Early diagnosis is assumed to improve patient survival, but whether symptom interval (SI i.e. the period between the onset of the first symptoms signs of the disease and its definitive diagnosis) has significant impact on outcome or not remain unclear.

Congenital infantile fibrosarcomas (CIFS) is a rare tumor of childhood that can be diagnosed from birth to 15 years. It has a ratio of 3.74/100 000 children and is well defined nosological entity with a well-defined pathogenetic patterns: translocation (12, 15) (p13, q25) with fusion of the gene ETV6-NTRK3.

The differential diagnosis of upper CIFS in infants must be made with lymphatic malformations, and when associated with the Kasabach-Merritt phenomenon’s (disseminated intravascular coagulopathy), haemangiomas, emangioendotelioma kaposiforme. In 26% of cases is congenital, while in 63% is diagnosed in the first 5 years. Unlike fibrosarcomas of the adult is characterized by a low rate of metastasis and a high survival rate (90% at 5 years). 74% of cases is observed in the limbs (upper> lower, distal> proximal).

The treatment of choice should to be, where possible, limb salvage and the recurrences are variable between 17% and 43%. The purpose of this paper is to present a case of CIFS, the clinical features, the oncological treatment, the reconstructive solutions and functional results obtained after reconstruction. Case report.

The child (Z.A. female), was diagnosed with a neoplasia of soft tissues of the right forearm before birth.

At birth the child underwent a needle biopsy with a diagnosis of CIFS.

The patient received four cycles of chemotherapy with reduction tumor mass of more than 50% of volume.

At month four she underwent an exeresi with wide margings and sacrifice of the radial nerve.

The reconstruction required a free flap of re-innervated latissimus dorsi muscle. After 30 months from the surgical procedure the child is disease free and has recovered extesion of the muscles of the hand and fingers.

Limb salvage has become the most important treatment for patients with malignant bone tumors of the lower limb. Reconstruction with endoprosthesis of the proximal femur and distal femur and proximal tibia is now the most common solution. The data of 180 consecutive patients with malignant bone tumors of the lower limbtreated between 4/1987 and 11/1998 were reviewed. The average follow up is of four years.

129 patients had surgery for primary bone sarcoma, six for aggressive GCT and 45 for metastatic carcinoma. 63 patients were reoperated for different complications. The main complications were: local recurrences in 10 patients, infection in 12 patients and mechanical complications in 35 patients. 28 patients were operated two times and 24 patients more than two times.

14 patients have undergone amputation: six because of local recurrences, four because of infection, and two for post-surgical ischemia.

Eight of the 12 infections occurred after a re-operation.

35 patients had mechanical complications: 14 patients were reoperated to replace the polyethylene bushings in of the first model of HMRTS prosthesis (Howmedica), five patients had ruptures of the femoral stem, three patients suffered mobilization of the tibial stem and two of the femoral stem, six patients required a patella prostheses for local pain.

Two patients had acetabulum wear and three had hip dislocation.

In our experience endoprosthesis reconstruction after resection of bone tumors of the lower limb is a feasible procedure for limb salvage. We must consider that more than 30% of these patients will be re-operated for different complications and that 50% of infections occours after a new surgical procedure.

Conservative treatment of neoplastic bone lesions in paediatric patients may require the sacrifice of growth cartilage with subsequent hypometria or axial deviation of the lower limb.

Segmental reconstructions can be made using acrylic cement and intramedullary nailing or allograft. In case of involvement of the joint, reconstruction can be performed with prosthesis or arthrodesis.

These reconstruction techniques can lead to a progressive deformity associated with shortening of the limb.

The resolution of legs discrepancy and axial defects in survived patients often requires more than one surgical procedure.

In our Institute, the patients affected by aforementioned defects, are treated with axial or circular external fixator at completed skeletal growth.

This paper refers complications and outcomes in five patients treated:

1st case. Male, 10 years: osteosarcoma of the distal femur healed with residual shortening of 8 centimeters.

– We proceeded with a double level lengthening (proximal femur and proximal tibia) using Ilizarov technique.

The results obtained in our patients show that the use of the external fixator increases the quality of life in long-term survivors.

EH of bone is a rare vascular neoplasm, subtype of hemangioendothelioma, characterized by mesenchimal cells that have an epithelioid endothelial appearance. There are different kinds of EH: the benign epithelioid hemangioma, and the malignant epithelioid angiosarcoma.

This tumors can occurs in soft tissue, lung, liver and bones and often are multicentric.

EH generally involve the bone of the spine and lower limb and is very rare in the upper limb and the hands. The main symptom is pain; pathological fracture may occur in aggressive lesions.

Radiographically the EH is a ostelytic lesion with variable peripheral sclerosis, cortical destruction and periosteal new bone.

Treatment of EH is curettage and local adjuvants in benign lesion, en bloc resection in the low-grade forms and wide or radical surgery in the high-grade forms. Radiation therapy is suggest in inoperable situations.

In the present report we describe the clinical features, the oncological treatment and the reconstructive solutions of two cases of EH of the hand treated in the Orthopedic Oncological Center of Gaetano Pini Institute of Milan. Both cases had multiple locations in the carpus, metacarpus and phalanges. The involvement of more joints caused a delayed diagnosis (> 1 year). Exer-esi and reconstruction of several segments of the wrist and hand has led to considerable technical difficulties resolved with the collaboration of the microsurgeon and plastic surgeon.

Introduction: Infection following major orthopaedic oncological surgery is a serious complication and every precaution should be taken to avoid it.

One potential source of infection is the biopsy procedure, particularly when is carried out of a referring centre. In fact up to 30 per cent of patients with soft tissues problems following a biopsy is reported. As an infected biopsy may make subsequent limb preservation surgery impractical, the greatest care should be taken in carrying out the biopsy.

The implantation of foreign materials (prostheses, grafts, acrilic cement, metallic devices, etc) as the duration of the surgical procedure, intraoperative bleeding, possible deep haematomas, presence of drains, increase the risk of infection. Also the importance of haematogenous spread from other sites of infection to joint pros-thesis is well estabilished.

Materials and methods: In this paper the Authors present their experience regarding septic complications after orthopaedic oncologie surgery. From 1988 to 2002, 143 patients underwent a major surgery for the treatment of skeletal neoplasms (wide resection and reconstruction employing modular or composite prostheses, osteoarticular or intercalary allografts, acrylic cement and osteosynthesis devices, major spinal surgery, internal hemipelvectomy, etc).

Results: The patients were followed-up to detect the presence of a septic complication. Also patient’s files were revised to evaluate paramethers related to infection as bleeding, duration of surgery, postoperative fever, neutropenia, ESR and CRP, antibiotic prophylaxis, etc. The overall infection rate was approximately to 15 per cent.

Conclusions: Immunosuppression from previous chemotherapy may predispose the patient to infection which may be occult, but which must be diligently sought.

It has to be also emphasized that if at any stage the patient has had local radiotherapy, the tissues may be fibrosed and avascular and unable to combat local infection effectively.

The Authors retain that the infection after major orthopaedic oncologie surgery could represent a serious threat to the implant and to the limb. The importance of meticolous asepsis practised at every stage has to be emphasized, together with prolonged use of prophylactic antibiotic, specially in immunosuppressed patients or chemotherapy.