G. Ulrich Exner

Osteosarcoma Relapse After Combined Modality Therapy: An Analysis of Unselected Patients in the Cooperative Osteosarcoma Study Group (COSS)

PURPOSE To evaluate the impact of patient, tumor, and treatment-related factors on outcome in unselected patients with recurrent osteosarcoma. PATIENTS AND METHODS Five hundred seventy-six consecutive patients who had achieved a first complete surgical remission (CR) during combined-modality therapy on neoadjuvant Cooperative Osteosarcoma Study Group (COSS) protocols and then developed recurrent osteosarcoma were analyzed (median time from biopsy to relapse, 1.6 years; range, 0.1 to 14.3 years). There were 501 patients with metastases, 44 with local recurrences, and 31 with both. Metastases involved lungs (469 patients), bones (90 patients), and/or other sites (54 patients). RESULTS After a median follow-up of 1.2 years for all patients and 4.2 years for survivors, actuarial overall survival (OS) rates at 2, 5, and 10 years were 0.38, 0.23, and 0.18, respectively. Five-year OS was 0.39 for 339 patients with and 0.00 for 229 patients without a second surgical CR (P < .0001). A long time to relapse, a solitary lesion, and, in the case of pulmonary metastases, unilateral disease and the absence of pleural disruption, were of positive prognostic value in uni- and multivariate analyses, as were a second surgical CR and the use of second-line chemotherapy. Radiotherapy was associated with moderately prolonged survival in patients without a second CR. The very limited prognostic differences associated with the use of second-line chemotherapy appeared to be more pronounced with polychemotherapy. CONCLUSION Time to relapse and tumor burden correlate with postrelapse outcome in osteosarcoma. Complete surgery is an essential component of curative second-line therapy. Chemotherapy, particularly chemotherapy with more than one agent, may contribute to limited improvements in outcome.

Osteosarcoma of the Pelvis: Experience of the Cooperative Osteosarcoma Study Group

PURPOSE To define patients and tumor characteristics as well as therapy results, patients with pelvic osteosarcoma who were registered in the Cooperative Osteosarcoma Study Group (COSS) were analyzed. PATIENTS AND METHODS Sixty-seven patients with a high-grade pelvic osteosarcoma were eligible for this analysis. Fifteen patients had primary metastases. All patients received chemotherapy according to COSS protocols. Thirty-eight patients underwent limb-sparing surgery, 12 patients underwent hemipelvectomy, and 17 patients did not undergo definitive surgery. Eleven patients received irradiation to the primary tumor site: four postoperatively and seven as the only form of local therapy. RESULTS Local failure occurred in 47 of all 67 patients (70%) and in 31 of 50 patients (62%) who underwent definitive surgery. Five-year overall survival (OS) and progression-free survival rates were 27% and 19%, respectively. Large tumor size (P =.0137), primary metastases (P =.0001), and no or intralesional surgery (P <.0001) were poor prognostic factors. In 30 patients with no or intralesional surgery, 11 patients with radiotherapy had better OS than 19 patients without radiotherapy (P =.0033). Among the variables, primary metastasis, large tumor, no or intralesional surgery, no radiotherapy, existence of primary metastasis (relative risk [RR] = 3.456; P =.0009), surgical margin (intralesional or no surgical excision; RR = 5.619; P <.0001), and no radiotherapy (RR = 4.196; P =.0059) were independent poor prognostic factors. CONCLUSION An operative approach with wide or marginal margins improves local control and OS. If the surgical margin is intralesional or excision is impossible, additional radiotherapy has a positive influence on prognosis.

Arthropathy of the knee joint caused by synovial hemangioma.

Degenerative changes resembling hemophilic arthropathy may be a complication of synovial hemangioma in the knee. It is thought that arthropathy is caused by repeated bleeding episodes similar to the joint disease in hemophilia. Four children aged 4 to 9 years at surgery were treated by anterior open synovectomy for intra-articular synovial hemangiomas. At open surgery the cartilage of all patients showed changes on the surface with yellow hematin staining in all cases, without ulceration in three patients but one, who showed severe degenerative changes at age 9 years. At 15 months follow-up the patient with severe degenerative changes had severely impaired flexion and mild extension deficit. The three others were asymptomatic at 1 to 6 years follow-up. The magnetic resonance images were typical and can be considered pathognomonic; however, the referral diagnosis included the correct diagnosis in none of them. Treatment should be initiated as early as possible to reduce the risk of damage to the cartilage. Treatment by complete open synovectomy in these four patients was performed without significant bleeding problems, and no recurrence was seen.

Die intertrochantäre Korrekturosteotomie nach Imhäuser

ZusammenfassungOperationszielOperative Korrektur der durch den epiphysären Gleitprozess entstandenen Deformität des proximalen Femurs mittels einer wiederaufrichtenden (zumeist flektierenden und innenrotierenden) Osteotomie auf intertrochantärem Niveau mit dem Ziel einer Reduktion der impingementverursachenden Offsetstörung des koxalen Femurendes bzw. der Prävalenz einer sekundären Koxarthrose.IndikationenChronische und subakute Verlaufsformen der Epiphyseolysis capitis femoris mit einer Epiphysendislokation in der axialen Röntgenaufnahme von 30–60° (ET-Winkel) und in der anteroposterioren Aufnahme von ΔED > 20° (CCD-Winkel minus ED-Winkel).KontraindikationenAkute Phase bzw. Verlaufsform der Epiphyseolysis capitis femoris.Sekundäre Koxarthrose (nach Epiphyseolysis capitis femoris).Traumatische Epiphysenlösung.OperationstechnikExposition des proximalen Femurs (ventraler Schenkelhals, intertrochantäre Ebene, proximale Femurdiaphyse) durch einen anterolateren Zugang; Fixation der Femurepiphyse mit zwei 3,0-mm-Steinmann-Nägeln in paralleler dorsomedialer Richtung; Einschlagen des Plattensitzinstruments gemäß geplanter, vornehmlich flektierender Achsenkorrektur; intertrochantäre keilförmige Osteotomie unter Ausrichtung der Keilentnahme entsprechend der Operationsplanung; Angleichung der Osteotomieebenen und Osteosynthese mit 90°-AO-Adoleszentenplatte unter interfragmentärer Kompression.WeiterbehandlungMobilisation an Unterarmgehstützen unter Entlastung der Extremität für 2 Monate postoperativ; stufenweiser Belastungsaufbau nach Maßgabe der knöchernen Konsolidierung der Osteotomie; Röntgenkontrollen 2 und 4 Monate postoperativ, weitere klinisch-radiologische Nachkontrollen bis Wachstumsabschluss (Epiphysenverschluss); Entfernung des Osteosynthesematerials ab 1 Jahr postoperativ.ErgebnisseRetrospektive Nachuntersuchung von 51 Patienten mit unilateraler mittel- bis hochgradiger Epiphyseolysis capitis femoris (Indikation: epiphysärer Gleitwinkel 30–60°, mittlerer präoperativer Gleitwinkel 45°), welche mit einer intertrochantären Osteotomie nach Imhäuser und Epiphysennagelung operativ korrigiert wurden. Nach einem durchschnittlichen Beobachtungszeitraum von 24 Jahren (Bereich 20–29 Jahre) erwiesen sich 28 (55%) der 51 Hüftgelenke als klinisch asymptomatisch und radiologisch frei von degenerativen Veränderungen; bei 14 Hüftgelenken (28%) waren moderate, bei neun (17%) fortgeschrittene klinische und radiologische Veränderungen zu verzeichnen.Operativ bedingte Komplikationen traten bei insgesamt sechs Hüftgelenken auf: In einem Fall Bruch eines Steinmann-Nagels nach Schädigung desselben durch die Plattenklinge; in zwei Fällen Notwendigkeit einer Revisionsosteosynthese der intertrochantären Osteotomie infolge inkorrekter Implantatlage; bei einem Hüftgelenk Entwicklung einer partiellen Femurkopfnekrose; in zwei Fällen „low-grade“ Osteomyelitis, nach Implantatentfernung ausgeheilt.AbstractObjectiveSurgical correction of deformities of the proximal femur caused by epiphyseal displacement by restorative (usually inflection and internal rotation) osteotomy at the intertrochanteric level with the aim of reducing both the offset disorder of the coxal end of the femur that is causing impingement and the prevalence of secondary coxarthrosis.IndicationsChronic and subacute manifestations of slipped capital femoral epiphysis with an epiphyseal dislocation in the radiographic axial view of 30–60° (ET angle) and ΔED > 20° (CCD angle minus ED angle) in the anteroposterior view.ContraindicationsAcute phase or course of slipped capital femoral epiphysis.Secondary coxarthrosis (after slipped capital femoral epiphysis).Traumatic epiphyseolysis.Surgical TechniqueExposure of the proximal femur (anterior femoral neck, intertrochanteric plane, proximal femoral diaphysis) through an anterolateral approach; fixation of the femoral epiphysis with two 3.0-mm Steinmann nails positioned parallel in a posteromedial direction; the seating chisel is hammered in according to the planned, usually inflectional axial correction; intertrochanteric osteotomy by excision of a wedge oriented in accordance with preoperative planning; adaptation of the osteotomy surfaces and osteosynthesis with a 90° AO adolescent plate under interfragmentary compression.Postoperative ManagementMobilization on underarm crutches with unloading of the extremity for 2 months postoperatively; gradual increasein loading depending on osseous consolidation of the osteotomy; radiographic assessment 2 and 4 months postoperatively, further clinical and radiologic follow-up until growth is completed (closure of the growth plate); removal of implants at 1 year postoperatively or thereafter.ResultsRetrospective follow-up of 51 patients with unilateral, moderate to severe slipped capital femoral epiphysis (indication: epiphyseal displacement angle of 30–60°, average preoperative displacement angle 45°) that was corrected surgically by Imhäuser intertrochanteric osteotomy and epiphyseal nailing. After an average observation time of 24 years (range: 20–29 years), 28 (55%) of the 51 hip joints were clinically asymptomatic and radiologically free of degenerative changes; moderate clinical and radiologic changes were recorded for 14 hip joints (28%), and advanced changes for nine (17%).Complications related to surgery were apparent in a total of six hip joints: in one case, the Steinmann nail broke after being damaged by the plate blade; in two cases, revision osteosynthesis of the intertrochanteric osteotomy was necessary due to incorrect implant positioning; partial femoral head necrosis developed in one hip joint; there were two cases of low-grade osteomyelitis that healed after implant removal.

Rotationplasty (Borggreve/Van Nes and modifications) as an alternative to amputation in failed reconstructions after resection of tumours around the knee joint

Failure of reconstructions as a result of infective or aseptic loosening and massive bone loss may make amputation necessary. If neurovascular structures can be preserved to keep a functional foot, rotationplasty may be considered an option. Four patients treated for malignant bone tumours (two osteosarcomas, one Ewing sarcoma, and one malignant fibrous histiocytoma) of the proximal tibia and distal femur (n=2 each) at the ages of 13 to 21 years had reconstructions that failed 3, 4, 5, and 15 years later. In three patients the cause was intractable infection, and in one loosening with shortening and deficiency of the extensor mechanism. The patients had the option to contact patients who had had rotationplasty as the primary procedure for tumours or severe femoral deficiencies. In two patients an AI-type rotationplasty was done, in one a type AII rotationplasty, and in the fourth a modification with shortening of the lower leg but retention of the knee joint. There were no postoperative complications such as persisting infections, fractures, or pseudarthrosis. All patients are active and are able to go alpine skiing or snowboarding. The main advantage of procedures in which a sensory-motor functional foot is retained is to avoid neuroma pain or phantom sensations. The foot allows for active knee movement of the orthoprosthesis and full weight bearing. It is of great psychological help for the patients to have contact during the decision-making with patients who have had similar procedures. It should be considered as an alternative to amputation.

Metabolic and orthopedic management of X-linked vitamin D-resistant hypophosphatemic rickets

PurposeTherapy of vitamin D-resistant hypophosphatemic rickets (VDXLR) consists of oral phosphate and vitamin D supplements. Bone deformities, pain, and small stature can occur even in children with good compliance, requiring surgical correction and bone lengthening. However, only few surgical reports are available.MethodsTwelve patients (three males) with VDXLR were followed at our institution. Median age at diagnosis was 3 9/12 years (range, birth to 11 10/12) with a follow-up period of 7 8/12 years (1 9/12–30) and age at last follow-up of 13 6/12 years (2–30). Eight patients underwent surgical correction, three of them in combination with bone lengthening. The corrections were performed at the end of growth in three patients. Clinical endpoints were height, leg axis, and pain.ResultsSingle bilateral surgical correction was performed in six patients; one patient each had three and five corrections. Bone lengthening was performed in three patients. At last follow-up, the height of seven operated patients was within normal range. In addition, leg axis was normalized in six patients with mild genua vara in two. Only one patient complained of intermittent pain. Bone healing was excellent; surgical complications were rare. There was no radiological evidence of degenerative arthropathy.ConclusionsMedical treatment remains the main pillar of therapy in children with VDXLR. In case of bone deformity, surgery can safely be performed, independent of age or bone maturation. All patients were satisfied with the results of axial corrective surgery and bone lengthening, and in the majority only one corrective intervention was needed.

Extra-articular en bloc resection of the talocrural and the talocalcaneonavicular joints for primary malignant synovial tumour (myxoinflammatory fibroblastic sarcoma).

A 13-year-old boy presented with a diagnosis of intra-articular myxoinflammatory fibroblastic sarcoma of the ankle. There had been no previous description of a sarcoma arising directly from the synovium of the ankle and limb salvage for malignant tumours of the ankle has rarely been reported. We treated him by peritalar extra-articular resection, and draw attention to this rare tumour and to a technique of limb-sparing resection of the ankle joint.

Extra-articular resection of the hip with a posterior column-preserving technique for treatment of an intra-articular malignant lesion. A report of two cases.

A malignant lesion extending into the hip may necessitate en bloc resection of the joint, which may result in substantial loss of pelvic bone stock and compromise pelvic stability. Few reports have dealt specifically with the technical aspects of extra-articular resection of the hip1-4. In this report, we present a technique that allows en bloc resection of the hip with use of a periacetabular osteotomy, reconstruction of the acetabulum with an acetabular allograft, and total hip arthroplasty through a combined ilioinguinal and iliofemoral approach. Fig. 1 The utilitarian incision described by Enneking is used. This incision is well suited for a combination of a modified Smith-Petersen approach, representing the standard approach for the Bernese periacetabular osteotomy, and the ilioinguinal approach described by Judet et al., allowing plate application along the anterior column of the pelvis. We present the cases of two patients in whom we performed an extra-articular resection of the hip for the treatment of intra-articular extension of a proximal femoral malignant lesion. By employing the principles of the Bernese periacetabular osteotomy, we preserved the posterior acetabular column and maintained the continuity and stability of the pelvic ring5. Both patients were informed that data concerning the cases would be submitted for publication. Fig. 2 En bloc resection of the hip was performed with use of a periacetabular osteotomy, as described by Ganz et al., leaving the posterior column intact. The osteotomies were performed in the following order: ischial (a), pubic (b), anterior aspect of the ilium (c), and posterior aspect of the ilium (d). Fig. 3 An acetabular allograft (hatched area) is used for reconstruction of the acetabular bone stock. The graft is fixed with screws (not shown in the diagram but can be seen in Figures 4-B and 5-C) augmented with an anterior reconstruction plate. Fig. 4-A Figs. 4-A and …

Acute correction of severe neglected club feet using a circumferential incision.

Severe neglected club feet in older children are a problem frequently seen in less privileged countries. Gradual correction using external fixation is a reliable method but needs sufficient infrastructure and professional supervision for follow-up. Acute corrections may be justified if such infrastructure is not available and a single stage solution is needed. A circumferential fasciocutaneous flap has been developed to allow skin coverage for acute corrections, which has been used besides a modification of other flaps used in correction of severe club feet.

Multiple subungual soft tissue metastases from a chondrosarcoma.

Chondrosarcoma metastasises to the lungs and from there to other organs. A patient with several metastases in the soft tissues of the fingers and toes had previously been treated for a chondrosarcoma of the foot. Subungual metastases of chondrosarcoma are unusual and there is no evidence based treatment. We therefore treated the lesion of the finger by total resection of the nail (R0).