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TÜRKÇE
Poster - 66
Multimodal management of desmoplastic small round cell tumor in children: A single-center experience including cytoreductive surgery and HIPEC
Mhd Faez Alsarraj 1, Ayten Ceren Bakır 1, Nurşah Eker 2, Dilek Gül 3, Özde Nisa Türkkan 4, Gürsu Kıyan 1, Kıvılcım Karadeniz Cerit 1
1 Marmara University Faculty of Medicine, Department of Pediatric Surgery, Istanbul
2 Marmara University School of Medicine, Department of Pediatric Oncology, İstanbul
3 Marmara University School of Medicine,Department of Radiation Oncology
4 Marmara University School of Medicine, Department of Pediatrics, Division of Pediatric Nephrology, Istanbul
Purpose: Desmoplastic small round cell tumor (DSRCT) is an extremely rare and aggressive malignancy in the pediatric population. Due to its rarity, no standardized treatment protocol exists. This study presents our institution’s multidisciplinary treatment approach, including cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC), alongside systemic chemotherapy.
Methods: We retrospectively analyzed patients diagnosed with DSRCT between January 2020 and June 2025. Demographics, clinical, surgical and oncological data were collected and analyzed.
Results: Our cohort included 3 patients with a median age of 13,6 years. The mean peritoneal cancer index (PCI) was 26. Complete cytoreduction (CC-0) was achieved in 2 patients. While one patient remained disease-free for 18 months, however one patient died due to recurrence even after two CRS/HIPEC procedures. Additionaly, one patient died of disease progression. Data is summarized in Table 1.
Conclusions: CRS combined with HIPEC is a feasible and safe option for selected pediatric DSRCT patients, offering the potential for improved local control when complete cytoreduction is achieved.
Table 1:
|
Patient |
Age (years)/Sex |
Preoperative biopsy |
Neoadjuvant therapy |
Operations/duration |
HIPEC |
Adjuvant therapy |
Outcome |
|
1 |
12/M |
Yes |
Yes |
Unresectable/6 hours |
No |
Yes |
DOD |
|
2 |
16/M |
No |
No |
Omentectomy, complete tumor resection/4 hours |
No |
Yes WART |
|
|
Peritonectomy / 7.5 hours |
Yes |
Complete remission
(18 months) |
|||||
|
3 |
13/M |
Yes |
Yes |
Omentectomy, small-large bowel resections, pelvic tumor resection, segmental liver resection, segmental ureteral resection, partial diaphragmatic resection, peritonectomy/23 hours |
Yes |
Yes WART |
|
|
Small and large bowel resections/16 hours |
Yes |
Yes |
DOD Relapse after 15th month of remission |
DOD: Dead of disease
WART: Whole abdominal radiotherapy
M: Male