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Natl J Integr Res Med. 2016; 7(2): 44-51

Secondary Malignancies Developing After Acute Lymphoblastic Leukemia And Its Treatment With Constitutional Mismatch Repair Deficiency Syndrome In Siblings

Mitul Modi*, Tarang Patel*, Priti Trivedi*, Mridul Anand**, Sameer Dalsaniya*, Tapan Varlekar***, Dhaval Jetly*.

Background & Objectives: Acute Lymphoblastic Leukemia (ALL) is the most common childhood malignancy, with an annual rate of 3 to 4 cases per 1,00,000 children. ALL patients are treated with chemotherapeutic agents and cranial irradiation. Long-term sequelae of treatment are impaired intellectual and psychomotor functioning, neuroendocrine abnormalitites, impaired reproductive capacity, cardiotoxicity and second malignant neoplasms are being reported with increased frequency in the survivors. Among second neoplasms observed after treatment of ALL, central nervous system (CNS) tumors in patients treated with cranial irradiation are the most common. Others are Lymphoma, Acute Myeloid Leukemia (AML) and Thyroid Cancer. Methodology & Results: We are reporting four cases ,who developed ALL at the age of 8,6,4 and 25 years respectively(among that case 1 & case 2 are siblings) and were treated with chemotherapy and cranial irradiation. They developed Astrocytoma Grade-4 , Alveolar Soft-Part Sarcoma(ASPS) , Anaplastic Large Cell Medulloblastoma & Ewing’s Sarcoma at, 12 , 15, 8, 12 & 26 years of age respectively. Conclusion: Oncologists should make sure about the radiation doses before starting treatment and they should keep CMMR-D as a possibility in pediatric patients with siblings having h/o malignancy. [Modi M NJIRM 2016; 7(2):44-51]

Key words: Alveolar Soft part Sarcoma, Constitutional mismatch repair deficiency syndrome, Acute Lymphoblastic Leukemia, Neural tumor.

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