M1047: 1. Brain

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Clinical history : A 33 year old male with nausea, headache and weak vision.
A cerebellar hemangioblastoma was removed and 6 months prior to death the patient presented with misbalance, double vision nausea, headache and heart failure.
After intensive investigation, an atrial septum defect was detected as well as a recurrent tumor in the cerebellum.
A full body scan showed a cyst in the pancreas and cystic kidney tumor and a bilateral retinal angioma and later developed respiratory failure.
Autopsy findings : 1. Cerbellar hemangioblastoma.
2. Bilateral renal hematoma.
3. Multiple pacreas cysts with single bright cell hematomas in the pancreas.
4. Retinal hemangioblastoma.
Diagnosis: Brain: cerebellar hemangioblastoma.
Kidney: renal hematoma von Hippel- Lindau syndrome.
Pancreas: single cyst von Hippel- Lindau syndrome.
Eye: Retinal hemangioblastoma von Hippel- Lindau syndrome

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