Desmoplastic Small Round Cell Tumor. A Case Report and Literature Review

Authors

  • Juan Camilo Rendón Toro Universidad Militar Nueva Granada, Bogotá, Colombia.
  • Gydnea Lourdes Aguirre Dávila Universidad Militar Nueva Granada, Bogotá, Colombia.
  • Ángela María Puerta Rico Universidad Militar Nueva Granada, Bogotá, Colombia.
  • Luis Rodrigo Arias Universidad Militar Nueva Granada, Bogotá, Colombia.
  • Daniela Arias Ramírez Fundación Valle del Lili, Cali, Valle.
  • Juan Francisco Ayala Pontificia Universidad Javeriana, Bogotá, Colombia.

DOI:

https://doi.org/10.53903/01212095.1

Keywords:

Desmoplastic small round cell tumor, Abdpminal neoplasms, Diagnostic imaging

Abstract

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.

Downloads

Download data is not yet available.

Author Biographies

Juan Camilo Rendón Toro, Universidad Militar Nueva Granada, Bogotá, Colombia.

Médico residente de Radiología, Universidad Militar Nueva Granada, Hospital Militar Central. Bogotá, Colombia

Gydnea Lourdes Aguirre Dávila, Universidad Militar Nueva Granada, Bogotá, Colombia.

Médica radióloga, Hospital Militar Central, docente titular de pregrado en Medicina y posgrado en Radiología de la Universidad Militar Nueva Granada. Bogotá, Colombia.

Ángela María Puerta Rico, Universidad Militar Nueva Granada, Bogotá, Colombia.

Médica residente de Radiología, Universidad Militar Nueva Granada, Hospital Militar Central. Bogotá, Colombia.

Luis Rodrigo Arias, Universidad Militar Nueva Granada, Bogotá, Colombia.

Médico residente de Radiología, Universidad Militar Nueva Granada, Hospital Militar Central. Bogotá, Colombia.

Daniela Arias Ramírez, Fundación Valle del Lili, Cali, Valle.

Médica residente de Medicina interna, Universidad ICESI, Fundación Valle del Lili. Cali, Valle.

Juan Francisco Ayala, Pontificia Universidad Javeriana, Bogotá, Colombia.

Médico, Pontificia Universidad Javeriana. Bogotá, Colombia.

References

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

Published

2020-12-12

How to Cite

(1)
Rendón Toro, J. C.; Aguirre Dávila, G. L.; Puerta Rico, Ángela M.; Arias, L. R.; Arias Ramírez, D.; Ayala, J. F. Desmoplastic Small Round Cell Tumor. A Case Report and Literature Review. Rev. colomb. radiol. 2020, 31, 5469-5472.

Issue

Section

Case reports
Crossref Cited-by logo
QR Code