Jevremovic Vasa, Yousuf Adnan, Hussain Zulfiqar, Abboud Amer, Chedrawy Edgar G
Department of Surgery, Weiss Memorial Hospital, Chicago, IL, USA.
Department of Oncology, Meadville Medical Center, Meadville, PA, USA.
J Med Case Rep. 2017 Mar 7;11(1):61. doi: 10.1186/s13256-017-1223-5.
Myxofibrosarcoma is an aggressive soft tissue neoplasm, classified as a variant of malignant fibrous histiocytoma. Most often, it occurs in middle to late adult life peaking in the seventh decade and involving the lower extremities (77%), trunk (12%), and retroperitoneum or mediastinum (8%). We report the first case of thoracic myxofibrosarcoma presenting as a Pancoast tumor.
A 48-year-old non-tobacco smoking African-American man presented with a slow-growing mass in his neck along with 11 kg weight loss over 9 months. A review of his systems was positive for hoarseness and lowgrade intermittent fever without any shortness of breath or cough. A physical examination revealed a mass on the left side of his neck superior to his sternoclavicular joint measuring 3 × 3 × 1 cm. He had ptosis and miosis of his left eye. His breath sounds were decreased and coarse at the left apex. A neurological examination revealed 3/5 strength in his left upper arm. The remainder of the physical examination was unremarkable. Ultrasound of his neck showed an ill-defined heterogeneous mass lateral to his left thyroid lobe. A computed tomography scan of his chest showed a large multiloculated pleural-based mass in his left lung surrounding the adjacent neurovascular structures. A percutaneous biopsy was non-diagnostic. Subsequently, he underwent a left thoracotomy with biopsy. The mass extended from his anterior mediastinum medially at the level of the pulmonary trunk, superiorly into the superior sulcus and posteriorly into his chest wall. Surgical pathology confirmed the diagnosis of myxofibrosarcoma.
Here we present a case of Pancoast tumor with myxofibrosarcoma as the underlying etiology. Pancoast syndrome generally entails an infiltrating lesion in the superior sulcus presenting with upper extremity pain, atrophy of the hand muscles, and Horner's syndrome. The differential diagnosis of Pancoast syndrome includes inflammatory and infectious etiologies, as well as neoplasms of benign and malignant nature. Of the neoplasms implicated, the most common are non-small cell lung carcinomas; myxofibrosarcoma presenting as a Pancoast tumor has not been reported in the literature.
黏液纤维肉瘤是一种侵袭性软组织肿瘤,被归类为恶性纤维组织细胞瘤的一种变体。它最常发生于成年中晚期,发病高峰在七十岁左右,且多累及下肢(77%)、躯干(12%)以及腹膜后或纵隔(8%)。我们报告首例表现为潘科斯特瘤的胸段黏液纤维肉瘤病例。
一名48岁不吸烟的非裔美国男性因颈部缓慢生长的肿块就诊,9个月内体重减轻了11千克。系统回顾显示有声音嘶哑和低热间歇性发热,无呼吸急促或咳嗽。体格检查发现其颈部左侧锁骨胸锁关节上方有一肿块,大小为3×3×1厘米。左眼有上睑下垂和瞳孔缩小。左肺尖呼吸音减弱且粗糙。神经系统检查显示左上臂肌力为3/5。其余体格检查未见异常。颈部超声显示左侧甲状腺叶外侧有一个边界不清的不均匀肿块。胸部计算机断层扫描显示左肺有一个大的多房性胸膜下肿块,包绕相邻的神经血管结构。经皮活检未明确诊断。随后,他接受了左胸开胸活检。肿块从肺动脉水平的前纵隔内侧延伸,向上进入肺尖沟,向后进入胸壁。手术病理确诊为黏液纤维肉瘤。
我们在此报告一例以黏液纤维肉瘤为潜在病因的潘科斯特瘤病例。潘科斯特综合征通常表现为肺尖沟的浸润性病变,伴有上肢疼痛、手部肌肉萎缩和霍纳综合征。潘科斯特综合征的鉴别诊断包括炎症和感染性病因,以及良性和恶性肿瘤。在相关肿瘤中,最常见的是非小细胞肺癌;文献中尚未报道过表现为潘科斯特瘤的黏液纤维肉瘤。