SARCOMA SINOVIAL EN ADOLESCENTE. PRESENTACIÓN DE UN CASO Y REVISIÓN DE LA LITERATURA

M. Hart., Ángela María Mesa González, R. Sanchez, Luigi Romano Flores, M. M. Molina, F. Stock
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Abstract

Synovial sarcoma (SS) is a mesenchymal spindle cell malignant tumor with areas of epithelial differentiation with variable grades of dysplasia. It represents the second most common malignant neoplasia in adolescents and young adults, after rhabdomyosarcoma. SS affects primarily the extremities, especially the lower limbs. According with surgical stages, the III / IVA stage has been associated with a much more precarious prognosis. For patients with unresectable disease at diagnosis (grade III), survival rates has been reported between 50 and 70%. The objective is to present a rare clinical case and understand the behavior of the tumor. 16 year old female patient with a medial and distal left arm mass, not mobile and painful on palpation. The Ultrasound reported soft tissue heterogeneous space-occupying lesion adjacent to 1/3 of the left humerus. The biopsy reported a Primary monophasic synovial sarcoma grade I ST III. Chemotherapy and radiotherapy were indicated for treatment, with satisfactory evolution. The patient is currently asymptomatic, without evidence of tumor activity. Synovial sarcoma is a rare tumor, the pathogenesis of SS is still unknown and there are not well-established risk factors. Its treatment is multidisciplinary and complex.
青少年滑膜肉瘤。案例介绍和文献综述
滑膜肉瘤(SS)是一种间充质梭形细胞恶性肿瘤,具有上皮分化区和不同程度的不典型增生。它是青少年和年轻人中第二常见的恶性肿瘤,仅次于横纹肌肉瘤。SS主要影响四肢,尤其是下肢。根据手术分期,III / IVA期预后更不稳定。对于诊断时无法切除的患者(III级),据报道生存率在50%至70%之间。目的是提出一个罕见的临床病例,并了解肿瘤的行为。16岁女性患者,左臂内侧和远端有肿块,不能动,触诊疼痛。超声显示左肱骨1/3附近软组织异质占位性病变。活检报告原发性单相滑膜肉瘤I级ST III。经化疗、放疗治疗,进展满意。患者目前无症状,无肿瘤活动迹象。滑膜肉瘤是一种罕见的肿瘤,其发病机制尚不清楚,危险因素尚未确定。其治疗是多学科和复杂的。
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