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Case Reports
. 2015 Sep 2:16:112-6.
doi: 10.1016/j.rmcr.2015.08.010. eCollection 2015.

Desmoplastic small round cell tumor of the lung: A case report and literature review

Miguel Angel Ariza-Prota  1 , Ana Pando-Sandoval  1 , David Fole-Vázquez  1 , Pere Casan  1
Affiliations
Case Reports

Desmoplastic small round cell tumor of the lung: A case report and literature review

Miguel Angel Ariza-Prota et al. Respir Med Case Rep. .

Abstract

Desmoplastic small round cell tumor (DSRCT) is a rare, aggressive and malignant tumor that is characterized by nests of small tumor cells surrounded by a cellular and vascular collagenous stroma and predominantly affects young adolescent males. This tumor most commonly originates in the abdomen; however, in rare cases, DSRCT can originate in other body regions. The main manifestations of DSRCT are chest pain and respiratory symptoms, and patients' average survival after diagnosis is less than two years. In this report, we describe a case involving DSRCT of the lung that proved to be difficult to diagnose, and we conduct a literature review.

Keywords: Adenosine deaminase, ADA; Computed tomography, CT; Desmoplastic small round cell tumor; Desmoplastic small round cell tumor, DSRCT; Enzyme-linked immunosorbent assay, ELISA; Fluorodeoxyglucose positron emission tomography/CT, FDG-PET/CT; Human immunodeficiency virus, HIV; Lactate dehydrogenase, LDH; Leukocyte common antigen, LCA; Lung cancer; Magnetic resonance imaging, MRI; Pleural effusion; Polymerase chain reaction, PCR; Primitive neuroectodermal tumor, PNET; Smooth muscle actin, SMA; Transbronchial needle aspiration, TBNA.

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Figures

Fig. 1
Fig. 1
Chest X-ray depicting a left pleural effusion.
Fig. 2
Fig. 2
CT scan depicting a large left pulmonary mass infiltrating the left pulmonary artery, left main bronchus and main carina as well as a severe pericardial effusion that is primarily localized around the left ventricle. An enlarged subcarinal lymph node is also visible.
Fig. 3
Fig. 3
Bronchoscopy revealed a whitish exophytic lesion that almost completely obstructs the left main bronchus.
Fig. 4
Fig. 4
Autopsy revealed a tumor mass with large dimensions that weighed 1750 g. The tumor occupied the entire left hemithorax; surrounded and infiltrated the left main bronchus and left great vessels; and was firmly adhered to the chest wall, diaphragm, pericardium and left atrium wall.
Fig. 5
Fig. 5
(A,B) The biopsy revealed a cellular tumor comprising of nests and groups of polygonal to spindle shaped pleomorphic cells, separated by fibrous septa, having high nuclear to cytoplasmic ratio, high mitosis and some areas of necrosis.
Fig. 6
Fig. 6
Immunohistochemistry indicated that tumor cells were positive for CK7 (A), 34B12 (B), desmin (C) and vimentin (D), confirming the diagnosis of desmoplastic small round cell tumor.
See this image and copyright information in PMC

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