Malign Feokromasitoma ve Paragangliomalar
Özet
Feokromasitomalar adrenal medulladan, paragangliomalar ise adrenal dışı sempatik veya parasempatik zincirlerden köken alan kromaffin hücreli tümörlerdir. Histopatolojik olarak benzerlik gösterseler de, paragangliomaların malignite riski (%25), feokromasitomalara (%10) kıyasla daha yüksektir. En önemli malignite belirteci akciğer, karaciğer, kemik ve lenf nodlarına yapılan metastazlardır. Klinik olarak paroksismal hipertansiyon, epizodik baş ağrısı, terleme ve taşikardi üçlüsü ile prezente olurlar. Tanıda ilk adım plazma veya idrarda fraksiyone metanefrin ölçümüyken, anatomik lokalizasyon için BT ve MRG, fonksiyonel değerlendirme için ise Ga68-DOTA-TATE PET/BT altın standarttır. Küratif tedavisi olmayan metastatik hastalıkta semptom kontrolü için ilk etapta kombine alfa ve beta-adrenerjik blokaj uygulanmalıdır. Tedavi yönetimi multidisipliner olup cerrahi rezeksiyon, lokal ablatif yöntemler (RFA, radyoterapi) ve sistemik tedavileri (I-131 MIBG, Lu-177 DOTATATE, CVD kemoterapisi ve sunitinib gibi tirozin kinaz inhibitörleri) içerir. Tüm hastalara genetik test önerilmektedir.
Pheochromocytomas are tumors originating from the chromaffin tissue of the adrenal medulla, whereas paragangliomas are extra-adrenal tumors arising from sympathetic or parasympathetic chains. Although histopathologically indistinguishable, the risk of malignancy is higher in paragangliomas (25%) than in pheochromocytomas (10%). The most definitive indicator of malignancy is metastasis, typically to the lungs, liver, bones, and lymph nodes. Clinically, they present with paroxysmal hypertension and the classic triad of episodic headache, sweating, and tachycardia. Initial biochemical diagnosis relies on plasma or urinary fractionated metanephrines, while CT and MRI are used for anatomical localization. Ga68-DOTA-TATE PET/CT is recommended as the gold standard for functional imaging. Symptom management requires combined alpha- and beta-adrenergic blockade before any intervention. Although there is no cure for metastatic disease, management requires a multidisciplinary approach including surgical resection, local ablative therapies (RFA, radiotherapy), and systemic treatments such as I-131 MIBG, Lu-177 DOTATATE, CVD chemotherapy, or sunitinib. Genetic testing is strictly recommended for all patients.
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