Adelani Muyibat A, Schultenover Stephen J, Holt Ginger E, Cates Justin M M
Department of Orthopaedic Surgery, Washington University in St Louis, St Louis, Missouri, USA.
Arch Pathol Lab Med. 2009 Sep;133(9):1448-56. doi: 10.5858/133.9.1448.
Leiomyosarcoma most commonly involves the female genital tract and occasionally arises within the retroperitoneum, abdominal cavity, or the soft tissues of the extremity. Presentation as a primary bone tumor is extraordinarily uncommon, potentially leading to misdiagnosis. The prognosis is traditionally thought to be dismal. However, this conclusion is largely based on individual case reports and small series, in some of which the pathologic diagnosis is not well documented.
To review the clinicopathologic features of well-established cases of primary skeletal leiomyosarcoma and reevaluate the prognostic implications thereof.
A National Center for Biotechnology Information PubMed search of the English language literature identified 104 authenticated cases of primary leiomyosarcoma of extragnathic bone. An additional 3 cases are reported and illustrated herein.
Approximately half of all patients with primary skeletal leiomyosarcoma either presented with metastatic disease or developed metastases within 1 year of diagnosis. The 5-year overall and disease-free survival rates were 59% and 41%, respectively, comparable to that of other skeletal sarcomas. As for other bone and soft tissue sarcomas, high histologic grade and tumor stage are predictive of poor outcome.
平滑肌肉瘤最常累及女性生殖道，偶尔发生于腹膜后、腹腔或四肢软组织。表现为原发性骨肿瘤极为罕见，可能导致误诊。传统上认为其预后不佳。然而，这一结论主要基于个别病例报告和小样本系列研究，其中一些病例的病理诊断记录并不完善。
回顾已确诊的原发性骨平滑肌肉瘤病例的临床病理特征，并重新评估其预后意义。
美国国立生物技术信息中心的PubMed数据库检索英文文献，共识别出104例经证实的颌骨外原发性平滑肌肉瘤病例。本文另报告并展示了3例病例。
所有原发性骨平滑肌肉瘤患者中，约半数在诊断时已出现转移性疾病或在诊断后1年内发生转移。5年总生存率和无病生存率分别为59%和41%，与其他骨肉瘤相当。与其他骨和软组织肉瘤一样，高组织学分级和肿瘤分期预示预后不良。
