Nucl Med Mol Imaging.  2023 Oct;57(5):235-242. 10.1007/s13139-023-00810-9.

⁶⁸Ga‑DOTANOC PET/CT for Screening and Surveillance of Von Hippel‑Lindau (VHL) disease

• Shamim S
• Arora G
• Kumar N
• Hussain J
• Gupta S
• ST A
• Shankar K
• Goyal A
• Khadgawat R
• Sagar S
• Bal C

• KMID: 2547012
• DOI: http://doi.org/10.1007/s13139-023-00810-9

Abstract

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 ⁶⁸Ga-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 ⁶⁸Ga-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.

Keyword

Von Hippel-Lindau (VHL) disease; ⁶⁸Ga-DOTANOC PET/CT; Hemangioblastoma; SUVmax