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1型神经纤维瘤病相关的肺肉瘤样癌:1例报告

Carcinosarcoma of the lung associated with neurofibromatosis type 1: a case report.

作者信息

Cıtıl Rana, Ciralik Harun, Karsligıl Ahmet, Oltulu Pembe, Yenıbertız Derya, Sayar Hamide, Gürbüz Esma

机构信息

Department of Pathology, Kahramanmaraş State Hospital, Kahramanmaraş, Turkey.

出版信息

Turk Patoloji Derg. 2012;28(1):90-4. doi: 10.5146/tjpath.2012.01106.

Abstract

Neurofibromatosis or von Recklinghausen's disease is the most common inherited syndrome predisposing to neoplasia. Carcinosarcoma is a rare malignant mixed tumor of the lung. Association of carcinosarcoma of lung with Neurofibromatosis-1 is not common. A 57-year-old man presented with history of fever, cough, hemoptysis, breathlessness, weight loss, chest pain. Multiple cutaneous neurofibromas and café au lait spots were revealed by physical examination. A homogeneous opacity was found in the right middle and right upper zone on posterior-anterior chest radiography. A 8x8x7 cm mass that had irregular borders in right upper posterior and apical segment was seen on contrast enhanced chest computed tomography. On bronchoscopy, the lumen of right upper apical segment was obstructed with vegetating tumoral lesion. The biopsy taken from this region was diagnosed as carcinosarcoma by histopathological and immunohistochemical examination.A rare case with carcinosarcoma of the lung and Neurofibromatosis-1 was reported.

摘要

神经纤维瘤病或冯·雷克林豪森病是最常见的易患肿瘤的遗传性综合征。癌肉瘤是一种罕见的肺恶性混合性肿瘤。肺癌肉瘤与神经纤维瘤病1型的关联并不常见。一名57岁男性,有发热、咳嗽、咯血、呼吸困难、体重减轻、胸痛病史。体格检查发现多处皮肤神经纤维瘤和咖啡斑。后前位胸部X线片显示右中肺野和右上肺野有均匀性密度增高影。胸部增强计算机断层扫描显示右上后段和尖段有一个8×8×7cm的肿块,边界不规则。支气管镜检查显示右上叶尖段管腔被赘生性肿瘤病变阻塞。从该区域取的活检组织经组织病理学和免疫组化检查诊断为癌肉瘤。报道了一例罕见的肺癌肉瘤合并神经纤维瘤病1型的病例。

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