Soft tissue sarcomas in children
Last reviewed: 23.04.2024
All iLive content is medically reviewed or fact checked to ensure as much factual accuracy as possible.
We have strict sourcing guidelines and only link to reputable media sites, academic research institutions and, whenever possible, medically peer reviewed studies. Note that the numbers in parentheses ([1], [2], etc.) are clickable links to these studies.
If you feel that any of our content is inaccurate, out-of-date, or otherwise questionable, please select it and press Ctrl + Enter.
Soft tissue sarcomas are a group of malignant tumors originating from primitive mesenchymal tissue. They account for about 7-11% of all malignant neoplasms of childhood. Half of the soft tissue sarcomas are represented by rhabdomyosarcoma. Along with rhabdomyosarcoma, most often children develop synovial sarcomas, fibrosarcomas and neurofibrosarcomas. Non-rhabdomyosarcoma soft tissue tumors are more common in adults. 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 revealed. General symptoms (fever, weight loss, etc.) are observed rarely, often in advanced stages of the disease.
Histological classification of soft tissue sarcomas
Histologically, soft tissue sarcomas are extremely heterogeneous. Below are variants of malignant sarcomas and histogenetically corresponding to them types of tissues.
Soft-tissue sarcomas are also referred to as extra-osseous tumors from bone and cartilaginous tissue (extraossar osteosarcoma, myxoid and mesenchymal chondrosarcoma).
Morphologically, it is difficult to make differential diagnosis between non-bloomingosarcoma soft-tissue tumors. To clarify the diagnosis, use electron microscopy, immunohistochemical and cytogenetic studies.
The histological appearance of a tumor of soft tissues (with the exception of rhabdomyosarcoma) does not give a clear idea of the clinical course and prognosis of the disease. To determine the correlation between the histological variant and the tumor behavior, the POG multidisciplinary research group (Pediatric Oncology Group, USA) identified three variants of histological changes that could be used as forecasting factors in a prospective study. 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 the tumors of the third group (degree III) in comparison with the first and second have a much worse prognosis.
Diagnosis of soft tissue sarcomas
Verification of the diagnosis of soft tissue sarcoma presents significant difficulties. When carrying out 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.
What do need to examine?
Who to contact?
Treatment of soft tissue sarcomas
The strategy of treating tumors in children and adults is different, which is connected with several points:
- carrying out organ-saving operations in children is associated with great technical difficulties due to anatomical and functional features;
- the use of radiotherapy in young children can lead to serious complications (for example, disruption in the growth of individual organs and the body as a whole), more pronounced than in adults;
- In children's oncology, more stringent schemes of high-dosage chemotherapy have been developed. Including multicomponent regimens (carrying out similar chemotherapeutic treatment in adults is often impossible in connection with the worst tolerability);
- the long-term effects of all therapies in children are socially more significant due to the potentially longer life expectancy compared with adults.
Drugs
Использованная литература