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:

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)

Experts

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 01/01/2012 - "Efficacy of trabectedin in patients with advanced or metastatic alveolar soft-part sarcoma."
2.	sunitinib (Sutent)FDA Link 07/01/2011 - "The purpose of this study was to confirm sunitinib activity in alveolar soft part sarcoma (ASPS) and to report on new insights into the molecular bases thereof. " 03/01/2012 - "Alveolar soft part sarcoma in childhood: is Sunitinib-Sutent® treatment an effective approach?" 01/01/2012 - "Treatment of multidrug resistant advanced alveolar soft part sarcoma with sunitinib." 07/01/2011 - "Sunitinib in advanced alveolar soft part sarcoma: evidence of a direct antitumor effect." 02/01/2009 - "Response to sunitinib malate in advanced alveolar soft part sarcoma."
3.	Microphthalmia-Associated Transcription FactorIBA 07/15/2015 - "Alveolar soft-part sarcoma (ASPS) and clear cell sarcoma (CCS) are rare mesenchymal malignancies driven by chromosomal translocations that activate members of the microphthalmia transcription factor (MITF) family. " 02/01/2011 - "The DNA-binding factor TFE3 is closely related to microphthalmia-associated transcription factor (MiTF) and is over-expressed in alveolar soft part sarcoma (ASPS) and select renal cell carcinomas. " 01/01/2014 - "Recent studies have demonstrated that cathepsin K seems to be a powerful marker in identifying the microphthalmia associated transcription factor (MITF) family tumors such as renal perivascular epithelioid cell neoplasms (PEComas), alveolar soft part sarcoma, and translocation-associated renal cell carcinomas. " 12/01/2012 - "Microphthalmia transcription factor (MITF)-associated (MiT) tumors are a family of rare malignancies, including alveolar soft part sarcoma (ASPS), clear cell sarcoma (CCS), and translocation-associated renal cell carcinoma (tRCC) that have dysregulated expression of oncogenic MITF family proteins. "
4.	Cathepsin KIBA 02/01/2013 - "Cathepsin K is consistently and diffusely expressed in alveolar soft part sarcoma (ASPS) and a subset of translocation renal cell carcinomas (RCCs). " 10/01/2011 - "Expression of cathepsin K distinguishes alveolar soft part sarcoma from the ASPSCR1-TFE3 carcinoma, harboring the same gene fusion. " 10/01/2011 - "However, the expression of cathepsin K in specific genetic subtypes of Xp11 translocation carcinomas, as well as alveolar soft part sarcoma, which harbors the same ASPSCR1-TFE3 gene fusion as some Xp11 translocation carcinomas, has not been addressed. " 01/01/2014 - "Recent studies have demonstrated that cathepsin K seems to be a powerful marker in identifying the microphthalmia associated transcription factor (MITF) family tumors such as renal perivascular epithelioid cell neoplasms (PEComas), alveolar soft part sarcoma, and translocation-associated renal cell carcinomas. "
5.	Tannins (Tannic Acid)FDA Link 01/01/1990 - "Alveolar soft part sarcoma: an electron microscopic study especially of uncrystallized granules using a tannic acid-containing fixative."
6.	Retinaldehyde (Retinal)IBA 02/01/2012 - "The parotid gland masses in this study included pleomorphic adenoma, Warthin's tumour, retention cyst, haemangiomas, chronic granuloma, lymphoma, fibrolipoma, abscess, basal cell adenoma, oncocytoma, lymphatic tuberculosis, myoepithelioma, neurilemmoma, mucoepidermoid carcinoma, adenoid cystic carcinoma, alveolar soft part sarcoma and retinal blastoma (metastasis). "
7.	Monoclonal AntibodiesIBA 04/01/1984 - "Alveolar soft part sarcoma: ultrastructural and immunohistological studies with monoclonal antibodies."
8.	Transcription Factors (Transcription Factor)IBA 11/01/2015 - "It is characterized by a specific chromosomal alteration, der(17)t(X:17)(p11:q25), resulting in fusion of the transcription factor E3 (TFE3) with alveolar soft part sarcoma critical region 1 (ASPSCR1) at 17q25. " 12/01/2009 - "The molecular signature of alveolar soft part sarcoma (ASPS) is a specific der(17)t(X;17)(p11.2;q25) translocation, resulting in a chimeric transcription factor (ASPSCR1-TFE3). " 06/01/2007 - "Alveolar soft part sarcoma (ASPS) is a rare soft tissue tumour with unique morphology and a recurrent, non-reciprocal translocation der(17)t(X;17)(p11.2;q25) leading to the fusion of ASPSCR1 (also known as ASPL) to the transcription factor TFE3. " 01/04/2001 - "The der(17)t(X;17)(p11;q25) of human alveolar soft part sarcoma fuses the TFE3 transcription factor gene to ASPL, a novel gene at 17q25." 01/01/2015 - "Xp11.2 or TFE3 translocation renal cell carcinomas (RCC) and alveolar soft part sarcoma (ASPS) are characterized by chromosome translocations involving the Xp11.2 breakpoint resulting in transcription factor TFE3 gene fusions. "
9.	ParaffinIBA 03/01/2012 - "Technique for differentiating alveolar soft part sarcoma from other tumors in paraffin-embedded tissue: comparison of immunohistochemistry for TFE3 and CD147 and of reverse transcription polymerase chain reaction for ASPSCR1-TFE3 fusion transcript." 06/01/2010 - "Dual-color, break-apart FISH assay on paraffin-embedded tissues as an adjunct to diagnosis of Xp11 translocation renal cell carcinoma and alveolar soft part sarcoma." 12/01/2008 - "Alveolar soft part sarcoma: a bimarker diagnostic strategy using TFE3 immunoassay and ASPL-TFE3 fusion transcripts in paraffin-embedded tumor tissues." 12/01/2004 - "To investigate the significance of detecting chimeric mRNA resulting from t(X;17)(p11.2;q25) in paraffin-embedded tumor tissues of alveolar soft part sarcoma (ASPS). " 12/01/2004 - "[Detection of ASPL-TFE3 fusion gene by reverse transcriptase polymerase chain reaction in paraffin-embedded tumor tissues of alveolar soft part sarcoma]."
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) 04/01/2010 - "Lingual alveolar soft part sarcoma in a child managed successfully with surgery and chemotherapy." 04/01/2004 - "Alveolar soft-part sarcoma responsive to intensive chemotherapy." 03/01/1991 - "A diagnosis of alveolar soft part sarcoma was made, and chemotherapy was started. " 03/01/1991 - "Alveolar soft part sarcoma is primarily treated by surgery, and reports of effective chemotherapy have been infrequent. " 03/01/1991 - "[A case of effective preoperative chemotherapy in alveolar soft part sarcoma]."
2.	Radiotherapy 01/01/1994 - "A clinical experience with radiotherapy in 18 patients with alveolar soft-part sarcoma is presented. " 12/01/2014 - "Management of recurrent alveolar soft-part sarcoma of the tongue after external beam radiotherapy with iodine-125 seed brachytherapy." 06/15/1984 - "Alveolar soft part sarcoma following radiotherapy for a spinal hemangioma. " 01/01/2005 - "Treatment included surgery, followed by postoperative radiotherapy (RT) and chemotherapy (CT) in 1, S and RT in 1, S in 1, RT and CT in 6, RT in 1, CT in 1 and no treatment in 5. Only one patient with alveolar soft part sarcoma is alive with disease 20 months from diagnosis of brain metastasis. " 11/01/2013 - "Ovarian function preserved by carbon-ion radiotherapy for alveolar soft-part sarcoma."
3.	Solid-State Lasers 01/01/1997 - "Between 1985 and 1993, six patients were surgically treated for alveolar soft-part sarcoma (ASPS) arising from the thighs or buttocks, four of whom underwent aggressive excision of multiple metastases using a neodymium:yttrium-aluminum garnet (Nd:YAG) laser. "
4.	Limb Salvage 02/01/1999 - "A report of alveolar soft part sarcoma of the hip region with extension to the ilium is presented to highlight an unusual presentation for this sarcoma and to discuss the treatment options for limb salvage for large malignant neoplasma involving the periacetabular region of the pelvis. "
5.	Heterologous Transplantation (Xenotransplantation) 08/01/2009 - "In vivo growth of alveolar soft part sarcoma (ASPS) was achieved using subcutaneous xenografts in sex-matched nonobese diabetic severe combined immunodeficiency mice. "