Özet:

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. 

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