Paratesticular/testicular ​desmoplastic round small cell malignant tumor: case report and literature review.

Authors

  • Handerson Rafael Osma Charris Instituto Nacional de Cancerología
  • Fernando Contreras Mejía Instituto Nacional de Cancerología
  • Camilo Vallejo Yepes Instituto Nacional de Cancerología
  • Carolina Bautista Saiz Fundación Universitaria Ciencias de la Salud (FUCS).
  • Vilma Medina Boada Instituto Nacional de Cancerología
  • Tatiana Roncancio Velandia Instituto Nacional de Cancerología

DOI:

https://doi.org/10.35509/01239015.691

Keywords:

Desmoplastic Small Round Cell Tumor; Sarcoma; Sarcoma, Small Cell; Sarcoma, Ewing; Testicular Neoplasms; Drug Therapy.

Abstract

Desmoplastic small round cell tumor (DSRCT) is a rare and aggressive malignant neoplastic disease. It predominantly affects men between the second and third decades of life. Patients with it have a poor prognosis, with a 5-year survival of up to 30%. It generally presents as a mass in the abdominal cavity, often multifocal. A multimodal approach is recommended for its treatment, with surgery, chemotherapy and radiotherapy. Just over 20 cases of testicular/paratesticular DSRCT have been reported in the literature. Next, we present an illustrative case in this location, the case is discussed and a review of the literature is carried out.

Author Biographies

Handerson Rafael Osma Charris, Instituto Nacional de Cancerología

Fellow Oncología Clínica.

Fernando Contreras Mejía, Instituto Nacional de Cancerología

Oncólogo Clínico.

Camilo Vallejo Yepes, Instituto Nacional de Cancerología

Fellow Oncología Clínica. 

 

Carolina Bautista Saiz, Fundación Universitaria Ciencias de la Salud (FUCS).

Residente de Patología.

Vilma Medina Boada, Instituto Nacional de Cancerología

Departamento de Genética y Oncología molecular.

Tatiana Roncancio Velandia, Instituto Nacional de Cancerología

Departamento de Genética y Oncología molecular.

References

Zhang G, Zhu Y, Gan H, Ye D. Testicular desmoplastic small round cell tumor: a case report and review of literature. World J Surg Oncol. 2014;12:227.

Gerald W, Rosai J. Case 2. Desmoplastic small cell tumor with divergent differentiation. Pediatr Pathol. 1989;9:177–183.

Ladanyi M., Gerald W.L. Specificity of the EWS/WT1 gene fusion for desmoplastic small round cell tumour. J Pathol. 1996;180(4):462.

Gerald W, Ladanyi M, de Alava E, 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;16:3028–3036.

Antonescu C, Gerald W, Magid M, Ladanyi M. Molecular variants of the EWS-WT1 gene fusion in desmoplastic small round cell tumor. Diagn Mol Pathol. 1998;7:24-28.

Zhang J, Xu H, Ren F, Yang Y, Chen B, Zhang F. Analysis of clinicopathological features and prognostic factors of desmoplastic small round cell tumor. Pathology oncology research: POR. 2014;20(1):161–168.

Gerald W, Miller H, Battifora H, Miettinen M, Silva E, 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;15:499–513.

Bulbul A, Fahy B, Xiu J, et al. Desmoplastic small round blue cell tumor: a review of treatment and potential therapeutic genomic alterations. Sarcoma. 2017:1278268.

Al-Ibraheemi A, Broehm C, Tanas M, et al. Desmoplastic small round cell tumors with atypical presentations: a report of 34 cases. Int J Surg Pathol. 2019;27(3):236–243.

Cummings O, Ulbright T, Young R, Dei T, Fletcher C, Hull M. Desmoplastic small round cell tumors of the paratesticular region. A report of six cases. Am J Surg Pathol. 1997;21:219–225.

Lal D, Su W, Wolden S, Loh K, Modak S, LaQuaglia M. Results of multimodal treatment for desmoplastic small round cell tumors. J Pediatr Surg. 2005;40:251-255.

Kadem S, Habash S. Paratesticular desmoplastic small round cell tumor as an incidental finding during imaging of an obstructed inguinal hernia in an old-age patient. Saudi Surg J 2018;6:108-110.

Sedig L, Geiger J, Mody R, Jasty-Rao R. Paratesticular desmoplastic small round cell tumors: A case report and review of the literature. Pediatr Blood Cancer. 2017 Dec;64(12).

Huang Y, Huang W, Mao W, Gong W, Gu C. Paratesticular desmoplastic small round cell tumor: report of a case. Zhonghua Bing Li Xue Za Zhi. 2009 Aug;38(8):559-60.

Morani A, Bathala T, Surabhi V, et al. Desmoplastic small round cell tumor: imaging pattern of disease at presentation. AJR Am J Roentgenol. 2019;212(3):W45–W54.

Gerald W, Rosai J. Desmoplastic small cell tumor with multi-phenotypic differentiation. Zentralbl Pathol. 1993;139(2):141–151.

Thway K, Noujaim J, Zaidi S, et al. Desmoplastic Small Round Cell Tumor: Pathology, Genetics, and Potential Therapeutic Strategies. Int J Surg Pathol. 2016;24:672–684.

He L, Wen S, Hu X, Guo C, Yi C. Primary desmoplastic small round cell tumor of the testis: A case report and review of the literature. Oncol Lett. 2013 Aug;6(2):565-567.

Chang F. Desmoplastic small round cell tumors: cytologic, histologic, and immunohistochemical features. Arch Pathol Lab Med. 2006;130(5):728–732.

Ordonez N. Desmoplastic small round cell tumor: I: a histopathologic study of 39 cases with emphasis on unusual histological patterns. The American journal of surgical pathology. 1998;22(11):1303–13.

Ito E, Honma R, Imai J, et al. A tetraspanin-family protein, T-cell acute lymphoblastic leukemia-associated antigen 1, is induced by the Ewing’s sarcoma-Wilms’ tumor 1 fusion protein of desmoplastic small round-cell tumor. Am J Pathol. 2003;163(6):2165–2172.

Mohamed M, Gonzalez D, Fritchie K, et al. Desmoplastic small round cell tumor: evaluation of reverse transcription-polymerase chain reaction and fluorescence in situ hybridization as ancillary molecular diagnostic techniques. Virchows Arch. 2017; 471: 631–40

Ostermeier A, McCarville M, Navid F, Snyder S, Shulkin B. FDG PET/CT imaging of desmoplastic small round cell tumor: findings at staging, during treatment and at follow-up. Pediatr Radiol. 2015; 45: 1308–15.

Thomas R, Rajeswaran G, Thway K, Benson C, Shahabuddin K, Moskovic E. Desmoplastic small round cell tumour: the radiological, pathological and clinical features. Insights Imaging. 2013;4(1):111–118.

Cliteur VP, Szuhai K, Baelde HJ, van Dam J, Gelderblom H, Hogendoorn P. Paratesticular desmoplastic small round cell tumour: An unusual tumour with an unusual fusion; cytogenetic and molecular genetic analysis combining RT-PCR and COBRA-FISH. Clin Sarcoma Res. 2012;2(1):3.

Subbiah V, Lamhamedi-Cherradi S, Cuglievan B, et al. Multimodality treatment of desmoplastic small round cell tumor: chemotherapy and complete cytoreductive surgery improve patient survival. Clin Cancer Res. 2018;24:4865–73.

Arora V, Price A, Fleming S, et al. Characteristic imaging features of desmoplastic small round cell tumour. Pediatr Radiol. 2013;43(1):93–102.

Prat J, Matias-Guiu X, Algaba F. Desmoplastic small round-cell tumor. Am J Surg Pathol. 1992;16(3):306–307.

Rais H, Elmansouri F, Belaabidia B, Essadki O, Oussehal A, Sarf I. Paratesticular desmoplastic small round cell tumour: Case report with literature review. Cancer Radiother. 2010;14(2):111–114.

Thuret R, Renaudin K, Leclere J, Battisti S, Bouchot O, Theodore C. Uncommon malignancies: Case 3. Paratesticular desmoplastic small round-cell tumor. J Clin Oncol. 2005;23(25):6253– 6255.

Yue X, Wang J, Tian Y, Wang K. Paratesticular desmoplastic small round cell tumor with metastasis: A report of two cases. Kaohsiung J Med Sci. 2014;30(2):104–105.

Farhat F, Culine S, Lhomme C, et al. Desmoplastic small round cell tumors: results of a four-drug chemotherapy regimen in five adult patients. Cancer. 1996;77:1363-1366.

Ordonez N, el-Naggar A, Ro J, Silva E, Mackay B. Intra-abdominal desmoplastic small cell tumor: a light microscopic, immunocytochemical, ultrastructural, and flow cytometric study. Hum Pathol. 1993;24:850-865.

Roganovich J, Bisogno G, Cecchetto G, et al. Paratesticular desmoplastic small round cell tumor: case report and review of the literature. J Surg Oncol. 1999;71:269–272.

Manjula M, Pawar Y. Primary desmoplastic small round cell tumor of the testis: First case in India and review of the literature. J Can Res Ther. 2015;11:650.

Amin M, Edge S, Greene F, Byrd D, Brookland R, Washington M, Gershenwald J, Compton C, Hess K, et al. (Eds.). AJCC Cancer Staging Manual (8th edition). Springer International Publishing: American Joint Commission on Cancer; 2017. p543.

Honoré C, Amroun K, Vilcot L, et al. Abdominal desmoplastic small round cell tumor: multimodal treatment combining chemotherapy, surgery, and radiotherapy is the best option. Ann Surg Oncol. 2015;22:1073-1079.

Hayes-Jordan A, Green H, Fitzgerald N, Xiao L, Anderson P. Novel treatment for desmoplastic small round cell tumor: hyperthermic intraperitoneal perfusion. J Pediatric Surg. 2010;45:1000–1006.

Tun A, Thein W, Guevara E. Optimal Multimodal Treatment for Desmoplastic Small Round Cell Tumors-Reply. JAMA Oncol. 2018 Sep 1;4(9):1301-1302.

Hayes-Jordan A, Green H, Lin H, et al. Complete cytoreduction and HIPEC improves survival in desmoplastic small round cell tumor. Ann Surg Oncol. 2014;21:220–224.

Kushner B, LaQuaglia M, Wollner N, et al. Desmoplastic small round-cell tumor: prolonged progression-free survival with aggressive multimodality therapy. J Clin Oncol. 1996;14:1526-1531.

Lettieri C, 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;2014:680126.

Hayes-Jordan A, Anderson P. The diagnosis and management of desmoplastic small round cell tumor: a review. Curr Opin Oncol. 2011; 23: 385–389.

Assi1 T, Le Cesne1 A, Nassif E, et al. Adriamycin and ifosfamide-based regimen as induction chemotherapy in desmoplastic small round cell tumors: Results of a retrospective single-center study on 34 patients. Ann Oncol. 2018. Oct;29 Suppl 8:viii589.

Scheer M, Vokuhl C, Blank B, et al. on behalf of the Cooperative Weichteilsarkom Studiengruppe. Desmoplastic small round cell tumors: multimodality treatment and new risk factors. Cancer Med. 2019;8:527–542.

Bisogno G, Ferrari A, Rosolen A, et al. Sequential intensified chemotherapy with stem cell rescue for children and adolescents with desmoplastic small round-cell tumor. Bone Marrow Transplantation. 2010;45(5):907–911.

Verret B, Honoré C, Dumont S, et al. Trabectedin in advanced desmoplastic round cell tumors: a retrospective single‐center series. Anticancer Drugs. 2017;28(1):116‐119.

Frezza A, Benson C, Judson I, et al. Pazopanib in advanced desmoplastic small round cell tumours: a multi-institutional experience. Clin Sarcoma Res.2014;4:7.

Shi C, Feng Y, Zhang L, Ding D, Yan M, Pan L. Effective treatment of apatinib in desmoplastic small round cell tumor: a case report and literature review. BMC Cancer. 2018;18:338.

Italiano A, Kind M, Cioffi A, et al. Clinical activity of sunitinib in patients with advanced desmoplastic round cell tumor: a case series. Target Oncol. 2013;8(3):211‐213.

Tap W, Demetri G, Barnette P, et al. Phase II study of ganitumab, a fully human anti-type-1 insulin-like growth factor receptor antibody, in patients with metastatic Ewing family tumors or desmoplastic small round cell tumors. J Clin Oncol. 2012;30:1849–1856.

Thijs A, van der Graaf W, van Herpen C. Temsirolimus for metastatic desmoplastic small round cell tumor. Pediatr Blood Cancer. 2010;55(7):1431–1432.

Naing A, LoRusso P, Fu S, et al. Insulin growth factor-receptor (IGF-1R) antibody cixutumumab combined with the mTOR inhibitor temsirolimus in patients with refractory Ewing's sarcoma family tumors. Clin Cancer Res. 2012;18:2625–2631.

Fine R, Shah S, Moulton T, et al. Androgen and c-Kit receptors in desmoplastic small round cell tumors resistant to chemotherapy: novel targets for therapy. Cancer chemotherapy and pharmacology. 2007;59(4):429–537.

Magnan H, Chou T, LaQuaglia M, et al. Elevated expression of VEGFR-2 and VEGFA in desmoplastic small round cell tumor (DSRCT) and activity of bevacizumab and irinotecan in a xenograft model of DSRCT. J Clin Oncol. 2009, 27: 15s-suppl; abstr 10016.

Loktev A, Shipley J. Desmoplastic small round cell tumor (DSRCT): emerging therapeutic targets and future directions for potential therapies. Expert Opin Ther Targets. 2020 Mar;11:1-5.

van Erp AEM, Versleijen-Jonkers Y, Hillebrandt-Roeffen M et al. Expression and clinical association of programmed cell death-1, programmed death-ligand-1 and CD8+ lymphocytes in primary sarcomas is subtype dependent. Oncotarget. 2017. Jul 7;8(41):71371-71384.

Wedekind M, Haworth K, Arnold M, Stanek J, Lee D, Cripe T. Immune profiles of desmoplastic small round cell tumor and synovial sarcoma suggest different immunotherapeutic susceptibility upfront compared to relapse specimens. Pediatr Blood Cancer. 2018 Nov;65(11):e27313.

Honoré C, Delhorme JB, Nassif E, et al. Can we cure patients with abdominal Desmoplastic Small Round Cell Tumor? Results of a retrospective multicentric study on 100 patients. Surg Oncol. 2019 Jun;29:107-112.

Honoré C, Atallah V, Mir O, et al. Abdominal desmoplastic small round cell tumor without extraperitoneal metastases: Is there a benefit for HIPEC after macroscopically complete cytoreductive surgery? PLoS ONE. 2017;12(2):e0171639.

Pinnix CC, Fontanilla HP, Hayes-Jordan A, et al. Whole Abdominopelvic Intensity-Modulated Radiation Therapy for Desmoplastic Small Round Cell Tumor after Surgery. Int J Radiat Oncol Biol Phys. 2012 May;83(1):317-326.

Osborne E, Briere T, Hayes-Jordan A, et al. Survival and toxicity following sequential multimodality treatment including whole abdominopelvic radiotherapy for patients with desmoplastic small round cell tumor. Radiother Oncol. 2016;119(1):40-4.

Dufresne A, Cassier P, Couraud L, et al. Desmoplastic small round cell tumor: current management and recent findings. Sarcoma. 2012;2012:714986.

Kallianpur A, Shukla N, Deo S, et al. Updates on the multimodality management of desmoplastic small round cell tumor. J Surg Oncol. 2012;105:617–621.

Hayes-Jordan A, Anderson P, Curley S, et al. Continuous hyperthermic peritoneal perfusion for desmoplastic small round cell tumor. J Pediatr Surg. 2007;42:E29–32.

Hayes-Jordan A, Coakley B, Green H, et al. Desmoplastic small round cell tumor treated with cytoreductive surgery and hyperthermic intraperitoneal chemotherapy: results of a phase 2 trial. Ann Surg Oncol. 2018;25:872–827.

Mazuryk M, Paterson A, Temple W, Arthur K, Crabtree T, Stewart D. Benefit of aggressive multimodality therapy with autologous stem cell support for intra-abdominal desmoplastic small round cell tumor. Bone Marrow Transplantation. 1998;21(9):961–963.

LaQuaglia M, Brennan M. The clinical approach to desmoplastic small round cell tumor. Surg Oncol. 2000;9:77–81.

How to Cite

[1]
Osma Charris, H.R. et al. 2020. Paratesticular/testicular ​desmoplastic round small cell malignant tumor: case report and literature review. Revista Colombiana de Cancerología. 24, 3 (Jul. 2020), 125–134. DOI:https://doi.org/10.35509/01239015.691.

Downloads

Download data is not yet available.

Published

2020-07-03

Issue

Section

Reportes de caso
Crossref Cited-by logo