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Solid-pattern desmoplastic small round cell tumor of the orbit: a case report.
Pei Wang1, Yixiong Liu2, Xiquan Liu3
1Department of Pathology, Weinan Central Hospital Weinan, Shaanxi, P. R. China.
International Journal of Clinical and Experimental Pathology
|January 16, 2020
Summary
Desmoplastic small round cell tumor (DSRCT), a rare abdominal cancer, can occur in the orbit. This case highlights the diagnostic challenge of purely solid variants in unusual locations.
Area of Science:
- Oncology
- Pathology
Background:
- Desmoplastic small round cell tumor (DSRCT) is a rare, aggressive sarcoma typically originating in the peritoneum.
- DSRCT predominantly affects young males and is characterized by a polyphenotypic immunoprofile and the EWS-WT1 gene fusion.
Observation:
- This study reports a rare case of DSRCT presenting as an orbital mass in a young adolescent male.
- Histological examination revealed a purely solid tumor variant, lacking the typical desmoplastic stroma.
Findings:
- The diagnosis was confirmed by a polyphenotypic immunoprofile (desmin and neural markers) and the EWS-WT1 gene fusion.
- The solid variant of DSRCT can mimic other orbital tumors, posing a diagnostic challenge.
Implications:
- This case underscores the importance of considering DSRCT in the differential diagnosis of orbital tumors, especially in pediatric and adolescent patients.
- Recognizing extrabdominal DSRCT variants is crucial for timely diagnosis and appropriate management.
