WHO classification of soft tissue tumors (2002)

WHO classification of soft tissue tumors (2002)

1. adipocytic tumors (fatty tumors)

2. fibroblastic tumors/myofibroblastic tumors

3. so-called fibrohistiocytic tumors

4. smooth muscle tumors

4A. benign smooth muscle tumors

 leiomyoma (cutaneous, deep, pleomorphic)
 angiomyoma (vascular leiomyoma)
 epithelioid leiomyoma
 intravenous leiomyomatosis
 leiomyomatosis peritonealis disseminata

4B. malignant smooth muscle tumors

 leiomyosarcoma

• epithelioid leiomyosarcoma

5. pericytic tumors (perivascular tumors)

 glomus tumor
 glomangiomyoma
 hemangiopericytoma
 malignant glomus tumor
 malignant hemangiopericytoma

6. skeletal muscle tumors

 rhabdomyoma
 rhabdomyosarcomas

• embryonal rhabdomyosarcoma
• alveolar rhabdomyosarcoma

6A. benign skeletal muscle tumors

 adult rhabdomyoma
 genital rhabdomyoma
 fetal rhabdomyoma
 intermediate rhabdomyoma or cellular rhabdomyoma

6B. malignant skeletal muscle tumors (rhabdomyosarcomas)

 embryonal rhabdomyosarcoma
 alveolar rhabdomyosarcoma
 morphological variants

• botryoid rhabdomyosarcoma
• spindle cell rhabdomyosarcoma
• pleomorphic rhabdomyosarcoma
• rhabdomyosarcoma with ganglionic differentiation (ectomesenchymoma)

6. vascular tumors (tumors of blood and lymph vessels)

6A. Benign vascular tumors
 papillary endothelial hyperplasia
 hemangiomas

• capillary hemangioma
• juvenile hemangioma
• cavernous hemangioma
• venous hemangioma

 epithelioid hemangioma (angiolymphoid hyperplasia, histiocytoid hemangioma)
 granulation-type hemangioma (pyogenic granuloma)
 tufted hemangioma
 deep hemangiomas

• intramuscular hemangioma
• synovial hemangioma
• perineural hemangioma

 lymphangioma
 lymphangiomyoma
 lymphangiomyomatosis
 angiomatosis
 lymphangiomatosis
 hemangioendothelioma

• epithelioid hemangioendothelioma
• endovascular papillary angioendothelioma (Dabska tumor)
• spindle cell hemangioendothelioma

6B. maligant vascular tumors
 angiosarcoma
 lymphangiosarcoma
 Kaposi sarcoma

7. synovial tumors

 tenosynovial giant cell tumor

• localized tenosynovial giant cell tumor
• diffuse tenosynovial giant cell tumor (extraarticular pigmented villonodular synovitis, florid tenosynovitis)

 synovial sarcoma
 malignant giant cell tumor of tendon sheath

8. mesothelial tumors

 solitary fibrous tumor of pleura and peritoneum (localized fibrous mesothelioma)
 multicystic mesothelioma
 adenomatoid tumor
 well-differentiated papillary mesothelioma
 malignant solitary fibrous tumor of pleura and peritoneum
 diffuse mesothelioma

9. neural tumors and neuroectodermal tumors

 traumatic neuroma
 Morton neuroma
 multiple mucosal neuromas
 neuromuscular hamartoma (benign Triton tumor)
 nerve sheath ganglion
 schwannoma (neurilemoma)
 schwannomatosis
 neurothekeoma (nerve sheath myxoma)
 neurofibroma
 granular cell tumor
 melanocytic schwannoma
 ectopic meningioma
 ectopic ependymoma
 ganglioneuroma
 pigmented neuroectodermal tumor of infancy (retinal anlage tumor, melanotic progonoma)
 malignant peripheral nerve sheath tumor (MPNST) (malignant schwannoma, neurofibrosarcoma)
 malignant granular cell tumor
 clear cell sarcoma (malignant melanoma of soft parts)
 malignant melanocytic schwannoma
 gastrointestinal autonomous nerve tumor (GANT or plexosarcoma)
 peripheral primitive neuroectodermal tumor (pPNET)
 neuroblastoma
 ganglioneuroblastoma
 peripheral neuroectodermal tumor (neuroepithelioma)
 extraskeletal Ewing sarcoma

10. paragangliomas (paraganglionic tumors)

 paraganglioma (extra-adrenal pheochromocytoma)
 malignant paraganglioma

11. Extraskeletal cartilaginous and osseous tumors

11A. Benign tumors
 Panniculitis ossificans and myositis ossificans
 Fibroosseous pseudotumor of the digits
 Fibrodysplasia (myositis) ossificans progressiva
 Extraskeletal chondroma or osteochondroma
 Extraskeletal osteoma

11B. Malignant tumors
 Extraskeletal chondrosarcoma
 Well-differentiated chondrosarcoma
 Myxoid chondrosarcoma
 Mesenchymal chondrosarcoma
 Extraskeletal osteosarcoma

13. Pluripotential mesenchymal tumors

 mesenchymoma
 malignant mesenchymoma

14. Miscellaneous tumors

14A. Benign tumors

 Congenital granular cell tumor
 Tumoral calcinosis
 Myxoma

• Cutaneous myxoma
• Intramuscular myxoma
• Juxta-articular myxoma

 Angiomyxoma
 Amyloid tumor
 Parachordoma
 Ossifying and nonossifying fibromyxoid tumors
 Palisaded myofibroblastoma of lymph node

14B. Malignant tumors

 Alveolar soft part sarcoma
 Epithelioid sarcoma
 Malignant extrarenal rhabdoid tumor
 Desmoplastic small cell tumor

15. Unclassified tumors