Alveolar Soft Part Sarcoma

A variety of rare sarcoma having a reticulated fibrous stroma enclosing groups of sarcoma cells, which resemble epithelial cells and are enclosed in alveoli walled with connective tissue. It is a rare tumor, usually occurring between 15 and 35 years of age. It appears in the muscles of the extremities in adults and most commonly in the head and neck regions of children. Though slow-growing, it commonly metastasizes to the lungs, brain, bones, and lymph nodes. (DeVita Jr et al., Cancer: Principles & Practice of Oncology, 3d ed, p1365)
Also Known As:
Sarcoma, Alveolar Soft Part
Networked: 101 relevant articles (1 outcomes, 5 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1. Neoplasms (Cancer)
2. Renal Cell Carcinoma (Grawitz Tumor)
3. Neoplasm Metastasis (Metastasis)
4. Perivascular Epithelioid Cell Neoplasms
5. Neurilemmoma (Schwannoma)


1. Argani, Pedram: 6 articles (02/2013 - 06/2003)
2. Ladanyi, Marc: 4 articles (10/2011 - 06/2003)
3. Demetri, George D: 2 articles (07/2015 - 12/2012)
4. Goldberg, John M: 2 articles (07/2015 - 12/2012)
5. Fisher, David E: 2 articles (07/2015 - 02/2007)
6. Wagner, Andrew J: 2 articles (07/2015 - 12/2012)
7. Rao, Qiu: 2 articles (01/2015 - 01/2014)
8. Gronchi, Alessandro: 2 articles (06/2014 - 02/2009)
9. Casali, Paolo G: 2 articles (06/2014 - 02/2009)
10. Lazar, Alexander J: 2 articles (01/2014 - 12/2009)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Alveolar Soft Part Sarcoma:
1. trabectedin (ecteinascidin 743)IBA
2. sunitinib (Sutent)FDA Link
3. Microphthalmia-Associated Transcription FactorIBA
4. Cathepsin KIBA
5. Tannins (Tannic Acid)FDA Link
6. Retinaldehyde (Retinal)IBA
7. Monoclonal AntibodiesIBA
8. Transcription Factors (Transcription Factor)IBA
9. ParaffinIBA
10. Formaldehyde (Formol)FDA Link
01/01/2014 - "To aid in diagnosis, a break-apart strategy fluorescence in situ hybridization (FISH) probe set specific for TFE3 rearrangement and a reflex dual-color, single-fusion strategy probe set involving the most common TFE3 partner gene, ASPSCR1, were validated on formalin-fixed, paraffin-embedded tissues from nine alveolar soft part sarcoma, two suspected Xp11.2 renal cell carcinoma, and nine tumors in the differential diagnosis. "
06/01/2003 - "We performed immunohistochemistry using a polyclonal antibody to the C-terminal portion of TFE3 in 40 formalin-fixed, paraffin-embedded tumors characterized by TFE3 gene fusions, including 19 alveolar soft part sarcoma (of which nine were molecularly confirmed) and 21 renal carcinomas with cytogenetically confirmed characteristic Xp11.2 translocations and/or fusion transcripts involving TFE3 (11 PRCC-TFE3, 7 ASPL-TFE3, 3 PSF-TFE3). "
12/01/2004 - "Formalin-fixed, paraffin-embedded tumor tissues from 8 cases of alveolar soft part sarcoma and 15 cases of controls (including 6 alveolar rhabdomyosarcomas, 6 renal cell carcinomas, 2 paragangliomas and 1 granular cell myoblastoma) were retrieved from the archival materials. "
03/01/2012 - "To identify the best marker for alveolar soft part sarcoma in formalin-fixed, paraffin-embedded tissues, we evaluated the sensitivity and specificity of the detection of the ASPSCR1-TFE3 fusion transcript along with the immunoreactivity for TFE3 and CD147 in 24 alveolar soft part sarcomas and 23 non-alveolar soft part sarcoma tumors, including 5 granular cell tumors, 5 paragangliomas, 3 clear cell sarcomas, and 10 clear cell renal cell carcinomas. "
03/01/2011 - "Detection of ASPL/TFE3 fusion transcripts and the TFE3 antigen in formalin-fixed, paraffin-embedded tissue in a series of 18 cases of alveolar soft part sarcoma: useful diagnostic tools in cases with unusual histological features."

Therapies and Procedures

1. Drug Therapy (Chemotherapy)
2. Radiotherapy
3. Solid-State Lasers
4. Limb Salvage
5. Heterologous Transplantation (Xenotransplantation)