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12例原发于眼附属器的横纹肌肉瘤,男11例,女1例。最大年龄9岁,最小年龄3个月,平均4.6岁。12例均为单侧,其中眼眶9例,眼睑2例,球结膜1例。12例组织病理学分型:胚胎型8例,腺泡状型2例,多形性型2例。8例作免疫组化检查:肌红蛋白(Myoglobin)5例阳性,3例可疑阳性;结蛋白(Desmin)8例均阳性。免疫组化检验对诊断及鉴别诊断有重要意义。本组术后随访10例,除2例化疗1疗程后失访外,其余8例2例复发,3例在放疗及或化疗过程中病情不能控制而放弃治疗,2例放疗后半年因复发死亡,1例放疗后8个月健在。本组病例疗效差,除肿瘤恶性程度高外,起病年龄低,治疗不及时及治疗经验不足也是重要因素。本文尚讨论其临床病理诊断及鉴别诊断。
Twelve cases of rhabdomyosarcoma primary in the eye appendage, 11 males and 1 female. The oldest is 9 years old, the youngest is 3 months old, with an average of 4.6 years old. 12 cases were unilateral, including 9 cases of orbital, eyelid in 2 cases, 1 case of conjunctival. 12 cases of histopathological classification: embryo in 8 cases, acinar in 2 cases, pleomorphic in 2 cases. Eight cases were examined by immunohistochemistry. Myoglobin was positive in 5 cases and suspicious in 3 cases. Desmin was positive in 8 cases. Immunohistochemical test for the diagnosis and differential diagnosis of great significance. This group was followed up in 10 cases, except 2 cases of chemotherapy after a course of treatment were lost, the remaining 8 cases of 2 cases of recurrence, 3 cases of radiotherapy and chemotherapy or uncontrollable disease and give up treatment, 2 cases of radiotherapy after 6 months due to recurrence of death , One case of 8 months after radiotherapy in the health. The poor efficacy of this group of patients, in addition to the high degree of malignancy, the onset of age, treatment is not timely and lack of experience is also an important factor. This article still discusses its clinical and pathological diagnosis and differential diagnosis.