Three cases are reported in which an osteosarcoma developed in relation to an enchondroma in a long bone. Two of the cases were in the proximal femur whilst one occurred in the proximal humerus, both recognised sites for old calcified enchondromas or "cartilage rests". The ages of the patients at presentation were 55, 63 and 84 years and all were women. Two patients died with pulmonary metastases within six months of the onset of clinical symptoms. Despite their intimate relationship to the enchondromas, none of the osteosarcomas could be shown histologically to have arisen from tumour cartilage. It appears probable that these are cases in which independently arising tumours have merged to form a so-called "collision" tumour, but the possibility that they could have been derived by dedifferentiation of a previously benign neoplasm cannot be discounted

Purpose of the study: The proximal humerus is a common localization for solitary endchondroma. Levy (. Clin Orthop. 2004. , . 431. ) emphasized the frequency of associated muscle and tendon disease. Treatment is generally curettage-autograft filling. Use of calcium phosphate bone substitute has been validated (A. Uchida et al. J Bone Joint Surg (Br) 90, F. Gouin Rev Chir Orthop 95, R. Mirzayan J Bone Joint Surg (Am) 2001). This retrospective analysis was conducted to determine the signs and symptoms and report the results of surgical treatment obtained in a consecutive series of 15 patients with metaphyseal enchondroma treated in the same unit. Material and methods: This series included twelve women and three men, mean age 48.2 years (range 38–73). All complained of pain. Two also had signs of calcification and six presented a cuff tendinopathy. Eight had had one or more joint injections. On average, the enchondromas measured 3.1 cm on the ap view and 3.6 cm on the lateral view. Magnetic resonance imaging (MRI) demonstrated the presence of a subacromial effusion in 13/16 shoulders, supraspinatus tendinopathy in six, calcifications in three, and acromioclavicular arthropathy in three. Curettage was followed by filling with biphased tricalcium phosphate (SBM, Lourdes) associated in nine shoulders with acromioplasty-bursectomy and in two with resection of a calcification. Results: There were no postoperative complications. Mean follow-up was six months. All patients recovered joint motion, seven were pain free, six complained of pain at exercise and two had episodic pain. There were no local signs of substitute intolerance. Follow-up was greater than one year in 12 patients and greater than two years in eight: seven shoulders were pain free, three presented pain at exercise, and two required analgesic drugs. Radiographically, the limit between the bone substitute and the cancellous bone was imprecise; the bone substitute could not be readily visualized in four shoulders, had faded out in three, and was visible in five. Discussion: The association of enchondroma and a rotator cuff pathology is common suggesting the tumor could affect disease expression. Imaging provides strong arguments favoring a benign disease. Use of bone substitute for filling is reliable and avoids the need for an iliac graft. Conclusion: A fortuitously discovered or painful enchondroma of the humerus should be treated by curettage-filling with bone substitute as soon as the nature of the tumor has been clearly identified and/or strong uptake on scintigraphy visualized. This is a supplementary operative argument suggesting an associated cuff pathology

Introduction: Surgical removal by means of curettage is the mainstay of treatment of enchondromas of the hand. Methods of reconstruction after tumor removal usually entail no reconstruction or filling of the tumor cavity with a bone graft. These techniques necessitate a prolonged period of protected activity until bone healing of the tumor cavity occurs. The authors have utilized hardware and bone cement for the purpose of reconstruction of the tumor cavity. This technique provides immediate mechanical stability and allows early mobilization. Methods: Between 1986 and 1999 the authors treated 13 patients (8 females, 4 males) who ranged in age from 23 to 58 years (median, 32 years) and diagnosed with enchondroma of the hand. Eight patients presented with a pathological fracture. Anatomic locations included: metacarpal bones – 5, proximal phalanx – 4, and middle phalanx – 4. Tumors were approached through the retained thinned or destroyed cortex to minimize additional bone loss. Surgery included removal of all gross tumor with hand curettes; this was followed by high speed burr drilling of the inner reactive bone shell. Reconstruction included intramedullary metal wire along the longitudinal axis of the cavity and polyme-hylmethacrylate (PMMA). Full activity as tolerated was allowed immediately after surgery. All patients were followed for more than 2 years. Follow-up included physical and radiological evaluation and functional evaluation. Results: Following surgery, all patients returned to their presurgical functional capability within two weeks. At the last follow-up, none of the patients had local tumor recurrence and although three patients had 15° to 20° decrease in flexion of the metacarpophalangeal joint, none reported a functional limitation. There were no postoperative infections or fractures. Conclusions: Reconstruction of the tumor cavity, remaining after curettage of enchondroma of the hand, with intramedullary hardware and PMMA provides immediate mechanical stability and allows early mobilization. This technique is associated with good short- and long-term functional outcomes

Introduction: Enchondromas are benign cartilaginous tumors, which are most often localized, in the small bones of the hand. Treatment methods fall into a broad spectrum ranging from conservative, medical therapies, to a variety of surgical procedures, which may or may not employ the use of local adjuvant treatment or bone grafting. Material & Method: We present thirty four patients with hand enchondromas treated surgically in our department during the last eleven years. Their medical records and radiographs were reviewed retrospectively and the patients were contacted and interviewed by telephone. Results: The patients were treated operatively with an intralesional approach, with meticulous curettage of the bone lesion, use of phenol 5% and grafting using coralline hydroxyapatite. X-rays taken over the first postoperative year revealed adequate bone synthesis at the site of enchondroma excavation, and there was no evidence of recurrence, fracture, infection or other complication related to the procedure. Conclusion: We conclude that the combination of curettage of the lesion with the use of phenol as local adjuvant and grafting using coralline hydroxyapatite during the surgical treatment of enchondromas, is a safe technique to reduce or prevent recurrence and allow adequate and uncomplicated local new bone formation

1. Five cases of calcified enchondroma are described. In all except one the condition was symptomless and was discovered accidentally. 2. The radiographic features are described and the differential diagnosis is discussed. 3. It is emphasised that when the diagnosis is in doubt biopsy should be undertaken

A prospective single-cohort study was designed to include 20 patients with enchondromas but was stopped because of poor early results. Four patients with an enchondroma, three in the proximal humerus and one in the distal femur, were treated by curettage and filling of the defect with Norian SRS cement. Clinical and radiological follow-up including CT and MRI was carried out for 18 months. All three patients with lesions in the proximal humerus had severe pain and limited movement of the shoulder. The radiological and CT appearances of the cement were unchanged at follow-up. There were characteristic appearances of synovitis and periosteitis on MRI in two patients. Since the cement induces a soft-tissue reaction the bony cavity should be sealed with the curetted and burred bone after curettage and introduction of Norian cement, especially in sites where a tourniquet cannot be applied

Malignant change in existing benign enchondroma of phalanx of hand to chondrosarcoma of hand is extremely rare. Books suggest that chondrosarcoma does not arise in small bones of hands and feet although in literature few cases have been reported but not described comprehensively. We report a rare case of chondrosarcoma in distal phalanx of ring finger in a 75- year old healthy female who had cystic lesion for past 25 years with recurrent fractures. Patient came to us with severe pain and tender, hard swelling of distal phalanx of left ring finger. X ray showed pronounced expansion of the terminal phalanx surrounded partially by a shell of bone, with focal spotty calcification with in the lesion. Because of sudden increase in size and pain of swelling, an amputation was performed at distal inter phalangeal joint. Histopathology showed grade II myxoid chondrosarcoma with pre-existing enchondroma. Wound healed nicely. Extensive investigation in form of CT chest and bone scan did not show any metastasis. Five year follow up did not show any local recurrence or distant metastasis. Clinical suspicion should be aroused in an older individual with a previously relatively quiescent lesion that becomes larger and painful. Usually course of the tumour is slow and metastasis to lungs is late. Treatment of choice is disarticulation a joint proximal to lesion. Prognosis is good if metastasis has not occurred. Once diagnosis is made, patient should be investigated to look for any possible metastasis and must be regularly followed up. A literature review and discussion of salient diagnostic and treatment issues is included

The pre-operative differentiation between enchondroma, low-grade chondrosarcoma and high-grade chondrosarcoma remains a diagnostic challenge. We reviewed the accuracy and safety of the radiological grading of cartilaginous tumours through the assessment of, first, pre-operative radiological and post-operative histological agreement, and second the rate of recurrence in lesions confirmed as high-grade on histology. We performed a retrospective review of major long bone cartilaginous tumours managed by curettage as low grade between 2001 and 2012. A total of 53 patients with a mean age of 47.6 years (8 to 71) were included. There were 23 men and 30 women. The tumours involved the femur (n = 20), humerus (n = 18), tibia (n = 9), fibula (n = 3), radius (n = 2) and ulna (n = 1). Pre-operative diagnoses resulted from multidisciplinary consensus following radiological review alone for 35 tumours, or with the addition of pre-operative image guided needle biopsy for 18. The histologically confirmed diagnosis was enchondroma for two (3.7%), low-grade chondrosarcoma for 49 (92.6%) and high-grade chondrosarcoma for two (3.7%). Three patients with a low-grade tumour developed a local recurrence at a mean of 15 months (12 to 17) post-operatively. A single high-grade recurrence (grade II) was treated with tibial diaphyseal replacement. The overall recurrence rate was 7.5% at a mean follow-up of 4.7 years (1.2 to 12.3). Cartilaginous tumours identified as low-grade on pre-operative imaging with or without additional image-guided needle biopsy can safely be managed as low-grade without pre-operative histological diagnosis. A few tumours may demonstrate high-grade features histologically, but the rates of recurrence are not affected. Cite this article: Bone Joint J 2014; 96-B:1098–105

Aims

The purpose of this study was to determine if clinical and radiological surveillance of cartilage tumours with low biological activity is appropriate.

Cartilage lesions vary in the spectrum from benign enchondromas to highly malignant dedifferentiated chondrosarcomas. From the treatment perspective, enchondromas are observed, Grade 1 chondrosarcomas are curetted like aggressive benign tumors, and rest are resected like other sarcomas. Although biopsy for tissue diagnosis is the gold standard for diagnosis and grade determination in chondrosarcoma, tumor heterogeneity limits the grading in patients following a biopsy. In the absence of definite pre-treatment grading, a surgeon is therefore often in a dilemma when deciding the best treatment option. Radiology has identified aggressive features and aggressiveness scores have been used to try and grade these tumors based on the imaging characteristics but there have been very few published reports with a uniform group and large number of cases to derive a consistent scoring and correlation. The authors asked these study questions :(1) Does Radiology Aggressiveness and its Score correlate with the grade of chondrosarcoma? (2) Can a cut off Radiology Agressiveness Score value be used to guide the clinician and add value to needle biopsy information in offering histological grade dependent management?. A retrospective analysis of patients with long bone extremity intraosseous primary chondrosarcomas were correlated with the final histology grade for the operated patients and Radiological parameters with 9 parameters identified a priori and from published literature (radiology aggressiveness scores - RAS) were evaluated and tabulated. 137 patients were identified and 2 patients were eliminated for prior surgical intervention. All patients had tissue diagnosis available and pre-treatment local radiology investigations (radiographs and/or CT scans and MRI scans) to define the RAS parameters. Spearman correlation has indicated that there was a significant positive association between RAS and final histology grading of long bone primary intraosseous chondrosarcomas. We expect higher RAS values will provide grading information in patients with inconclusive pre-surgery biopsy to tumor grades and aid in correct grade dependant surgical management of the lesion. Prediction of dedifferentiated chondrosarcoma from higher RAS will be attempted and a correlation to obtain a RAS cut off, although this may be challenging to achieve due to the overlap of features across the intermediate grade, high grade and dedifferentiated grades. Radiology Aggressiveness correlates with the histologic grade in long bone extremity primary chondrosarcomas and the correlation of radiology and biopsy can aid in treatment planning by guiding us towards a low-grade neoplasm which may be dealt with intralesional extended curettage or high-grade lesion which need to be resected. Standalone RAS may not solve the grading dilemma of primary long bone intraosseous chondrosarcomas as the need for tissue diagnosis for confirming atypical cartilaginous neoplasm cannot be eliminated, however in the event of a needle biopsy grade or inconclusive open biopsy it may guide us towards a correlational diagnosis along with radiology and pathology for grade based management of the chondrosarcoma

Ollier disease is a rare skeletal disorder. It is characterized by the occurrence of multiple enchondromas with a marked unilateral predominance mainly affecting medulla of the metaphyses and diaphyses of the short and long tubular bones of the limbs, especially the hands and feet. The risk of malignant transformation is suggested to be up to 35%. We hypothesise that Ollier disease is a mosaic condition as it is polyostotic and because of its unilateral predominance. Here we aimed to identify molecular defects in Ollier disease related enchondromas and chondrosarcomas using high resolution single nucleotide polymorphism (SNP) array approach. Affymetrix SNP 6.0 was performed on 67 samples which include 10 blood samples and 3 matched blood-saliva samples as a control; 13 enchondromas and 26 chondrosarcomas of different grades from 30 Ollier patients and normal DNA from 12 Ollier patients for paired comparison. All samples were divided into three groups: normals, enchondromas and chondrosarcomas. The number of numerical genomic changes in the chromosomes were not different for the enchondromas (p=0.36) while large genomic aberrations were seen in chondrosarcomas as compared to normals (p=0.01). Copy number variation (CNV) analysis showed 95K amplification at 4q13 in 5 out of 13 enchondromas and a 2K deletion at 14q11 in 6 out of 13 enchondromas. Paired loss of heterozygosity (LOH) analysis failed to show LOH in 5 enchondromas at higher resolution. Paired LOH was observed at 3q, 5p, 6p, 6q, 7q, 9p, 12p, 13p and 13q in 7 high grade chondrosarcomas associated with loss of chromosomes. The results of this study indicate involvement of chromosomes 4 and 14 for the development of enchondromas. We were unable to detect LOH in enchondromas at 1Mb resolution containing approximately 500 SNP probes. High grade chondrosarcomas showed LOH at different chromosomes. In future, we will study LOH and CNV changes at gene level and select candidate genes

Introduction. Enchondromas located in the phalangeal bones may be more cellular than non-digital locations necessitating clinical and radiological correlation to determine diagnosis. Atypical enchondromas have increased cellularity and atypia relative to simple enchondromas but no evidence of permeation. Chondrosarcomas of the phalanges are thought to have a more indolent course than chondrosarcomas in other locations. The aim of the study was to determine the outcome of atypical enchondromas and grade 1 chondrosarcomas of the phalanges treated surgically. Methods. Data was collected prospectively on patients with a cartilage lesion of the phalanges. Typical enchondromas, grade 2 or 3 chondrosarcomas and patients with Ollier's disease were excluded. Results. There were twenty two cases of atypical enchondroma or grade 1 chondrosarcoma. Ten of the patients were female and twelve male with a mean age of 41. There were fourteen atypical enchondromas and eight grade 1 chondrosarcomas. Sixteen of the lesions were in the hand and six were in the foot. Seventeen tumours, including four cases of grade 1 chondrosarcoma, were treated with extended curettage utilising a high speed burr. Five cases were managed by digital amputation as the degree of bone loss precluded retention of the phalanx. At a mean follow up of 30 months there has been one case of local recurrence occurring in an atypical enchondroma of the foot. There have been no cases of local recurrence in the four cases of grade 1 chondrosarcoma treated by curettage. There have been no cases of distant metastasis. All patients remain under long term clinical review. Discussion. There are few published results on the treatment of atypical enchondromata and low-grade chondrosarcomas of the phalanges. In selected cases extended curettage has a low recurrence rate in the treatment of atypical enchondromata and grade 1 chondrosarcomas of the phalanges

Enchondromatosis is a non-hereditary disease, characterised by the presence of multiple enchondromas. While Ollier Disease is typified by multiple enchondromas, in Maffucci Syndrome they are combined with haemangioma. Due to the rarity of these diseases, systematic studies on clinical behaviour providing information how to treat patients are lacking. This study intends to answer the following questions: What are predictive factors for developing chondrosarcoma? When is extensive surgery necessary? How often patients die due to dedifferentiation or metastasis?. Twelve institutes in eight countries participated in this descriptive retrospective EMSOS-study. 118 Patients with Ollier Disease and 15 patients with Maffucci Syndrome were included. Unilateral localization of disease was found in 60% of Ollier patients and 40% of patients with Maffucci Syndrome. One of the predictive factors for developing chondrosarcoma is the location of the enchondromas; the risk increases especially when enchondromas are located in the scapula (33%), humerus (18%), pelvis (26%) or femur (15%). For the phalanges, this risk is 14% in the hand and 16% in the feet. The decision whether or not to perform extensive surgery is difficult, especially in patients who suffer multiple chondrosarcomas. Malignant transformation was found in fourty-four patients with Ollier Disease (37%) and eight patients with Maffucci Syndrome (53%). Multiple synchronous or metachronous chondrosarcomas were found in 15 patients. Nine patients died (range 21–54 yrs). Seven of them died disease related due to pulmonary metastasis (2 humerus, 2 pelvis, 3 femur). Two patients died from glioma of the brain. In conclusion, one important predictive factor for developing chondrosarcoma is the location of the enchondromas; interestingly, only patients with chondrosarcoma outside the small bones died of their disease. In this series, no dedifferentiation of chondrosarcoma was seen. A first design flow-chart how to approach chondrosarcoma in patients with Ollier Disease and Maffucci Syndrome is in preparation

Aim: The differential diagnosis between chondroma and grade I chondrosarcoma still represents a challenge. There are always cases in which a perfect diagnosis can’t be done for sure. This cases are defined in literature with different synonyms such as: borderline chondrosarcoma, grade 0 chondrosarcoma, atypical enchondroma or in situ chondrosarcoma. Enchondroma are benign lesions that do not require a surgical treatment. Low grade chondrosarcoma is a malignant tumour that can recur and also if in a low percentage of cases can metastasize. Methods: The Authors reviewed 22 cases of chondrosarcoma of the limbs for clinical, radiographycal and histological features. Results: Pain was present in 80% of cases of low grade chondrosarcoma, while was absent in enchondroma. Radiographic analysis was not significative. Bone scan was often positive in low grade chondrosarcoma as in enchondroma. Histology demonstrated a permeative pattern in chondrosarcoma with infiltration of the bone trabeculae. Conclusions: Only the complete evaluation of the patient resulted in a correct diagnosis. Follow-up of patients confirmed our findings

Abstract. Background. Benign osteolytic lesions of bone represent a diverse group of pathological and clinical entities. The aim of this study is to highlight the importance of intraoperative endoscopic assessment of intramedullary osteolytic lesions in view of the rate of complications during the postoperative follow up period. Methods. 69 patients (median age 27 years) with benign osteolytic lesion had been prospectively followed up from December 2017 to December 2018 in a university hospital in Cairo, Egypt and in a level-1 trauma center in United Kingdom. All patients had been treated by curettage with the aid of endoscopy through a standard incision and 2 portals. Histological analysis was confirmed from intraoperative samples analysis. All patients had received bone allografts from different donor sites (iliac crest, fibula, olecranon, etc). None of them received chemo or radiotherapy. Results. Most of lesions were enchondroma (n=29), followed by Aneurysmal bone cyst (ABC) (n=16), Fibrodysplasia (n=13), Chondromyxoid fibroma (n=3), simple bone cyst (n= 3), non-ossifying fibroma (n= 3), giant cell tumour (n= 1) and chondromyxoid fibroma (n = 1). Site of lesion varied from metacarpals (n = 29), femur (n= 1), lower leg (n= 31), and upper limb (n=18). Complications happened only in 9 cases (pathological fractures (n=2), infection (n= 1), recurrence (n=3, all aneurysmal bone cyst), residual pain (n= 3, all in tibia). None of cases developed malignant transformation. Conclusion. Endoscopy is recommended in management of benign osteolytic bone lesions; as it aids in better visualization of the hidden lesions that are missed even after doing apparently satisfactory blind curettage. From our study the recurrence rate is 2% compared to the known 12–18% recurrence rate in the blind technique from literature

Introduction: Both enchondromas and chondrosarcomas are mesenchymal neoplasms which originate from cartilage cells, and they occur mainly in the extremities. Both these tumours are resistant to chemotherapy and radiotherapy, and surgery is the only treatment option. In the last few years limb saving procedures have become the treatment of choice. Intra-operative cryosurgery has been introduced as a local adjuvant therapy for skeletal benign and low-grade malignant tumours. It is applied after curettage of the lesion to destroy any remaining tumour cells, and to enlarge the oncological margin of resection. Since the introduction of cryosurgery as an adjuvans, oncological and functional results of this extremity sparing surgery are significantly enhanced. Patients and Methods: A retrospective study was conducted to evaluate the oncological and functional results, and the complications of cryosurgical treatment. Data were prospectively collected from the tumour register and patient records. Functional scores of the affected limbs were assessed according to the Musculo-Skeletal Tumour Society scoring system. Results: Between 1994 and 2003 123 patients (47 men, 76 women, average age 49 years; range 13–83 yrs) were treated with curettage and cryosurgery for an Enneking stage 3 enchondroma (75 patients) or a low-grade chondrosarcoma (55 patients). The minimal follow up was two years, and the average follow up 50 months (range 24–119 months). At follow up three recurrences had occurred in patients treated for enchondroma. One residual tumour was diagnosed in a patient with chondrosarcoma grade Ib. All patients were treated again with curettage and cryosurgery and disease free at the latest follow-up. Of the 37 complications the most common were a fracture at the surgical site (18), fracture of osteosynthesis (6), 3 wound infection (3), delayed soft tissue healing (3), and transient nerve palsy (3). Functional MSTS scores increased in time to an average of 28 points (94%) at two year follow up. No significant difference in scores were found regarding to localisation of the lesion, age or gender. A significant discrepancy in functional scores was observed between patients who did suffer from one or more complications and patients who did not. Conclusion: We believe that the use of cryosurgery is an excellent adjuvant therapy after curettage to achieve local control of aggressive enchondromas and low grade chondrosarcomas. It avoids the need for segmental resection, making reconstruction of the bony defect easier and therefore results in excellent functional outcome. Due to the initial high fracture rate osteosynthesis at the surgical site is used more often, and weight baring mobilisation is postponed until full consolidation is reached

Enchondroma of the hand is a common benign tumour composed of mature cartilage; it is usually asymptomatic and found accidentally or after pathologic fractures. Malignant transformation may occur, though only very rarely. The age of the patients varies widely. The small bones of the hand are the most frequent anatomic site for this pathology. Enchondromas are conventionally treated by curettage and the bone defect is then filled with morceellised autologous bone chips from the iliac crest or with an allograft. Recently, bone substitutes have also been used instead of autologous or allogenic bone graft. Calcium phosphate cement is a promising injectable biomaterial able to increase the number of osteoblasts without inducing a marked de-differentiation, an effect that is useful when a high number of bone forming cells are required. This bone substitute has been used successfully for the treatment of distal radius fractures and or mal-unions, femoral neck fractures, tibial plate fractures, complex calcaneal fractures and enchondromas. From 2001 we treated 12 patients who were diagnosed as having solitary enchondromas, nine in the hand (four metacarpal bones and five finger bones); 6 patients had an associated pathologic fracture that occurred as a result of simple trauma. A surgical treatment with a complete removal of the tumour and the injection of Norian SRS cement into the cavity and fluoroscopic control was performed as a standardised procedure in all cases. The patients allowed to perform complete range of motion 3 weeks after surgery. At follow-up they were evaluated by clinical examination, X-ray and the DASH questionnaire. None of the patients had swelling or deformity or tendon injuries or wound infection. Four patients had a slight loss of flexion at the MF joint but none considered this a functional limitation; all the others regained a complete ROM. All the patients returned to their presurgical occupation. Five of them complained of a hypertrophic scar. The X-ray showed a complete fill of the bone gap. They were satisfied and the mean DASH score was 6.06. Clinical results have been reported with simple curettage without bone grafting or bone substitutes, but these two elements minimise the volume of the bone defect, maintain bone strength and promote new bone formation. Autologous bone graft caused pain in the donor site. Reconstruction of the tumour cavity provides immediate mechanical stability and good functional outcome

Aims

Due to its indolent clinical behaviour, the treatment paradigm of atypical cartilaginous tumours (ACTs) in the long bones is slowly shifting from intralesional resection (curettage) and local adjuvants, towards active surveillance through wait-and-scan follow-up. In this retrospective cohort study performed in a tertiary referral centre, we studied the natural behaviour of ACT lesions by active surveillance with MRI. Clinical symptoms were not considered in the surveillance programme.

Purpose: Hand localisations predominate in Ollier disease. There have however been few studies devoted to this topic and the one report available only presented a few cases. Treatment of multiple enchondromatosis is sometimes discussed with the treatment of solitary enchondromas despite different recurrence behaviour and aggressiveness. A specific approach is indicated. Material and methods: We present a retrospective series of 22 children treated for multiple enchondromatosis of the hand. One hand was involved in 11 and both hands in 11. We thus identified 246 enchondromas on 33 hands. One girl with Maffucci syndrome died at the age of six years due to angiosarcomatous degeneration of the leg. Results: At diagnosis, mean age was six years nine months. Pain and pathological fractures were exceptional, observed in three patients. Overall hand function remained satisfactory. We performed surgery for 14 patients (mean age 8 yr 8 mo) due to increasing symptoms and tumour volume. Five patients required two operations and one a third. We thus performed 18 interventions on 37 hand bones (51 surgical procedures). Simple curettage was performed in 21 cases, combined with autologous cancellous grafting in 30 others. One finger had to be amputated due to aggressive recurrence. Radio-lucent residual lesions were found in 62% of the children at mean 19 months follow-up. Discussion: Hand function remained satisfactory despite a worrisome radiographic aspect. The decision for surgery was basically made to improve the aesthetic aspect of the hand. Many surgical techniques have been proposed. In our experience, it is difficult to eradicate a treated lesion but grafting appears to be more effective than simple curettage. Conclusion: Function remains good during growth, fractures are exceptional. Lesions stabilise at the end of growth. We have not observed any case of chondrosarcomatous degeneration in our series of paediatric patients. There is however a risk of malignant degeneration which should be suspected in the event of pain, increased tumour volume or pathological fracture

Ollier’s disease is characterised by severe deformity of the extremities and retarded growth because of multiple enchondromas. For correction of deformity, the Ilizarov method has been used although it has many complications. A 17-year-old boy with Ollier’s disease had a limb-length discrepancy of 17.4 cm, with a valgus deformity of the right knee and recurvatum of the femur of 23°. He had undergone three unsuccessful attempts to correct the deformities by using external fixators. We used a fully implantable, motorised, lengthening and correction nail (Fitbone) to achieve full correction of all the deformities without complications. We decided to carry out the procedure in three stages. First, we lengthened the femur by 3.6 cm and the tibia by 4 cm. We then exchanged the femoral nail for a longer implant and achieved a further 6 cm of length. This reduced the shortening to 3.8 cm. When the boy has finished secondary school we will adjust the remaining discrepancy