Diagnosis and treatment of a patient with epithelioid sarcoma

The authors describe epithelioid soft tissue sarcoma in a child with emphasis on diagnostic difficulties associated with morphological verification and differential diagnosis, as well as evaluate the effectiveness of complex treatment, including radical surgery and adjuvant radiotherapy. An 11-year-old boy was diagnosed with epithelioid sarcoma of soft tissues of the right shoulder. Anamnesis, clinical examination, ultrasound, MRI, CT of the chest and abdominal cavity, scintigraphy and PET-CT were used to make the diagnosis. Additionally, bone marrow puncture was performed to exclude systemic spread. Treatment included total excision of tumor with defect closure and subsequent adjuvant radiotherapy. Assessment of effectiveness was based on clinical data and follow-up survey. The diagnosis was confirmed only after repeated morphological revisions and expanded immunohistochemical analysis. The patient underwent total resection of tumor and defect closure with subsequent radiotherapy. There were no major complications. The authors achieved local control of disease and good quality of life in their patient. This case illustrates the difficulties of early diagnosis of epithelioid sarcoma in childhood. This example complements available clinical base and has practical value for improving approaches to management of children with rare soft tissue tumors.