RESUMEN
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.
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Though survival in bilateral retinoblastoma (RB) has improved due to advancement in diagnostics and treatment modalities, children require long-term follow-ups for recurrence and second malignancies. We report a case of bilateral RB in a 7-month-old baby who was treated with chemotherapy, transpupillary thermotherapy, and periocular carboplatin for both eyes following which there was complete regression of tumour. Six and a half years after treatment, the child presented with metastatic recurrence of tumour in the left ulna. He was treated successfully with chemotherapy, extracorporeal radiation and reimplantation therapy. A less aggressive treatment approach for isolated bone relapse may be considered in selected cases.
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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.
RESUMEN
PURPOSE: Whilst much is known on the multiple variables associated with a poor prognosis in soft tissue sarcomas, little evidence exists on the impact of fungation at the time of presentation. The aim of this study was to assess the impact of fungation on overall and disease free survival in soft tissue sarcomas. METHODS: The study comprised a retrospective review of all patients presenting with a soft tissue sarcoma between 1996 and 2014 managed at a single institution. Over the 18-year study period, 2661 patients were diagnosed with a STS. Eighty-six patients (3.2%) presented with a fungating tumour. RESULTS: The five year survival for all patients with a fungating sarcoma was 15.5% (95% confidence interval: 4.6-26.4), which compared to 65.6% (95% confidence interval 63.2-67.8%) (p = 0.0001) for non-fungating tumours. The incidence of metastasis at presentation in the non-fungating tumour group was 11.3% of patients, compared to 20.0% in those with a fungating tumour (p = 0.0113). Factors associated with a poor prognosis in the fungating group included size, grade and tumour-type, patient age, tumour depth, and metastases at presentation. As an independent risk factor, tumour fungation was associated with a poor prognosis when compared to non-fungating tumours. The rate of limb sacrifice as primary treatment was higher in the fungating group (23% versus 73%). In spite of this, the incidence of local recurrence was higher in the fungating group (20% versus 16.5%). CONCLUSIONS: 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.