A case report of chest wall desmoplastic small round cell tumor in children.

ABSTRACT

INTRODUCTION: Desmoplastic small round cell tumor (DSRCT) is a rare sarcoma predominantly afflicting young males. CASE PRESENTATION In this current report, a two-year-old boy was admitted to the hospital for the evaluation of a left chest wall mass. Imaging revealed the tumor's presence in the left chest, compressing lung tissue. Subsequently, histological analysis confirmed the DSRCT diagnosis following a biopsy. The patient underwent a comprehensive management strategy centered around surgery, successfully completing the entire treatment course without experiencing relapse during subsequent follow-up assessments. DISCUSSION: When chest wall tumors are inoperable upon initial diagnosis, a biopsy is essential to clarify the pathology and assist in the diagnostic process. If a patient is diagnosed with DRSCT and conventional chemotherapy fails with surgical resection still not feasible, timely adjustment of the chemotherapy regimen coupled with targeted drug administration can reduce the tumor, enable complete resection, and improve the overall prognosis. CONCLUSION: DSRCT is a rare malignancy associated with a generally poor prognosis. The administration of a combined treatment approach involving oral targeted medication (anlotinib), chemotherapy, radiotherapy, and aggressive surgical resection holds the potential to enhance the prognosis for pediatric patients with this condition.