Alveolar soft-part sarcoma in the sacrum: a case report and review of the literature.
Skeletal Radiol
; 43(1): 115-20, 2014 Jan.
Article
in En
| MEDLINE
| ID: mdl-24092236
ABSTRACT
Alveolar soft part sarcoma (ASPS) is a rare disease of the soft tissue. Although the disease is rare, it is refractory to chemotherapy and radiation. En bloc surgical resection offers the best chance of cure. In this article we report the case of a 28-year-old woman who presented with buttock and leg pain, bowel, bladder and gait impairment and a large mass in the sacrum. Following surgical excision, the lesion was proven to be ASPS. On pathology, the mass was TFE3 (transcription factor E3) positive, indicating the presence of the ASPL-TFE3 (novel gene-transcription factor) translocation. Following surgery, the patient had improvement in her pain and ambulation; however, she refused adjuvant therapy to pursue hospice care and succumbed to her disease 2 years after surgery. On a review of the literature, it was found that ASPS of the bone constitutes a rare and formidable subset of this disease. Further, metastases related to ASPS are common in the lungs, liver, brain, and lymph nodes. The degree of dissemination is a predictor of outcome, with 5-year survival of 81-88% in patients with local disease and only 20-46% in patients with metastatic disease at the time of presentation. Brain metastases at the time of presentation portend the worst prognosis.
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Sacrum
/
Spinal Neoplasms
/
Magnetic Resonance Imaging
/
Sarcoma, Alveolar Soft Part
Type of study:
Prognostic_studies
Limits:
Adult
/
Female
/
Humans
Language:
En
Journal:
Skeletal Radiol
Year:
2014
Document type:
Article
Affiliation country:
United States