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下肢被忽视的巨大皮下平滑肌肉瘤的外生性生长。病例报告。

Exophytic growth of a neglected giant subcutaneous Leiomyosarcoma of the lower extremity. A case report.

作者信息

Angeloni Marina, Muratori Francesco, Magarelli Nicola, Chalidis Byron E, Ricci Riccardo, Rossi Barbara, Maccauro Giulio

机构信息

Orthopaedic Department, Catholic University of Rome, Italy.

出版信息

Int Semin Surg Oncol. 2008 May 21;5:11. doi: 10.1186/1477-7800-5-11.

Abstract

BACKGROUND

Superficial leiomyosarcoma is an exceedingly uncommon malignant tumor which could be located either to cutaneous or subcutaneous tissues. Increased mass size and depth, advanced tumor staging and inadequate surgical excision are the main prognostic factors for poor result.

CASE PRESENTATION

We report a rare case of a 71-year-old man with an extensive exophytic lesion (12 x 10 cm) in the anterior-medial side of the proximal right tibia. The lesion was painless and consistently neglected by the patient until a skin trauma caused ulceration of the affected area. Magnetic Resonance Imaging revealed a soft-tissue mass which was well defined from the surrounding bone and muscles. As initial biopsy in another hospital hadn't clarified the true nature of the lesion, new samples were taken and the diagnosis of leiomyosarcoma was established. Laboratory examination showed no distant metastasis and wide excision of the neoplasm was decided. After tumor resection, the remaining soft tissue and skin defect was covered with a gastrocnemius myocutaneous flap. The postoperative period was uneventful and wound healing was followed by local radiotherapy and systemic chemotherapy. At 3 years follow up, no recurrence or metastasis was identified and the patient was able to walk and stand without impairment of his ambulatory status.

CONCLUSION

Proper surgical management of soft tissue leiomyosarcoma continues to remain the cornerstone of treatment efficacy and the most important prognostic factor for patients' survival. Reconstruction of the remaining soft tissue defect should be always performed at the same operative time when removal of giant size tumors leaves an uncovered cavity with an inadequate sleeve of muscular and skin tissues.

摘要

背景

浅表性平滑肌肉瘤是一种极为罕见的恶性肿瘤,可位于皮肤或皮下组织。肿块大小增加、深度增加、肿瘤分期进展以及手术切除不充分是预后不良的主要因素。

病例报告

我们报告一例罕见病例,一名71岁男性,在右胫骨近端前内侧有一个广泛的外生性病变(12×10厘米)。该病变无痛,患者一直未在意,直到皮肤创伤导致病变部位溃疡。磁共振成像显示一个软组织肿块,与周围骨骼和肌肉界限清晰。由于另一家医院的初步活检未能明确病变的真实性质,遂重新取材并确诊为平滑肌肉瘤。实验室检查显示无远处转移,决定对肿瘤进行广泛切除。肿瘤切除后,用腓肠肌肌皮瓣覆盖剩余的软组织和皮肤缺损。术后恢复顺利,伤口愈合后进行局部放疗和全身化疗。随访3年,未发现复发或转移,患者能够行走和站立,活动状态未受影响。

结论

软组织平滑肌肉瘤的恰当手术处理仍然是治疗效果的基石,也是患者生存的最重要预后因素。当切除巨大肿瘤后留下一个肌肉和皮肤组织覆盖不足的无覆盖腔隙时,应在同一手术时间对剩余软组织缺损进行修复。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/53ff/2409359/82bbf0f95dcd/1477-7800-5-11-1.jpg

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