Clinical Outcome of paediatric ewing sarcoma and significance of pathological necrosis for mortality after neoadjuvant chemotherapy: Single institutional study.

OBJECTIVE: To determine the clinical outcome and significance of pathological necrosis after neoadjuvant chemotherapy. METHODS: The retrospective study was conducted in Shaukat Khanum Memorial Cancer Hospital and Research Center and comprised data from January 2010 to December 2015 related to young with newly diagnosed Ewing sarcoma on histopathology. Data was collected on patients aged <20 years of either gender along with primary tumour site, metastatic status, tumour volume, mode of local therapy, degree of necrosis post-surgery, tumour margins after resection, outcome at the end of treatment and at last follow-up visit. Tumours were categorised as grade I = little or no necrosis, grade II = 50-90% necrosis, grade III = 90-99% necrosis, and grade IV = 100% necrosis. Data was analysed using SPSS 20. RESULTS: Of the 124 patients, 89(72%) were non-metastatic; 35(28%) were metastatic; 37(29.8%) underwent surgery; 58(46%) received radiotherapy; 7(5.6%) received both surgery and radiotherapy; 22(17.7%) received no treatment. Histopathology report post-surgery showed little grade 1 necrosis in 10(8%) patients, grade II in 8(6.5%), grade III in 8(6.5%) and grade IV in 14(11%). Event-free survival in grade IV necrosis was 93% in 14 (11.3%) patients, EFS in grade III necrosis was 71% in 8(6.5%) patients , EFS in grade II necrosis was 22% seen in 9(7.3%) patients and EFS in grade- I necrosis was 35% seen in 14 (11.3%) patients. Overall survival in grade IV necrosis was 93% in 14 (11.3%) patients, OS in grade III 75% seen in 8(6.5%), OS in grade II 25% seen in 9(7.3%) and OS in grade I was 50% in 14(11.3%) patients. Event-free survival was 48 (38%) patients and overall survival of Ewing sarcoma patients was 52 (46.6%) patients respectively. CONCLUSION: Tumour necrosis and histopathological changes post-surgery were found to have great impact on survival outcome in Ewing Sarcoma.

Clinical Features and Treatment Outcomes of Children with Anaplastic Large Cell Lymphoma.

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. STUDY DESIGN: A retrospective-observational study. PLACE AND DURATION OF STUDY: Shaukat Khanum Memorial Cancer Hospital and Research Centre, Lahore, from January 2005 to December 2015. METHODOLOGY: Medical records of pediatric patients with anaplastic large cell lymphoma was retrospectively collected after IRB approval. Data was reviewed for patients confirmed on histopathology and age less than 20 years at the time of diagnosis to see clinical features and treatment outcomes. Descriptive statistics were applied. RESULTS: A total of 40 children, 27 males (67.5%) and 13 females (32.5%) with ALCL (CD30 +), were reviewed. B symptoms were present in 32 (80%) patients, nodal involvement in 39 (97.5%), and mediastinum involvement was present in 8 (20%) patients. Visceral (lung, liver, spleen) and cutaneous involvement was seen in 16 (40%) and 6 (15%) patients, respectively. ALK was positive in 19 patients (48%) and Bone marrow was involved in 3 patients (7.5%). Stage III was seen in 29 (72.5%). All patients were treated on ALCL 99 protocol. Five-year EFS (event-free survival) and OS (overall survival) was 30 and 60%, respectively. There were 7 relapses, 2 progressive disease, 16 death and 3 refusal for treatment. CONCLUSION: This analysis shows poor outcomes in pediatric ALCL. The most common cause of mortality was hematological toxicity and febrile neutropenia associated with it. Supportive care needs to be improved. Key Words: Clinical features, Outcomes, Anaplastic large cell lymphoma (ALCL), Children.

Retrospective analysis of clinical features and treatment outcomes of children with Hodgkin's Lymphoma treated with different chemotherapy protocols at a tertiary care center in Pakistan.

PURPOSE: Hodgkin lymphoma (HL) is one of the most curable paediatric cancers, with long-term survival rates now exceeding 90% after treatment with chemotherapy alone or combined with radiotherapy (RT). Treatment options for Hodgkin's Lymphoma differ among various study groups and there is still no consensus regarding the standard treatment for Hodgkin's lymphoma. Taking into account the impact of treatment-related mortality in low- and middle-income countries we propose to study the the clinical features and treatment outcomes by using different chemotherapy protocols in Hodgk in s' s Lymphoma children's at Shaukat khanam hospital Lahore.. METHODS: Clinical data from a large regional cancer center Pediatrics patients with Hodgkin's Lymphoma from January 2009 till December 2015 was retrospectively collected after Institutional Review Board (IRB) approval. RESULTS: A total of 748 patients were reviewed retrospectively. Mostly (45%) were in 6-10 years age group. Male showed predominance ,male to female ratio was 4:1. B symptoms were present in 51%, bulky disease in 44% and ESR was more than 30mm in 26% of patients. CD 30 was positive in 95%, Bone marrow involved in 13% of patients. Stage I in 8%, stage- II in 27%, stage -III in 39% and stage IV in 26% was seen. COPDAc/ABVD was given in 412 patients, CHLVPP/ABVD in 176 patients, OEPA/COPP in 57 patients, OEPA in 35 patients, OEPA/COPDAC in 33 Patients and remaining 33 received various chemotherapy protocol combination. XRT was given in 17% of patients. Of these 86% of patients were alive ,5% patients died , 3% patients abandoned, 6% patients relapsed ,3% patients progressed while on chemotherapy. Five years Overall survival was 94% and 5 Years Event free survival was 91%. Minimum haematological and other toxicity was seen in patients who had received COPDac/ABVD when compared to other regimen. CONCLUSIONS: Hodgkin's lymphoma patients had good outcome with different chemotherapy regimens, however our experience showed that the COPDac/ABVD regimen wass better tolerated with minimum toxicity.

Primary Extra-renal Wilms' Tumor in Urinary Bladder: Rare Presentation of a Common Pediatric Malignancy.

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.