Von hippel - lindau disease, adrenal involvement

(Eugen von Hippel, 1867 - 1939, German ophthalmologist, Arvid Lindau, 1892 - 1958, Swedish pathologist). Phaeochromocytoma in von Hippel Lindau disease is often multiple and extra-adrenal. CT, MRI and MIBG scans may be used to demonstrate the tumour. CT demonstrates a solid enhancing mass and its detection in the adrenal or extra adrenal location is highly accurate. MRI shows characteristic findings on high signal intensity on T2-weighted images. Characterization of phaeochromocytoma by MRI is considered highly specific, but the most specific modality is MIBG. MIBG is of particular value in the case of extra-adrenal phaeochromocytoma (see radionuclide adrenal imaging). For a general description of the disease, see von Hippel Lindau disease CNS manifestation. Also, see von Hippel Lindau disease.

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