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Ewing sarcoma (ES) of the jaw bones comprises a small fraction of ES at all sites. Due to their rarity, a specific policy for local treatment is lacking. The aim of this study was to evaluate the local therapy for ES and recommend measures to individualize treatment options. Patients with primary non-metastatic ES of the jaw bones treated between August 2005 and February 2015 were analyzed. All patients received primary induction chemotherapy, following which lesions amenable to resection based on specific radiological criteria were resected; those with unresectable lesions were offered definitive radiotherapy. The maxilla was the primary site in 13 patients and the mandible in eight. The median age of patients was 11.6 years (range 5–17 years). Overall, surgery was performed in 17 patients and definitive radiotherapy was used in four patients. Postoperative radiotherapy was administered to 12 patients and was avoided in five patients with 100% tumour necrosis. The 3-year overall survival, event-free survival, and local control were 68.1%, 63.6%, and 80.2%, respectively. Mandible primary and a histological response to chemotherapy were significant prognostic factors. The stratification of patients based on radiological criteria aids in selecting local therapy. In eligible patients, surgery with contemporary reconstruction results in optimal oncological and functional outcomes. Surgery also has the added advantage of identifying patients who may not need radiotherapy. [ABSTRACT FROM AUTHOR]

To evaluate treatment outcomes of children diagnosed with unilateral retinoblastoma. Retrospective study of children diagnosed with unilateral retinoblastoma registered at the Tata Memorial Hospital (TMH), Mumbai from January 2013 to December 2018 and completed the planned curative treatment protocol. For the 98 cases that were analyzed, the median age of presentation was 24 months. The majority of patients had the intraocular disease (n = 72), whereas orbital retinoblastoma was in 26 patients. At the time of presentation, on imaging extra scleral spread was observed in 16 patients whereas 18 patients had optic nerve involvement, 11 patients had both extra scleral invasion and optic nerve involvement. We used the International Classification of Retinoblastoma for grouping. Out of 98 patients, 71 patients were in Group E, 21 were in Group D and 4 were in Group B and 2 were in Group C. For Staging of Retinoblastoma, we used International Retinoblastoma Staging System (IRSS) in our study and 14 patients had Stage 0 disease, 52 patients had Stage I disease, 10 patients had Stage II, 21 patients had Stage III A and 1 patient had Stage III B disease. High-risk features on surgical specimen histopathology were optic nerve cut margin positive in 6 patients, optic nerve involvement in 21 patients, extra scleral spread in 3 patients, Choroidal invasion in 38 and Iris involvement in 10 patients. Primary enucleation was offered in 52 patients whereas 26 patients underwent secondary enucleation. Systemic chemotherapy was received by the patient in the neoadjuvant setting in 17 patients as a form of chemo-reduction and 51 patients received systemic chemotherapy in the adjuvant setting. Intra-arterial chemotherapy was offered to 27 patients as a primary treatment or in conjugation with focal therapy (n = 11). Definitive radiotherapy was offered to only 2 patients and 22 patients received adjuvant radiotherapy. At a median follow-up of 62 months, 2patients had local relapse, which was salvaged by focal therapy in one and enucleation in the other. Ten patients who had leptomeningeal relapse had died. The 5-year local control (LC) is 97.6%, event-free survival (EFS) is 88% and overall survival (OS) is 89.5%. Globe was salvaged in only 16 cases. On univariate analysis, we observed a significant association between overall survival and extraocular and intraocular disease (p-value 0.0), Extra scleral spread (p-value 0.0), optic Nerve involvement (imaging), and an optic nerve cut margin positive (p-value 0.045), ICRB Groups (p-value 0.0) and IRSS stage (p- value 0.024). Retinoblastoma is curable if detected early. Extra ocular disease and high-risk features are associated with inferior outcomes and poor globe salvage rates. [ABSTRACT FROM AUTHOR]

Aim To assess the diagnostic accuracy of whole-body magnetic resonance imaging (WB-MRI) for metastatic disease in patients with solid small round cell tumours (SRCT) by comparing it with routine staging procedures (standard of care). Materials and methods Eligible cases of neuroblastoma, primitive neuroectodermal tumour, and rhabdomyosarcoma were enrolled in the study after obtaining informed consent. WB-MRI was undertaken using overlapping coronal T1 and short-tau inversion recovery (STIR) sequences. Lesions were classified into skeletal, pulmonary, and soft-tissue types. Conventional staging, which consisted of combined positron-emission tomography & computed tomography (PET-CT), bone scintigraphy & bone marrow biopsy for bone metastases, CT thorax for lung metastases, combined PET-CT, metaiodobenzylguanidine (MIBG) scintigraphy (in neuroblastoma) for soft tissue metastases and clinical evaluation was used as the reference standard. Parameters for diagnostic accuracy were calculated. Results Thirty-four out of forty patients enrolled were included in final analysis, half of them having metastatic disease. The sensitivity, specificity, positive and negative predictive value, and the diagnostic accuracy of WB-MRI and PET-CT for skeletal metastases as compared to reference standard were 91.9%, 99.8%, 97.4%, 99.6%, and 95.5% and 99.1%, 99.9%, 99.1%, 99.9%, and 99.9%, respectively. The sensitivity of MRI, only PET and PET-CT with plain CT thorax was 30%, 40%, and 100%, respectively, for lung metastases. The sensitivity of MRI for soft-tissue lesions was 76.9%. Conclusion WB-MRI is a radiation-free tool with high diagnostic accuracy for the evaluation of metastatic disease to the marrow. The rate of detection of soft-tissue metastases, such as nodal metastases, is less when WB-MRI is compared with conventional staging using coronal STIR images. CT thorax is essential for accurate evaluation of lung metastases. [ABSTRACT FROM AUTHOR]

The management of Wilms' tumour has evolved through thorough systematic research, predominantly lead by two groups: the Wilms' Tumour Study Committee of the International Society of Paediatric Oncology (SIOP) and National Wilms' Tumor Study Group (NWTSG) of North America. These two groups differ in their approach: SIOP advocates initial chemotherapy of 4–6 weeks followed by surgery, whereas the NWTSG advocates upfront surgery, with certain exceptions. This review briefly discusses the principles, and pros and cons of each approach. Both the treatment approaches have equivalent outcomes (in the form of event-free survival and overall survival), when compared stage-wise. With this knowledge, modern imaging can be used for individualizing treatment in anticipation of minimizing complications. The review identifies critical imaging features and discusses the reliability of imaging based on current reports in the literature. [Copyright &y& Elsevier]