A. Babinet

Real frequency of ordinary and atypical sub-trochanteric and diaphyseal fractures in France based on X-rays and medical file analysis.

PURPOSE Atypical sub-trochanteric and femoral shaft fractures have been reported in patients treated with bisphosphonates. Their incidence has been determined from registered data analysis using international codes. Therefore, the aim of our study was to estimate the real frequency of typical and atypical sub-trochanteric or diaphyseal fractures, based on radiological and clinical data compared to registered data. METHODS In the registers of three large French University Hospitals, patients identified with International Classification of Diseases, 10th Revision diagnosis codes for sub-trochanteric or diaphyseal fracture were selected. Frequencies of ordinary and atypical fractures were calculated after both registered data, radiological and clinical files analysis. RESULTS Among the 4592 patients hospitalized for a femoral fracture over 5 years, 574 were identified to have had a sub-trochanteric or femoral shaft fracture. 47.7% of the sub-trochanteric and femoral shaft fractures were misclassified, predominantly in the sub-trochanteric fractures subset. 12 patients had an atypical fracture (4% of the sub-trochanteric and femoral shaft fractures) and 11 fractures presented radiological features of atypical fractures, whereas clinical files analysis revealed they were pathological or traumatic fractures. CONCLUSION Atypical fractures frequency is very low. Because of their low frequency and the unreliability of registered databases, the risk of atypical fractures is very difficult to estimate retrospectively. A prospective study is needed to clarify the risk factors associated with these fractures.

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.

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.

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.

Limited Arthrodesis of the Wrist for Treatment of Giant Cell Tumor of the Distal Radius

PURPOSE To present the functional results of a technique of radiocarpal arthrodesis and reconstruction with a structural nonvascularized autologous bone graft after en bloc resection of giant cell tumors of the distal radius. METHODS A total of 13 patients with a mean age of 37 years with aggressive giant cell tumor (Campanacci grade III) of distal radius were managed with en bloc resection and reconstruction with a structural nonvascularized bone graft. The primary outcome measure was the disability evaluated by the Musculoskeletal Tumor Society rating score of limb salvage. Secondary outcomes included survival of the reconstruction measured from the date of the operation to revision procedure for any reason (mechanical, infectious, or oncologic). Other outcomes included active wrist motion and ability to resume work. RESULTS Mean follow-up period was 6 years (range, 2-14 y). The median arc of motion at the midcarpal joint was 40°, median wrist flexion was 20°, and median extension was 10°. The median Musculoskeletal Tumor Society score based on the analysis of factors pertinent to the patient as a whole (pain, functional activities, and emotional acceptance) and specific to the upper limb (positioning of the hand, manual dexterity, and lifting ability) was 86%. Five patients underwent a second surgical procedure. The cumulative probability of reoperation for mechanical reason was 31% at similar follow-up times at 2, 5, and 10 years. CONCLUSIONS This technique provided a stable wrist and partially restored wrist motion with limited pain. However, further surgical procedures may be necessary to reach this goal. TYPE OF STUDY/LEVEL OF EVIDENCE Therapeutic IV.

Our experience of solitary plasmacytoma of the bone: improved PFS with a short-course treatment by IMiDs or proteasome inhibitors combined with intensity-modulated radiotherapy

Emmanuelle Le Ray, Lisa Belin, Corine Plancher, Philippe Anract, Antoine Babinet, Val erie Dumaine, J erôme Tamburini, B en edicte Deau Fischer, Lise Willems, Leonardo Magro, Thierry Facon, Xavier Leleu, Didier Bouscary and Youlia M. Kirova Service d’H ematologie, Hôpital Cochin, AP-HP, Paris, France; Facult e de M edecine Sorbonne Paris Cit e, Universit e Paris Descartes, Paris, France; Department of Biostatistics, Institut Curie, Paris, France; Service de chirurgie orthop edique, Hôpital Cochin, AP-HP, Paris, France; Institut Cochin, D epartement D eveloppement Reproduction Cancer, CNRS UMR8104, INSERM U1016, Paris, France; Service d’H ematologie, CHRU Lille, Lille, France; Service d’h ematologie, CHU de Poitiers, Poitiers, France; Department of Radiation Therapy, Institut Curie, Paris, France

Proximal femoral osteosarcoma: Diagnostic challenges translate into delayed and inappropriate management

BACKGROUND The proximal femuris is an uncommon site of osteosarcoma. The unusual manifestations at this site may lead to diagnostic and therapeutic mistakes. We therefore performed a retrospective study to estimate the proportions of patients with imaging study findings and/or clinical manifestations typical for osteosarcoma and/or inappropriate treatment decisions. HYPOTHESIS Proximal femoral osteosarcoma often produces atypical clinical and radiological presentations. MATERIAL AND METHODS Consecutive patients who underwent surgery at our center to treat proximal femoral osteosarcoma were included. For each patient, we collected the epidemiological characteristics, clinical symptoms, imaging study findings, treatment, and tumor outcome. Proportions were computed with their confidence intervals. RESULTS Twelve patients had surgery for proximal femoral osteosarcoma between 1986 and 2015. Imaging findings were typical in 1 (8%) patient; they consisted of ill-defined osteolysis in 11/12 (92%) patients, a periosteal reaction in 1/12 (8%) patient, soft tissue involvement in 7/12 (58%) patients, and immature osteoid matrix in 11/12 (92%) patients. No patient had the typical combination of pain with a soft tissue swelling. Management was inappropriate in 2/12 (17%) patients, who did not undergo all the recommended imaging studies before surgery and were treated in another center before the correct diagnosis was established. At last follow-up, 4 patients had died (after a mean of 7 years) and 8 were alive (after a mean of 4 years). CONCLUSION Proximal femoral osteosarcoma is uncommon and rarely produces the typical clinical and imaging study findings. The atypical presentation often results in diagnostic errors and inappropriate treatments. Ill-defined osteolysis on standard radiographs should prompt computed tomography or magnetic resonance imaging of the proximal femur. Treatment in a specialized center is imperative. LEVEL OF EVIDENCE IV, retrospective study.

Tumores malignos óseos del fémur proximal: exéresis y reconstrucciones

En el femur proximal puede localizarse un tumor oseo maligno primario o, con mayor frecuencia, una metastasis. El tratamiento quirurgico puede ser paliativo o curativo. En el ultimo caso, comprende dos etapas: la reseccion del tumor (objetivo oncologico) y la reconstruccion osea y de los tejidos blandos (objetivo funcional). En el tratamiento curativo la exeresis sera amplia, con margenes de reseccion suficientes (en el hueso y los tejidos blandos). En caso de invasion articular la exeresis sera extraarticular, con extraccion de la articulacion coxofemoral en un solo bloque a efectos de garantizar margenes de reseccion sanos. El tratamiento quirurgico de las metastasis esta basicamente indicado en caso de fractura patologica o de lesion con riesgo de fractura. En la mayoria de estos casos, la cirugia consiste en una exeresis intralesional destinada a reducir el volumen tumoral, asociada a una reconstruccion que garantice una buena estabilidad mecanica. La reconstruccion puede efectuarse de diversas maneras. La reconstruccion del femur proximal se puede hacer con una protesis compuesta o con una protesis masiva, a su vez modular o a medida. Para las lesiones de pequeno tamano limitadas a la epifisis y al cuello femoral, asi como para el tratamiento paliativo de las metastasis femorales proximales, se puede usar un vastago femoral estandar, corto o largo. Para la reconstruccion articular se practica una hemiartroplastia con protesis intermedia o, en algunos casos, con protesis total de cadera.

Principios terapéuticos quirúrgicos de los tumores óseos de los miembros (incluyendo la cintura escapular y el hueso coxal)

Los tumores oseos malignos primarios son tumores infrecuentes. Su baja incidencia y la necesidad de un tratamiento multidisciplinario, a cargo de medicos con experiencia en esta afeccion, imponen el traslado de estos pacientes a los centros de referencia o, como minimo, el analisis de los casos en reuniones de concertacion multidisciplinaria especializada. La reseccion quirurgica de los tumores malignos oseos debe ser amplia, es decir, habra que dejar en contacto con el tumor una capa de tejido sano (margen). En mas del 80% de los casos es posible reconstruir el segmento oseo o la articulacion y, por tanto, conservar el miembro. Las diafisis se reconstruyen con preferencia mediante una combinacion de auto y aloinjerto con osteosintesis. En la mayoria de los casos, las articulaciones se reconstruyen con una protesis masiva o se efectua una reconstruccion compuesta (protesis rodeada por un aloinjerto). Cuando, ademas de la articulacion, la reseccion incluye los musculos periarticulares, se prefiere la artrodesis. Las resecciones de la pelvis, si se limitan al ala iliaca o al marco isquiopubico, no necesitan reconstruccion. En cambio, las resecciones de la region acetabular y del ala iliaca, que interrumpen la continuidad del anillo pelvico, imponen la reconstruccion a efectos de lograr un resultado funcional aceptable. La reconstruccion de la cadera tras la reseccion del acetabulo es mucho mas dificil. En caso de reseccion de la region acetabular, sola o asociada a la del marco isquiopubico, los autores de este articulo prefieren la reconstruccion con un autoinjerto de femur proximal y una protesis. Tambien es posible usar las protesis en silla de montar, las de tipo Mac Minn con auto o aloinjerto, las protesis modulares y a medida, los aloinjertos masivos con o sin protesis y las artrodesis femoroiliacas. En caso de reseccion del ala iliaca y de la region acetabular, las reconstrucciones posibles son: la artrodesis isquiofemoral y femorosacra, la reconstruccion mediante autoinjerto de femur proximal y protesis, la medializacion de la cabeza femoral, los aloinjertos y las protesis masivas. Las amputaciones se tratan en otro articulo de la EMC.