CASE 6883 Published on 26.08.2008

Pleural desmoplastic small round cell tumour

Section

Chest imaging

Case Type

Clinical Cases

Authors

Andrea Canelas, Miguel Seco, Bruno Graça, Luís Curvo-Semedo

Patient

71 years, male

Clinical History
A 71-year-old man with intense left chest pain and marked weight loss.
Imaging Findings
A 71-year-old man with progressive effort dyspnoea and easy fatigue with several years of duration, with worsening in the last 7 months. Associated findings included marked weight loss and intense left chest pain with one month of evolution.
In the emergency room he was submitted to a routine chest radiogram, which showed a nodular pleural thickening and a left pleural effusion with contralateral mediastinal shift. The effusion consisted of a sero-hematic fluid on thoracocentesis.
During the hospital stay, he was submitted to a thoracic computed tomography (CT), which revealed a widespread nodular thickening of both visceral and parietal pleural leaflets, extending into the interlobular fissure and encasing the entire lung surface. Pleural effusion and left rib destruction with a soft-tissue component were also disclosed, as well as multiple nodular lesions in the contralateral lung and several hypoattenuating nodular liver lesions.
He was then submitted to a transthoracic biopsy. Immunohistochemical studies revealed large ammounts of citoplasmatic vimentin, suggesting the diagnosis of a sarcomatous lesion, namely a desmoplastic small round cell tumour.
Discussion
Desmoplastic small round cell tumour is an extremely rare and highly aggressive neoplasm, usually affecting young males and presenting as single or multiple abdominal soft-tissue masses, growing along serosal membranes with multiple nodules attached to the peritoneal surface. Because of their dissemination along serous surfaces, the development of serous effusions is a common clinical finding. At CT, areas of low attenuation within the tumour, suggestive of haemorrhage or necrosis, are frequent. In contrast-enhanced studies, the degree of uptake by the tumour is modest, approximately equal in attenuation to that of the abdominal wall musculature. The most common symptoms include abdominal pain and symptoms of gastrointestinal obstruction. Although rare, other primary sites have been reported, such as pleura, paratesticular region, bone, soft tissues and ovary. They commonly spread to the liver, lung and bone. The cell of origin for this tumour is unclear. This neoplasm shows features of epithelial, mesenchymal and neural differentiation. A characteristic translocation between chromosome 11 and 22 has been associated with it. Effective treatment must rely upon chemotherapy and radiation therapy, since due to the diffuse involvement of the abdomen at the time of diagnosis, complete resection is rarely possible. Overall survival rates for desmoplastic small round cell tumour is poor, less than 20 percent at 5 years.
Differential Diagnosis List
Pleural desmoplastic small round cell tumour.
Final Diagnosis
Pleural desmoplastic small round cell tumour.
Case information
URL: https://www.eurorad.org/case/6883
DOI: 10.1594/EURORAD/CASE.6883
ISSN: 1563-4086