概述
Enchondroma is a benign indolent intramedullary hyaline cartilage neoplasm拒迅。Accounts for 10% of all benign osseous tumors骚秦。Limited growth, most lesions are less than 5 cm in maximal dimension她倘。
一般沒癥狀,除非發(fā)生病理性骨折作箍。大約50%發(fā)生在手或足上硬梁,而這其中大部分發(fā)生在指/趾骨上。肱骨及股骨近端是最常見的長骨發(fā)病部位胞得。中軸骨(骨盆荧止、脊柱、肋骨)等不常見懒震,但該部位的腫瘤常常具有惡性傾向(軟骨肉瘤)罩息。
分型
Solitary Enchondroma
Multiple Enchondromas (Enchondromatosis/Ollier's Disease)
Maffucci Syndrome (Multiple Enchondromas and Soft Tissue Hemangiomas)
影像學特征
髓腔內(nèi)生軟骨瘤表現(xiàn)為溶骨性。腫瘤上層皮質(zhì)骨變薄个扰,一旦出現(xiàn)病理性骨折瓷炮,皮質(zhì)骨仍保存完整,也不會出現(xiàn)軟組織腫塊递宅。若皮質(zhì)骨受到侵蝕或變厚娘香,出現(xiàn)軟組織團塊,往往是內(nèi)生軟骨瘤惡變的征象办龄。
組織學特征
內(nèi)生軟骨瘤由分化良好的透明軟骨組成烘绽。長骨內(nèi)生軟骨瘤表現(xiàn)為軟骨細胞減少和無細胞非典型增生,豐富的藍染軟骨樣基質(zhì)俐填,很少能找到雙核細胞安接。和內(nèi)生軟骨瘤相比,手足和骨膜等處的軟骨瘤表現(xiàn)為細胞非典型增生英融,但仍不能診斷為惡性盏檐。在長骨的軟骨瘤昙读,基質(zhì)粘液樣改變高度提示惡變大脉;但在手足的小塊骨,小幅度基質(zhì)粘液樣變不能斷定是軟骨肉瘤敬飒。惡性腫瘤可以穿透骨小梁痕鳍,良性腫瘤不會穿透骨小梁破壞皮質(zhì)骨硫豆。
鑒別診斷
The main differential is with a low grade (Grade 1) chondrosarcoma
Can be very difficult to differentiate from a Grade 1 Chondrosarcoma from an enchondroma based on histology
It is easy to differentiate from a grade 2 or 3 chondrosarcoma by means of histology which are hypercellular, pleomorphic and demonstrate mitotic figures
Radiographs and clinical history are important for differentiation
Biopsies are not useful for differentiating an enchondroma from a low grade chondrosarcoma
Features consistent with chondrosarcoma
Pain attributable to lesion
Age greater than 50
Cortical destruction and a soft tissue mass
Periosteal reaction and thickening
Endosteal erosion>2/3 cortical thickness on a CT scan
Size greater than 5 cm
Bone Scan: Lesion that is hotter than ASIS
治療
內(nèi)生軟骨瘤應(yīng)行刮除術(shù),必要時骨移植修復骨缺損笼呆。如果腫瘤生長活躍或患者感覺疼痛熊响,活檢和刮除術(shù)應(yīng)同時應(yīng)用。如果刮除干凈诗赌,術(shù)后腫瘤復發(fā)率極低汗茄。因為腫瘤很少侵犯皮質(zhì)骨,所以只需刮除腫瘤即可境肾,有時手術(shù)邊界可擴展到周圍1~2mm皮質(zhì)骨剔难。
Enchondromas are benign, indolent (not growing) tumors
Indications for surgery:Digits: Impending or actual pathological fracture,Intralesional curettage and bone graft or cement奥喻;Long bones: Rare to fracturea偶宫,usually observe
If grows it is considered chondrosarcoma and would recommend surgery accordingly
預后
Recurrence rate following curettage is <5%。
Recurrence of an enchondroma suggests malignancy环鲤。
There are rare cases where enchondromas can dedifferentiate into a chondrosarcoma or dedifferentiated chondrosarcoma (low grade chondrosarcoma with an osteosarcoma, fibrosarcoma or malignant fibrous histiocytoma arising adjacent to it)纯趋。
參考文獻:
1、http://www.tumorsurgery.org/
2冷离、王正義 主編《足踝外科學》人民衛(wèi)生出版社吵冒,2006年