Primary desmoplastic small round cell tumor of the femur Journal Article
| Authors: | Yoshida, A.; Edgar, M. A.; Garcia, J.; Meyers, P. A.; Morris, C. D.; Panicek, D. M. |
| Article Title: | Primary desmoplastic small round cell tumor of the femur |
| Abstract: | Desmoplastic small round cell tumor (DSRCT) is a rare malignant neoplasm typically involving the abdominal cavity of a young male. Extra-abdominal occurrence of this tumor is very rare. We report a 10-year-old girl with primary DSRCT arising within the left femur. The patient presented with knee pain, and radiological findings were strongly suggestive of osteogenic sarcoma. In addition to the typical microscopic appearance and immunophenotype, RT-PCR demonstrated the chimeric transcript of EWS-WT1, which is diagnostic of DSRCT. Pulmonary metastases were present at initial staging studies, but no abdominal or pelvic lesion was present. Despite chemotherapy and complete tumor excision, the patient developed progressive lung and bone metastases and died 3 years after initial presentation. This is the second reported case of primary DSRCT of bone with genetic confirmation. © 2008 ISS. |
| Keywords: | immunohistochemistry; child; human tissue; school child; bone neoplasms; bone tumor; histopathology; case report; doxorubicin; adjuvant therapy; nuclear magnetic resonance imaging; computer assisted tomography; multiple cycle treatment; diagnosis, differential; etoposide; lung neoplasms; cyclophosphamide; vincristine; diagnostic imaging; biopsy; ifosfamide; reverse transcriptase polymerase chain reaction; fatal outcome; tumor diagnosis; femur tumor; bone scintiscanning; bone radiography; desmoplastic small round cell tumor; sarcoma, small cell; femoral neoplasms; ews-wt1 fusion protein |
| Journal Title: | Skeletal Radiology |
| Volume: | 37 |
| Issue: | 9 |
| ISSN: | 0364-2348 |
| Publisher: | Springer |
| Date Published: | 2008-09-01 |
| Start Page: | 857 |
| End Page: | 862 |
| Language: | English |
| DOI: | 10.1007/s00256-008-0501-0 |
| PUBMED: | 18470511 |
| PROVIDER: | scopus |
| DOI/URL: | |
| Notes: | --- - "Cited By (since 1996): 1" - "Export Date: 17 November 2011" - "CODEN: SKRAD" - "Source: Scopus" |
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