Steiner Michael A, Giles Henry W, Daley William P
Department of Diagnostic Radiology, University of Mississippi, Jackson, MS 39216, USA.
Pediatr Radiol. 2009 Apr;39(4):396-9. doi: 10.1007/s00247-008-1141-1. Epub 2009 Feb 10.
Low-grade fibromyxoid sarcoma (LGFMS) is a rare soft-tissue sarcoma that usually presents in young adults as a painless, slow-growing mass. Evans first described LGFMS in 1987 as a spindle-cell sarcoma with bland histological features and paradoxically aggressive behavior. Although young adults are most frequently affected, recent reports describe pediatric cases being increasingly more common. Males and females are affected approximately equally and common locations include the deep soft tissue of the lower extremity, particularly the thigh and trunk. Primary occurrence within the chest cavity is exceedingly rare. Local recurrence and metastasis are not uncommon and present the clinician and radiologist with challenges regarding follow-up recommendations. Review of the literature reveals many cases of slowly progressive symptoms related to a mass effect. We present a healthy 12-year-old African-American girl who interestingly developed acute symptoms of shortness of breath and chest pain while playing with her brother.
低度纤维黏液样肉瘤（LGFMS）是一种罕见的软组织肉瘤，通常在年轻人中表现为无痛、生长缓慢的肿块。埃文斯于1987年首次将LGFMS描述为一种具有温和组织学特征但行为却异常侵袭性的梭形细胞肉瘤。尽管年轻人最常受累，但最近的报告显示儿童病例越来越普遍。男性和女性受影响的几率大致相等，常见部位包括下肢深部软组织，尤其是大腿和躯干。胸腔内原发性发病极为罕见。局部复发和转移并不少见，给临床医生和放射科医生在随访建议方面带来挑战。文献回顾显示，许多病例都有与肿块效应相关的缓慢进展症状。我们报告一名健康的12岁非裔美国女孩，有趣的是，她在和哥哥玩耍时出现了急性呼吸急促和胸痛症状。
