In pediatric population, the most common solid malignant tumor of the kidney is Wilms' tumor (WT). Incidence of Extra-Renal Wilms' Tumor (ERWT) is immensely unusual. Prognosis of ERWT is comparable to renal WT. Hence, diagnosis, staging workup and start of treatment in a timely manner is very essential. The most common location of ERWT is the retroperitoneum. The staging of ERWT is done according to National Wilms' Tumor Study group (NWTS). ERWT is considered as stage II or higher; because it is outside renal capsule. A 6-year boy presented with complaints of increased frequency of micturition and difficulty in urination for 15 months. Biopsy of bladder mass showed WT by immunohistochemistry stains such as WTI and Desmin. Cross-sectional imaging showed both kidneys clear of any tumor. Hence, the final diagnosis of ERWT was made.
Purpose: Tumor necrosis and histopathological changes in Ewing sarcoma following neoadjuvant chemotherapy are important predictors of disease outcome. The aim of our study is to determine the clinical outcome and significance of pathological necrosis after neoadjuvant chemotherapy as it has not been reported in our country so far.
Methods: Data was reviewed after IRB approval from January 2010 to December 2015 were retrospectively reviewed for patients with newly diagnosed Ewing sarcoma on histopathology and less than 20 years of age at the time of diagnosis.
Results: A Total of 124 patients were included, in which 89 patients (72%) were non metastatic and 35 patients were metastatic (28%). Histopathology report after doing surgery showed Little or no (Grade 1 ) necrosis seen in 14 patients (11%) and 50-90% (Grade II) necrosis seen in 9 patients (7%), 90-99% (Grade III) necrosis seen in 8 patients (5.5%), and 100% (Grade IV) necrosis in 14 patients (11%). EFS of grade 4 necrosis was 93%, grade-3 71%, grade- 2 22% and grade-1 35%. OS of grade 4 necrosis was 93%, grade -3 75%, grade-2 25 % and grade-1 50%. EFS of Ewing sarcoma patients were 38% and OS was 38%.
Conclusion: Tumor necrosis and histopathological changes after surgery has great impact on survival outcome in Ewing Sarcoma.
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Introduction: MRI plays an important role in characterising spinal dysraphisms.The reason behind this is quality of MRI showing very high contrast resolution of soft tissuesand the ability to detect fat in the lesion.T2 weighted images provide excellent contrastbetween the sub-arachnoid spaces and neural tissue while evaluating spinal dysraphisms.Objectives: To determine the frequency of rare disorders of spinal dysraphism in view of MRIfindings of spinal dyspharism patients. Study Design: retrospective. Duration: the study wasconducted from 1st September 2015 to 30th November 2015. Settings: Radiology department,Allied hospital Faisalabad. Methods: A total of 20 consecutive pediatric age group patientspresented with clinical suspicion of spinal dysraphism were included. MRI L/S spine was carriedout on 1.5Tesla Philips and diagnosis of various disorders of spinal dysraphism was made.Conclusion: MRI is the first choice examination for the detection of occult spinal dysraphism.Its timely diagnosis improving surgical & medical outcome as well as better & long term careof patients with spinal dysraphism. Majority of patients in the current study had tethered cord(75%), myelomeningocele (20%), myelocele (10%), lipomyelocele (10%) Dermal sinus tract(10%) & diastematomyelia (10%) followed by associated spinal lipomas (5%), caudal agenesis(5%) & anterior meningocele (5%).
Objective: To describe clinical features and treatment options in pediatric patients with ALCL (Anaplastic large cell lymphoma) and their outcome over a span of 10 years.
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