Fois Alessandro Giuseppe, Pirina Pietro, Arcadu Antonella, Becciu Francesca, Manca Sandra, Marras Viviana, Canu Sara, Castagna Gaetano, Ginesu Giorgio Carlo, Zinellu Angelo, Paliogiannis Panagiotis
Department of Clinical and Experimental Medicine, University of Sassari, Viale San Pietro 43, 07100 Sassari, Italy.
Department of Biomedical Sciences, University of Sassari, Viale San Pietro 43, 07100 Sassari, Italy.
Multidiscip Respir Med. 2017 Sep 9;12:22. doi: 10.1186/s40248-017-0103-6. eCollection 2017.
Desmoplastic small round cell tumor of the pleura is a rare malignancy, with only a few cases reported in the scientific literature. The aim of the present review is to discuss the demographic, pathological, clinical, and therapeutic features of this rare tumor. English-language articles published since 1989, when the first case of desmoplastic small round cell tumor of the pleura was described, were retrieved, and fifteen cases included in fourteen articles were revised. The mean age of the patients was 25.5 years, out of them 60% were males. Chest pain, pleural effusion, and dyspnea were the most common clinical manifestations, while chest roentgenogram and computed tomography were the imaging techniques most commonly used. Surgical biopsy was employed in 80% of the cases for diagnosis. A multidisciplinary approach consisting in a combination of surgery with chemotherapy and radiation therapy was adopted in most cases. Only two patients (13.3%) were alive at 3 years from diagnosis, reflecting the aggressiveness of the disease, and the poor outcomes of the treatments currently available. Desmoplastic small round cell tumors of the pleura are extremely aggressive and challenging to diagnose, because of their rarity and unspecific demographic, clinical, and radiological features. An in-depth knowledge of such features is necessary for the optimal management of patients with this rare malignancy.
胸膜促纤维增生性小圆细胞肿瘤是一种罕见的恶性肿瘤，科学文献中仅报道了少数病例。本综述的目的是讨论这种罕见肿瘤的人口统计学、病理学、临床和治疗特征。检索了自1989年首例胸膜促纤维增生性小圆细胞肿瘤被描述以来发表的英文文章，并对14篇文章中包含的15例病例进行了分析。患者的平均年龄为25.5岁，其中60%为男性。胸痛、胸腔积液和呼吸困难是最常见的临床表现，而胸部X线片和计算机断层扫描是最常用的影像学检查方法。80%的病例采用手术活检进行诊断。大多数病例采用手术联合化疗和放疗的多学科治疗方法。只有两名患者（13.3%）在确诊后3年存活，这反映了该疾病的侵袭性以及目前可用治疗方法的不良预后。胸膜促纤维增生性小圆细胞肿瘤极具侵袭性，诊断具有挑战性，因为其罕见性以及非特异性的人口统计学、临床和放射学特征。深入了解这些特征对于优化管理这种罕见恶性肿瘤患者至关重要。
