Background:Osteosarcoma (OS) is the most common primary malignant tumor of bone. The survival of OS patients has steadily improved from <20% in the early 20th century to around 70% with current treatment. There are very few studies in pediatric OS from India analyzing various aspects of the disease. This study focuses on the clinical profile, treatment options, and their complications and survival outcomes in pediatric osteosarcoma (OS) patients.Materials and Methods:This was a retrospective observational study which included pediatric patients <14 years of age, with newly diagnosed OS confirmed by histological diagnosis. Medical records of all patients were reviewed for clinical profile, treatment data, surgical management, and treatment complications. Patients alive at the end of treatment were followed up and overall (OAS) and disease-free survival (DFS) were analyzed.Results:Sixty-two patients were diagnosed with OS during the study of whom 55 opted for treatment. Cisplatin, adriamycin, and ifosfamide (PAI) was offered as chemotherapy and was completed as planned in the majority of patients. Limb salvage surgery was performed in most patients (87%, n = 40). The local recurrence occurred in 7 patients. The 3 years overall survival for the cohort was 54.6% ± 7.8% and DFS was 43.4% ± 7.9%, with females and those with the localized disease having a significantly better DFS.Conclusions:High dose methotrexate free chemotherapy can give good OAS in localized disease and LSS is feasible in most of the pediatric OS patients. However the modest DFS even for localized disease with PAI chemotherapy and extremely poor outcomes in the metastatic OS, demand further research and innovations in systemic therapy to improve outcomes.

Fungation is associated with a poor prognosis with a high incidence of metastases at presentation and a high rate of local recurrence. As an independent variable, fungation confers a poor prognosis when compared to non-fungation in soft tissue sarcomas.

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With the use of potent chemotherapeutic drugs, newer imaging modalities and improved surgical techniques limb preserving surgeries for malignant bone tumours have become the norm. Endoprosthesis still remains the commonest method of reconstruction after tumour resection. But when one is able to an oncologically safe intercalary resection for malignant bone tumours one method of reconstruction is reimplanting the resected tumour bone after sterilisation. Radiation given outside the body to sterilise the tumour bone is called Extra Corporeal Radiotherapy (ECRT). After resection the bone is cleaned of all its soft tissue and marrow contents and sent in a plastic container to the Radiotherapy department where it is subjected to 50 Gy of radiation which kills all cells including the tumour cells. The bone is brought back to the theatre and reimplanted after augmenting it with either bone cement or fibular grafts and stabilised by appropriate fixation devices. The advantage of reimplanting the same bone is that you get an exact match to the resected bone which is tumour free. Post operatively the joint is mobilised immediately and weight bearing started as appropriate to the fixation used. The diaphyseal end takes more time to unite than the metaphyseal end. In our series of 16 patients who had undergone ECRT and reimplantation for malignant bone tumours of the extremity and had completed two year follow up, the metaphyseal end took an average of 6.2 months (4 -12 months) for union while the diaphyseal end united in 10.6 months (5 -15 months). If the tumour has caused extensive destruction of the bone or has a pathological fracture, it may be mechanically not sound to reimplant it after ECRT. ECRT is an oncologically safe and mechanically stable procedure in biologically reconstructing bony defects after tumour resection.

Background In the surgical removal of primary malignant tumours involving long bones, intraoperative frozen sections are used to ascertain the adequacy of tumour clearance. However, with the improved imaging modalities that provide better foreknowledge of the tumour extent, it is possible that the arduous task of performing frozen sections can be safely avoided. This would not only save procedural time but also reduce hospital costs. Presently, there are no clear guidelines regarding the modality required intraoperatively to assess tumour margins in these cases. Hence, in our retrospective multicentre analysis, we aimed at determining the usefulness of frozen sections in these cases. Materials and methods Our study is a 3-centre retrospective analysis of 475 cases (513 tumour margins) involving the surgical removal of primary malignancies of long bones. The preoperative Magnetic Resonance Imaging (MRI) and intraoperative assessment of the split specimen of the tumours were used to determine marginal clearance in all the cases in addition to frozen sections in 410 of the margins. Results Of the 410 frozen sections (centres 1 and 2), only one margin was reported positive and another reported indeterminate. All other margins were reported negative. In the first case, a 2 cm additional bone-cut was done whereas in the second, the procedure was proceeded based on the intraoperative agreement without re-cutting the margin. All these margins were negative in the final histopathology. In addition, in Centre 3, where frozen sections were not available, all the 103 cases had negative margins in the final histopathology. Conclusion In primary malignancies involving long bones, intraoperative decision making with the aid of MRI has been sufficiently accurate in identifying the required tumour margin without frozen sections. Hence, the added time and cost incurred by doing an additional procedure can be avoided in these cases.