Карциноидный синдром у ребенка с соматостатиномой поджелудочной железы

Карциноидный синдром обусловлен повышенной секрецией вазоактивных веществ нейроэндокринными опухолями (НЭО). Соматостатинома (крайне редкий вариант НЭО) проявляется в типичных случаях триадой симптомов - холелитиазом, стеатореей, сахарным диабетом. Частота встречаемости соматостатином 1:40 000; такие НЭО описаны только у взрослых. Карциноидный синдром не является типичным проявлением соматостатиномы. Представлено первое в мире описание случая соматостатиномы у ребенка, дебютировавшей карциноидным синдромом. Все лечебные мероприятия оказались безуспешными из-за поздней диагностики (распространенный метастатический процесс). Трудности в диагностике были связаны с низким уровнем биохимических маркеров данного заболевания (хромогранина А, 5-ГИУК, серотонина) и малого размера первичной опухоли поджелудочной железы, не визуализируемой при МСКТ. Только иммуногистохимические исследования определили секрецию опухолью соматостатина.

нейроэндокринные опухоли, карциноидный синдром, соматостатинома, дети

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