ED-18 * PRIMARY INTRACRANIAL SOFT TISSUE SARCOMA IN CHILDREN AND YOUNG ADULTS: RETROSPECTIVE ANALYSIS - MD ANDERSON CANCER CENTER EXPERIENCE

Abstract

INTRODUCTION: Primary intracranial sarcoma (ISTS) is rare with unknown origin and ill-defined incidence in pediatric population. There is paucity of literature reporting outcomes of ISTS for children and young adults. Multimodality approach is used in most of the studies to treat this tumor with no well-defined treatment consensus. PATIENTS AND METHODS: A retrospective analysis of pediatric patients with primary diagnosis of ISTS treated at MD Anderson Cancer Center was conducted using descriptive measures, to describe clinical findings, treatment and long-term outcomes of children and adolescents with ISTS. RESULTS: Eleven patients (median age 6 years, range 1-20 year) with primary diagnosis of ISTS treated at our center between 2000 and 2014. Six patients were male, primary diagnosis as follows: Undifferentiated sarcoma (n = 5), Primitive neuroectodermal tumor (n = 2), Hemangiopericytoma (n = 1), Chondrosarcoma (n = 1), Aggressive mesenchymal tumor (n = 1), Neurofibrosarcoma (n = 1). Two patients had extraneural spread to the bone and the spine. Nine patients underwent gross total resection at time of diagnosis. Six cycles of the 3 drug regimens (Etoposide/ifosfamide/ Carboplatin) was the most common chemotherapy given at primary diagnosis or recurrence. Local radiation therapy with/without temozolomide were given to nine patients either after primary surgery or following surgery and adjuvant chemotherapy. Second look surgery was pursued in two patients before starting radiation and microscopic disease was found at site of the primary tumor despite of no radiologic evidence of residual disease. Five patients had disease progression, of whom four died of their disease. CONCLUSION: ISTS is rare neoplasm with dismal outcome. Despite Multimodality therapy, ISTS remains a challenge to treat with high rate of recurrence or treatment failure. Primary surgery followed by systemic chemotherapy and second look surgery at time of local control along with radiation therapy are associated with best chance for complete cure.