Desmoplastic small round cell tumor: A case report of a rare differential diagnosis of solid tumors of the pleura

Abstract

Abstract. Desmoplastic small round cell tumor (DSRCT) presents as a rare separate clinical pathological entity, and pleural DSRCT is very rare. Following review of the English literatures it was revealed that, to date, <15 cases of primary DSRCT of the pleura have been reported worldwide. Among these, there are few computed tomography (CT) findings of pleural DSRCT which have previously been described in detail. The present study reports a pathologically proven case of pleural DSRCT, with varying contrast CT findings in a 72-year-old female, which appeared as a large (12.0x10.0x6.5 cm), smooth, oval mass in the left lower thorax with slight-moderate uniform enhancement on contrast-enhanced CT. To the best of our knowledge, the present report is the first to describe the large solid-tumor pattern and the patient is the eldest reported case of pleural DSRCT.

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

“… 34 Free of disease Quarssani et al, 2011 [ 14 ] Radiation, chemotherapy 53 Alive with disease Benbrahim et al, 2012 [ 15 ] Chemotherapy 5 Alive with disease Jian et al [ 16 ] Chemotherapy 22 Dead. Cao et al [ 17 ] Surgery 32 Free of disease Won et al, 2015 [ 18 ] Chemotherapy 16 Dead Ikeue et al [ 19 ] Chemotherapy, surgery, radiation 6 Alive with disease …”

“…Both these conditions clinically manifest with chest pain, dyspnea, and pleural effusion, which represent also the most common clinical picture in patients with pleural DSRCT. Furthermore, the clinical examination of the patients is commonly unspecific, as it evidences the absence or reduction of breath sounds in the hemithorax involved or, in advanced cases, bilaterally, with or without crackles in the areas corresponding to residual inflated lungs [ 16 , 17 ]. Other clinical manifestations, like back or neck pain, arm weakness, and chest compression have been reported, but they are highly unspecific and it is extremely difficult to associate them to DSRCT, localized fibrous tumors or malignant pleural mesothelioma.…”

“…Moreover, compression of the adjacent lung tissues and enlarged mediastinal lymph nodes were often detected. In cases of advanced tumors contrast enhancement is often heterogeneous, showing low attenuation areas that correspond to myxoid alterations, hemorrhage, necrosis or cystic degeneration; these findings and the pleural effusion can be bilateral in such cases [ 6 , 17 ]. CT scans for the detection of distant metastases have been performed in most of the cases reviewed; nevertheless, distant metastases are rare, but extension along the crura of the diaphragm to the retroperitoneum has been reported [ 19 ].…”

Case Report: A pericardial effusion secondary to cardiac desmoplastic small round cell tumor

“…6,7,9 Concurrent sites of distant metastases at presentation commonly involve lymph nodes, the liver, and the lungs. [6][7][8][9][10] Few cases of primary DSRCT have been reported in the lungs and pleura, [11][12][13] with fewer reports of mediastinal cases. 6,14,15 However, primary cardiac DSRCT is highly uncommon, with only three cases published in the literature.…”

“…On the other hand, fibrous tumors present as a homogeneous mass with intermediate to high attenuation on unenhanced CT scans. [12][13][14][15][16][17][18][19][20][21][22][23][24][25][26][27][28][29] MRI has been useful in delineating the extension of the disease, including local invasion of osseous anatomical structures if surgical resection is being considered. MRI of DSRCTs often demonstrates high signal intensity on T2 weighted sequences vs. hypo-or iso-intensity relative to skeletal muscle on T1 weighted images.…”