Myxoinflammatory fibroblastic sarcoma and acral fibromyxoma typically involve the distal extremities and the subcutaneous tissues 2. Myxoid liposarcomas and extraskeletal myxoid chondrosarcomas are typically located within the lower extremities hips, buttocks and trunk 2,6. Intramuscular myxomas will most often occur intramuscularly and in rare occasions intermuscular or subcutaneous the thigh is the most commonly affected extremity 2,5.Īggressive angiomyxomas are typically found in the perineal area of women in their 20s to 40s 2,3.
Most myxoid soft tissue tumors will be found in the extremities and less often in the trunk 2,3. Macroscopic, microscopic appearances markers and genetics will depend on each separate entity. It is often only a secondary phenomenon in the latter and is more often an intrinsic part of the respective mesenchymal soft tissue tumor 4. The myxoid extracellular matrix itself is a histological feature in diverse physiological and pathological conditions 6 and is also found in mesenchymal and epithelial tumors. Myxoid tumors produce a myxoid extracellular matrix, which is quite heterogeneous among different tumors basically composed of different proteoglycans and glycosaminoglycans (GAGs) 1-4 of which none is specific for a lesion and hyaluronic acid is the most common 4. The diagnosis is established by histological evidence of a myxoid extracellular matrix.
Some tumors as extraskeletal myxoid chondrosarcomas or aggressive angiomyxomas can cause pain and tenderness the latter in the perineum 2,3. Clinical presentationĭepending on the entity most patients will present with a lump or other painless soft tissue mass somewhere in their extremities or trunk, or they are entirely asymptomatic.
Myxoid chondrosarcomas, ossifying fibromyxoid tumors, low-grade fibromyxoid sarcoma and myxoinflammatory fibroblastic sarcomas are rare variants from the malignant group 2,3 and superficial angiomyxoma, myxolipoma and acral fibromyxoma are rare benign myxoid tumors 2. Fairly frequent malignant myxoid tumors include myxoid liposarcoma making up 20-50% of all liposarcomas 3, myxofibrosarcoma, formerly known as myxoid malignant fibrous histiocytoma, which is a common malignant soft tissue tumor in the elderly 3. Aggressive angiomyxoma is a locally aggressive benign variant affecting mostly women 2,3. Incidence depends entirely on the specific tumor with intramuscular myxoma being the most common 2,3 from the benign lesions.
0 kommentar(er)
