Does cytoreductive surgery and hyperthermic intraperitoneal chemotherapy improve survival in children with desmoplastic small round cell tumors? First experiences from Turkey

Year 2025, Volume: 38 Issue: 1, 29 - 35, 29.01.2025

Kıvılcım Cerit Karadeniz , Nurşah Eker , Ahsen Karagözlü Akgül , Dilek Gül Özde Nisa Türkkan , Ayten Ceren Bakır Ali Eyvazov Gürsu Kıyan

https://doi.org/10.5472/marumj.1627763

Abstract

Objective: Desmoplastic small round cell tumor (DSRCT) is a rare form of highly aggressive sarcoma and despite multimodal therapy,
mortality still remains high. The aim of the study is to review our experience in cytoreductive surgery (CRS) and hyperthermic
intraperitoneal chemotherapy (HIPEC) in children with DSRCT.
Patients and Methods: A retrospective review of patients with DSRCT followed in our institution between January 2020-January
2024 was performed. Demographics, radiological imaging, histopathology results, cytogenetic analyses, chemotherapy/radiotherapy
protocols, operative data and outcomes were analyzed.
Results: Three patients with DSRCT were identified. The median age was 13.3 years (12-16 years). HIPEC was performed after
CRS in two patients, while HIPEC procedure could not be applied in one patient with unresectable tumor. One patient underwent
reoperation/re-HIPEC for recurrence after seven months. One patient had the diagnosis after complete removal of the tumor. Patient
required peritonectomy and HIPEC as a second operation due to peritoneal recurrence. The patients who underwent CRS and HIPEC
are still alive for 33 and 34 months since initial diagnosis, nevertheless the patient who had an unresectable tumor died after 8 months.
Conclusion: Cytoreductive surgery and HIPEC may be considered as a safe and feasible treatment option in children with DSRCT in
experienced centers.

Keywords

Desmoplastic small round cell tumor, Cytoreductive surgery, Hyperthermic intraperitoneal chemotherapy, children

References

• Bent MA, Padilla BE, Goldsby RE, et al. Clinical characteristics and outcomes of pediatric patients with desmoplastic small round cell tumor. Rare Tumors 2016; 8: 6145. doi: 10.4081/ rt2016.6145.
• Hayes-Jordan A, Anderson PM. The diagnosis and management of desmoplastic small round cell tumor: a review. Curr Opin Oncol 2011; 23: 385-9. doi: 10.1097/ CCO.0b013e3283477aab.
• Gerald WL, Rosai J. Desmoplastic small cell tumor with divergent differentiation. Pediatr Pathol 1989; 9: 177-83. doi: 10.3109/155.138.18909022347.
• Ladanyi M, Gerald W. Fusion of the EWS and WT1 genes in the desmoplastic small round cell tumor. Cancer Res 1994; 54: 2837-40. PMID: 8187063.
• Hayes-Jordan A, LaQuaglia MP, Modak S. Management of desmoplastic small round cell tumor. Semin Pediatr Surg 2016; 25: 299-304. doi: 10.1053/j.sempedsurg.2016.09.005.
• Honoré C, Delhorme J, 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; 29: 107-12. doi: 10.1016/j.suronc.2019.04.002.
• Byrwa DJ, Twist CJ, Skitzki J, et al. A review of the use of hyperthermic intraperitoneal chemotherapy for peritoneal malignancy in pediatric patients. Cancers 2023; 15: 2815. doi: 10.3390/cancers15102815.
• Hayes-Jordan A, Green H, Fitzgerald N, et al. Novel treatment for desmoplastic small round cell tumor: Hyperthermic intraperitoneal perfusion. J Pediatr Surg 2010; 45: 1000-6. doi: 10.1016/j.jpedsurg.2010.02.034.
• Msika S, Gruden E, Sarnacki S, et al. Cytoreductive surgery associated to hyperthermic intraperitoneal chemoperfusion for desmoplastic round small cell tumor with peritoneal carcinomatosis in young patients. J Pediatr Surg 2010; 45: 1617-21. doi: 10.1016/j.jpedsurg.2010.03.002.
• Harmon RL, Sugarbaker PH. Prognostic indicators in peritoneal carcinomatosis from gastrointestinal cancer. Int Semin Surg Oncol 2005; 2: 3. doi: 10.1186/1477-7800-2-3.
• Glehen O, Kwiatkowski F, Sugarbaker PH, et al. Cytoreductive surgery combined with perioperative intraperitoneal chemotherapy fort he management of peritoneal carcinomatosis from colorectal cancer: a multi-institutional study. J Clin Oncol 2004; 22: 3284-92. doi: 10.1200/ JCO.2004.10.012.
• Rodríguez SC, Moreno RFJ, Bellido EI, et al. Are there intraoperative hemodynamic differences between the Coliseum and closed HIPEC techniques in the treatment of peritoneal metastasis? A retrospective cohort study. World J Surg Oncol 2017; 15: 51. doi: 10.1186/s12957.017.1119-2.
• Granowetter L, Womer R, Devidas M, et al. Dose-intensified compared with standard chemotherapy for nonmetastatic Ewing sarcoma family of tumors: A Children’s Oncology Group Study. J Clin Oncol 2009; 27:2536-41. doi: 10.1200/ JCO.2008.19.1478.
• Hayes-Jordan A, Green H, Ludwig J, et al. Toxicity of hyperthermic intraperitoneal chemotherapy (HIPEC) in pediatric patients with sarcomatosis/carcinomatosis: early experience and phase 1 results. Pediatr Blood Cancer 2012; 59: 395-7. doi: 10.1002/pbc.24160.
• Hayes-Jordan A, Pappo A. Management of desmoplastic small round cell tumors in children and young adults. J Pediatr Hematol Oncol 2012; 34: 73-75. doi: 10.1097/ MPH.0b013e31824e38ad.
• Kushner BH, LaQuaglia MP, Wollner N, et al. Desmoplastic small round-cell tumor: prolonged progression-free survival with aggressive multimodality therapy. J Clin Oncol 1996; 14: 1526-31. doi: 10.1200/JCO.1996.14.5.1526.
• Goodman KA, Wolden SL, La Quaglia MP, et al. Whole abdominopelvic radiotherapy for desmoplastic small round-cell tumor. Int J Radiat Oncol Biolo Phys 2002; 54: 170- 6. doi: 10.1016/s0360-3016(02)02871-7.
• Shen XZ, Zhao JG, Wu JJ, et al. Clinical and computed tomography features of adult abdominopelvic desmoplastic small round cell tumor. World J Gastroenterol 2014; 20: 5157- 64. doi: 10.3748/wjg.v20.i17.5157.
• 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; 83: 317-26. doi: 10.1016/j. ijrobp.2011.06.1985.
• Stiles ZE, Murphy AJ, Anghelescu DL, et al. Desmoplastic small round cell tumor: Long-term complications after cytoreduction and hyperthermic intraperitoneal chemotherapy. Ann Surg Oncol 2020; 27: 171-8. doi: 10.1245/ s10434.019.07339-2.
• Lal DR, Su WT, Wolden SL, et al. Results of multimodal treatment for desmoplastic small round cell tumors. J Pediatr Surg 2005; 40: 251-5. doi: 10.1016/j.jpedsurg.2004.09.046.
• Hayes-Jordan A, Green HL, Lin H, et al. Complete cytoreduction and HIPEC improves survival in desmoplastic small round cell tumor. Ann Surg Oncol 2014; 21: 220-4. doi: 10.1245/s10434.013.3269-y.
• Hayes-Jordan A, Coakley BA, Green HL, 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: 872-7. doi: 10.1245/ s10434.018.6333-9.
• Hayes-Jordan A, Green H, Lin H, et al. Cytoreductive surgery and Hyperthermic Intraperitoneal Chemotherapy (HIPEC) for children, adolescents, and young adults: the first 50 cases. Ann Surg Oncol 2015; 22: 1726-32. doi: 10.1245/ s10434.014.4289-y.
• Scalabre A, Philippe-Chomette P, Passot G, et al. Cytoreductive surgery and hyperthermic intraperitoneal perfusion with chemotherapy in children with peritoneal tumor spread: A French nationwide study over 14 years. Pediatr Blood Cancer 2018; 65. doi: 10.1002/pbc.26934.