[Unusual intra-abdominal tumor]

Area of Science:

• Oncology
• Pathology

Background:

• Desmoplastic small round cell tumor (DSRCT) is an aggressive, rare neoplasm predominantly affecting the abdominal cavity.
• Diagnosis can be challenging, necessitating detailed morphological and immunohistochemical analysis.

Observation:

• A case study of an 18-year-old presenting with a large intra-abdominal mass (20 cm).
• Radiologic and histopathologic findings confirmed a proliferation of small round cells within a desmoplastic stroma.
• Immunohistochemistry was positive for pancytokeratin (KL1), EMA, vimentin, NSE, and desmin, supporting the DSRCT diagnosis.

Findings:

• The patient underwent chemotherapy and surgical reduction, but experienced recurrence and metastasis.
• The patient succumbed to hepatic failure 18 months post-diagnosis, highlighting the aggressive nature of DSRCT.
• The study underscores the importance of suspecting DSRCT in young patients with intra-abdominal small round cell tumors.

Implications:

• Accurate diagnosis of DSRCT requires a combination of imaging, histology, and immunohistochemistry.
• Multidisciplinary management is essential for DSRCT patients.
• The poor prognosis associated with DSRCT emphasizes the need for further research into effective therapeutic strategies.