Notarnicola Angela, Moretti Lorenzo, Cocca Maria Pia, Martucci Antonio, Orsini Umberto, Moretti Biagio
Department of Clinical Methodology and Surgical Technique, Orthopaedics Section, University of Bari, Piazza G. Cesare 11, Bari, Italy.
Musculoskelet Surg. 2010 Nov;94(2):109-12. doi: 10.1007/s12306-010-0075-6. Epub 2010 Apr 8.
Low-grade fibromyxoid sarcoma (LGFMS) was first described by Evans in 1987, and since then, just over 150 cases have been reported, showing the rarity of this tumor. We report the clinical case of a 56-year-old man with a swelling on the distal third of the left thigh. The mass had grown in the course of 1 year and was surgically excised, obtaining complete resolution. Immuno-histological examination demonstrated the lesion to be a LGFMS, defined as a benign tumor but with a high incidence of local and distant recurrence (5-10%). At 2-year follow-up the patient was disease-free, confirming the efficacy of the surgical excision. In literature, the frequency of incomplete excision of LGFMS due to inappropriate diagnosis is stressed. It is this element that contributes most to the high rate of recurrence. We recommend that the eventuality of observing this rare tumor be borne in mind, and that care be taken to perform a complete, ample excision.
低度纤维黏液样肉瘤(LGFMS)于1987年由埃文斯首次描述,自那时起,仅报告了150多例病例,表明这种肿瘤很罕见。我们报告了一名56岁男性的临床病例,其左大腿远端三分之一处有肿胀。肿块在1年的时间里逐渐增大,随后通过手术切除,获得了完全缓解。免疫组织学检查显示该病变为低度纤维黏液样肉瘤,虽被定义为良性肿瘤,但局部和远处复发率较高(5%-10%)。在2年的随访中,患者无疾病复发情况,证实了手术切除的有效性。文献中强调了因诊断不当导致低度纤维黏液样肉瘤切除不完全的频率。正是这一因素导致了高复发率。我们建议应牢记观察这种罕见肿瘤的可能性,并注意进行完整、充分的切除。