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 .
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).
Pulmonary artery intimal sarcoma
diagnosed by percutaneous transcatheter aspiration.
, 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)).