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Soft tissue sarcomas in children
Last reviewed: 07.07.2025
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Soft tissue sarcomas are a group of malignant tumors originating from primitive mesenchymal tissue. They account for about 7-11% of all malignant neoplasms in childhood. Half of soft tissue sarcomas are rhabdomyosarcoma. Along with rhabdomyosarcoma, synovial sarcomas, fibrosarcomas, and neurofibrosarcomas most often develop in children. Non-rhabdomyosarcoma soft tissue tumors are more common in adults. The localization of soft tissue sarcomas is very diverse.
[ Symptoms of soft tissue sarcomas
Clinical manifestations depend on the location of the tumor and its relationship to surrounding tissues. As a rule, local symptoms of tumor growth are detected. General symptoms (fever, weight loss, etc.) are rarely observed, more often in advanced stages of the disease.
Histological classification of soft tissue sarcomas
Histologically, soft tissue sarcomas are extremely heterogeneous. Below are presented variants of malignant sarcomas and the histogenetically corresponding tissue types.
Soft tissue sarcomas also include extraosseous tumors of bone and cartilage tissue (extraosseous osteosarcoma, myxoid and mesenchymal chondrosarcoma).
It is morphologically difficult to perform differential diagnostics between non-rhabdomyosarcoma soft tissue tumors. Electron microscopy, immunohistochemical and cytogenetic studies are used to clarify the diagnosis.
The histological type of a soft tissue tumor (except for rhabdomyosarcoma) does not provide a clear picture of the clinical course and prognosis of the disease. In order to determine the correlation between the histological type and tumor behavior, the multicenter research group POG (Pediatric Oncology Group, USA) in the course of a prospective study identified three types of histological changes that can be used as prognostic factors. The degree of histological changes was determined by the indices of cellularity, cellular pleomorphism, mitotic activity, severity of necrosis and invasive tumor growth. It was shown that tumors of the third group (grade III) have a significantly worse prognosis compared to the first and second.
Diagnosis of soft tissue sarcomas
Verification of the diagnosis of soft tissue sarcoma presents significant difficulties. When performing a biopsy, it is important to obtain a sufficient amount of tumor tissue not only for routine histological, but also for immunohistochemical, cytogenetic and molecular biological studies.
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Treatment of soft tissue sarcomas
The treatment strategy for tumors in children and adults is different, which is due to several factors:
- performing organ-preserving operations in children is associated with great technical difficulties due to anatomical and functional features;
- the use of radiation therapy in young children can lead to serious complications (for example, disruption of the growth of individual organs and the body as a whole), more pronounced than in adults;
- In pediatric oncology, more stringent high-dose chemotherapy regimens have been developed, including multicomponent regimens (carrying out such chemotherapy treatment in adults is often impossible due to poorer tolerability);
- The long-term effects of all types of therapy in children are socially more significant due to their potentially longer life expectancy compared to adults.
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