Endocrine Abstracts

A 41-year-old woman was admitted with headache and hypertension. She described worsening of headaches in the last 2 months. On physical examination blood pressure was 150/90 mmHg, and grade 1 hypertensive retinopathy was present. Her sister died at the age of 24 due to pancreas neuroendocrine tumour and her mother was followed up with multiple pancreatic cysts. Abdomen MRI revealed a 6 cm phaeochromocytoma lesion on the right surrenal and multiple cysts in the pancreas, kidneys and ovaries. Urinary catecholamin levels were elevated by sixfold. Other laboratory results were normal. Doxazosin 4 mg (b.i.d.) and amlodipin 5 mg were initiated. Patient’s family history, presence of multiple visceral cysts and phaeochromocytoma led us to consider Von Hippel Lyndau sydrome. Cranium MRI which was performed due to severe headaches revealed a 5 cm serebellar hemanjioblastoma and hydrocephalus of the third ventricle. Urgent surgical intervention was indicated due to presence of hydrocephalus and risk of herniation. Coexistence of phaeochromocytoma, risk of hypertensive crisis and deterioration of herniation with anaesthesia induction made it mandatory to perform the two surgeries sequentially in the same surgical session. As the patient had been receiving α blockers for a sufficient time of preoperative period, laparoscopic right surrenalectomy was performed, followed by hemangioblastoma excision in the same session. Pathological evaluation revealed, right surrenal phaeochromocytoma with a Ki score of 3% and grade 1 serebellar hemanjioblastoma. Two months after the surgery patient was normotensive without any antihypertensive treatment and symptoms of headache were omitted. Screening of the family members revealed multiple pancreas, renal and epididymal cysts, serebellar hemangioblastoma and phaeochromocytoma in her brother compatible with Von Hippel Lindau syndrome and treatment was initiated. Evaluation of the other family members were normal and a surveillance programme was scheduled.

Conclusion: Hemangioblastoma of central nervous system which may complicate the surgery of phaeochromocytoma must be considered in patients who present with phaeochromocytoma and multisystemic manifestations implicating presence of a genetic syndrome.