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KMID : 1148920230570050235
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Nuclear Medicine and Molecular Imaging
2023 Volume.57 No. 5 p.235 ~ p.242
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68Ga-DOTANOC PET/CT for Screening and Surveillance of Von Hippel-Lindau (VHL) disease
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Shamim Ahmed Shamim
Geetanjali Arora
Naresh Kumar
Jhangir Hussain
Shreya Datta Gupta
Arun Raj ST
Kritin Shankar
Alpesh Goyal
Rajesh Khadgawat
Sambit Sagar
Chandrasekhar Bal
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Abstract
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Purpose : Hereditary tumor syndrome Von Hippel-Lindau (VHL) disease is characterized by various benign and malignant tumors that are known to express somatostatin receptors (SSTR). We evaluated the role of 68Ga-DOTANOC PET/CT scan in patients with positive germline mutation of the VHL gene, presented initially or on follow-up, for the detection of recurrent or synchronous/metachronous lesions.
Methods : Fourteen patients (8 males; 6 females) with mean age 30?±?9.86 years were retrospectively analyzed, were tested positive for VHL on gene dosage analysis, and underwent 68 Ga-DOTANOC PET/CT scan for disease evaluation. The number and site of lesions were determined. The tracer uptake was analyzed semi-quantitatively by calculating the maximum standardized uptake values (SUVmax) of lesion.
Results : Four of the 14 patients underwent scan for initial diagnosis as baseline, 6 patients for post-therapy disease status, and 4 patients for initial diagnosis as well as follow-up evaluation of the disease. A total of 67 lesions were detected in 14 patients. The sites of lesions were cerebellar/vertebral/spinal (17; mean SUVmax?=?7.85); pancreatic neuroendocrine tumor (NET) (11; mean SUVmax?=?20.64); retina (3; mean SUVmax?=?10.46); pheochromocytoma (10; mean SUVmax?=?16.32); paragangliomas (3; mean SUVmax?=?10.65); pancreatic cyst (9; mean SUVmax?=?2.54); and renal cyst (8; mean SUVmax?=?1.56) and miscellaneous lesions constituted 6 lesions.
Conclusion : Our results show that 68 Ga-DOTANOC PET/CT may be a useful modality for screening and follow-up of associated tumors in patients with germline gene mutation for VHL. It can be used as a one-stop imaging modality for VHL patients and may substitute for separate radiological investigations, making it more convenient for patients in terms of time and cost.
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KEYWORD
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Von Hippel-Lindau (VHL) disease, 68 Ga-DOTANOC PET/CT, Hemangioblastoma, SUVmax
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