松果体原发性横纹肌肉瘤
Primary rhabdomyosarcoma of the pineal gland.
作者信息
Lau Steven K M, Cykowski Matthew D, Desai Shiv, Cao Ying, Fuller Gregory N, Bruner Janet, Okazaki Ian
机构信息
From the Departments of Surgery,
Department of Pathology and Genomic Medicine, Houston Methodist Hospital, Houston, TX; and Department of Pathology, The University of Texas MD Anderson Cancer Center, Houston.
出版信息
Am J Clin Pathol. 2015 May;143(5):728-33. doi: 10.1309/AJCP9ZON4ZIHODIG.
OBJECTIVES
To report a case of primary rhabdomyosarcoma (RMS) of the pineal gland in an adult, as well as review the literature on this rare entity.
METHODS
The case is compared with previous reports of similar entities, with emphasis on this patient's characteristics and clinical presentation, investigations, and management.
RESULTS
Diagnosis of primary RMS of the pineal gland was based on the presence of strap cells and multinucleated myotube-like structures, as well as tumor cell expression of skeletal muscle markers consistent with myogenic differentiation. Multimodality treatment was initiated based on pediatric protocols. Unfortunately, the disease progressed on treatment, and the patient survived only 5 months from diagnosis.
CONCLUSIONS
Pineal RMS is a rare disease with poor prognosis. Optimal management is unknown but likely to involve aggressive multimodality therapy.
目的
报告一例成人松果体原发性横纹肌肉瘤（RMS）病例，并复习关于这一罕见实体的文献。
方法
将该病例与先前类似实体的报告进行比较，重点关注该患者的特征、临床表现、检查及治疗。
结果
松果体原发性RMS的诊断基于束状细胞和多核肌管样结构的存在，以及肿瘤细胞表达与肌源性分化一致的骨骼肌标志物。基于儿科方案启动了多模式治疗。不幸的是，疾病在治疗过程中进展，患者自诊断后仅存活了5个月。
结论
松果体RMS是一种预后不良的罕见疾病。最佳治疗方案尚不清楚，但可能需要积极的多模式治疗。
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