intimal sarcoma

intimal sarcoma

malignant mesenchymal tumor arising in large arteries, composed of fibroblastic or myofibroblastic cells, characterized by intraluminal growth and hematogenous embolization to distal sites.
References in periodicals archive ?
Anna Hackett, 33, was diagnosed with intimal sarcoma - an extremely rare condition which affects one in 10 million.
Intimal sarcoma is a rare mesenchymal malignancy arising in the intimal layer of the aorta or pulmonary artery and predominantly shows intraluminal growth [1].
A histological diagnosis was finally determined to be unclassified sarcoma, which included a possible intimal sarcoma. The patient was diagnosed with PAIS on the basis of clinical, radiological, and pathological correlations.
Dallaudiere, "Multiple skeletal muscle metastases revealing a cardiac intimal sarcoma," Skeletal Radiology, vol.
For example, MDM2 or CDK4 can show positivity in intimal sarcoma, pleomorphic rhabdomyosarcoma, a subset of malignant peripheral nerve sheath tumor, and myxofibrosarcoma.
MDM2 Positivity by IHC and FISH in Soft Tissue and Bone Tumors Tumor Type MDM2 by MDM2 by IHC FISH ALT/WD liposarcoma + + (nuclear) Dedifferentiated liposarcoma + + (diffuse, nuclear) Pleomorphic rhabdomyosarcoma - + Intimal sarcoma + + (up to 70%) Malignant peripheral nerve sheath tumor - + (subset) Myxofibrosarcoma - + (subset) Low-grade central osteosarcoma + + Conventional osteosarcoma + (10%) - Parosteal osteosarcoma + (>85%) + Undifferentiated high-grade pleomorphic + (17%) - sarcoma of bone Abbreviations: ALT/WD, atypical lipomatous tumor/well differentiated; FISH, fluorescence in situ hybridization; IHC, immunohistochemistry; MDM2, mouse double minute 2 homolog; +, positive; -, negative.
Pathology evaluation revealed intimal sarcoma of pulmonary artery, being undifferentiated in 7 cases (Table 2).
Intimal sarcoma, a malignant neoplasm arising from large central vessels, most commonly the pulmonary artery, shows MDM2 amplification and overexpression in 75% (6 of 8) of cases.
The tumor was diagnosed as intimal sarcoma and its histological findings, proliferation of atypical spindle or polygonal cells with high mitotic activity, were also compatible with the diagnosis (Figure 3(c)).