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Sarcomas rise from mesenchymal layers and may transform to variable malignant lesions in multiple organs systems. Although they are mostly localized, many of them have predilection of distant sites away from their primary origins and subsequent metastasis to different anatomical locations of body. Soft Tissue Sarcomas (STSs) represent one percent of adult and about 7% of pediatric malignancies. Although insignificant in number, these tumors may complicate patient’s overall health status compared with other malignancies, because of their numerous subtypes and variable features which may pose an everlasting challenge to both clinical and surgical treatment strategies. Existence of these complications and a relatively small available data regarding Central Nervous System’s (CNS) sarcomas, encouraged authors to conduct a retrospective study in a 6-year period to evaluate the specific epidemiological features of CNS sarcomas, including brain, spinal cord and meningeal layer sarcomas extracted from Iranian National Cancer Registry (INCR). Our study revealed CNS sarcoma’s trend to affect males more than females, affecting them most in their midlife and a higher prevalence of brain involvement compared to spinal cord and meningeal layers. Also, we have provided detailed morphological features of the tumors, as well as patient’s geographical distribution, with Northern parts of Iran have the lowest incidence rate (10.06%). Moreover, our analysis of crude rate revealed lower Age Specific Incidence Rate (ASIR) of CNS Sarcomas than expected number of cases compared to standard world population by World Health Organization (WHO)’s ASIR as 0.035 person per 1-years. Authors believe this report of epidemiological assessment of CNS sarcomas in Iran could act as a foundation for better understanding the underlying pathophysiological mechanism as it is the first survey in developing countries and selection of optimal treatment strategies based on improved understanding of these neoplasms.

Epidemiologic Evaluation of Central Nervous System Sarcomas in Iran: A 2009-2014 Survey