学特征。在我们的病理数据库中查询了骨外骨肉瘤的病例。结果19例患者符合纳入标准(年龄59 ± 15岁(28-85岁)；男性15例，女性4例)。肿瘤发生在下肢(12/19，63%)、骨盆/臀部(3/19，16%)、上肢(2/19，5%)、胸部(1/19，5%)和颈部(1/19，5%)。19名患者中有2名(11%)以前曾接受过肿瘤部位的放射治疗。病理检查19个肿瘤中16个为高级别(84%)。肿瘤表现为软组织肿块，直径9.5 ± 6.8(2~29)cm。肿瘤矿化5例(26%)，局部侵犯1例(6%)。在MRI上，肿瘤在T2加权序列上典型地表现为高信号，15例(100%)增强扫描中有15例强化，19例中有10例(53%)有中央坏死。低级别肿瘤较小(4 cm；3/3,100%)，且缺乏中央坏死(3/3,100%)。19例患者中有8例(42%)有转移，最常见的是肺(7/19，37%)和骨(2/19，11%)。8例有转移者中2例(25%)无复发生存，11例无转移者中有8例(73%)无复发生存(平均随访3.8 ± 4.0[0.2~14.2]年)。结论骨外骨肉瘤是一种罕见的高度恶性肿瘤，常转移至肺部和骨骼。低级别和无转移的肿瘤预后较好。MRI表现无特异性，呈T2高信号，强化不均匀。与传统的骨肉瘤不同，矿化很少见。
ObjectiveTo evaluate clinical and radiological features of pathology-proven extraskeletal osteosarcomas.MethodsThis retrospective study was IRB-approved and HIPAA-compliant. Our pathology database was queried for cases of extraskeletal osteosarcoma. Tumor location, size, imaging appearance, presence of metastases, and clinical outcome were documented.ResultsNineteen patients met inclusion criteria (age 59 ± 15 (range 28–85) years; 15 male, 4 female). Tumors occurred in the lower extremities (12 out of 19, 63%), pelvis/gluteal region (3 out of 19, 16%), upper extremity (2 out of 19, 5%), thorax (1 out of 19, 5%), and neck (1 out of 19, 5%). Two out of 19 (11%) patients had undergone radiation to the tumor site previously. According to pathology, 16 out of 19 tumors were high-grade (84%). Tumors presented as soft-tissue masses measuring 9.5 ± 6.8 (2–29) cm. Tumor mineralization was present in 5 out of 19 cases (26%) and local invasion was found in 1 out of 19 cases (6%). On MRI, tumors typically appeared hyperintense on T2-weighted sequences with enhancement in 15 out of 15 (100%) contrast-enhanced studies, and with central necrosis in 10 out of 19 (53%) cases. Low-grade tumors were smaller (<4 cm; 3 out of 3, 100%) and lacked central necrosis (3 out of 3, 100%). 8 out of 19 patients (42%) had metastases, most commonly to the lung (7 out of 19, 37%) and bone (2 out of 19,11%). Two out of 8 patients (25%) with metastases and 8 out of 11 (73%) without metastases achieved recurrence-free survival (mean follow-up 3.8 ± 4.0 [0.2–14.2]) years. No metastases or deaths occurred in patients with low-grade histology.ConclusionsExtraskeletal osteosarcomas are rare, typically high-grade malignancies that commonly metastasize to lung and bones. Low-grade tumors and those without metastases have a good prognosis. MRI appearance is nonspecific, with T2 hyperintense signal and heterogeneous enhancement. Unlike conventional osteosarcoma, mineralization is rare.