Individuals presenting with<unk>  tumors are 3 to 87 years old (median age 44.6 years) with most individuals aged 30-60 years. These tumors involve the lower limb and shoulder areas (28.3% of cases), trunk (18.7%), head and neck areas (11.7%), lung and its pleura (10.0%), bone (including the spinal vertebrae) (10.0%), soft tissues (9.1%), upper limb and shoulder areas (7.5%), kidney (3.9%), pancreas (0.9%), liver (0.4%), and brain (0.4%). Rare cases of<unk>  tumors have developed in the ovary and lower gastrointestinal tract. In two separate studies, 17% and 27% of patients presented with metastases at the time of the initial diagnosis of their disease. While most individuals present with a painless mass, about 33% report having a painful and enlarging mass. The tumor may have been noticed and even painful for a few months to years. Tumors developing in the head, abdominal cavity, or other space-constrained sites often present with symptoms and signs related to their tumors' mass effects. For example, 5 of 5 patients diagnosed with<unk>  tumors in the spinal vertebrae presented with pain that they had experienced for 3 to 6 months and 2 of 2 patients with a<unk>  located in the buttock presented with highly painful sciatica.<unk>  tumors have varied in size from 1 to 25 cm in diameter (average diameter, 8.3 cm). In one small study, all individuals treated with surgical resection of their tumors, re-presented with recurrences of their tumors at the sites of their surgical removal and 91% re-presented with metastases (67% to the lung, 50% to bone, and 1 case each to the liver, brain and abdomen).
Sclerosing epithelioid fibrosarcoma
Angiomyofibroblastoma
Perivascular epithelioid cell tumour
Low-grade fibromyxoid sarcoma
Lymphoepithelioma-like carcinoma
Multinucleate cell angiohistiocytoma
Undifferentiated pleomorphic sarcoma
Plexiform angiomyxoid myofibroblastic tumor