alveolar soft part sarcoma


Also found in: Dictionary, Thesaurus, Encyclopedia, Wikipedia.

sarcoma

 [sahr-ko´mah] (pl. sarcomas, sarco´mata.)
A tumor, often highly malignant, composed of cells derived from connective tissue such as bone, cartilage, muscle, blood vessel, or lymphoid tissue; sarcomas usually develop rapidly and metastasize through the lymph channels. Different types are named for the specific tissue they affect: fibrosarcoma in fibrous connective tissue; lymphosarcoma in lymphoid tissues; osteosarcoma in bone; chondrosarcoma in cartilage; rhabdosarcoma in muscle; and liposarcoma in fat cells. adj., adj sarco´matous.
Abernethy's sarcoma a malignant fatty tumor occurring mainly on the trunk.
alveolar soft part sarcoma one with a reticulated fibrous stroma enclosing groups of sarcoma cells enclosed in alveoli walled with connective tissue.
botryoid sarcoma (sarcoma botryoi´des) an embryonal rhabdomyosarcoma arising in submucosal tissue, usually in the upper vagina, cervix uteri, or bladder neck in young children and infants, presenting grossly as a polypoid grapelike structure.
endometrial stromal sarcoma a pale, polypoid, fleshy, malignant tumor of the endometrial stroma.
Ewing's sarcoma a malignant tumor of the bone that arises in medullary tissue, usually found in cylindrical bones, with pain, fever, and leukocytosis as prominent symptoms; called also Ewing's tumor.
giant cell sarcoma a malignant form of giant cell tumor of bone.
granulocytic sarcoma chloroma.
immunoblastic sarcoma of B cells an aggressive B-cell lymphoma believed to arise from transformed interfollicular B lymphocytes, which in many cases is associated with a preexisting immunologic disorder, e.g., Sjögren's syndrome, systemic lupus erythematosus, or Hashimoto's thyroiditis, or with an immunocompromised state.
immunoblastic sarcoma of T cells a group of T-cell lymphomas comprising tumors derived from T lymphocytes in the paracortical area arising from a mixture of small lymphocytes and many large transformed cells; the latter are characterized by one or more small but distinctly eosinophilic nucleoli.
Kaposi sarcoma a multicentric, malignant neoplastic vascular proliferation characterized by bluish red cutaneous nodules, usually on the lower extremities, most often on the toes or feet; the nodules slowly increase in size and number and spread to more proximal sites. Tumors often remain confined to skin and subcutaneous tissue, but widespread visceral involvement may occur. The condition occurs endemically in certain parts of Central Africa and Central and Eastern Europe, and a particularly virulent and disseminated form occurs in immunocompromised patients such as transplant recipients and those with acquired immunodeficiency syndrome. Human herpesvirus 8 has been implicated as a causative agent.
lymphatic sarcoma diffuse lymphoma.
osteogenic sarcoma a malignant primary tumor of bone composed of a malignant connective tissue stroma with evidence of osteoid, bone, and/or cartilage formation; depending upon the dominant component, classified as osteoblastic, fibroblastic, or chondroblastic.
pseudo–Kaposi sarcoma unilateral subacute to chronic dermatitis, often with postinflammatory hyperpigmentation, occurring in association with underlying arteriovenous fistula, which closely resembles Kaposi sarcoma both clinically and histologically.
reticulum cell sarcoma histiocytic lymphoma.

al·ve·o·lar soft part sar·co·ma

[MIM*606243]
a malignant tumor formed of a reticular stroma of connective tissue enclosing aggregates of large round or polygonal cells; occurs in subcutaneous and fibromuscular tissues.

alveolar soft part sarcoma

A sarcoma most common in deep tissue of paediatric/adolescent legs (especially female), or paediatric head and neck; less common in extremities of males <age 50.
Histogenesis Uncertain.
Management Wide excision.
Prognosis Larger tumours are more aggressive; grading is not recommended.

al·ve·o·lar soft part sar·co·ma

(al-vē'ŏ-lăr sawft pahrt sahr-kō'mă)
A malignant tumor formed of a reticular stroma of connective tissue enclosing aggregates of large round or polygonal cells; occurs in subcutaneous and fibromuscular tissues.
References in periodicals archive ?
Ordonez, "Alveolar soft part sarcoma: a review and update," Advances in Anatomic Pathology, vol.
Wang, "Imaging findings and histologic appearances of alveolar soft part sarcoma in the prostate: a case report and review of the literature," Clinical Genitourinary Cancer, vol.
Alveolar soft part sarcoma is a rare soft tissue sarcoma that presents most commonly within the lower extremity fascial planes or skeletal muscle of young females.
Alveolar soft part sarcoma is consistently positive for an antibody that detects the carboxyl terminal portion of the transcription factor E3 (TFE3) gene retained in the fusion protein.
Primary renal neoplasms with the ASPL-TFE3 gene fusion of alveolar soft part sarcoma: a distinctive tumor entity previously included among renal cell carcinomas of children and adolescents.
Nuclear expression of TFE3 is seen in a variety of different tumors, most of which harbor TFE3 gene fusions, including alveolar soft part sarcoma, Xp11 translocation renal cell carcinoma, "melanotic" Xp11 translocation renal cell carcinoma, and a subset of PEComas and epithelioid hemangioendotheliomas.
JSA was set up by Cramlington's Jessica Robson before she died from alveolar soft part sarcoma aged 18 last May, and has been taken on by mum Julie, dad Trevor and sister Nicole in an eort to raise awareness of the illness and support other ill children in the North East.
Jessica died last May, aged 18, after a near four-year battle with alveolar soft part sarcoma. She had set up her own charity to raise awareness of the condition and help other children suffering from it.
Jessica Robson, of Cramlington, lost her near four-year battle with alveolar soft part sarcoma in May - but not before she raised more than PS28,000 for the Teenage Cancer Trust (TCT) and Jessica's Sarcoma Awareness, which she set up herself.
Alveolar soft part sarcoma is another rare tumor of uncertain histogenesis, most commonly diagnosed in the extremities of young adults, and with a tendency to metastasize.
Jessica Robson, of Cramlington, lost her near four-year battle with alveolar soft part sarcoma in May - but not before she raised more than [pounds sterling]28,000 for the Teenage Cancer Trust (TCT) and Jessica's Sarcoma Awareness, which she set up herself.
While was ill, The 18-year-old passed away following a near four-year battle with alveolar soft part sarcoma, which began when she discovered a small lump in her right leg while on holiday.