PDF(Pubmed)
Abstract：
UNASSIGNED: Alveolar Rhabdomyosarcoma is a profoundly malignant soft-tissue sarcoma that predominantly affects children and adolescents. However, the medical field lacks consensus regarding the optimal surgical approach to be undertaken in cases where this tumor causes local bone destruction in the upper limb.
UNASSIGNED: A 17-year-old male presented a mass in his left forearm and CT and MRI indicated that the mass had penetrated the ulnar cortex and infiltrating the medulla, resulting in the formation of an eccentric trans-ventricular tumor focus. The sizable tumor affected the volar muscles of the forearm as well as the ulnar bone marrow, exerting pressure on the ulnar artery and vein. It was confirmed by needle biopsy that the mass is alveolar rhabdomyosarcoma. Following two courses of neoadjuvant chemotherapy, the tumor was widely excised en bloc. Autologous fibula with a vascular pedicle was utilized for reconstruction during the procedure. In the postoperative follow-up, no local recurrence of the tumor was observed. Furthermore, the patient retained satisfactory wrist flexion and pronation function in the left forearm.
UNASSIGNED: Alveolar rhabdomyosarcoma is an uncommon and highly aggressive form of soft tissue sarcoma. Scientific management necessitates a multidisciplinary approach, combining chemotherapy with surgery. In cases where the tumor invaded into compartment of the bone, careful consideration should be given to the boundaries of tumor resection, the extent of osteotomy, and the approach to musculoskeletal reconstruction when designing the surgical plan. Through reporting our own case and thoroughly reviewing previous clinical experiences, we aim to provide valuable insights for the treatment of this particular disease.
UNASSIGNED: A 17-year-old male presented a mass in his left forearm and CT and MRI indicated that the mass had penetrated the ulnar cortex and infiltrating the medulla, resulting in the formation of an eccentric trans-ventricular tumor focus. The sizable tumor affected the volar muscles of the forearm as well as the ulnar bone marrow, exerting pressure on the ulnar artery and vein. It was confirmed by needle biopsy that the mass is alveolar rhabdomyosarcoma. Following two courses of neoadjuvant chemotherapy, the tumor was widely excised en bloc. Autologous fibula with a vascular pedicle was utilized for reconstruction during the procedure. In the postoperative follow-up, no local recurrence of the tumor was observed. Furthermore, the patient retained satisfactory wrist flexion and pronation function in the left forearm.
UNASSIGNED: Alveolar rhabdomyosarcoma is an uncommon and highly aggressive form of soft tissue sarcoma. Scientific management necessitates a multidisciplinary approach, combining chemotherapy with surgery. In cases where the tumor invaded into compartment of the bone, careful consideration should be given to the boundaries of tumor resection, the extent of osteotomy, and the approach to musculoskeletal reconstruction when designing the surgical plan. Through reporting our own case and thoroughly reviewing previous clinical experiences, we aim to provide valuable insights for the treatment of this particular disease.
摘要：
■肺泡横纹肌肉瘤是一种严重恶性软组织肉瘤，主要影响儿童和青少年。然而,在这种肿瘤导致上肢局部骨破坏的情况下，医学领域缺乏关于最佳手术方法的共识。
一名17岁男性左前臂有肿块，CT和MRI显示肿块穿透尺骨皮质，浸润髓质，导致形成偏心的跨心室肿瘤病灶。相当大的肿瘤影响了前臂的掌侧肌肉以及尺骨骨髓，对尺动脉和静脉施加压力。穿刺活检证实肿块为肺泡横纹肌肉瘤。经过两个疗程的新辅助化疗，肿瘤被广泛切除。在手术过程中，使用带有血管蒂的自体腓骨进行重建。在术后随访中,未观察到肿瘤局部复发.此外，患者在左前臂保留了令人满意的腕关节屈曲和内旋功能。
■肺泡横纹肌肉瘤是一种罕见且高度侵袭性的软组织肉瘤。科学管理需要多学科的方法,化疗与手术相结合。如果肿瘤侵入骨腔，应该仔细考虑肿瘤切除的边界，截骨的范围，以及设计手术计划时肌肉骨骼重建的方法。通过报告我们自己的病例并彻底回顾以前的临床经验，我们旨在为这种特殊疾病的治疗提供有价值的见解。
一名17岁男性左前臂有肿块，CT和MRI显示肿块穿透尺骨皮质，浸润髓质，导致形成偏心的跨心室肿瘤病灶。相当大的肿瘤影响了前臂的掌侧肌肉以及尺骨骨髓，对尺动脉和静脉施加压力。穿刺活检证实肿块为肺泡横纹肌肉瘤。经过两个疗程的新辅助化疗，肿瘤被广泛切除。在手术过程中，使用带有血管蒂的自体腓骨进行重建。在术后随访中,未观察到肿瘤局部复发.此外，患者在左前臂保留了令人满意的腕关节屈曲和内旋功能。
■肺泡横纹肌肉瘤是一种罕见且高度侵袭性的软组织肉瘤。科学管理需要多学科的方法,化疗与手术相结合。如果肿瘤侵入骨腔，应该仔细考虑肿瘤切除的边界，截骨的范围，以及设计手术计划时肌肉骨骼重建的方法。通过报告我们自己的病例并彻底回顾以前的临床经验，我们旨在为这种特殊疾病的治疗提供有价值的见解。
