Botev Boijo, Casale Manuele, Vincenzi Bruno, D'Ascanio Luca, Santini Daniele, Esposito Vincenzo, Di Marino Maria Pia, Baldi Alfonso, Rinaldi Vittorio, Tonini Giuseppe, Salvinelli Fabrizio
Ear, Nose and Throat Department, University Hospital Zariza Joanna, Sofia, Bulgaria.
In Vivo. 2006 Nov-Dec;20(6B):907-10.
Primary low-grade fibromyxoid sarcoma (LGFMS) is an extremely rare tumour of the salivary glands and arises from undifferentiated pluripotential mesenchymal cells. We report a case of a huge (17 x ll x 10 cm) primary LGFMS of the parotid gland of a 57-year-old woman. Clinically, a 12x8 cm firm, non-tender, left parotid mass was observed, facial nerve function was not impaired and no enlarged lymph nodules were palpated on the neck. A contrast axial CT scan showed intense enhancement of the enlarged left parotid gland. The fine needle aspiration biopsy (FNAB) was compatible with sarcomatous tissue. The tumour was removed en bloc and the facial nerve was sacrificed; surgical margins were free from disease. Microscopically, the tumour consisted of fibrous and myxoid tissue with varying grades of cellularity and gradual transitions between the two. Post-operative radiotherapy was carried out on the left neck region and parotid tumour bed. Thirty-six months after surgery the patient presents good general conditions without any evidence of local or regional disease.
The largest primary LGFMS of the parotid gland described in the literature is reported and a review of the literature on such neoplasms is also provided.
原发性低度纤维黏液样肉瘤(LGFMS)是一种极其罕见的涎腺肿瘤,起源于未分化的多能间充质细胞。我们报告一例57岁女性腮腺原发性巨大(17×11×10 cm)LGFMS病例。临床上,观察到一个12×8 cm质地坚硬、无压痛的左侧腮腺肿块,面神经功能未受损,颈部未触及肿大淋巴结。增强轴向CT扫描显示左侧腮腺肿大并强化明显。细针穿刺活检(FNAB)结果与肉瘤组织相符。肿瘤整块切除,牺牲面神经;手术切缘无肿瘤残留。显微镜下,肿瘤由纤维组织和黏液样组织组成,细胞密度不同,两者之间有逐渐过渡。术后对左侧颈部区域和腮腺肿瘤床进行了放射治疗。手术后36个月,患者一般情况良好,无局部或区域疾病迹象。
报告了文献中描述的最大的腮腺原发性LGFMS病例,并对这类肿瘤的文献进行了综述。