Mazabraud's syndrome. A case with multiple myxomas.

Mazabraud's syndrome is defined as the combination of one or more intramuscular myxomas and fibrous dysplasia of bone. The diagnosis is important given the increased risk of malignant transformation of the bone lesions. We report a case in a 56-year-old patient with a 14-year follow-up during which multiple surgical procedures were required to remove myxomas (present at more than 15 sites). The resected myxomas were large and progressive. Unique features in this case include the long follow-up and the number of myxomas considerably above the average for this disease. Eighty other cases of Mazabraud's syndrome have been reported. The condition predominantly affects middle-aged women (mean age, 44 years). The bone lesions may be monostotic or polyostotic. Mazabraud's syndrome may be difficult to distinguish from soft-tissue sarcoma or neurofibromatosis. Identification of the underlying genetic abnormality provides diagnostic confirmation, as shown in our patient. The management consists in surgery to remove the myxomas and magnetic resonance imaging at regular intervals to monitor the lesions.

Intercalary defects reconstruction of the femur and tibia after primary malignant bone tumour resection. A series of 13 cases.

INTRODUCTION: Performing intercalary segment reconstruction after malignant bone tumour resection results in both mechanical and biological challenges. Fixation must be solid enough to avoid short-term or mid-term mechanical failure. The use of an allograft or autograft must ensure long-term survival of the reconstruction. The goal of this study was to analyse the clinical and radiological outcomes of these reconstructions. PATIENTS AND METHODS: Thirteen patients were operated on eight femurs and five tibias. The median age was 20 years old (range 14-50). The most common diagnosis was osteosarcoma. The median resection length was 15cm (Q1-Q3: 6-26). A plate was used for fixation in nine cases and an intramedullary locked nail in four cases. An isolated bone autograft was used in two cases, an isolated bone allograft in one case, a dual autograft-allograft composite in six cases, and vascularised fibula and allograft combination in four cases. RESULTS: The cumulative probability of union was 46% (95% CI: 0-99%) at 1 year; at the final follow-up, union was achieved in 12 patients (92%). Because of non-unions, 13 iterative procedures were needed to obtain these results. A non-displaced fracture of a cuboid-shaped tibial graft occurred in one patient, which was treated conservatively. Three infections occurred. DISCUSSION: The results of intercalary segmental defects reconstruction after bone tumour resection were good, both from an oncologic and radiological point-of-view. One or more iterative procedures are sometimes needed to finally obtain bone union. We prefer to use a free rectangular cuboidal tibial graft since reconstruction with a vascularised autograft is technically more difficult. The choice of fixation methods is still controversial and no approach was found to be superior. LEVEL OF EVIDENCE: Level IV. Retrospective study.

Primary or recurring extra-abdominal desmoid fibromatosis: assessment of treatment by observation only.

INTRODUCTION: Extra-abdominal desmoid fibromatosis (EADF) is a benign tumoral condition, classically managed by more or less radical and sometimes mutilating excision. This treatment strategy is associated with a recurrence rate of nearly 50% according to various reports. HYPOTHESIS: EADF may show spontaneous stabilization over time. METHODS: A retrospective series of 26 cases of EADF managed by simple observation was studied to assess spontaneous favorable evolution and identify possible factors impacting evolution. Eleven cases were of primary EADF with no treatment or surgery, and 15 of recurrence after surgery with no adjuvant treatment. MRI was the reference examination during follow-up. RESULTS: Twenty-four cases showed stabilization at a median 14 months; there were no cases of renewed evolution after stabilization. One primary tumor showed spontaneous regression, and one recurrence still showed evolution at end of follow-up (23 months). The sole factor impacting potential for evolution was prior surgery. No radiologic or pathologic criteria of evolution emerged from analysis. DISCUSSION: The present series, one of the largest dedicated to EADF managed by observation, confirmed recent literature findings: a conservative "wait-and-see" attitude is reasonable and should be considered when large-scale resection would entail significant functional or esthetic impairment. LEVEL OF EVIDENCE: Level IV, retrospective study.

Distally-based vastus lateralis muscle flap: a salvage alternative to address complex soft tissue defects around the knee.

Repeated soft tissue injuries around the knee represent challenging clinical situations where functional prognosis of the joint is often poor, especially in the presence of total knee arthroplasty (TKA). When gastrocnemius muscle flap techniques have already been used and failed, therapeutic solutions become rare. The authors suggest a regional solution to salvage these cases by the transfer of distally-based vastus lateralis muscle flaps. Four clinical operations of this muscle flap transfer are described, with three around TKA revision and one case of a post-traumatic knee amputation resulting from a compound open knee injury. Technical details of the intervention are presented. In all cases, skin closure was achieved, even if distal marginal necrosis was seen in two cases related to the type of muscle flap vascularisation.Final joint mobility was always poor (45 grades on average). Distally-based vastus lateralis muscle flaps represent a salvage procedure to correct iterative soft tissue defects around the knee that threaten short-term joint function. These muscle flaps do not require microsurgical anastomosis.

Pelvic chondrosarcomas: surgical treatment options.

INTRODUCTION: Chondrosarcoma (CS) is a primary malignant bone tumor with cartilaginous differentiation. The only available treatment is carcinological surgical resection since the usual adjuvant treatments are ineffective. The pelvic location creates specific technical difficulties both for exeresis and reconstruction. Our objective was to evaluate the carcinological and functional outcomes of inter-ilioabdominal amputation and conservative surgery. MATERIALS AND METHODS: We retrospectively studied 59 cases of pelvis chondrosarcoma managed in our department between 1968 and 2003. Demographic, anatomopathological, surgical and survival data were analyzed. Survival was estimated by the Kaplan-Meier curves and the cumulative incidence method. Multivariate analysis was used to identify all possible independent prognostic variables. RESULTS: There were 33 men and 26 women, with an average age of 48 years. The average follow-up duration was 94 months. Eleven patients had a grade 1 chondrosarcoma, 36 a grade 2 chondrosarcoma, five were grade 3, and seven were dedifferentiated chondrosarcoma. Eleven patients underwent an inter-ilioabdominal disarticulation, and 48 had a more conservative surgery. Resection margins proved healthy in 46 patients (78%). Eighteen patients (31%) had a local recurrence, and 12 (20%) had metastases. At last follow-up, 30 patients (51%) were still alive without any sign of recurrence. Twenty-three patients (39%) died from the disease. Multivariate analysis showed that margin invasion was associated with a definitely increased local recurrence rate. A high tumoral grade was correlated with a greater risk of metastases occurrence. These two last factors (margin status and tumor grade) as well as acetabulum involvement were correlated with a reduced survival rate. Function was better among patients treated by conservative surgery, and among them, even better when the peri-acetabular area remained intact. Our study confirmed that resection margins quality is a major prognostic factor both for local control and for survival. On the other hand, local recurrence is an adverse survival prognosis factor and is itself correlated with resection margins quality. Peri-acetabular chondrosarcoma location (in zone 2) appears to be a poor oncological prognosis factor since, in this location, obtaining healthy margins appears particularly difficult. Compared to resection, inter-ilioabdominal amputation did not prove its superiority concerning resection margins quality or survival. However, resection guaranteed a better functional outcome. CONCLUSION: Chondrosarcoma of the pelvic girdle remains of worse prognosis than peripheral bones chondrosarcoma since the critical prognosis factor is the resection margins quality. This location, and especially the peri-acetabular zone, poses difficult specific technical problems when conservative surgery is selected. Various imaging techniques should help better envision tumor resection extent. Inter-ilioabdominal amputation should only be resorted to in non-metastatic patients, when the tumor does not seem to be removable with sufficient healthy margins guarantee, or when local conditions make it impossible to hope for a good quality reconstruction. LEVEL OF EVIDENCE: Level IV; therapeutic retrospective study.

Surgical management of pelvic primary bone tumors involving the sacroiliac joint.

INTRODUCTION: Pelvic primary malignant bone tumours, especially when involving the sacroiliac joint are difficult to treat. Abdominoperineal amputations are today used, only in life-threatening situations. HYPOTHESIS: A precisely planed surgical technique can save the affected extremity without compromising the resection quality and subsequent patient survival. OBJECTIVE: To assess the procedures used for resection and reconstruction of bone tumours invading the sacroiliac joint as well as their effects on cancer outcome and functional results. MATERIALS AND METHODS: This is a continuous and retrospective analysis of 24 patients treated between 1986 and 2003. Six tumours affected the sacral body and 18 tumours involved the wing of the ilium. The joint articular surface was invaded in only six cases. Seventeen patients received neoadjuvant chemotherapy. The procedure was performed through an enlarged iliac crest incision, giving access to two sections of the pelvic ring. Six cases required neurological sacrifice. Initial tumour grading was based on the Enneking classification, and the functional results, on the Musculoskeletal Tumour Society (MSTS) scoring system. RESULTS: The average operation lasted 5.27 hours. Reconstruction was performed with bone autograft and instrumentation. Resection was large with adequate margins 11 times, marginal 12 times, and contaminated once. Average follow-up was 4.77 years. The 5-year survival rate was 50%. Twelve patients either died from their disease or were in the metastatic stage at final follow-up. Survival was linked to the quality of resection and initial tumour staging. Hemisacrectomy did not affect patient survival. Local recurrences had a poor prognosis with eight cases of secondary metastases out of 11. Bone healing occurred in 13 patients, 10 of whom survived. Of the 12 patients who survived and were in complete remission at final follow-up, the average MSTS score was 61%. The score was at 38.6% in cases involving neurological sacrifice, and at 77.1% for the rest of the group. It was at 64% in healed cases and 13% in nonunion cases. DISCUSSION: The survival of patients presenting with a sacroiliac joint tumour is substantially related to both tumour histology and resection quality. Local recurrences carry a poor prognosis with a high rate of secondary metastatic dissemination. In situations where disease control can be achieved, the proposed method of reconstruction allows, satisfactory bone healing and fair functional recovery, provided no major neurological sacrifice has taken place. LEVEL OF EVIDENCE: level IV: Retrospective Therapeutic Study.

[Bizarre parosteal osteochondromatous proliferation (Nora's lesion): two cases]. / La lésion de Nora ou Bizarre Parosteal Osteochondromatous Proliferation: à propos de 2 cas

The lesion Nora described in 1983 as a bizarre parosteal osteochondromatous proliferation (BPOP) is a member of a group of osteocartilaginous surface lesions. BPOP is infrequent but new cases are regularly reported. We report two new cases with an unusual localization (ilion and distal humerus) and unusual size (9 cm for the iliac lesion). In light of these cases and reports in the literature, the main differential diagnoses of BPOP are exostosis and parosteal osteosarcoma.

[Surveillance of operative site infections in an orthopedic and traumatology surgery department: an example of methodology]. / Proposition d'un protocole de suivi des infections avérées de site opératoire en chirurgie orthopédique et traumatologique.

PURPOSE OF THE STUDY: A program for the prevention of nosocomial infections, including operative site infections (OSI) is a legal obligation in France. According to the CDC, in orthopedic surgery, nosocomial infection is defined as any infection occurring within 30 days of operation, or within one year in the event of material implantation. No surveillance system has been validated and the rate of OSI is unknown in orthopedic surgery. We report the number of OSI observed during a three year period in our unit and describe the characteristic features. MATERIAL AND METHODS: Data were collected from the bacteriology reports on operative site samples with a positive culture. A group of specialists determined the infective nature of the germ and the nosocomial nature of the OSI. Clinical and bacteriological data were noted on a standard datasheet used for prospective follow-up of the number of cases and data processing. During a three-year period (2000, 2001, 2002), among 9397 orthopedic and traumatology operations performed, 86 OSI were identified. Mean patient age was 58 years and mean body mass index was 25.7. The ASA score was >or=II for 72% of patients. RESULTS: The OSI involved an arthroplasty in 23 cases, a traumatology procedure in 21, and tumor treatment in 24. The diagnosis was established within 30 days of operation for 75% and after discharge from hospital in 65.4%. Single-germ infections predominated (n=59). Staphylococcus aureus was isolated in 80.23% of infections. For tumor surgery, the statistically more frequent multiple-germ infections associated coagulase negative Staphylococcus and Gram-negative bacilli. There were six OSI-related deaths. DISCUSSION: Two criticisms can be formulated concerning our surveillance system. First, infections with no identified germ could be missed. The frequency of such infections has been estimated at 2.8 to 19% by different authors. Although patients are automatically recalled for consultation, we were unable to determine the number of patients lost to follow-up at one year. It was thus not possible to determine a precise rate of OSI. Data in the literature have not demonstrated any system providing an exhaustive surveillance, particularly because of the long postoperative period after material implantation. Excepting tumor surgery, Staphylococcus aureus infections predominated. Factors of risk of OSI include the patient's general status, particularly for arthroplasty. We had a mortality rate of 7% for our OSI, corroborating earlier studies and illustrating the severity of such infections. CONCLUSION: Surveillance of OSI in orthopedic surgery requires the development of a system responding to the problem of a long observation period. It would be important to know the precise number of OSI and their characteristic features in order to develop comparison tools.

[Multicentric giant-cell tumor]. / Tumeur à cellules géantes multifocale.

Nearly all (99) giant-cell tumors are solitary. The multicentric presentation is exceptional and not well elucidated. We report the case of a patient presenting five foci identified over an eleven-year period (right tibia, left 4th metacarpal, sacrum, right femur, left femur). The proliferation index was identified for each focus. A review of the literature shows that the development of a new center after treatment for giant-cell tumor is a well-known event, with a wide variability in localizations, number and time to development. The most recent data suggest that each center would be independent progressing according to an individual course. Since histological transformation is not observed and multicenter forms are very exceptional, we suggest that it would not be necessary to propose specific screening for patients with a giant-cell tumor but that it would be good to inform the patient of the possibility of multicentric presentations.

[Twenty nine shoulder reconstructions after resection of the proximal humerus for neoplasm with mean 7-year follow-up]. / Les reconstructions après résection tumorale de l'extrémité supérieure de l'humérus.

PURPOSE OF THE STUDY: Techniques available for shoulder reconstruction after resection of a tumor of the proximal humerus include scapulohumeral arthrodesis, humerus prosthesis with or without an allograft, inverted prostheses, and massive allografts. The purpose of this study was to review clinical and radiological outcomes in a series of 29 patients (20 men and 9 women) who underwent resection-reconstruction of the proximal humerus and to establish from these cases a decision making algorithm for therapeutic indications as a function of tumor invasion. MATERIAL AND METHODS: The tumors were 20 chondrosarcomas, five osteosarcomas, two Ewing sarcomas and one malignant hemangiopericytoma. In 17 patients epiphyso-metaphyseal or epiphyso-metaphyso-diaphyseal resection was performed with preservation of the abductor muscles (type S34A or S345A according to the Musculoskeletal Tumor Society classification). For 12 patients epiphyso-metaphyseal or epiphyso-metaphyso-diaphyseal resection was performed without preservation of the abductor muscles (type S34B or S345B). Reconstruction was achieved using a centromedullary cemented nail in one patient, scapulohumeral arthrodesis in three, a massive humerus prosthesis in 15, and composite humerus prosthesis in three and an inverted prosthesis in seven. The functional score of the Musculoskeletal Tumor Society (MSTS) and standard x-rays were used to assess outcome. RESULTS: Mean follow-up was 85 months (range 16-300). The mean MSTS score was 88% for inverted prostheses, 76% for composite prostheses, 72.6% for massive prostheses, 75% for scapulohumeral arthrodeses, 67% for massive prostheses, and 80% for cemented centromendullary nail. Five patients died from their malignant disease and one from another cause. Four patients are alive but with active disease after a mean follow-up of 108 months and 19 patients (65.5%) are alive and free of locoregional recurrence or metastasis after a mean 83.5 months. We had 28 complications. Glenohumeral instability was the most frequent (11 cases). DISCUSSION: Resection of the upper portion of the humerus should be performed to achieve cancerologically satisfactory tumor resection and enable shoulder resection, if possible, with preservation of a viable and functional abductor system. The functional outcome after such reconstruction depends on the type of bony resection, but also on the sacrifice of the rotator cuff and the deltoid muscle. In light of our experience and results in the literature, we advocate, despite the small number of cases for the different reconstructions, the following decision-making algorithm after resection of the proximal humerus without joint invasion: when the resection removes the rotator cuff and the deltoid (or the axillary nerve), there are two options: scapulohumeral arthrodesis or massive humerus prosthesis for patients who do not desire a complex therapy with a long postoperative period; when the resection preserves the rotator cuff and/or the deltoid muscle, reconstruction can be achieved with a composite (inverted or not) prosthesis with suture of the cuff tendons. We prefer the inverted composite prosthesis; if the deltoid muscle can be preserved but not the rotator cuff, the composite inverted prosthesis appears to be the most logical solution, but scapulohumeral arthrodesis can be proposed in selected cases.

[Trauma-induced tears of the quadriceps tendon: 47 cases]. / Ruptures traumatiques du tendon quadricipital. A propos de 47 cas.

PURPOSE OF THE STUDY: A torn quadriceps tendon is an exceptional finding generally observed after high-energy knee trauma in patients over 40 who present a sprain-like syndrome or after low-energy trauma in elderly subjects who experience knee instability. We reviewed a series of 47 cases of traumatic quadriceps tendon tears treated between 1976 and 1996 in order to evaluate outcome after surgical repair. MATERIAL AND METHODS: Clinical diagnosis was the rule. Forty-two patients, mean age 55 years (range 17-92) were treated for tears of one or both quadriceps tendons subsequent to low-energy trauma (40 tears) or high-energy trauma in younger subjects. The diagnosis was established early in all cases except eight (diagnosis at three weeks to one year). Surgical repair was performed in all cases except one. After surgery, the knee was either immobilized with a plaster cast or held in a removable splint to allow early mobilization. RESULTS: Average time to recovery compatible with daily life or occupational activities was four months. Recovery was not complete at this time. Long-term follow-up revealed that complete recovery with very good or good subjective results was achieved in 90% of the cases. Complete joint motion and normal quadriceps force was achieved in 80% of the cases. Patients who started rehabilitation exercises early generally achieved less satisfactory results although no significant correlation was identified with objective clinical variables. DISCUSSION: Quadriceps tendon tear is a clinical diagnosis which does not require complementary exploration for confirmation. Plain x-rays may be useful to identify associated bony lesions and specific signs of tendon tears. Early surgical repair followed by complete immobilization appears to be preferable for functional recovery allowing better recovery of muscle force without compromising flexion.

Partial anterior trochanteric osteotomy in total hip arthroplasty: surgical technique and preliminary results of 127 cases.

The authors describe a new approach to the hip joint arthroplasty performed in 127 cases of total hip arthroplasty without major complication. A small anterior fragment of greater trochanter, maintaining the insertions of the gluteus minimus and vastus lateralis muscles, is detached. The whole insertion of the gluteus medius is preserved intact, providing good prosthetic stability and rapid recovery of abductor power and gait. Three months after surgery, 74% of patients had recovered good abductor strength with a Merle d'Aubigné and Postel score of 17 points. This surgical approach is technically easy to perform and provides good exposure of the hip. The osteotomized fragment is easily reattached using 2 cerclage wires, and upward displacement after operation was rarely seen.

[Primary bone lymphoma. Retrospective immunohistochemical study of 22 cases]. / Les lymphomes osseux primitifs. Etude rétrospective sur 22 cas avec étude immuno-histochimique récente et homogène.

PURPOSE OF THE STUDY: The clinical, biological, radiological, MRI, and histological features of 22 primary bone lymphomas were reviewed retrospectively. MATERIAL AND METHODS: The standard pathology slides were reexamined with an immunohistochemistry study. Results of two treatments, chemotherapy alone versus chemotherapy then radiotherapy, were compared in terms of local control and patient survival. RESULTS: Mean age of the patients was 53 years; male gender predominated (M/F=2/1). The most frequent clinical sign was pain and 32% of the patients had a pathological fracture. The long bones were generally involved. The typical radiographic image was a permeable bone defect. MRI demonstrated heterogeneous signals on the T2 images. Technetium scintigraphy (performed in all patients) was very sensitive. Histology generally described diffuse large centroblastic lymphoid cells, all with B phenotype. Twelve patients were treated with chemotherapy alone and eight with chemotherapy and radiotherapy. One patient was only given palliative treatment with corticosteroids due to major immunodepression. The last patient, a 79-year-old woman, developed a tumor of the femoral neck; at surgery section of the neck removed the entire tumor, but radiotherapy (30 Gy) centered on the femur was nevertheless performed. Only patients with fractures were operated. Mean follow-up in this series was 48 months (range 4-120). Mean 5-year Kaplan-Meier survival was 74.41% for the overall series, 83.33% for the chemotherapy group and 82.50% for the chemotherapy-radiotherapy group. DISCUSSION: We were unable to demonstrate any significant difference in survival between the two types of treatment. None of the patients experience local relapse. Our observations suggest that technetium scintigraphy is more sensitive than MRI and more specific for diagnosis. Radiography, together with clinical examination and is indicated to monitor these patients during and after treatment. Based on this series, patients with primary bone lymphoma should be given chemotherapy, either alone or combined with radiotherapy, rather than radiotherapy alone. Surgery is not indicated except to obtain a biopsy and to treat mechanical complications.

Complications and functional evaluation of 17 saddle prostheses for resection of periacetabular tumors.

Seventeen saddle prostheses were inserted between 1988 and 1997 after resection of periacetabular tumors. The tumors involved the zones II and III of Enneking classification in 13 patients, the zones I and II in 2 patients, and the zone II in 2 patients. The tumors included 11 chondrosarcomas, 3 Ewing sarcomas, 2 giant cells tumors, and 1 metastasis of renal carcinoma. The tumoral resection was wide "en bloc" in 14 cases, marginal in 2 cases, and intratumoral in 1 case. The mean follow-up period of the patients is 42 months ranging from 8 to 84 months. Local recurrences occurred in five cases and metastases in four cases. Five patients died of tumoral disease and one of intercurrent disease. Complications were observed in 11 cases (65%) including nerve damages (3 cases), deep infections (3 cases), upward migrations of the saddle (4 cases), saddle dislocations (3 cases), sacroiliac subluxations (2 cases), and mechanical failures (2 cases). The modified Musculoskeletal Tumor Society Score (MSTS) and the Toronto Extremity Salvage Score (TESS) were used for functional analysis. Functional results were available for only nine patients of the series with a mean MSTS of 17 points ranging from 11 to 23 points and a mean TESS of 58 points ranging from 39 to 95 points. The saddle prosthesis provided in all cases of this series an early painfree weight-bearing reconstruction with minimal limb shortening, but the functional results remained fair in most patients due to a limited range of motion and a poor abductor strength.

Long-term disability assessment after surgical treatment of low grade spondylolisthesis.

The aim of this study was to determine whether assessment of back surgery with disability scores is relevant. We also attempted to answer the question of whether this evaluation should be conducted by a surgeon or a medical doctor. This retrospective study analyzes the long-term outcome (average follow-up 7 years, range: 3-12) of 40 patients (mean age: 46.2 years) treated by posterior surgical decompression, posterolateral arthrodesis, with or without instrumentation, for symptomatic low-grade spondylolisthesis. All patients were interviewed postoperatively and examined the same day by an orthopedic surgeon, who was not involved in the patients' treatment, as well as by a medical doctor rehabilitation specialist. Impairment was assessed by a standardized clinical examination and by visual analog scales (VAS) of pain. Disability was assessed using two scales: the Quebec disability scale and the Beaujon scale. Anxiety and depression were assessed with a validated specific questionnaire (HAD). Patient's perceived handicap was assessed on a 100-mm VAS. Our results show that the scores of the two disability scales were highly correlated with the patient's overall satisfaction ( r = 0.73 and 0.77 for the Quebec scale and the Beaujon scale, respectively). The intraclass correlation coefficient showed very good or excellent correlation between the data collected by the surgeon and the rehabilitation specialist, ranging from 0.8 to 0.97. This finding clearly demonstrates that interview by a surgeon who is not involved in the patient's treatment does not influence the patient's assessment in terms of impairment, disability, or handicap. Moreover, our results suggest that disability scales are the most relevant outcome measures in the assessment of spine surgery.

Knee reconstruction with prosthesis and muscle flap after total arthrectomy.

A massive prosthesis and medial gastrocnemius muscle transfer were used to reconstruct the knee after extracapsular en bloc excision for bone sarcoma. Magnetic resonance images showed intraarticular involvement. This technique was used in nine patients, six men and three women aged 18 to 51 years, with primary malignant bone tumors of the knee. Extraarticular resection of the knee, including the patella, was done in every case. A knee prosthesis was implanted, and the extensor mechanism was reconstructed by transfer of the medial gastrocnemius muscle and pes anserinus tendons. All resections had negative margins. There were no local recurrences, but metastases occurred in two patients. Infection was the only major complication and was seen in two patients. The mean postoperative Musculoskeletal Tumor Society score was 61% (range, 36%-100%). The mean postoperative range of flexion was 62 degrees (range, 30 degrees-90 degrees), and the mean extensor lag was 12 degrees (range, 0 degrees-40 degrees). Three patients required a crutch to walk. The functional outcome was poor in the two patients whose proximal tibia was removed with the joint, suggesting that arthrodesis may be best in this situation. In properly selected patients, prosthesis and muscle flap reconstruction provides acceptable function and a good cosmetic result.

[Sacral chordoma: retrospective review of 11 surgically treated cases]. / Chordome du sacrum: une étude rétrospective de onze cas traités chirurgicalement.

PURPOSE OF THE STUDY: Chordoma is a malignant neoplasm believed to arise from notochord remnants. It accounts for approximately 3 to 4 p. 100 of primary bone tumors and is localized along the axial skeleton, 50 p. 100 being sacrococcygeal. Clinical, radiographical and histological findings have been well established since the first description by Ribbert in 1894. Sacral chordomas are however difficult to manage and remain a challenge for surgeons and radiotherapists alike. The purpose of this study was to evaluate the long-term results of surgical treatment and patterns of failure in patients treated for chordoma of the sacrum in our department. MATERIALS AND METHODS: This retrospective study included 11 cases of sacral chordomas treated from 1973 to 1998. Patient age ranged from 36 to 77 years (mean 59 years). Six patients were female and five male. The initial treatment was surgery in all cases including intralesional removal in two cases, marginal resection in seven and complete en bloc resection in two. RESULTS: Median follow-up was 6 years (1 month to 14 years). Tumoral recurrences were observed in nine cases 5 months to 8 years after treatment. In two cases, recurrence was observed 8 years after radical sacrectomy. Treatment of recurrences was partial surgical removal with radiotherapy (40 to 70 Grays). Three patients developed metastases in lungs, liver and bone, respectively. Seven patients died, two from metastatic disease. The 5-year overall survival was 64 p. 100 but only 18 p. 100 of the patients survived 10 years. Average disease-free survival was 18 p. 100 at 5 years and 0 p. 100 at 10 years. DISCUSSION: Chordoma is a slow-growing tumor allowing survival for several years despite recurrent disease. However, only 10 to 20 p. 100 of the patients survive free of disease at 5 years. Recurrences are frequent (45 to 80 p. 100) and often multiple. Chordoma inevitably recurs and eventually leads to death after intralesional removal or marginal resection. Radical surgery should be attempted whenever technically feasible. When performed early, particularly for smaller lesions, it offers the best chance for cure. However, tumoral recurrence can occur postoperatively despite a macroscopically complete resection. Because radiation therapy seems to be more successful in controlling microscopic disease, it should be considered as a pre- or postoperative adjuvant to a macroscopically complete resection.