Table 1 Clinical features of 11 cases of primary bone PEComa
Author/Reference | Age (y)/Sex | Site | Size (cm) | Presentation | Radiologic features | Treatment | Follow-up |
|---|---|---|---|---|---|---|---|
Insabato et al. [2] | 30/M | Right proximal tibia | 2 | Pain | Osteolytic with cortical destruction | Local resection | ANED, 12mo |
Torri et al. [5] | 28/M | Right 6th rib | 2 | Pain | Osteolytic | Complete resection | ANED, not reported |
Righi et al. [4] | 92/F | Right fibula | NA | NA | NA | Local resection | NA |
Lian et al. [3] | 52/F | Right mid-shaft fibula | 6.3 | Progressive swelling | Extension through the cortex forming a soft-tissue mass | Wide excision | ANED, 3mo |
Yamashita et al. [6], case 1 | 35/M | 7th thoracic vertebra | 1.8 | Bilateral leg weakness, back pain | Osteolytic, destructive enhancing lesion (MRI) | CRT | Pelvic bone metastases, AWD,12mo |
Yamashita et al. [6], case 2 | 39/F | Right tibia | 6.5 | Pain | Enhancing mass with areas of breakthrough of the cortex forming a soft-tissue mass | RT + Excision | ANED,34mo |
Yamashita et al. [6], case 3 | 48/F | Right distal tibia | Very small | Pain | Permeable destructive lesion with soft tissue extension (recurrent lesion) | Excisional biopsy, amputation | Recurred 3 times in 3 y, ANED, 3y |
Kazzaz et al. [7] | 26/M | 5th lumbar vertebra | Large | Lower back pain, left leg weakness | Destructive lesion with extra-osseous mass | Conservative | Lung metastases, ANED, not reported |
Desy et al. [8], case 1 | 93/F | Right distal fibula | NA | Progressive pain, swelling | Expansile lytic lesion | Local resection | ANED,2y |
Desy et al. [8], case 2 | 29/M | Left acetabulum | 5 | Progressive hip pain | Extensive lytic with soft tissue expansion | Left hemipelvectomy + temsirolimus | Lung metastases, DOD, 8mo |
Lao et al. (our case) | 47/M | Left femur | 5.2 | Pain, progressive swelling | Osteolytic mass, destruction of cortex forming a soft-tissue mass | Curettage + CRT | Lung metastases, AWD, 3.5y |