The nonrhabdomyosarcoma soft tissue sarcomas constitute a heterogenous group of rare mesenchymal tumors that account for less than %5 of pediatric cancers. Their biology and optimal treatment is not well understood. This study retrospectively analyses a small subset of surgically treated patients.

Fifteen patients with a mean age of 11.4 years (4 months – 16 years) were followed-up for a mean of 48 (2–124) months. The histologic diagnosis was synovial sarcoma in 5 patients; soft tissue Ewing Sarcoma in 3; fusiform cell sarcoma in 3; malignant peripheral nerve sheath tumor in 2; fibromyxoid sarcoma in 1 and myxoid liposarcoma in 1. The tumor was located in the upper extremity in 6 patients; thigh in 4; inguinal region in 2, foot in 2 and gluteal region in 1. Eight patients received preoperative chemotherapy and 5 received preoperative radiotherapy. Two patients had pulmonary metastasis at the time of admission. Thirteen patients were operated by limb salvage procedures and 2 underwent amputation.

Tumor resection was radical in 2 patients, wide in 12 and marginal in 1. Five patients received adjuvant chemotherapy and 5 received adjuvant radiotherapy. There were 5 local recurrences after 23.8 (14–40) months; three patients underwent wide resection for recurrence and two received chemotherapy and radiotherapy. Three patients had systemic metastases after a mean of 32.3 (27–40) months. There were no major complications but local wound problems were encountered. Three patients died of disease after a mean of 65.3 months (32–124 months). Two patients had metastatic disease and 10 had no evidence of disease in the last follow-up visit.

Pediatric soft tissue sarcomas can be treated following the principles of adult soft tissue sarcomas, except for wide utilization of radiotherapy in neoadjuvant and adjuvant setting. Treatment results are similar to adult patients.

Aneurysmal bone cysts are benign lesions of bone that tend to recur if they display an aggressive behaviour. In this study, an aggressive lesion is described as one with almost completely lost cortices on standard x-rays, mimicking ‘vanishing bone disease’. Purpose of this study is to retrospectively analyse the treatment results in this special patient subgroup.

Sixteen patients with a mean age of 23.1 (6–44) were included in this study. Femur (%25) was the most commonly affected bone. Spinal lesions were excluded. All lesions were diagnosed by preoperative tru-cut biopsy, however open biopsy was also done if requested by the pathologist. All lesions were preoperatively examined by contrast enhanced MRI. They were evaluated as having almost no cortical bone rim but a periosteum like soft tissue envelope. Intraoperatively, following an extended curettage, this tissue was observed as an alive periosteal layer. Phenolisation was added and cavities were filled with allograft bone chips. The periosteum was sutured around the grafts. Additional stabilisation was performed by external fixator in 3 patients, dynamic compression hip screw in 1.

Patients were followed up for a mean period of 47.8 months (9–84). Lesions healed after a mean period of 28.2 weeks (6–126). There was recurrence in 5 patients. The number of additional procedures necessary for relapsed lesions was 3 for one patient, 2 for another patient and 1 for the remaining 2 patients. Time to healing was not included for 1 patient who refused surgery for relapse and another patient who recently underwent surgery for relapse. One patient healed with a deformity.

Aneurysmal bone cysts may present as highly aggressive local bone lesions. Even in such a subgroup, resection to prevent relapses seems an exaggerated procedure. Extended curretage and packing may yield satisfactory results, with acceptable recurrence rates.

In an era where the survival rates of oncologic patients are improving, biologic reconstruction is the treatment of choice, however, it has its complications and fortunately we have the solutions.

Biological reconstruction was performed on 52 patients with a mean age of 11.3 (1.5–16) after malignant bone tumor resection in our institution between 1991 and 2008. Patients were followed up for a mean period of 49 months (3–216). Twenty-nine patients were diagnosed with osteosarcoma, 22 with Ewing sarcoma and 1 with adamantinoma. A wide range of vascular and nonvascular autografts, allografts, fibular transposition, bone regeneration and bone recycling techniques were utilised alone or in combination for reconstruction. Crucial anatomical parts (epiphyses, apophyses, triradiate cartilage, glenoid) were preserved in 41 patients while maintaining safe surgical margins.

Wound problem was the most common early complication. The most common late complications were nonunion, limb length discrepancy, limitation of range of motion (ROM), deformity, implant or external fixator failure and fibular graft fracture. Local recurrence was seen in only 2 patients. Patients underwent a mean of 0.8 (1–10) additional surgical interventions for treatment of complications. Thirty-one out of 43 lower extremity patients became ambulatory with full weight bearing and near full ROM while 4 died of disease and 2 were disarticulated prior to healing or treating of complications. Six patients with reconstructions around the glenohumeral joint had functional outcomes varying from excellent to poor with defect pseudoarthrosis. Two patients with successful pelvic reconstructions suffered from co-morbidities and disease itself. Implants and fixators, orthoses, physiotherapy and antibiotherapy were used as necessary for treatment of complications.

Biologic reconstruction yields good functional outcomes and allows more flexibility while treating complications since it preserves bone stock and epiphyses.

Enchondromatosis is a non-hereditary disease, characterised by the presence of multiple enchondromas. While Ollier Disease is typified by multiple enchondromas, in Maffucci Syndrome they are combined with haemangioma.

Due to the rarity of these diseases, systematic studies on clinical behaviour providing information how to treat patients are lacking.

This study intends to answer the following questions: What are predictive factors for developing chondrosarcoma? When is extensive surgery necessary? How often patients die due to dedifferentiation or metastasis?

Twelve institutes in eight countries participated in this descriptive retrospective EMSOS-study. 118 Patients with Ollier Disease and 15 patients with Maffucci Syndrome were included. Unilateral localization of disease was found in 60% of Ollier patients and 40% of patients with Maffucci Syndrome.

One of the predictive factors for developing chondrosarcoma is the location of the enchondromas; the risk increases especially when enchondromas are located in the scapula (33%), humerus (18%), pelvis (26%) or femur (15%). For the phalanges, this risk is 14% in the hand and 16% in the feet. The decision whether or not to perform extensive surgery is difficult, especially in patients who suffer multiple chondrosarcomas.

Malignant transformation was found in fourty-four patients with Ollier Disease (37%) and eight patients with Maffucci Syndrome (53%). Multiple synchronous or metachronous chondrosarcomas were found in 15 patients.

Nine patients died (range 21–54 yrs). Seven of them died disease related due to pulmonary metastasis (2 humerus, 2 pelvis, 3 femur). Two patients died from glioma of the brain.

In conclusion, one important predictive factor for developing chondrosarcoma is the location of the enchondromas; interestingly, only patients with chondrosarcoma outside the small bones died of their disease. In this series, no dedifferentiation of chondrosarcoma was seen. A first design flow-chart how to approach chondrosarcoma in patients with Ollier Disease and Maffucci Syndrome is in preparation.

Autografts produced by recycling of tumor-bearing bone have been used for bridging intercalary bone defects but they are known to act as massive allografts after recycling procedures due to devitalisation. Recycled bone is superior to massive allografts since it allows anatomical reconstruction. Vascularised fibular grafts are inserted into recycled bone segments to provide biological support and to promote healing.

Twelve patients with a mean age of 13.3 years (6–31), who had undergone curative resection of malignant bone tumor followed by biological reconstruction comprised of recycled bone combined with vascularised fibula, were followed up for a mean period of 16.8 (6–46). The tumor was located in distal femur in 7 patients, proximal femur in 2, proximal tibia in 2 and mid-diaphyseal tibia in 1. Cryopreservation with liquid nitrogen was employed for all patients. Contralateral single strut vascularised fibular grafts were used in all except one patient for whom bilateral fibula grafts were harvested to span a longer defect. Plates were used for fixation in 11 patients, and intramedullary nailing in one case. Mean length of bony defect was 16.1 cm (9.0–25.0). Mean fibular graft length was 17.5 cm (10.0–23.0 cm).

Complete union and full weight bearing was achieved in 6 patients, and mean time to detect the commencement of union was 6 months (4–8). Incomplete union was detected in 4 patients and no union in 2. Five patients were complicated by implant failure, 1 with deep infection and 1 with drop foot.

In order to fill large defects after resection of bone tumors, recyled bone combined with vascularised fibular graft is an effective reconstructive tool. Union rate of this technique is quite satisfactory with good functional results.