Fibulocalcaneal Impingement in a Growing Child With Otherwise Asymptomatic Talocalcaneal Coalition.

Subfibular impingement has been described in patients with flatfoot. It possibly occurs with valgus deformity associated with talocalcaneal coalition. We observed symptomatic unilateral fibular impingement initially on the left foot of an 11-year-old female with an otherwise asymptomatic bilateral talocalcaneal coalition. From the age of 8 years, she had complained of pain around the left fibular tip. Magnetic resonance imaging showed a partial talocalcaneal coalition. At 10 years of age, it was questioned whether the pain was related to the coalition. However, imaging of the asymptomatic right foot also showed a talocalcaneal coalition, with the coalition in both feet appearing equal. Additionally, the peroneal trochlea appeared particularly prominent, more so on the left than on the right foot. Therefore, the symptoms were suspected to have been caused only by fibulocalcaneal impingement owing to a relatively long fibula. Subperiosteal shortening of the fibula was performed at when she was 11 years old. A bed for the peroneal tendons was created around the remaining epiphysis of the fibula, and the fibular ligaments were reattached with Arthrex® anchors (Arthrex, Naples, FL). At 14 months postoperatively, the patient was free of pain with unrestricted movement, although the follow-up imaging studies showed complete bony fusion on the medial aspect of the coalition between the talus and calcaneus. Approximately 1.5 years after surgery, our female patient at 12.5 years old complained of the same problems on her right foot, definitely occurring only around the fibula. The same procedure was performed as she had undergone on the left foot. At the last follow-up examination, she was asymptomatic 2.5 years after the initial surgery of the left foot and 11 months after surgery on the right. Talocalcaneal coalition can cause moderate to severe hindfoot deformity, leading to fibulocalcaneal impingement. Hence, treatment should be determined accordingly.

Resection of Renal Cell and Prostate Carcinoma Sternum Metastases with Long-Term Follow-Up: A Report of 2 Cases.

Introduction: Rarely solitary sternum metastases are addressed by resection. Two additional cases are presented as they are interesting because of their long-term follow-up. Case Presentation: Case 1: A renal cell carcinoma was treated by transabdominal nephrectomy at age 64. Right iliac bone and sternum metastases were diagnosed 7 months later and treated by internal hemipelvectomy followed by sternum metastasectomy 6 weeks after the internal hemipelvectomy. At 12-year follow-up, the patient appears disease free. Case 2: Prostate cancer was treated by prostatectomy at age 67. A subsequent solitary sternum metastasis was resected 10 years later for persistent PSA-activity despite repeated radiotherapy. The patient remains asymptomatic for 3 years now. Conclusion: Resection of sternum metastases may have curative potential and should be considered in tumours known to be rather resistant to chemo- and/or radiotherapy.

Bending osteotomy through the distal tibial physis for stable reduction of the ankle joint in fibular hemimelia. Update at skeletal maturity.

Fibular hemimelia is a complex longitudinal malformation of the lower extremity with partial or complete deficiency of the fibula resulting in dorso-fibular dislocation of the hindfoot. Typically associated are talocalcaneal coalition, absence of rays of the foot, diaphyseal tibial deformity of valgus-procurvatum type and longitudinal growth deficiency. We have addressed the deformity of the distal tibial epiphysis surgically by a metaphyseal osteotomy to bend through the physis inspired by the Pemberton's acetabular osteotomy in 7 to 21-month-old children. Short-term results of a maximum of 42 months of follow-up have been published. Meanwhile, the first 4 patients thus treated have reached skeletal maturity, and the long-term results are presented. Three patients with unilateral and one patient with bilateral fibular hemimelia were operated on as described before at ages 7, 9, 15, and 18 months. Subsequently, several other procedures have been performed on all patients mainly consisting of lengthening of the tibia combined with axial corrections as well as additional foot alignment if needed. The osteotomy leads to stable axial retainment of the hindfoot in all patients without premature closure of the physis. Most of them need adaptation of footwear. None of the patients at present would favor to have been treated by amputation. The technique has shown good clinical results with preserved growth of the physis of the distal tibia and full axial weight bearing at long-term. In selected cases, this technique should be considered as a valuable alternative to other reconstructions.

Prognostic Factors in High-Grade Osteosarcoma of theExtremities or Trunk: An Analysis of 1,702 Patients Treatedon Neoadjuvant Cooperative Osteosarcoma Study GroupProtocols.

PURPOSE: To define prognostic factors for response and long-term outcome for a wide spectrum of osteosarcomas, extending well beyond those of the typical young patient with seemingly localized extremity disease. PATIENTS AND METHODS: A total of 1,702 consecutive newly diagnosed patients with high-grade osteosarcoma of the trunk or limbs registered into the neoadjuvant studies of the Cooperative Osteosarcoma Study Group before July 1998 were entered into an analysis of demographic, tumor-related, and treatment-related variables, response, and survival. The intended therapeutic strategy included preoperative and postoperative chemotherapy with multiple agents as well as surgery of all operable lesions. RESULTS: Axial tumor site, male sex, and a long history of symptoms were associated with poor response to chemotherapy in univariate and multivariate analysis. Actuarial 10-year overall and event-free survival rates were 59.8% and 48.9%. Among the variables assessable at diagnosis, patient age (actuarial 10-year survival ≥ 40, 41.6%; < 40, 60.2%; P = .012), tumor site (axial, 29.2%; limb, 61.7%; P < .0001), and primary metastases (yes, 26.7%; no, 64.4%; P < .0001), and for extremity osteosarcomas, also size (≥ one third, 52.5%; < one third, 66.7%; P < .0001) and location within the limb (proximal, 49.3%; other, 63.9%; P < .0001), had significant influence on outcome. Two additional important prognostic factors were treatment related: response to chemotherapy (poor, 47.2%; good, 73.4%; P < .0001) and the extent of surgery (incomplete, 14.6%; macroscopically complete, 64.8%; P < .0001). All factors except age maintained their significance in multivariate testing, with surgical remission and histologic response emerging as the key prognostic factors. CONCLUSION: Tumor site and size, primary metastases, response to chemotherapy, and surgical remission are of independent prognostic value in osteosarcoma.

Physeal distraction for joint preservation in malignant metaphyseal bone tumors in children.

BACKGROUND: Physeal distraction facilitates metaphyseal bone tumor resection in children and preserves the adjacent joint. The technique was first described by Cañadell. Tumor resection procedures allowing limb-sparing reconstruction have been used increasingly in recent years without compromising oncologic principles. QUESTIONS/PURPOSES: We report our results with Cañadell's technique by assessing tumor control, functional outcome, and complications. METHODS: Six consecutive children with primary malignant metaphyseal bone tumors underwent physeal distraction as a part of tumor resection. Tumor location was the distal femur in four patients, the proximal humerus in one patient, and the proximal tibia in one patient. The functional outcome was evaluated after a minimum of 18 months (median, 62 months; range, 18-136 months) using the Musculoskeletal Tumor Society (MSTS) score and the Toronto Extremity Salvage Score (TESS). RESULTS: At latest followup, five patients were alive and disease-free and one had died from metastatic disease. All tumor resections resulted in local control; there were no local recurrencies. The mean MSTS score was 79% (range, 53%-97%) and corresponding mean TESS was 83% (range, 71%-92%). In one case, postoperative infection required amputation of the proximal lower leg. All physeal distractions were successful except for one patient in whom distraction resulted in rupturing into the tumor. This situation was salvaged by transepiphyseal resection. CONCLUSIONS: We consider Cañadell's technique a useful tool in the armamentarium to treat children with malignant tumors that are in close proximity to an open physis. LEVEL OF EVIDENCE: Level IV, therapeutic study. See Guidelines for Authors for a complete description of levels of evidence.

Serpentine fibula-polycystic kidney syndrome caused by truncating mutations in NOTCH2.

Serpentine fibula-polycystic kidney syndrome (SFPKS) is a rare disorder characterized by the association of craniofacial anomalies, radiological findings (wormian bones, elongated and bowed fibulae), polycystic kidneys, and normal intelligence. SFPKS shares many similarities with Hajdu-Cheney syndrome (HCS). We and others recently showed that truncating mutations in the last exon of NOTCH2 cause HCS. Here, we identify by Sanger sequencing two different heterozygous truncating mutations in the last exon of NOTCH2 in two unrelated patients with SFPKS. In one family, we show that the mutation occurred de novo. These findings demonstrate that SFPKS and HCS are both conditions caused by NOTCH2 mutations.

Cement Spacer Formed in a 3D-Printed Mold for Endoprosthetic Reconstruction of an Infected Sarcomatous Radius: A Case Report.

CASE: The ulcerated recurrent clear cell sarcoma of the forearm with bony invasion of the radius needed an uncontaminated resection and control of infection. A mold was printed based on CT-reconstructed 3D models of the patient's anatomy to create an antibiotic-loaded cement spacer as endoprosthetic replacement used in combination with soft-tissue reconstruction and systemic antibiotics. CONCLUSION: This then undescribed novel technique allowed for fast local recovery of the patient's hand function and return to work. In selected cases, such an anatomically formed spacer may be preferred for faster functional recovery and longer intervals before definitive reconstruction is possible.

When SUV Matters: FDG PET/CT at Baseline Correlates with Survival in Soft Tissue and Ewing Sarcoma.

INTRODUCTION: The role of positron-emission tomography/computed-tomography (PET/CT) in the management of sarcomas and as a prognostic tool has been studied. However, it remains unclear which metric is the most useful. We aimed to investigate if volume-based PET metrics (Tumor volume (TV) and total lesions glycolysis (TLG)) are superior to maximal standardized uptake value (SUVmax) and other metrics in predicting survival of patients with soft tissue and bone sarcomas. MATERIALS AND METHODS: In this retrospective cohort study, we screened over 52'000 PET/CT scans to identify patients diagnosed with either soft tissue, bone or Ewing sarcoma and had a staging scan at our institution before initial therapy. We used a Wilcoxon signed-rank to assess which PET/CT metric was associated with survival in different patient subgroups. Receiver-Operating-Characteristic curve analysis was used to calculate cutoff values. RESULTS: We identified a total of 88 patients with soft tissue (51), bone (26) or Ewing (11) sarcoma. Median age at presentation was 40 years (Range: 9-86 years). High SUVmax was most significantly associated with short survival (defined as <24 months) in soft tissue sarcoma (with a median and range of SUVmax 12.5 (8.8-16.0) in short (n = 18) and 5.5 (3.3-7.2) in long survival (≥24 months) (n = 31), with (p = 0.001). Similar results were seen in Ewing sarcoma (with a median and range of SUVmax 12.1 (7.6-14.7) in short (n = 6) and 3.7 (3.5-5.5) in long survival (n = 5), with (p = 0.017). However, no PET-specific metric but tumor-volume was significantly associated (p = 0.035) with survival in primary bone sarcomas (with a median and range of 217 cm3 (186-349) in short survival (n = 4) and 60 cm3 (22-104) in long survival (n = 19), with (p = 0.035). TLG was significantly inversely associated with long survival only in Ewing sarcoma (p = 0.03). DISCUSSION: Our analysis shows that the outcome of soft tissue, bone and Ewing sarcomas is associated with different PET/CT metrics. We could not confirm the previously suggested superiority of volume-based metrics in soft tissue sarcomas, for which we found SUVmax to remain the best prognostic factor. However, bone sarcomas should probably be evaluated with tumor volume rather than FDG PET activity.

Efficacy of a CDK4/6 Inhibitor in a Patient with Breast Cancer and Liposarcoma: A Case Report and Review of the Literature.

BACKGROUND: The cyclin D/cyclin-dependent kinase (CDK)4/6 inhibitor of the CDK4 (INK4)/retinoblastoma (Rb) pathway plays a crucial role in cell cycle progression. Selective CDK4/6 inhibitors specifically target a variety of tumors, with the main focus on hormone receptor(HR)-positive and human epidermal growth factor receptor 2(HER2)-negative breast cancer (BC). CASE REPORT: We report on the efficacy of neoadjuvant palbociclib and letrozole application in a patient suffering from invasive estrogen receptor (ER)+/HER2- BC and concurrent well-differentiated and dedifferentiated liposarcoma (WD-DDLPS) of the thigh. Clinical and histological workup upon surgery revealed significant regressive changes in both the liposarcoma and the BC. The 24-month follow-up shows no signs of disease. CONCLUSION: CDK4/6 inhibitors exhibit a high therapeutic potential, although reliable prognostic markers need to be identified.

Extra-abdominal desmoid tumours - further evidence for the watchful waiting policy.

PURPOSE: Extra-abdominal desmoid tumours are benign and rare, and lead to a persistent treatment dilemma because of their high recurrence rate and their heterogeneous behaviour. The goal of this retrospective study was to evaluate the results of different treatment modalities for extra-abdominal desmoid tumours at four sarcoma treatment centres. METHODS: The mean follow-up time for the 96 patients included in the study (63.5% female; mean age 38.9 years) was 8.4 years (2.0–40.5 years). The initial treatments were surgery (n = 44), surgery with radiation (n = 16), watchful waiting (n = 15), radiation only (n = 9), or systemic treatment (n = 12). Patient demographics, tumour sites, and the follow-up status of all patients were reviewed and evaluated for each of the treatment modalities. RESULTS: The local recurrence rate was 45.5% in patients with primary surgical treatment and 37.5% following surgery combined with irradiation. Patients who were treated with radiation alone showed regressive (33.3%) or stable disease (66.6%). Systemic treatment alone resulted in disease progression in 41.7% of our patients. In the watchful waiting group, 73.3% showed stable disease, 20.0% showed spontaneous regression, and 6.7% showed progression after a mean follow-up of 4.1 years (2.0–11.5 years). CONCLUSIONS: Our results suggest that a watchful waiting approach should be the first line treatment in asymptomatic desmoid tumours. However, radiation can help improve local control rates in patients who have undergone surgery. Progression and local recurrence rates following systemic treatment were comparable to those observed in surgery combined with radiation.

Progressive genu valgum resulting from idiopathic lateral distal femoral physeal growth suppression in adolescents.

BACKGROUND: Progressive genu varum with disturbance of medial tibial physeal growth (classic Blount disease) is a well-studied phenomenon, and in rare cases, genu varum can occur because of medial distal femoral physeal growth disturbance (so-called femoral Blount disease). To our knowledge, progressive genu valgum caused by disturbance of lateral distal femoral physeal growth has not been described. METHODS: This article presents the history, clinical findings, imaging studies, results of surgical treatment, and speculation regarding the etiology of this disorder in 2 girls. RESULTS: Both patients were overweight girls without a history of injury or prior medical disorder who presented with relatively rapid progression of knock-kneed deformity. Magnetic resonance imaging studies demonstrated unusual circular peglike disturbances of the lateral distal femoral physes. Treatments in both cases included corrective distal femoral osteotomy and epiphysiodesis to prevent further deformity. CONCLUSIONS: Obesity and repetitive microtrauma, superimposed on genetic factors (physis that is sensitive to compressive forces), may contribute to the etiology of this rare disorder.

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.

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.

Results of treatment of malignant soft tissue tumours in the groin.

Soft tissue sarcomas of the inguinal region are a challenge with regard to achieving clear margins, reconstruction of the femoral vessels, and soft tissue coverage. Six men aged 39 to 48 years and one woman of 56 were treated for soft tissue sarcomas of the groin. All patients were treated with local en bloc resections including the femoral artery, vein, and nerve. In two patients the soft tissue defect was covered primarily with an ipsilateral rectus abdominis muscle flap, in two others (because of wound dehiscence) coverage was achieved with the opposite rectus abdominis muscle pedicle flap as we were afraid of closure of the ipsilateral deep epigastric vessels. In the others local measures were sufficient, however, wound healing was usually delayed. Histopathological examination showed tumour-free margins in each case. One patient developed a local recurrence, but had had no radiotherapy because of problems with wound healing. A high rate of local tumour control in soft tissue sarcomas of the inguinal region can be achieved with the combination of surgical resection and radiotherapy. No compromise should be made with aggressive soft tissue coverage to protect the vascular reconstruction, control wound healing after neoadjuvant radiotherapy, or allow immediate adjuvant radiotherapy. At primary wound closure we would generally use an ipsilaterally distally pedicled rectus abdominis muscle flap if the deep epigastric vessels can be preserved or - if the ipsilateral vessels need be resected to achieve clearance of tumour - use a contralateral flap.

Congenital unilateral pseudarthrosis of the olecranon.

Congenital pseudarthrosis of the olecranon is a rare condition. Three isolated cases with bilateral presentation, different treatment modalities and variable outcome were reported in the orthopaedic literature. In this presented case a pseudarthrosis of the right olecranon in a 13-year-old boy was treated by pseudarthrosis resection, bone graft interposition and tension band osteosynthesis. Hardware removal was performed nine months after the initial procedure. At 6-year follow-up at the end of growth, the patient has no pain and a free range of motion of his dominant right elbow. This (to our knowledge) first case of unilateral pseudarthrosis of the olecranon had an excellent clinical result with stable fixation and support of bone healing by a bone graft interposition.

Osteofibrous Dysplasia with Rhabdoid Elements in a 38-Year-Old Man with Spontaneous Regression Over Five Years: A Case Report.

CASE: A 38-year-old man presented with multifocal, partially confluent osteolytic lesions in the proximal dia-meta-epiphyseal region of the proximal aspect of the left tibia, which had been found incidentally when a radiograph was made after a rotational knee injury. When the results of a percutaneous core needle biopsy proved inconclusive, an open biopsy was performed. Osteofibrous dysplasia (OFD) with scattered groups of plump cells with a rhabdoid phenotype, shown to express both vimentin and pan-cytokeratin, was found. Because the lesion was an incidental finding, we decided to proceed with observation. Three months after the open biopsy, imaging showed marked regression of the lesion; there was nearly complete normalization 5 years later. CONCLUSION: To our knowledge, there has been only 1 prior reported case with these pathologic features, and there have been no reports of complete spontaneous regression in an adult patient with OFD. Treatment recommendations for OFD and for OFD-like adamantinoma range from observation to aggressive resection.

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 (RO).

Corrective Imhäuser intertrochanteric osteotomy.

OBJECTIVE: Surgical 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. INDICATIONS: Chronic and subacute manifestations of slipped capital femoral epiphysis with an epiphyseal dislocation in the radiographic axial view of 30-60 degrees (ET angle) and DeltaED > 20 degrees (CCD angle minus ED angle) in the anteroposterior view. CONTRAINDICATIONS: Acute phase or course of slipped capital femoral epiphysis. Secondary coxarthrosis (after slipped capital femoral epiphysis). Traumatic epiphyseolysis. SURGICAL TECHNIQUE: Exposure 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 degrees AO adolescent plate under interfragmentary compression. POSTOPERATIVE MANAGEMENT: Mobilization on underarm crutches with unloading of the extremity for 2 months postoperatively; gradual increase in 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. RESULTS: Retrospective follow-up of 51 patients with unilateral, moderate to severe slipped capital femoral epiphysis (indication: epiphyseal displacement angle of 30-60 degrees, average preoperative displacement angle 45 degrees) 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.

The tactics and technique of musculoskeletal biopsy.

The treatment of musculoskeletal neoplasms and infection is usually based on an initial diagnostic biopsy.Prior to biopsy, a hypothesis should be formed about the most likely diagnosis and a differential diagnosis. These deliberations should consider whether the lesion is a primary benign or malignant tumour, a metastasis, a haematological problem or an infection.A tactical plan should be developed which evaluates the necessity, the risk, the approach and finally defines the technique of biopsy most likely to achieve a representative result in the clinical case.In developing this technical approach, the pitfalls should be anticipated, i.e. inadequate sampling, difficulty of pathological interpretation and contamination.The tactical approach should be developed in conjunction with a multi-disciplinary team together with appropriate pre-biopsy imaging. Cite this article: EFORT Open Rev 2017;2:51-57. DOI: 10.1302/2058-5241.2.160065.

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.