Complementary role of 18F-FDG and 68Ga-DOTATATE PET/CT in the surveillance of patients with von Hippel-Lindau syndrome

Georgios Z. Papadakisab, Samira M. Sadowksic, Apostolos H. Karantanasab, Corina Millod

Medical School, University of Crete, Heraklion, Crete, Greece; Foundation for Research and Technology Hellas (FORTH), Computational Biomedicine Laboratory (CBML), Heraklion, Crete, Greece; Surgical Oncology Program, National Institutes of Health (NIH), Bethesda, Maryland, USA; Clinical Center (CC), National Institutes of Health (NIH), Bethesda, Maryland, USA

aDepartment of Radiology, Medical School, University of Crete, Heraklion, Crete, Greece (Georgios Z. Papadakis, Apostolos H. Karantanas); bFoundation for Research and Technology Hellas (FORTH), Computational Biomedicine Laboratory (CBML), Heraklion, Crete, Greece (Georgios Z. Papadakis, Apostolos H. Karantanas); cDepartment of Thoracic and Endocrine Surgery, University Hospital of Geneva and Faculty of Medicine, University of Geneva, Geneva, Switzerland (Samira S. Sadowksi); dPositron Emission Tomography (PET) Department, Clinical Center (CC), National Institutes of Health (NIH), Bethesda, Maryland, USA (Corina Millo)

Correspondence to: Georgios Z. Papadakis, MD, MPH, PhD, Foundation for Research and Technology Hellas (FORTH), Computational Biomedicine Laboratory (CBML), N. Plastira 100, Vassilika Vouton, GR-70013, Crete, Greece, e-mail: gzpapadakis@gmail.com
Received 17 February 2020; accepted 23 February 2020; published online 14 March 2020
DOI: https://doi.org/10.20524/aog.2020.0465
© 2020 Hellenic Society of Gastroenterology

A 45-year-old woman with a history of von Hippel-Lindau (VHL) syndrome was evaluated during follow up with whole-body positron-emission tomography/computed tomography (PET/CT) scans post administration of 68Ga-DOTATATE (targets cell surface overexpression of somatostatin receptors) (Fig. 1A,B) and 18F-FDG (targets metabolic activity) (Fig. 1C,D) within two consecutive days. 68Ga-DOTATATE was taken up by a cerebral hemangioblastoma (Fig. 1A) and revealed (Fig. 1A,B) 2 lesions in the head and the uncinate process of the pancreas; both were 18F-FDG negative, indicative of well-differentiated neuroendocrine tumors (NETs). Additionally, 18F-FDG revealed (Fig. 1C,D) a hypermetabolic tumor adjacent to the left adrenal gland, which was 68Ga-DOTATATE-negative. This tumor was surgically resected and was proven to be an extra-adrenal paraganglioma. VHL is a highly penetrant, autosomal, dominantly inherited disorder associated with a germline mutation of the VHL tumor suppressor gene on the short arm of chromosome 3. It is characterized by the development of several benign and malignant tumors, including hemangioblastomas, pancreatic NETs, and paragangliomas [1]. 68Ga-DOTATATE is a radiolabeled somatostatin analog suitable for PET imaging, that has emerged as the imaging standard of reference for the diagnosis of well-differentiated NETs [2,3]. Furthermore, hemangioblastomas, which are highly vascular benign tumors, are known to be 68Ga-DOATATE-positive, as in the present case [2,4]. In addition, 18F-FDG is the most widely used PET-tracer for oncologic purposes, as it can contribute significantly to the detection and follow up of various VHL-related tumors. The presented data highlights the complementary role of 68Ga-DOTATATE and 18F-FDG for the accurate diagnosis of uncertain lesions in the context of the surveillance for patients with VHL.

thumblarge

Figure 1 (A) Maximum intensity projection (MIP) 68Ga-DOTATATE positron-emission tomography (PET) image of the head and torso, showing a focal lesion in the left cerebellum (green arrow), corresponding to a known hemangioblastoma, and 2 foci of abnormally increased radiotracer uptake in the abdomen (red arrows). (B) Axial fused 68Ga-DOTATATE PET/CT image of the abdomen, showing (white arrow) a focal lesion in the pancreatic head (SUVmax: 41.7) and a second lesion (yellow arrow) in the uncinate process of the pancreas (SUVmax: 32.7), indicative of neuroendocrine tumors. (C) MIP 18F-FDG PET image of the head and torso, showing a focal, intensely hypermetabolic lesion in the left upper abdomen (red arrow). (D) Axial fused 18F-FDG PET/CT image of the abdomen, showing (white arrow) 18F-FDG avid lesion (SUVmax: 20.3) located below the pancreatic tail, adjacent to the left adrenal gland. The patient underwent surgery for the mass adjacent to the left adrenal gland, and histology revealed extra-adrenal paraganglioma

References

1. Ganeshan D, Menias CO, Pickhardt PJ, et al. Tumors in von Hippel-Lindau syndrome:from head to toe-comprehensive state-of-the-art review. Radiographics 2018;38:849-866.

2. Hofman MS, Lau WF, Hicks RJ. Somatostatin receptor imaging with 68Ga DOTATATE PET/CT:clinical utility, normal patterns, pearls, and pitfalls in interpretation. Radiographics 2015;35:500-516.

3. Tirosh A, Papadakis GZ, Millo C, et al. Prognostic utility of total 68Ga-DOTATATE-Avid tumor volume in patients with neuroendocrine tumors. Gastroenterology 2018;154:998-1008.

4. Papadakis GZ, Millo C, Jassel IS, et al.18F-FDG and68Ga-DOTATATE PET/CT in von Hippel-Lindau disease-associated retinal hemangioblastoma. Clin Nucl Med 2017;42:189-190.

Notes

Conflict of Interest: None