Desmoplastic Small Round Cell Tumor. A Case Report and Literature Review
DOI:
https://doi.org/10.53903/01212095.1Keywords:
Desmoplastic small round cell tumor, Abdpminal neoplasms, Diagnostic imagingAbstract
Desmoplastic small round cell tumor (DSRCT) is a rare malignant neoplasm with an aggressive clinical course and high mortality. The case of a 21-year-old man is presented, who consulted for abdominal pain of moderate intensity radiating to the right flank, fever and weight loss. Contrast abdominal tomography was performed, documenting a large intraperitoneal mass with areas of central necrosis and extension to the pelvis, in addition to secondary neoplastic liver lesions. The diagnosis was confirmed by ultrasound-guided percutaneous biopsy, which reported extensive infiltration by malignant tumor, consisting of cells with vesicular nuclei of clear chromatin, eosinophilic cytoplasm and immunohistochemistry compatible with said tumor. This case report is compared with the findings described in other series published in the literature and a clinical review of the subject is made.
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Iyer RS, Schaunaman G, Pruthi S, Finn LS. Imaging of pediatric desmoplastic small- round-cell tumor with pathologic correlation. Curr Probl Diagn Radiol. 2013;42(1):26- 32. https://doi.org/10.1067/j.cpradiol.2012.05.004
Gerald WL, Miller HK, Battifora H, Miettinen M, Silva EG, Rosai J. Intra-abdominal desmoplastic small round-cell tumor. Report of 19 cases of a distinctive type of high- grade polyphenotypic malignancy affecting young individuals. Am J Surg Pathol. 1991;15(6):499-513. https://doi.org/10.1097/00000478-199106000-00001
Pickhardt PJ, Fisher AJ, Balfe DM, Dehner LP, Huettner PC. Desmoplastic small round cell tumor of the abdomen: Radiologic-histopathologic correlation. Radiology. 1999;210(3):633-8. https://doi.org/10.1148/radiology.210.3.r99mr42633
Bellah R, Suzuki-Bordalo L, Brecher E, Ginsberg JP, Maris J, Pawel BR. Desmoplastic small round cell tumor in the abdomen and pelvis: Report of CT findings in 11 affected children and young adults. Am J Roentgenol. 2005;184(6):1910-4. https://doi.org/10.2214/ajr.184.6.01841910
Shen X-Z, Zhao J-G, Wu J-J, Liu F. Clinical and computed tomography features of adult abdominopelvic desmoplastic small round cell tumor. World J Gastroenterol. 2014;20(17):5157. https://doi.org/10.3748/wjg.v20.i17.5157
Morani AC, Bathala TK, Surabhi VR, Yedururi S, Jensen CT, Huh WW, et al. Des- moplastic small round cell tumor: Imaging pattern of disease at presentation. Am J Roentgenol. 2019;212(3):W45-54. https://doi.org/10.2214/AJR.18.20179
Gerald WL, Ladanyi M, De Alava E, Cuatrecasas M, Kushner BH, LaQuaglia MP, et al. Clinical, pathologic, and molecular spectrum of tumors associated with t(11;22) (p13;q12): Desmoplastic small round-cell tumor and its variants. J Clin Oncol. 1998;16(9):3028-36. https://doi.org/10.1200/JCO.1998.16.9.3028
Lettieri CK, Garcia-Filion P, Hingorani P. Incidence and outcomes of desmoplastic small round cell tumor: Results from the surveillance, epidemiology, and end results database. J Cancer Epidemiol. 2014;2014. https://doi.org/10.1155/2014/680126
Kis B, O'Regan KN, Agoston A, Javery O, Jagannathan J, Ramaiya NH. Imaging of desmoplastic small round cell tumour in adults. Br J Radiol. 2012;85(1010):187-92. https://doi.org/10.1259/bjr/57186741
Chen J, Wu Z, Sun B, Li D, Wang Z, Liu F, et al. Intra-abdominal desmoplastic small round cell tumors: CT and FDG-PET/CT findings with histopathological association. Oncol Lett. 2016;11(5):3298-302. https://doi.org/10.3892/ol.2016.4421
Murosaki N, Matsumiya K, Kokado Y, Yoshioka T, Yasunaga Y, Aozasa K, et al. Retrovesical desmoplastic small round cell tumor in a patient with urinary frequency. Int J Urol. 2001;8(5):245-8. https://doi.org/10.1046/j.1442-2042.2001.00293.x
Tateishi U, Hasegawa T, Kusumoto M, Oyama T, Ishikawa H, Moriyama N. Desmo- plastic small round cell tumor: Imaging findings associated with clinicopathologic features. J Comput Assist Tomogr. 2002;26(4):579-83. https://doi.org/10.1097/00004728-200207000-00018
Sabaté JM, Torrubia S, Roson N, Matias-Guiu X, Gómez A. Intra-abdominal desmo- plastic small round-cell tumor: A rare cause of peritoneal malignancy in young people. Eur Radiol. 2000;10(5):817-9. https://doi.org/10.1007/s003300051011
Arora VC, Price AP, Fleming S, Sohn MJ, Magnan H, LaQuaglia MP, et al. Charac- teristic imaging features of desmoplastic small round cell tumour. Pediatr Radiol. 2013;43(1):93-102. https://doi.org/10.1007/s00247-012-2485-0
Sharma S, Vikram NK, Thulkar S, Goel S. Case of the season. Semin Roentgenol. 2001;36(1):3-5. https://doi.org/10.1053/sroe.2001.20460
Thomas R, Rajeswaran G, Thway K, Benson C, Shahabuddin K, Moskovic E. Des- moplastic small round cell tumour: the radiological, pathological and clinical features. Insights Imaging. 2013;4(1):111-8. https://doi.org/10.1007/s13244-012-0212-x
Zhang W, Li C, Liu Q, Hu Y, Cao Y, Huang J. CT, MRI, and FDG-PET/CT imaging findings of abdominopelvic desmoplastic small round cell tumors: Correlation with histopathologic findings. Eur J Radiol. 2011;80(2):269-73. https://doi.org/10.1016/j.ejrad.2010.06.046
Gani F, Goel U, Canner JK, Meyer CF, Johnston FM. A national analysis of patterns of care and outcomes for adults diagnosed with desmoplastic small round cell tumors in the United States. J Surg Oncol. 2019;119(7):880-6. https://doi.org/10.1002/jso.25426
Magnan H, Abramson SJ, Price AP, Grewal RK, Merchant MS, LaQuaglia MP, et al. Positron emission tomography for response assessment in desmoplastic small round cell tumor. J Pediatr Hematol Oncol. 2013;35(5). https://doi.org/10.1097/MPH.0b013e3182707d4c

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