Treatment challenges and outcomes for pediatric intracranial ependymoma at a single institution in Iran.

BACKGROUND: The scarcity of information on pediatric ependymoma in Iran motivated this study. Our main objectives were to determine outcomes, identify clinical management challenges at a nongovernment hospital in Iran, and devise guidelines for improving care. PROCEDURE: A retrospective chart review was performed for pediatric patients with ependymoma who were younger than 15 years and treated at MPCTRC between 2007 and 2015. Records included patient demographics, treatment regimens used, duration of follow-up, and outcomes. Clinical outcomes [ie, 3-year overall survival (OS) and progression-free survival (PFS)] were determined based on the age at diagnosis (younger or older than 3 years) by using the Kaplan-Meier method. RESULTS: In total, 73 eligible patients were enrolled; 20 patients were in the younger group, and 53 were in the older group. The majority (91.8%, n = 67) of patients underwent initial gross-total or partial surgical resection, and 6 (8.2%) had a biopsy. Twenty-one patients experienced ependymoma recurrence. The median time to relapse was 1 year. The median duration of follow-up and PFS were 25 and 17 months, respectively. The 3-year OS and PFS were 61% and 59.5%, respectively. At the time of this project, 27 patients had died, and 35 were alive with no evidence of disease. CONCLUSION: Our study demonstrated inferior outcomes of Iranian children with ependymoma. To improve our care for these children, a paradigm shift must occur that includes radiation therapy as standard of care, second-look surgery, a multidisciplinary team approach, and potentially twinning initiatives.

Management and outcomes of treating pediatric medulloblastoma: an eight years' experience in an Iranian pediatric center.

PURPOSE: The clinical management of pediatric medulloblastoma requires a multidisciplinary approach, which can be challenging, especially in low- and middle-income countries. The aim of this study was to identify current challenges and describe the treatment and outcomes of Iranian pediatric patients with medulloblastoma who were referred to our center in Tehran, Iran. METHODS: Our retrospective review included 126 patient records from April 2007 to May 2015. The records were analyzed for epidemiologic features, treatment modalities, overall survival, and progression-free survival. Data were analyzed using SPSS 22.0 software. RESULTS: Median age at diagnosis was 6 years (male:female ratio, 2.3:1). At the time of diagnosis, 7 patients were 2 years or younger, and 76 (60.3%) were categorized as having high-risk disease. Overall, 100 patients had gross or near-total surgical resection. Cerebral spinal fluid involvement was detected in 22.2% of the patients tested, and spinal involvement was detected in 25% of the patients who underwent spinal MRI. Metastasis stages at the time of diagnosis were as follows: M0: 48.4% patients, M1: 16.7%, M2: 5.5%, and M3: 21.4%. Median times of follow-up and progression-free survival were 16 and 12 months, respectively. Probability of 7-year overall survival and progression-free survival were 59 and 53.8%, respectively. CONCLUSIONS: Results of the current retrospective study emphasize the need for implementing measures to improve outcome for our patients with medulloblastoma. Such measures include a multidisciplinary approach, unified national treatment guidelines, better disease and metastasis staging, twinning initiatives, and seeking a second opinion when needed.

CD7 aberrant expression led to a lineage switch at relapsed childhood acute pre-B lymphoblastic leukemia.

Immunophenotypic changes and lineage switch between diagnosis and relapse in acute lymphoblastic leukemia are uncommon and accompanied by poor outcomes. In this report, a 12-year-old boy with diagnosis of pre-B ALL with an aberrant expression of CD 7 is described. Patient was treated with the ALL-BFM 2000 protocol and suffered an episode of relapse with a lineage switch from pre-B ALL to T cell ALL. This report concludes that presence of aberrant expression of CD7 at diagnosis of pre-B ALL can have prognostic value of lineage switch to T cell ALL at relapse.

Family History and Relapse in Pediatric Acute Myeloid Leukemia.

We report the epidemiology and characteristics of acute myeloid leukemia and outcomes of its treatment with the AML-BFM 83 protocol at the Mahak Pediatric Cancer Treatment and Research Center, Tehran, Iran, from 2007 to 2012. A positive family history of cancer or leukemia was associated with the risk of relapse (family history of cancer in relapse: n = 11; 61%, P = 0.136, leukemia: n = 7; 39%; P = 0.016). Treatment-related mortality was 19% and associated with underweight patients (n = 5; 62.5%; P = 0.158). Event-free and overall survivals were 36% (SE = 3.5) and 44% (SE = 3.4), respectively. These data suggest a possible relationship between family history and relapse rate.