Role Of Ga-68-Dotanoc Pet/Ct Scan In Von Hippel-Lindau Disease

127 Aim: Hereditary tumor syndrome von Hippel-Lindau (VHL) disease is characterized by various benign and malignant tumors that are known to express somatostatin receptors (SSTR) like cerebellar and spinal hemangioblastoimas (HB), pheochromocytoma, paraganglioma and pancreatic NETs. Patients can present with different symptoms depending upon the characteristic tumor at presentation. We evaluated role of 68Ga-DOTANOC PET/CT scan in patients with positive germline mutation of VHL gene, presented initially or on follow up for detection of recurrent or metachronous lesions. Methods: Data of 13 patients (6 male; 7 female), who tested positive for VHL on gene dosage analysis and underwent 68Ga-DOTANOC PET/CT scan for disease evaluation, was retrospectively analyzed. All the scans were reported by two experienced nuclear medicine physicians. Number and site of lesions were determined. The tracer uptake was analyzed semi-quantitatively by calculating standardized uptake values (SUVmax) of lesion. Results: Mean age of patients was 33.61±13.18 years. Out of 13 patients, 3 patients were referred for baseline evaluation while 10 were referred for follow up evaluation. Seven of 13 patients had pancreatic cyst and 5/13 had renal cyst which did not show significant SSTR expression. Retinal angioma was present in 2/13 patients; paraganglioma in 3; pheochromocytoma in 5 and 6 patients had cerebellar/ spinal HB. Pancreatic NET was present in 6/13 patients. Additional finding of pancreatic NET was found in 4 patients; paraganglioma in 1 patient and cerebellar HB in 2 patients, which were not known previously. Of the 5 patients with pheochromocytoma on DOTANOC scan; 2 patients had recurrent lesions and 3 had newly diagnosed lesions. In an operated case of HB, DOTANOC scan showed similar lesion in contralateral cerebellar hemisphere in addition to local recurrence. Total of 31 68Ga-DOTANOC avid lesions were detected in 13 patients. The sites of lesions were pancreas (7; median SUVmax=5.3; IQR=1.8-19); retina (3; SUVmax range=1.3-14.9); adrenals (8; median SUVmax=9.7; IQR=3.4-21.7); cerebellar/vertebral/spinal (10; median SUVmax=7.5; IQR=2.1-15.4) and paragangliomas (3; SUVmax range=5.9-19). Conclusions: Our results show that 68Ga-DOTANOC PET/CT may be a useful modality for screening and follow up of associated tumours in patients with germline gene mutation for VHL.