| Autor: |
Sharma, Aarti E., deVries, John A., Agrawal, Caitlin, Haydon, Rex C., Krausz, Thomas, Pytel, Peter, Cipriani, Nicole A. |
| Předmět: |
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| Zdroj: |
International Journal of Surgical Pathology; May2022, Vol. 30 Issue 3, p317-325, 9p |
| Abstrakt: |
Background: Desmoplastic small round cell tumors (DSRCT) are malignant neoplasms of young males arising most commonly in the abdominopelvic cavity, with a subset originating from extra-abdominal soft tissues. As either primary or metastatic lesions, they are rare in intraosseous sites. Case Presentation: We describe the fifth report of primary DSRCT of bone. A healthy 18-year old male presented with a blastic, 17 cm lesion within the left distal femur, suspicious for osteosarcoma or Ewing sarcoma. Subsequent biopsy revealed nests of small round blue cells infiltrating through a desmoplastic stroma. These cells were diffusely positive for epithelial markers, with paranuclear staining for desmin and focal reactivity with NSE. Break-apart FISH revealed a rearrangement in EWSR1, and RNA fusion panel confirmed WT1 as its partner in the pathognomonic t(11;22)(p13;q12) rearrangement. PET/CT showed widespread metastatic disease to visceral and bony sites. Conclusions : Due to their rarity as well as clinicopathologic and immunomorphologic overlap, primary intraosseous DSRCT can create diagnostic challenges with the more frequently encountered tumors of bone. [ABSTRACT FROM AUTHOR] |
| Databáze: |
Complementary Index |
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