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病例报告与文献综述:股骨转子区原发性骨平滑肌肉瘤

Case report and literature review: Primary leiomyosarcoma of the bone in the trochanteric region of the femur.

作者信息

Wu Zhonghan, Cheng Li, Cao Qiliang, Ye Shuming, Yu Shuisheng, Sun Min, Jing Juehua

机构信息

Department of Orthopaedics, The Second Hospital of Anhui Medical University, Hefei, China.

Institute of Orthopaedics, Research Center for Translational Medicine, The Second Hospital of Anhui Medical University, Hefei, China.

出版信息

Front Surg. 2023 Jan 10;9:1045307. doi: 10.3389/fsurg.2022.1045307. eCollection 2022.

Abstract

BACKGROUND

Primary leiomyosarcoma of the bone (LMSB) is an extremely rare, invasive, and highly destructive primary osteosarcoma with limited treatment options and poor prognosis. Only a few case reports of LMSB have been described because of its rarity. Therefore, clinicians have a limited understanding of its diagnosis, treatment, and prognosis, and the final diagnosis depends on histopathological findings. In this report, we describe a rare case of primary LMSB in the trochanteric region of the femur. Reporting this case may increase the dissemination and understanding of information regarding LMSB and provide a reference for the diagnosis and treatment of similar cases.

CASE PRESENTATION

A 63-year-old woman presented with pain and limited movement of the left hip, which had lasted for 3 months, with no history of trauma or illness. Plain radiography and computed tomography revealed a solitary osteolytic lesion in the trochanteric area of the left femur with focal cortical destruction. Magnetic resonance imaging findings suggested invasion of the lesion into the bone cortex, forming a soft tissue mass, although no distant positive findings were observed on a whole-body bone scan. A bone tumor puncture biopsy was performed to obtain a final diagnosis, and histopathological evaluation revealed left femoral intertrochanteric leiomyosarcoma, classified as G1T2M0 and staged as IB (extracompartmental low-grade malignant) according to the Enneking staging system. Thus, we performed extensive debridement and left hip arthroplasty. Postoperative chemotherapy was administered, and the patient was followed up for 4 years. Four years later, the patient's left hip pain had resolved, joint activity was good, and no signs of recurrence or distant metastasis of the bone tumor were noted.

CONCLUSION

For proximal femoral Enneking stage IB LMSB, extensive tumor resection combined with tumor prosthesis replacement may be an effective treatment method to prolong the patient's lifespan and to restore joint function.

摘要

背景

原发性骨平滑肌肉瘤(LMSB)是一种极其罕见、具有侵袭性且高度破坏性的原发性骨肉瘤,治疗选择有限且预后较差。由于其罕见性,仅有少数LMSB的病例报告。因此,临床医生对其诊断、治疗和预后的了解有限,最终诊断依赖于组织病理学检查结果。在本报告中,我们描述了一例发生于股骨转子区的罕见原发性LMSB病例。报告此病例可能会增加对LMSB信息的传播和了解,并为类似病例的诊断和治疗提供参考。

病例介绍

一名63岁女性因左髋部疼痛和活动受限就诊,症状持续3个月,无外伤或疾病史。X线平片和计算机断层扫描显示左股骨转子区有一个孤立的溶骨性病变,伴有局灶性皮质破坏。磁共振成像结果提示病变侵犯骨皮质,形成软组织肿块,尽管全身骨扫描未发现远处阳性结果。进行了骨肿瘤穿刺活检以获得最终诊断,组织病理学评估显示为左股骨转子间平滑肌肉瘤,根据Enneking分期系统分类为G1T2M0,分期为IB(间室外低度恶性)。因此,我们进行了广泛的清创和左髋关节置换术。术后给予化疗,并对患者进行了4年的随访。4年后,患者左髋部疼痛缓解,关节活动良好,未发现骨肿瘤复发或远处转移的迹象。

结论

对于股骨近端Enneking分期为IB的LMSB,广泛的肿瘤切除联合肿瘤假体置换可能是延长患者寿命和恢复关节功能的有效治疗方法。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bd3d/9872517/93e94ddd09b4/fsurg-09-1045307-g001.jpg

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