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Primary Alveolar Soft-Part Sarcoma of the Liver : Anomalous Presentation of a Rare Disease
Alveolar soft-part sarcoma is a highly vascular soft-tissue tumor that is uniformly malignant. It comprises less than 1 per cent of all soft-tissue sarcomas. Patients with alveolar soft-part sarcoma most frequently are aged 15 to 35 years, and the soft tissues of the lower extremities typically are...
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| Published in: | The American surgeon 2008, Vol.74 (1), p.43-46 |
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| container_end_page | 46 |
| container_issue | 1 |
| container_start_page | 43 |
| container_title | The American surgeon |
| container_volume | 74 |
| creator | SHADDIX, Kyle K FAKHRE, G. Peter NIELDS, William W STEERS, Jeffery L HEWITT, Winston R MENKE, David M |
| description | Alveolar soft-part sarcoma is a highly vascular soft-tissue tumor that is uniformly malignant. It comprises less than 1 per cent of all soft-tissue sarcomas. Patients with alveolar soft-part sarcoma most frequently are aged 15 to 35 years, and the soft tissues of the lower extremities typically are affected. In the pediatric population, it most frequently occurs in the head and neck and particularly affects the tongue and orbit. Alveolar soft-part sarcoma has been described as a primary lesion in the trunk, upper extremities, and retroperitoneum; more novel locations include the mediastinum, female genital tract, stomach, bone, and larynx. Numerous case reports describe alveolar soft-part sarcoma in diverse anatomic locations, but this report is, to our knowledge, the first documentation of primary alveolar soft-part sarcoma of the liver. We describe a 47-year-old woman with such a manifestation. Despite surgical resection and numerous chemotherapeutic regimens, this patient had widespread metastasis and died approximately 2 years after the diagnosis was established. |
| doi_str_mv | 10.1177/000313480807400109 |
| format | article |
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Peter ; NIELDS, William W ; STEERS, Jeffery L ; HEWITT, Winston R ; MENKE, David M</creator><creatorcontrib>SHADDIX, Kyle K ; FAKHRE, G. Peter ; NIELDS, William W ; STEERS, Jeffery L ; HEWITT, Winston R ; MENKE, David M</creatorcontrib><description>Alveolar soft-part sarcoma is a highly vascular soft-tissue tumor that is uniformly malignant. It comprises less than 1 per cent of all soft-tissue sarcomas. Patients with alveolar soft-part sarcoma most frequently are aged 15 to 35 years, and the soft tissues of the lower extremities typically are affected. In the pediatric population, it most frequently occurs in the head and neck and particularly affects the tongue and orbit. Alveolar soft-part sarcoma has been described as a primary lesion in the trunk, upper extremities, and retroperitoneum; more novel locations include the mediastinum, female genital tract, stomach, bone, and larynx. Numerous case reports describe alveolar soft-part sarcoma in diverse anatomic locations, but this report is, to our knowledge, the first documentation of primary alveolar soft-part sarcoma of the liver. We describe a 47-year-old woman with such a manifestation. Despite surgical resection and numerous chemotherapeutic regimens, this patient had widespread metastasis and died approximately 2 years after the diagnosis was established.</description><identifier>ISSN: 0003-1348</identifier><identifier>EISSN: 1555-9823</identifier><identifier>DOI: 10.1177/000313480807400109</identifier><identifier>PMID: 18274427</identifier><identifier>CODEN: AMSUAW</identifier><language>eng</language><publisher>Atlanta, GA: Southeastern Surgical Congress</publisher><subject>Antineoplastic Agents - therapeutic use ; Biological and medical sciences ; Breast cancer ; Fatal Outcome ; Female ; General aspects ; Hepatectomy ; Hospice care ; Humans ; Liver Neoplasms - diagnosis ; Liver Neoplasms - drug therapy ; Liver Neoplasms - surgery ; Lungs ; Medical sciences ; Middle Aged ; Patients ; Radiation therapy ; Rare diseases ; Sarcoma, Alveolar Soft Part - diagnosis ; Sarcoma, Alveolar Soft Part - drug therapy ; Sarcoma, Alveolar Soft Part - surgery ; Studies ; Tissues ; Tomography</subject><ispartof>The American surgeon, 2008, Vol.74 (1), p.43-46</ispartof><rights>2008 INIST-CNRS</rights><rights>Copyright Southeastern Surgical Congress Jan 2008</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c402t-919a5a47dce6bcb962e0944d8f9b8c37f72bb6918754c5806291959dd178a7123</citedby><cites>FETCH-LOGICAL-c402t-919a5a47dce6bcb962e0944d8f9b8c37f72bb6918754c5806291959dd178a7123</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>314,776,780,4009,27902,27903,27904</link.rule.ids><backlink>$$Uhttp://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&idt=20001077$$DView record in Pascal Francis$$Hfree_for_read</backlink><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/18274427$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>SHADDIX, Kyle K</creatorcontrib><creatorcontrib>FAKHRE, G. 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Alveolar soft-part sarcoma has been described as a primary lesion in the trunk, upper extremities, and retroperitoneum; more novel locations include the mediastinum, female genital tract, stomach, bone, and larynx. Numerous case reports describe alveolar soft-part sarcoma in diverse anatomic locations, but this report is, to our knowledge, the first documentation of primary alveolar soft-part sarcoma of the liver. We describe a 47-year-old woman with such a manifestation. 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| ispartof | The American surgeon, 2008, Vol.74 (1), p.43-46 |
| issn | 0003-1348 1555-9823 |
| language | eng |
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| source | Sage Journals Online |
| subjects | Antineoplastic Agents - therapeutic use Biological and medical sciences Breast cancer Fatal Outcome Female General aspects Hepatectomy Hospice care Humans Liver Neoplasms - diagnosis Liver Neoplasms - drug therapy Liver Neoplasms - surgery Lungs Medical sciences Middle Aged Patients Radiation therapy Rare diseases Sarcoma, Alveolar Soft Part - diagnosis Sarcoma, Alveolar Soft Part - drug therapy Sarcoma, Alveolar Soft Part - surgery Studies Tissues Tomography |
| title | Primary Alveolar Soft-Part Sarcoma of the Liver : Anomalous Presentation of a Rare Disease |
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