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A rare presentation of myxofibrosarcoma as a Pancoast tumor: a case report

机译:罕见的黏液性原纤维肉瘤表现为胰腺癌:一例病例报告

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BackgroundMyxofibrosarcoma 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. Case presentationA 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. ConclusionsHere 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.
机译:背景粘液原纤维肉瘤是一种侵袭性软组织肿瘤,被分类为恶性纤维组织细胞瘤的变体。最常见的情况是,它发生在成年后的中晚期,在第七个十年达到高峰,涉及下肢(77%),躯干(12%)和腹膜后或纵隔(8%)。我们报告的第一例表现为Pancoast肿瘤的胸黏膜纤维肉瘤。病例介绍一名48岁非吸烟的非洲裔美国人在9个月内颈部缓慢增长,体重减轻11公斤。对他的系统进行的检查对声音嘶哑和低度间歇性发烧是积极的,没有呼吸急促或咳嗽。体格检查发现,颈部左侧的质量超过肩锁关节的3×3×1 cm。他的左眼有下垂和瞳孔缩小。他的呼吸音减弱,左顶点粗糙。神经系统检查显示他左上臂的力量为3/5。其余身体检查无异常。颈部超声显示左甲状腺叶旁有不明确的异质性肿块。胸部X线计算机断层扫描显示,左肺周围相邻神经血管结构周围有大量基于胸膜的肿块。经皮活检未确诊。随后,他接受了左胸腔穿刺术。肿物从他的前纵隔向内延伸至肺干水平,向上延伸至上沟,然后向后延伸至胸壁。手术病理证实了粘液性原纤维肉瘤的诊断。结论在此我们提出了一例以黏液性原纤维肉瘤为基础病因的Pancoast肿瘤。 Pancoast综合征通常在上沟中浸润病变,表现为上肢疼痛,手部肌肉萎缩和Horner综合征。 Pancoast综合征的鉴别诊断包括炎性和感染性病因,以及良性和恶性肿瘤。在涉及的肿瘤中,最常见的是非小细胞肺癌。文献中尚未报道表现为Pancoast肿瘤的粘液性原纤维肉瘤。

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