Саркома Юинга — агрессивная первичная опухоль костей, требующая комбинированного системного и локального лечения с самого начала. Терапия первой линии начинается незамедлительно, а ответ на химиотерапию определяет тактику локального лечения первичной опухоли.
Предпочтительная стратегия первой линии предполагает комбинированную химиотерапию с уплотнёнными интервалами в сочетании с окончательным локальным лечением первичной опухоли — хирургической резекцией и/или лучевой терапией — в зависимости от характеристик опухоли, анатомического расположения и состояния пациента.
DOI: 10.1038/s41416-024-02868-4
Greater treatment intensity is linked to better outcomes: a two-weekly interval-compressed VCD/IE induction was demonstrated to be more effective than the same regimen given three-weekly and VDC/IE induction followed by IE/VC consolidation has better outcomes than VIDE induction and VAI or VAC consolidation and is now the preferred first-line treatment for all patients who are medically fit to receive it.
Complete removal of the primary tumour (meaning the parts of all anatomical structures involved in the original tumour volume) provides optimal local control but is not always feasible, for example, because critical anatomical structures are involved.
Radiotherapy should be considered in addition to surgery if there is a poor radiological or histological response to chemotherapy, the surgical margins are inadequate, the tumour is large or is in a high-risk area (e.g. pelvis).
Definitive radiotherapy is frequently recommended for tumours judged to be inoperable, those in anatomic locations where complete removal would cause unacceptable morbidity (e.g. pelvic and sacral tumours), in patients at unacceptable risk of significant surgical complications, and if the prognosis is poor (e.g. widespread bone metastases) such that morbidity of surgery is not appropriate.
If there is a poor histological response (≤90% necrosis) to pre-operative chemotherapy, even if the resection margins are negative.
Tumour volume change can be seen on MRI, and reliably reflects chemotherapy response particularly if late.
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