Open AccessDesmoplastic small round cell tumors of the pleura: a review of the clinical literature
Author(s) -
Alessandro Giuseppe Fois,
Pietro Pirina,
Antonella Arcadu,
Francesca Becciu,
Salvatorica Manca,
Vincenzo Marras,
Sara Canu,
Gaetano Castagna,
Giorgio Carlo Ginesu,
Angelo Zinellu,
Panagiotis Paliogiannis
Publication year - 2017
Publication title -
multidisciplinary respiratory medicine
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.72
H-Index - 28
eISSN - 2049-6958
pISSN - 1828-695X
DOI - 10.4081/mrm.2017.248
Subject(s) - medicine , malignancy , radiology , pathological , cardiothoracic surgery , biopsy , interventional radiology , radiation therapy , surgery , pathology
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.