About: Alveolar Soft Part Sarcoma     Goto   Sponge   NotDistinct   Permalink

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AttributesValues
rdf:type
rdfs:label
  • Alveolar Soft Part Sarcoma
equivalentClass
Concept_In_Subset
Semantic_Type
  • Neoplastic Process
Preferred_Name
  • Alveolar Soft Part Sarcoma
UMLS_CUI
  • C0206657
Contributing_Source
  • CTEP
ALT_DEFINITION
  • A soft tissue tumor that is most common in older children and teenagers. It begins in the soft supporting tissue that connects and surrounds the organs and other tissues. Alveolar soft part sarcoma usually occurs in the legs, but can also occur in the arms, hands, head, or neck. It can cause the growth of new blood vessels that help the tumor grow and spread.NCI-GLOSS
  • A tumor predominantly affecting adolescents and young adults and composed of large, uniform epithelioid cells with abundant eosinophilic granular cytoplasm arranged in solid nests and/or solid structures. There is a recurrent cytogenetic aberration: der (17)t(X;17)(p11;q25).NCI
ICD-O-3_Code
  • 9581/3
Neoplastic_Status
  • Malignant
Legacy_Concept_Name
  • Alveolar_Soft_Part_Sarcoma
FULL_SYN
  • ASPSPTNCI-GLOSSCDR0000641934
  • alveolar soft part sarcomaPTNCI-GLOSSCDR0000641933
  • Alveolar Soft Part SarcomaPTNCI
  • ASPSABNCI
  • Alveolar soft part sarcomaPTCTEP10001886
DEFINITION
  • A rare malignant neoplasm characterized by the presence of large epithelioid cells with abundant cytoplasm forming nests and pseudoalveolar structures. The groups of the epithelioid cells are separated by thin-walled sinusoidal spaces. It occurs most often in adolescents and young adults. In adults the most common sites of involvement are the extremities, and in infants and children, the head and neck. It usually presents as a slowly growing mass and it frequently metastasizes to other anatomic sites. The most common sites of metastasis are the lungs, bone, and brain.NCI
code
  • C3750
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