Desmoplastic small round cell tumors of the pleura: a review of the clinical literature

Abstract

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.

One Case Report of Desmoplastic Small Round Cell Tumor of Pleura

“…4). This is similar to the previous results reported by immunohistochemistry [10]. The diagnosis of DSRCT of the pleura was eventually established with no rib invaded.…”

“…The disease most commonly involves the omentum and peritoneum, followed by the retroperitoneum [1]. Pleural DSRCT is a kind of extra-abdominal DSRCT, which is rare in the lung [2], paranasal sinuses [3], central nervous system [4], soft scalp tissue [5], salivary glands, and pleura [6]. The clinical manifestations are nonspeci c and usually related to the size and location of the tumor.…”

“…The clinical manifestations are nonspeci c and usually related to the size and location of the tumor. Among them, the translocation of T (11; 22) (P13; Q12) was regarded as its unique marker [6,7]. The disease often signi es a poor prognosis, and anticancer treatment is required, including surgery, chemotherapy, radiotherapy.…”

A series of patients with unusual lung cancers with unusual presentations

“…[10,11] Mostly arising in the abdominal and pelvic cavity, these tumours can also originate in other sites, such as the lung and pleura. [12] DSRCTs are rare and aggressive malignancies commonly affecting young males with only a few hundred cases reported in the literature. The prognosis is poor and therapy is still not well defined.…”

The Diagnosis of Pleural Tumors Other Than Mesothelioma

“…(cytokeratins, epithelial membrane antigen, MOC31, Ber EP4), muscle (perinuclear ''dot'' desmin), and neural (neuron-specific enolase, CD 57) markers. 107 WT-1 is typically positive and both myogenin and Myo-D1 are negative. There may be variable expression with CD99 and synaptophysin.…”