Addition of hyperthermic intraperitoneal chemotherapy (HIPEC) after complete cytoreductive surgery in a child with desmoplastic small round cell tumour

Yıl 2019, Cilt: 36 Sayı: 4, 131 - 135, 02.03.2020

İbrahim Kartal , Koray Topgül , Mustafa Kemal Aslan , Ayhan Dağdemir , Emel Özyürek Şaban Sarıkaya

Öz

Desmoplastic small round cell tumour (DSRCT) is a rare and aggressive sarcoma. No curative treatment has been reported yet; however, multi-agent chemotherapy, an aggressive surgical approach and radiotherapy have been used. Hyperthermic intraperitoneal chemotherapy (HIPEC) is an additional strategy used to remove microscopic disease after cytoreduction, especially in patients with peritoneal carcinomatosis and colorectal cancer; however, its application in paediatric patients has been reported rarely. In Turkey, some HIPEC studies have been performed in adult patients but not in paediatric patients. A 10-yearold girl presented to the hospital with a mass palpated in the left lumbar region. The mass was reported as a primitive neuroectodermal tumour after the biopsy. Left radical nephrectomy and lymph node dissection were performed after she failed to respond to five months of neoadjuvant chemotherapy. The pathological examination revealed a DSRCT. A multi-agent chemotherapy regimen was applied to the patient for one year. Myeloablative chemotherapy and autologous stem cell transplantation were performed in the patient, who subsequently entered remission. During the third month of follow-up, high-dose multi-agent chemotherapy protocols were initiated because of disease relapse. At 6 months after recurrence, HIPEC with irinotecan and oxaliplatin was performed after the excision of all tumour foci by intracavitary cytoreductive surgery. No complications were observed. The patient was prescribed a tyrosine kinase receptor inhibitor (sunitinib maleate). The patient died 8 months later. In conclusion, although the benefit of HIPEC is still unknown and requires evaluation in a prospective trial, HIPEC can be used as an alternative treatment in paediatric patients with peritoneal carcinomatosis. 

Anahtar Kelimeler

Child with desmoplastic small round cell tumour, hyperthermic intraperitoneal chemotherapy

Kaynakça

• Gerald, WL and Juan Rosai. "Case 2 desmoplastic small cell tumor with divergent differentiation." Pediatr Pathol 1989; 9 : 177-183.
• Ji-Won Kim, Jin Hyun Park, Hyeon Jin Cho et al. "A case of desmoplastic small round cell tumor diagnosed in a young female patient." Cancer Res Treat 2009; 41 : 233-236
• Gerald WL, Juan Rosai, and Marc Ladanyi. "Characterization of the genomic breakpoint and chimeric transcripts in the EWS-WT1 gene fusion of desmoplastic small round cell tumor." Proc Natl Acad Sci U S A 1995 14; 92: 1028-1032.
• Reingruber B, Boettcher MI, Klein P, Hohenberger W, Pelz JO. Hyperthermic intraperitoneal chemoperfusion is an option for treatment of peritoneal carcinomatosis in children. J Pediatr Surg 2007; 42: E17-21.
• Carolin Hartmann, Peter Weinel, Hansjo¨rg Schmid, Lorenz Grigull, Anette Sander, Christin Linderkamp, Karl Welte and Dirk Reinhardt. Oxaliplatin, Irinotecan, and Gemcitabine: A Novel Combination in the Therapy of Progressed, Relapsed, or Refractory Tumors in Children. J Pediatr Hematol Oncol. 2011 Jul;33(5):344-9.
• Richard Bellah, Lisa Suzuki-Bordalo, Eric Brecher, Jill P. Ginsberg, John Maris and Bruce R. Pawel. "Desmoplastic small round cell tumor in the abdomen and pelvis: report of CT findings in 11 affected children and young adults." AJR Am J Roentgenol 2005; 184: 1910-1914.
• Jellouli M, Mekki M, Krichene I, et al. "Intra-abdominal desmoplastic small round cell tumor in childhood: case report and review of the literature." Bull Cancer 2003; 90: 1063-1066.
• G Biswas, S Laskar, SD Banavali, S Gujral, PA Kurkure, M Muckaden, PM Parikh, Chandrika N Nair. "Desmoplastic small round cell tumor: extra abdominal and abdominal presentations and the results of treatment." Indian J Cancer 2005; 42: 78-84.
• Perry J. Pickhardt, Andrew J. Fisher, Dennis M. Balfe, Louis P. Dehner and Phyllis C. Huettner. "Desmoplastic small round cell tumor of the abdomen: radiologic-histopathologic correlation." Radiology 1999; 210: 633-638.
• OrdOnez, Nelson G. "Desmoplastic small round cell tumor: I: a histopathologic study of 39 cases with emphasis on unusual histological patterns." Am J Surg Pathol 1998; 22: 1303-1313.
• C.E. Stuart-Buttlea, C.J. Smarta, S. Pritchardb, D. Martinc, I.M. Welcha. "Desmoplastic small round cell tumour: a review of literature and treatment options." Surg Oncol 2008; 17: 107-112.
• Hiralal, Gamanagatti S, S. Thulkar, and S. K. Rao. "Desmoplastic round cell tumour of the abdomen." Singapore Med J 2007; 48: e19-21.
• 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; 24; 12: e0171639
• Lal DR, Su WT, Wolden SL, Loh KC, Modak S, La Quaglia MP."Results of multimodal treatment for desmoplastic small round cell tumors." J Pediatr Surg 2005; 40: 251-255.
• Irwin GJ, Mac Lennan AC and Carachi R: Interventional radiology cases from the Royal Hospital for Sick Children, Glasgow: Percutaneous metal biliary stent placement for palliation of intraabdominal desmoplastic small round cell tumour in a child. Eur J Pediatr Surg 2004; 14: 130-132.
• Lauridant-Philippin G, Ledem N, Lemoine F, Gouerant S, Guillemet C, Michel P, Scotte M, Di Fiore F. Optimal treatment with systemic chemotherapy, complete surgical excision and hyperthermic intraperitoneal chemotherapy for a desmplas-tic small round cell tumor in an adulte male patient. Gas-troentérologie Clinique et Biologique 2010; 34, 321-324
• Gil A, Paortilla A, Brun E, et al. Clinical perspective on desmoplastic small round cell tumour. Oncology 2004, 67, 231-242
• Hayes-Jordan A, Green H, Fitzgerald N, Xiao L.C. and Anderson P. Novel treatment for desmoplastic small round cell tumor: Hyperthermic intraperitoneal per- fusion. Journal of Pediatric Surgery 2010, 45, 1000-1006.
• Simon Msika, Enrico Gruden, Sabine Sarnacki, et al. Cytoreductive surgery associated to hyperthermic intraperitoneal chemoperfusion for desmoplastic round small cell tumor with peritoneal carcinomatosis in young patients. Journal of Pediatric Surgery (2010) 45, 1617–1621.