Khamashta Natalie, Dalal Ahmad, Alashwas Mo'men, Idkedek Mayar, Abu-Akar Firas
Medical Research Club, Faculty of Medicine, Al-Quds University, Jerusalem, Palestine.
Department of Cardiothoracic Surgery, Al-Makassed Charitable Society Hospital, Jerusalem, Palestine.
Front Surg. 2022 Sep 23;9:988881. doi: 10.3389/fsurg.2022.988881. eCollection 2022.
Low-grade fibromyxoid sarcoma is an extremely rare malignant neoplasm, with an incidence of 0.18 per million, and comprises 0.6% of all soft tissue sarcomas. It has a high recurrence rate and late metastatic spread and is chemotherapy and radiotherapy insensitive. This paper reports a case of an unusually large mediastinal low-grade fibromyxoid sarcoma in a 55-year-old patient. The tumor was engulfing the main blood vessels of the mediastinum, involving the lung, and extending beyond the chest cavity to involve the cervical and axillary regions. The patient has a 21-year history of frequent surgical resections for lesions that were repeatedly misdiagnosed as neurofibroma. The tumor was successfully resected by a challenging operation that involved mediastinal mass resection, chest wall mass resection, and wedge resection of the left upper lobe of the lung. The deceivingly benign-looking histology of this tumor makes it a commonly misdiagnosed one, requiring careful assessment by pathologists to reach the right diagnosis. Surgical resection with clear margins remains the treatment of choice for these lesions. Due to the behavior of this tumor, once detected and managed, extensive long-term follow-up is always recommended.
低度恶性纤维黏液样肉瘤是一种极其罕见的恶性肿瘤，发病率为百万分之0.18，占所有软组织肉瘤的0.6%。它具有高复发率和晚期转移扩散的特点，对化疗和放疗不敏感。本文报告一例55岁患者发生的异常巨大的纵隔低度恶性纤维黏液样肉瘤。肿瘤包绕纵隔主要血管，累及肺，并延伸至胸腔外累及颈部和腋窝区域。该患者有21年病史，因病变多次被误诊为神经纤维瘤而频繁接受手术切除。通过一项具有挑战性的手术成功切除了肿瘤，该手术包括纵隔肿物切除、胸壁肿物切除以及左肺上叶楔形切除。这种肿瘤看似良性的组织学表现使其常被误诊，需要病理学家仔细评估才能做出正确诊断。手术切缘阴性的手术切除仍然是这些病变的首选治疗方法。鉴于这种肿瘤的特性，一旦发现并进行处理，总是建议进行广泛的长期随访。
