Shish qayerdan keladi?
Xomilaning rivojlanishida asab hujayralari butun tana bo'ylab ko'chib o'tadi va simpatik asab tizimini, shu jumladan umurtqa pog'onasi bo'ylab buyrak usti medullasi va ganglionlarni keltirib chiqaradi. Agar bu hujayralarning ba'zilari kamolotga etmasa, ular neyroblastomaga aylanishi mumkin. Shuning uchun o'simta asosan yosh bolalarda uchraydi va simpatik zanjir bo'ylab joylashgan. Chaqaloqlarda ba'zan yetilmagan hujayralar kechroq pishib, o'simta yaxshi xulqli yoki yo'qoladi - bu boshqa xavfli o'smalarda deyarli uchramaydigan hodisa.
- Simpatik asab tizimining yetilmagan hujayralarining shishi
- Eng keng tarqalgan joylashuvi: buyrak usti bezlari
- Orqa miya, ko'krak, bo'yin va tos suyagi bo'ylab paydo bo'lishi mumkin
- Chaqaloqlar mustaqil ravishda etishishi mumkin
- O'simta harakati yoshga va molekulyar xususiyatlarga bog'liq
Semptomlar
Ko'rinishlar o'simtaning joylashishiga va uzoq o'choqlarning mavjudligiga bog'liq. Qorin bo'shlig'i shishi bilan ota-onalar ko'pincha o'zlari zich shakllanishni his qilishadi yoki qorin bo'shlig'ining kattalashishini sezishadi; Shu bilan birga, bolaning bezovtalanishi, yomon ovqatlanishi va vazni yo'qolishi mumkin. Ko'krak qafasidagi shish yo'tal va nafas qisilishi sabab bo'ladi; bo'ynidagi o'sma ko'z qovog'ining tushishiga va ko'z qorachig'ining siqilishiga olib keladi. Orqa miya kanaliga qarab o'sish oyoqlarda zaiflik va siydik chiqarishda qiyinchiliklarga olib keladi. Suyaklarga o'tganda og'riq va oqsoqlik paydo bo'ladi, ko'z atrofidagi ko'karishlar ko'z olmalarining chiqib ketishi tipik belgi hisoblanadi.
- Qorin bo'shlig'ining zich shakllanishi va kengayishi
- Pallor, letargiya, ishtaha va vazn yo'qolishi
- Suyak og'rig'i va oqsoqlik
- Ko'z atrofida ko'karishlar va ko'z olmasining chiqib ketishi
- Ko'z qovog'ining tushishi va ko'z qorachig'ining torayishi
- Orqa miya kanaliga o'sayotganda oyoqlarda zaiflik
- Gormonal faol o'smalar bilan doimiy diareya
Diagnostika
They start with an ultrasound, which is accessible and safe for the child, then perform a CT or MRI to accurately assess the extent and relationship to the vessels. An important feature is that most neuroblastomas produce catecholamines, the metabolic products of which are determined in the urine, and this helps in both diagnosis and observation. The bone marrow must be examined, since the tumor often spreads into it, and a special radioisotope study is carried out to search for foci. The diagnosis is confirmed by a biopsy with molecular testing, including the status of the MYCN gene, which determines the risk group.
- Ultrasound of the abdominal cavity and retroperitoneal space
- Ta'sir qilingan hududning KT yoki MRI
- Siydikda katexolaminlar va ularning metabolitlari
- LDH va ferritin
- Suyak iligi ponksiyon va biopsiya
- Issiq nuqtalarni topish uchun radioizotopni o'rganish
- MYCN gen testi bilan o'simta biopsiyasi
Xavf guruhlari va taktikasi
Neuroblastoma is divided into risk groups taking into account the child’s age, stage, histological structure and molecular features, primarily changes in the MYCN gene. Bu asosan davolanishga ta'sir qiladi. At low risk, especially in children in the first year of life, surgery and sometimes just observation are sufficient, since the tumor can mature or regress on its own. Intermediate risk requires moderate chemotherapy and surgery. High risk is treated intensively and for a long time, combining all available methods.
- Bolaning yoshi asosiy omil sifatida
- Shishning bosqichi va darajasi
- Gistologik tuzilish
- MYCN geni o'zgaradi
- Kam xavf: jarrohlik yoki kuzatish
- O'rta xavf: kimyoterapiya va jarrohlik
- Yuqori xavf: intensiv kombinatsiyalangan davolash
Davolash va kuzatish
If the risk is low, treatment is minimal and aimed at doing no harm: in infants with small, asymptomatic tumors, close monitoring with regular ultrasound is possible. If the risk is intermediate, chemotherapy is given and the tumor is removed. High-risk treatments include intensive chemotherapy, surgery, high-dose therapy with autologous stem cell support, radiation therapy, and subsequent supportive care to kill residual cells. Observation after treatment is long-term, with monitoring of urine tests and visualization according to the schedule.
- Chaqaloqlarda kichik o'smalarni kuzatish
- O'simtani jarrohlik yo'li bilan olib tashlash
- Protokol bo'yicha kimyoterapiya
- Ildiz hujayralarini qo'llab-quvvatlash bilan yuqori dozali terapiya
- Yuqori xavf ostida radiatsiya terapiyasi
- Asosiy davolashdan keyin parvarishlash terapiyasi
- Long-term follow-up with catecholamine monitoring
