Fig 1

Gabriel Ogun

and 6 more

Aim: To describe the pattern of paediatric soft tissue sarcomas with emphasis on the grade and stage at the first surgical or medical oncologic intervention. Methods: We retrospectively reviewed all cases of histologically confirmed paediatric Soft Tissue Sarcomas in the Department of Pathology, University College Hospital (UCH), Ibadan, Nigeria in children age 0-14 years. The study period was January 1991 to December 2016. Information obtained included age, gender, morphology and site of the tumours. The tumour grade and pathologic/clinical staging of all cases that had surgical biopsy or surgical resection were also obtained and verified from the clinical records. Tumour grading was carried out using the Fédération Nationale des Centres de Lutte Contre le Cancer (FNCLCC) Sarcoma group grading system and staging was done using TNM. Results: One hundred and twelve cases were seen with an almost equal male-to-female ratio and age range from 5 months to 14 years (median 8.5 years). The peak age group was 5-9 years. Rhabdomyosarcoma was by far the most common histological type (73.2%). Other types were undifferentiated sarcomas (6.3%), fibrosarcoma (5.4%), Kaposi sarcoma (4.5%), and 2.7% each for synovial sarcoma, dermatofibrosarcoma protuberans. The common primary sites were the head and neck (including the orbit) 51(45.5%), and the abdominopelvic 27(24.1%) regions. Other sites included the lower limb 13(11.6%), trunk 12(10.7%), upper limb 6(5.4%). Majority (67.9%) had histologic grade 3 at presentation based on the FNCLCC grading system. About 48.2% had Stage T2b while 9.8% were stage T2a based on American Joint Committee on Cancer Staging (AJCC) system. Conclusion: Rhabdomyosarcoma, undifferentiated sarcomas and Kaposi sarcoma are the commonest paediatric malignant soft tissue tumours in our practice. Majority of our patients present with late stage tumour and with high histologic grade thereby conferring poor prognosis at presentation/first surgical or medical oncologic intervention.