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Year : 2022  |  Volume : 5  |  Issue : 3  |  Page : 130-131

A rare case of desmoplastic small-round-cell tumor of pleura

Department of Chest Medicine and Thoracic Endoscopy, Ruby Hall Clinic, Pune, Maharashtra, India

Date of Submission30-Jun-2022
Date of Decision22-Jan-2023
Date of Acceptance01-Feb-2023
Date of Web Publication01-Mar-2023

Correspondence Address:
Dr. Rahul Kamal Jalan
604.605, Indraprasth Business House, Behind Rasranjan, National College Road, Memnagar, Ahmedabad - 380 009, Gujarat
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/japt.japt_21_22

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How to cite this article:
Khoja AM, Jalan RK, Goud SK. A rare case of desmoplastic small-round-cell tumor of pleura. J Assoc Pulmonologist Tamilnadu 2022;5:130-1

How to cite this URL:
Khoja AM, Jalan RK, Goud SK. A rare case of desmoplastic small-round-cell tumor of pleura. J Assoc Pulmonologist Tamilnadu [serial online] 2022 [cited 2023 Mar 21];5:130-1. Available from: https://www.japt.in//text.asp?2022/5/3/130/370808


Desmoplastic small-round-cell tumor (DSRCT) is a rare and highly aggressive mesenchymal tumor that was first described in 1989 by Gerald and Rosai.[1] It predominantly affects young adolescent males and most commonly originates in the abdomen.[2] Less than 10 cases of primary DSRCT in pleura have been described.[3] DSRCT is associated with a unique chromosomal translocation t(11:22) (p13; q12) that involves the Ewing sarcoma breakpoint region 1 and Wilm tumor 1 (WT1) genes.[4] Overall, the average survival of patients with DSRCT is <2 years.[5]

Our patient, a 22-year-old male, presented with chief complaints of low-grade fever, breathlessness, dry cough, decreased appetite, and significant weight loss for the past 4 months. The patient had similar complaints 6 months ago and underwent thoracentesis, an analysis of which revealed a lymphocytic, exudative effusion with adenosine deaminase (ADA) level of 67.3 U/L, and negative for malignant cells and organisms. Tubercular etiology was considered in view of long history and pleural fluid analysis and hence antitubercular therapy (isoniazid, rifampicin, pyrazinamide, ethambutol, and pyridoxine) in appropriate doses.

Due to the recurrent nature of pleural effusion, he underwent a tube thoracostomy. The patient was referred to us for further management. On examination, he had a body mass index of 13.67 and was afebrile, the trachea was shifted to the left with a stony dull note on percussion and decreased breath sounds all over the right hemithorax with an intercostal tube in situ.

His hematologic investigations showed a hemoglobin level of 9.4 g/dL, leukocytosis of 11,800 cells/cm3, and platelet count of 4.18 × 103/L. The enzyme-linked immunosorbent assay for human immunodeficiency virus was nonreactive. Chest X ray: Pleural-based lesions with intercostal tube in situ in the right hemithorax [Figure 1]. The patient underwent video-assisted thoracoscopic surgery which revealed multiple thick adhesions and multiple nodules varying from 1 cm to the size of cricket ball with pus in the thoracic cavity [Figure 2] and [Figure 3].
Figure 1: X-ray of chest shift of trachea to left side with pleural effusion and pleural-based lesions with Intercostal tube in situ in the right hemithorax (Red Arrow: Pleural based lesions)

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Figure 2: Multiple nodules varying from 1 cm to the size of cricket ball with pus in the thoracic cavity (Black Arrow: Thoracoscopy showing multiple pleural based lesions)

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Figure 3: Multiple nodules varying from 1 cm to the size of cricket ball with pus in the thoracic cavity (black arrow: Thoracoscopy showing large pleural based mass lesion)

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Biopsy of the lesion revealed tumor composed of sheets, cords, and islands of round-to-oval cells with pale-staining nuclei and scant-to-moderate pink cytoplasm [Figure 4]. Immunohistochemistry: Tumor cells express cytokeratins, epithelial membrane antigen, Vimentin, and WT1 [Figure 5]. These characteristics were suggestive of DSRCT.
Figure 4: Tumour composed of sheets, cords, and islands of round-to-oval cells with pale staining nuclei and scant-to-moderate pink cytoplasm

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Figure 5: Tumor cells express CK, EMA, Vimentin, and WT1. CK: Cytokeratins, EMA: epithelial membrane antigen, WT1: Wilm tumor 1

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On being told about the illness and its prognosis, the patient and his family members decided against getting any chemotherapy or surgery.

In a country like India, where pleural effusion is most commonly associated with tuberculosis, it is important to rule out other causes of lymphocytic exudative pleural effusion with high ADA levels such as malignancy before initiating antituberculosis treatment. Pleuroscopy with biopsy is a gold standard tool in the diagnosis of such conditions.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Gerald WL, Rosai J. Case 2. Desmoplastic small cell tumor with divergent differentiation. Pediatr Pathol 1989;9:177-83.  Back to cited text no. 1
Chang F. Desmoplastic small round cell tumors: Cytologic, histologic, and immunohistochemical features. Arch Pathol Lab Med 2006;130:728-32.  Back to cited text no. 2
Karavitakis EM, Moschovi M, Stefanaki K, Karamolegou K, Dimitriadis E, Pandis N, et al. Desmoplastic small round cell tumor of the pleura. Pediatr Blood Cancer 2007;49:335-8.  Back to cited text no. 3
Sawyer JR, Tryka AF, Lewis JM. A novel reciprocal chromosome translocation t(11;22)(p13;q12) in an intraabdominal desmoplastic small round-cell tumor. Am J Surg Pathol 1992;16:411-6.  Back to cited text no. 4
Kempson RL, Fletcher CD, Evans HL, Henrickson MR, Sibley RS. Tumors of the Soft tissue Atlas of Tumor Pathology. 3rd Series, Fascicle 30. Washington, DC: Armed Forces Institute of Pathology; 2001. p. 452-8.  Back to cited text no. 5


  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]


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