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Sinonasal desmoplastic small round cell tumor: a case report
Nicole M Finke1, Marick E Lae, Ricardo V Lloyd
1Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota 55905, USA.
The American Journal of Surgical Pathology
|May 23, 2002
Summary
This report details a rare sinonasal desmoplastic small round cell tumor in a young woman. The case, lacking serosal involvement, adds to the mystery surrounding this unusual cancer's origin.
Area of Science:
- Oncology
- Pathology
- Genetics
Background:
- Desmoplastic small round cell tumor (DSRCT) is a rare and aggressive malignancy.
- Typically, DSRCT presents in the abdominal or pelvic peritoneum.
- Extraperitoneal DSRCT is exceptionally uncommon.
Observation:
- A case of sinonasal DSRCT in a 21-year-old female is presented.
- Histology revealed nests of tumor cells within a desmoplastic stroma.
- Immunohistochemistry showed positivity for keratin, vimentin, desmin (perinuclear dot-like pattern), and focal neuron-specific enolase.
Findings:
- Genetic analysis confirmed the characteristic t(11;22)(p13;q12) translocation, resulting in the EWSR1-WT1 fusion transcript.
- This represents the third reported case of DSRCT arising outside of a serosal surface.
- The sinonasal location and lack of serosal involvement present unique diagnostic and histogenetic challenges.
Implications:
- This case expands the known clinical spectrum of DSRCT.
- Further research is needed to understand the histogenesis and behavior of extraperitoneal DSRCT.
- Understanding these rare presentations is crucial for accurate diagnosis and treatment planning in oncology.