Presentation
Although tumors grow rather slowly, patients often present with advanced disease. Patients presenting with stage IV disease reach nearly two thirds of the analyzed cases in some series [8,9]. [austinpublishinggroup.com]
Each presentation is accompanied by abundant histologic slides to display the diagnostic differences. [books.google.com]
Case Report A 25-year-old male presented with a rapidly increasing swelling over the left scapular region of six months duration. [scirp.org]
Discussion ASPS typically presents in young women as a slowly enlarging, well-circumscribed, and often encapsulated mass. Vascular invasion is frequent, and metastatic disease is frequently present at diagnosis. [ajnr.org]
Crystals were present in the cytoplasm of the tumor cells following periodic acid-Schiff (PAS) staining. [spandidos-publications.com]
Entire Body System
- Soft Tissue Mass
[…] of the lesion. c T2-weighted fat-suppressed axial image showed a hyperintense soft tissue mass with lobulated contour. [karger.com]
It accounts for less than 1% of all soft tissue malignancies, and generally occurs between 15 and 35 years of age with a notable female predominance [1] [2]. ASPS usually presents as a slowly growing, painless mass [1]. [scirp.org]
The duration of the disease ranged between 3 months to 3 years, and the main clinical manifestation was an increasingly soft-tissue mass with pain. [spandidos-publications.com]
Soft tissue masses, the underutilization of sonography. Semin in Musculoskelet Radiol [Internet] 1999 [citado 16 Oct 2016]; 3(2): 115-34. Disponible en: https://www.thieme-connect.com/DOI/DOI?10.1055/s-2008-1080056. Weatherhall P. [medigraphic.com]
[…] bladder, gastrointestinal, orbital area, and tongue. 4) ASPS is found as a slowly growing soft tissue mass. [synapse.koreamed.org]
- Falling
| Sioux Falls, South Dakota, 57117-5134, United States T C Thompson Children's Hospital | Chattanooga, Tennessee, 37403, United States East Tennessee Childrens Hospital | Knoxville, Tennessee, 37916, United States St. [ichgcp.net]
In children without metastases, the five-year survival rate is 91 percent, but falls to around 60 percent if the disease has spread. Local recurrence after surgical removal is uncommon; however, tumors can return many years after treatment. [danafarberbostonchildrens.org]
[…] the MD Anderson Cancer Center’s institutional database, which may overrepresent incidence as a tertiary care center for rare tumors, estimated 90 total new cases of ASPS in the United States in 2004; it predicted that about half of the patients would fall [curesarcoma.org]
60% 5 yr survival falls to 15% at 20 yrs (metastases reported 33 yrs after inital presentation) poor response to chemotherapy and/or radiation Novel txs Sunitinib Multitargeted receptor tyrosine kinase inhibitor Care must be taken postop with STSG as [orthopaedicsone.com]
- Fever
Good prognostic features include lesions that are isolated to skin and lymph nodes with minimal mucosal disease, a CD4 count higher than 200, no evidence of thrush, and no systemic symptoms (eg, fever, night sweats, weight loss >10% of body weight). [emedicine.medscape.com]
- Weight Loss
Good prognostic features include lesions that are isolated to skin and lymph nodes with minimal mucosal disease, a CD4 count higher than 200, no evidence of thrush, and no systemic symptoms (eg, fever, night sweats, weight loss >10% of body weight). [emedicine.medscape.com]
- Anemia
Case Report A 34-year-old woman noticed discomfort on the right side of her throat after choking on an apple and subsequently had several episodes of oral bleeding leading to anemia. [ajnr.org]
Respiratoric
- Dyspnea
Hayes D Jr, Kesler MV, Skinner SC, Attili AK: A 19-year-old woman with progressive dyspnea and multiple pulmonary nodules. Chest 2011 Jul;140(1):253-7. [orthopaedicsone.com]
Pleural effusion or ascites that causes respiratory compromise (≥ NCI-CTCAE version 4.03 grade 2 dyspnea) Centros participantes H. U. Canarias - J. Cruz H. U. Virgen del Rocío - J. Martín Broto H. de la Santa Creu i Sant Pau - A. López Pousa H. [grupogeis.org]
Attili, A 19-Year-Old Woman With Progressive Dyspnea and Multiple Pulmonary Nodules, Chest, 140, 1, (253), (2011). Susan Kenney, David T. Vistica, Luke H. Stockwin, Sandra Burkett, Melinda G. Hollingshead, Suzanne D. Borgel, Donna O. [doi.org]
Neurologic
- Headache
Parameningeal tumors may present with cranial nerve dysfunction, symptoms of sinusitis, ear discharge, headaches, and facial pain. Orbital tumors often present with orbital swelling and proptosis. [wikipredia.net]
Treatment
The mean treatment duration was 312 days in pazopanib treatment. In evaluable eight patients best RECIST response was PR in two, SD in five and PD in one. [oncologypro.esmo.org]
TREATMENT Treatment of ASP tumors depends on the specific type of tumor, its size, its location, and the amount that it has spread (metastasis). The standard treatment of these tumors is a complete resection of the primary tumor. [secure.ssa.gov]
A team of doctors from many specialties will work to determine a course of treatment for alveolar soft-part sarcoma. [stjude.org]
The ASPS of the patient in this case report was clearly progressive during a 4 months lasting treatment with three combination chemotherapy schemes and two periods of single-agent treatment. [nature.com]
Before you start treatment you will see the trial doctors and have some tests. [cancerresearchuk.org]
Prognosis
Diagnosis - Alveolar soft part sarcoma Prognosis - Alveolar soft part sarcoma Although ASPS displays a relatively indolent course, the ultimate prognosis is poor and is often characterized by late metastases. [checkorphan.org]
This variant has a better prognosis. [histopathology-india.net]
Conclusions Pediatric ASPS has a more favorable prognosis than its adult counterpart. In this series, tumor size correlates with metastatic disease at onset and is the major factor influencing survival. Surgery is the mainstay of therapy. [academic.oup.com]
Etiology
(Etiology) The exact cause and mechanism of Alveolar Soft Part Sarcoma formation, is unknown. The tumor is said to arise from embryonic connective tissues They are thought to occur as a result of gene fusion. [dovemed.com]
Approximately 80% of chondrosarcomas are primary lesions arising in healthy tissue, with no know etiologic factor. [emedicine.medscape.com]
Epidemiology
Relevant External Links for ASPSCR1 Genetic Association Database (GAD) ASPSCR1 Human Genome Epidemiology (HuGE) Navigator ASPSCR1 Atlas of Genetics and Cytogenetics in Oncology and Haematology: ASPSCR1 No data available for Genatlas for ASPSCR1 Gene The [genecards.org]
Alveolar soft part sarcoma is characterized by a translocation between the ASPL locus on chromosome 17 and the TFE3 locus on the X chromosome (der(17)t(X;17)(p11q25)) Epidemiology Most patients with alveolar soft part sarcoma probably have had the cancer [curesarcoma.org]
ASPS was first described and characterized in 1952. [1] Epidemiology ASPS is an extremely rare cancer. While sarcomas comprise about 1% of all newly diagnosed cancers, and 15% of all childhood cancers, ASPS comprises less than 1% of sarcomas. [ipfs.io]
[citation needed] Epidemiology[edit] Rhabdomyosarcoma is the most common soft-tissue sarcoma in children as well as the third most common solid tumor in children. [wikipredia.net]
Epidemiology Rare tumour: represents less than 1% of soft tissues sarcomas of adults and 1-2% of soft tissues sarcomas in children. Occurs most often in the young adult, less frequently in children. [atlasgeneticsoncology.org]
Pathophysiology
Clinical Information Discusses physiology, pathophysiology, and general clinical aspects, as they relate to a laboratory test Alveolar soft-part sarcoma (ASPS) is a rare malignant tumor typically occurring in patients in their 20s to 30s within the muscle [mayomedicallaboratories.com]
Prevention
Prevention - Alveolar soft part sarcoma Not supplied. [checkorphan.org]
If you speak up you might help prevent other ASPS patients from experiencing similar misfortune. [leiomyosarcoma.org]
Treatment principle was of a massive resection, and when the surgical margin is questionable, radiation treatment can be added for prevention of local recurrence. [synapse.koreamed.org]
If these were positives the inclusion is not recommended, remaining at investigators' discretion the preventive treatment with lamivudine. [grupogeis.org]