WHO肿瘤新分类由世界各国著名病理、肿瘤、遗传学家共同制定，于2000年由IARC开始出版并在全世界发行。该系列丛书中包括发病率、临床特点、分类、诊断标准、基因遗传学改变等，是目前国际上最具权威性的肿瘤分类，是病理及肿瘤诊断及治疗标准化的指南。目前已出版神经、消化、淋巴造血、软组织和骨、乳腺和女性生殖等12部。
   
肿瘤病理分类的变化总趋势是简化、易于操作。以往分类主要根据瘤细胞的形态学表现，WHO新分类充分考虑到形态学、免疫类型及遗传学特点，因此重复性更强，也有更好的临床相关性（病程发展、预后、疗效），已越来越得到国内外普遍的认可。

在过去的10～15年间，细胞遗传学和分子遗传学领域知识的迅猛增长对软组织肿瘤分类有着重要的影响，也影响了我们对软组织肿瘤病因学的理解。反映在新分类中的主要变化包括对生物学行为类型进行了修正，将中间性恶性的2种不同类型，分别认定为“局部侵袭性”和“偶尔有转移”。新分类最重要的是认可了恶性纤维组织细胞瘤（MFH，实际上代表多形性未分化肉瘤）和血管周细胞瘤（多数病例与孤立性纤维性肿瘤密切相关）定义不明的性质。所谓的血管瘤样MFH和骨外黏液样软骨肉瘤分化谱系的不确定性，使其再次归入不能确定分化的肿瘤类型。然而，在这些肿瘤类型能被更好地理解之前，工作组避免更改名称，因为担心对日常的临床实践造成混淆。自1994年分类以来，许多新确定的病种已经被采纳，而且进行与临床相关的更为明确的软组织肿瘤亚分类的趋势似乎在将来仍将继续下去。

2002年WHO肿瘤新分类中，推荐按生物学潜能将软组织肿瘤分为4种类型：良性、中间性(局部侵袭性)、中间性(偶见转移)和恶性。

This terminology should not be confused with the grading system mentioned above, in which grade 2 may be regarded as intermediate.

Fletcher CDM,Unni KK,Mertens F. World health organization classification of tumours. Pathology and genetics of tumours of soft tissue and bone. Lyon: IARC Press, 2002.

软组织肿瘤WHO新分类（2002）

脂肪细胞肿瘤（adipocytic tumours）
良性
脂肪瘤 （lipoma）                           8850/0
脂肪瘤病（lipomatosis）                   8850/0
神经脂肪瘤病（lipomatosis of nerve）                        8850/0
脂肪母细胞瘤（lipoblastoma）/脂肪母细胞瘤病（lipoblastomatosis）     8881/0
血管脂肪瘤（angiolipoma）                           8861/0
平滑肌脂肪瘤（myolipoma）                               8890/0
软骨样脂肪瘤（chondroid lipoma）                        8862/0
肾外血管平滑肌脂肪瘤（extrarenal angiomyolipoma）             8860/0
肾上腺外髓性脂肪瘤（extra-adrenal myelolipoma）                 8870/0
梭形细胞（spindle）/                                        8857/0
多形性脂肪瘤 （pleomorphic lipoma）                             8854/0
冬眠瘤（hibernoma）                                         8880/0

中间性（局部侵袭性）
非典型脂肪瘤性肿瘤（atypical lipomatous tumour）/
分化良好型脂肪肉瘤（well differetiated liposarcoma）                8851/3
恶性
去分化脂肪肉瘤（dedifferentiated liposarcoma）               8858/3
黏液样脂肪肉瘤（myxoid  liposarcoma）                        8852/3
圆细胞脂肪肉瘤（round cell liposarcoma）                      8853/0
多形性脂肪肉瘤（pleomorphic liposarcoma）                    8854/3
混合型脂肪肉瘤（mixed-type liposarcoma）                     8855/3
脂肪肉瘤，无其它特异性（liposarcoma,not otherwise specified)         8850/0

纤维母细胞/肌纤维母细胞肿瘤（fibroblastic/myofibroblastic tumours）
良性
结节性筋膜炎（nodular fasciitis）
增生性筋膜炎（proliferative fasciitis）
增生性肌炎（proliferative myositis）
房间隔缺损7（myositis ossificans）
指（趾）纤维骨性假瘤（fibro-osseous pseudotumour of digits）
缺血性筋膜炎（ischaemic fasciitis）
弹力纤维瘤 （elastofibroma）                             8820/0
婴儿纤维性错构瘤（fibrous hamartoma of infancy）
肌纤维瘤/肌纤维瘤病 （myofibroma/myofibromatosis）             8824/0
颈纤维瘤病（fibromatosis colli）
幼年性透明性纤维瘤病（juvenile hyaline fibromatosis）
包涵体纤维瘤病 （inclusion body fibromatosis）
腱鞘纤维瘤 （fibroma of tendon sheath）                     8810/0
纤维组织增生性纤维母细胞瘤（desmoplastic fibroblastoma）               8810/0
乳腺型肌纤维母细胞瘤 （mammary-type myo fibroblastoma）               8825/0
钙化性腱膜纤维瘤（calcifying aponeurotic fibroma）                8810/0
血管肌纤维母细胞瘤（angiomyo fibroblastoma）                        8826/0
细胞性血管纤维瘤（cellular angiofibroma）                          9160/0
项型纤维瘤  （nuchal-type fibroma）                             8810/0
Gardner纤维瘤 （Gardner fibroma）                           8810/0
钙化性纤维性肿瘤（calcifying fibrous tumour）
巨细胞血管纤维瘤 （giant cell angiofibroma）                    9160/0

中间性（局部侵袭性）
浅表性纤维瘤病（掌/跖）（superficial fibromatoses(palmar/plantar)）
韧带样型纤维瘤病 （desmoid-type fibromatoses）                     8821/1
脂肪纤维瘤病（lipofibromatosis）

中间性（偶尔有转移）
孤立性纤维性肿瘤（solitary fibrous tumour）                  8815/1
和血管周细胞瘤（haemangiopericytoma）
（包括脂肪瘤性血管周细胞瘤）(incl.lipomatous haemangiopericytoma)
炎性肌纤维母细胞性肿瘤（inflammatory myofibroblastic tumour）            8825/1
低度恶性肌纤维母细胞肉瘤（low grade myofibroblastic sarcoma）             8825/3
黏液样炎性纤维母细胞肉瘤（myxoinflammatory fibroblastic sarcoma）         8811/3
婴儿纤维肉瘤（infantile fibrosarcoma）                                8814/3

恶性
成人纤维肉瘤（adult fibrosarcoma）                               8810/3
黏液样纤维肉瘤 （myxofibrosarcoma）                           8811/3
低度恶性纤维黏液样肉瘤（low grade fibromyxoid sarcoma）                8811/3
透明性梭形细胞肿瘤（hyalinizing spindle cell tumour）
硬化性上皮样纤维肉瘤 （sclerosing epithelioid fibrosarcoma）             8810/3

所谓的纤维组织细胞性肿瘤（so-called fibrohistiocytic tumours）
良性
腱鞘巨细胞肿瘤（giant cell tumour of tendon sheath）                   9252/0
弥漫型巨细胞肿瘤（diffuse-type giant cell tumour）                  9251/0
深部良性纤维组织细胞瘤 （deep benign fibrous histiocytoma）            8830/0

中间性（偶尔有转移）
丛状纤维组织细胞肿瘤 （plexiform fibrohistiocyticv tumour）               8835/1
软组织巨细胞肿瘤 （giant cell tumour of soft tissues）                9251/1

恶性
多形性“恶性纤维组织细胞瘤”（pleomorphic ‘MFH’）/
未分化多形性肉瘤（undifferentiated pleomorphic sarcoma）              8830/3
巨细胞“恶性纤维组织细胞瘤”（giant cell ‘MFH’）/
未分化多形性肉瘤伴巨细胞（undifferentiated pleomorphic sarcoma with giant cells）         8830/3
炎症性“恶性纤维组织细胞瘤”（inflammatory ‘MFH’）/
未分化多形性肉瘤伴明显炎症（undifferentiated pleomorphic sarcoma with prominent inflammation） 8830/3

平滑肌肿瘤（smooth muscle tumours）
血管平滑肌瘤（angioleiomyoma）                           8894/0
深部平滑肌瘤（deep leiomyoma）                        8890/0
生殖道平滑肌瘤（genital leiomyoma）                       8890/0
平滑肌肉瘤（leiomyosarcoma）（不包括皮肤）               8890/3

周细胞（血管周细胞）肿瘤（pericytic(perivascular) tumoues）
血管球瘤（glomus tumour）（和变型）                8711/0
恶性血管球瘤（malignant glomus tumour）                 8711/3
肌周细胞瘤（myopericytoma）                                8713/1

骨骼肌肿瘤（skeletal muscle tumours）
良性
横纹肌瘤（rhabdomyoma）                                8900/0
 成人型（adult type）                                     8904/0
 胎儿型（fetal type）                                      8903/0
 生殖道型（genital type）                                  8905/0

恶性
胚胎性横纹肌肉瘤（embryonal rhabdomyosarcoma）                 8910/3
（包括梭形细胞、                                         8912/3
葡萄簇、间变性）                                               8910/3
腺泡状横纹肌肉瘤（alveolar rhabdomyosarcoma）（包括实性、间变性）      8920/3
多形性横纹肌肉瘤（pleomorphic rhabdomyosarcoma）                   8901/3

脉管肿瘤（vascular tumours）
良性
血管瘤（haemangiomas）
皮下/深部软组织                                           9120/0
毛细血管性                                              9131/0
海绵状                                                  9121/0
动静脉性                                                9123/0
静脉性                                                  9122/0
肌内                                                9132/0
滑膜                                        9120/0
上皮样血管瘤（epithelioid haemangioma）                     9125/0
血管瘤病（angiomatosis）
淋巴管瘤（lymphangioma）                                       9170/0

中间性（局部侵袭性）
卡波西样血管内皮瘤（kaposiform haemangioendothelioma）              9130/1

中间性（偶尔有转移）
网状血管内皮瘤（retiform haemangioendothelioma）                    9135/1
淋巴管内乳头状内皮瘤（papillary intralymphatic angioendothelioma）       9135/1
组合性血管内皮瘤（composite haemangioendothelioma）                   9130/1
卡波西肉瘤（kaposi sarcoma）                                            9140/3

恶性
上皮样血管内皮瘤（epithelioid haemangioendothelioma）                 9133/3
软组织血管肉瘤（angiosarcoma of soft tissue）                         9120/3

软骨-骨肿瘤（chondro-osseous tumours）
软组织软骨瘤（soft tissue chondroma）                              9220/0
间叶性软骨肉瘤（mesenchymal chondrosarcoma）                    9240/3
骨外骨肉瘤（extraskeletal osteosarcoma）                          9180/3

不能确定分化的肿瘤（tumours of uncertain differentiation）良性
肌内黏液瘤（intramuscular myxoma）                            8840/0
（包括细胞性变型）
关节旁黏液瘤（juxta-articular myxoma）                          8840/0
深部（“侵袭性”）血管黏液瘤（deep (‘aggressive’) angiomyxoma）      8841/0
多形性透明变性血管扩张性肿瘤（pleomorphic hyalinizing angiectatic tumour）
异位错构瘤性胸腺瘤（ectopic haemartomatous thymoma）           8587/0

中间性（偶尔有转移）
血管瘤样纤维组织细胞瘤（angiomatoid fibrous histocytoma）           8836/1
骨化性纤维黏液样肿瘤（ossifying fibromyxoid tumour）                 8842/0
（包括非典型/恶性）
混合性肿瘤（mixed tumour）/                                                           8940/1
   肌上皮瘤（myoepithelioma）/                                                       8982/1
   副脊索瘤（parachordoma）                                    9373/1

恶性
滑膜肉瘤（synovial sarcoma）                                  9040/3
上皮样肉瘤（epithelioid sarcoma）                            8804/3
腺泡状软组织肉瘤（alveolar soft part sarcoma）                 9581/3
软组织透明细胞肉瘤（clear cell sarcoma of soft tissue）          9044/3
骨外黏液样软骨肉瘤（extraskeletal myxoid chondrosarcoma）        9231/3
（“脊索样”型）
PNET/骨外尤文肿瘤（extraskeletal Ewing tumour）
 pPNET                                                                        9364/3
骨外尤文肿瘤（extraskeletal Ewing tumour）          9260/3
纤维组织增生性小圆细胞肿瘤（desmoplastic small round cell tumour）      8806/3
肾外横纹样肿瘤（extra-renal rhabdoid tumour）                        8963/3
恶性间叶瘤（malignant mesenchymoma）                          8890/3
具有血管周上皮样细胞分化的肿瘤（neoplasms with perivascular epithelioid cell differentiation，PEComa）
透明细胞肌黑色素细胞肿瘤（clear cell myomelanocytic tumour）
血管内膜肉瘤（intimal sarcoma）                        8800/3

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