Paediatric soft tissue sarcomas in a resource constrain setting: Grade
and stage at presentation and at oncologic intervention are usually of
poor prognostic characteristics.
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.