CURRENT KNOWLEDGE ON THE EPIDEMIOLOGY, HISTOLOGY, SYMPTOMS, DIAGNOSIS AND TREATMENT OF RHABDOMYOSARCOMA IN THE PAEDIATRIC POPULATION
Abstract
Introduction and Objective: Rhabdomyosarcoma (RMS) is the third most common solid and extracranial malignant tumour in children. It originates from mesenchymal tissue and is a heterogeneous tumour in terms of location, histology, clinical presentation and prognosis. The aim of this paper is to provide a comprehensive review of the current state of knowledge on this disease.
Description of The State of Knowledge: The total incidence rate of RMS is approximately 4.5 patients per million people under the age of 20. The tumour is most commonly located in the head and neck, less frequently in the urinary tract, limbs and trunk. Histologically, the embryonal type is the most common, accounting for 60-80% of all RMS cases. The symptoms of the disease depend primarily on the location of the primary tumour and may include exophthalmos, visual disturbances, pain and tissue swelling, nosebleeds, haematuria, visible tumours and general symptoms in advanced forms, i.e. weakness, fever and weight loss. One of the key factors influencing the prognosis is the presence of distant cancer metastases.
Summary: The diagnosis of RMS is difficult due to the rare occurrence of the disease and non-specific symptoms. Although the cure rate for local disease is generally > 70% of patients, metastatic disease is still associated with low overall survival despite intensive treatment strategies. Therefore, early diagnosis of the disease is very important for achieving the best possible treatment outcomes and full recovery.
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