Giraudet-Le Quintrec J S, Minoui A, Frenaux E, Anract P, Fadel E, Missenard G, Kerboull M, Dartevelle P, Kahan A
Service de Rhumatologie A, Groupe hospitalier Cochin, Paris.
Presse Med. 1998 Nov 7;27(34):1727-30.
Desmoid tumors are uncommon fibromatous tumors arising from musculoaponeurotic tissue characterized by spindle cell fibroblast and myofibroblast proliferation. The cause is unknown (trauma, hormonal factors, genetic anomaly...). Locally invasive, they tend to recur increasing morbidity or even mortality.
Stiff shoulder resulting from blockage of the scapulothoracic articulation was the inaugural sign. Three-phase bone scintigraphy demonstrated early uptake in the soft tissue in contact with the scapula. Magnetic resonance imaging and biopsy confirmed the diagnosis of desmoid tumor. Complete resection was performed. No adjuvant radiotherapy nor hormone treatment were given since no antiestrogen receptor antibodies were identified on the surgical specimen.
The clinical manifestation (stiff shoulder) and the scapular localization observed in this case are unusual for desmoid tumors. Initially tendinopathy of the shoulder, acromio-clavicular arthropathy and capsular retraction of the shoulder joint had been entertained. Early diagnosis and wide surgical resection are indicated in desmoid tumors.
硬纤维瘤是一种罕见的纤维瘤性肿瘤，起源于肌筋膜组织，其特征为梭形细胞成纤维细胞和肌成纤维细胞增殖。病因不明（创伤、激素因素、基因异常……）。具有局部侵袭性，容易复发，增加发病率甚至死亡率。
肩胛胸壁关节阻滞导致的肩部僵硬是首发症状。三相骨闪烁显像显示与肩胛骨接触的软组织早期摄取。磁共振成像和活检确诊为硬纤维瘤。进行了完整切除。由于手术标本上未发现抗雌激素受体抗体，因此未给予辅助放疗和激素治疗。
该病例中观察到的临床表现（肩部僵硬）和肩胛骨定位在硬纤维瘤中并不常见。最初考虑为肩部肌腱病、肩锁关节病和肩关节囊挛缩。硬纤维瘤需早期诊断并广泛手术切除。
