SpringerLink
Log in

Ga-68 Somatostatin Receptor PET/CT in von Hippel-Lindau Disease

  • Case Report
  • Published:
Nuclear Medicine and Molecular Imaging Aims and scope Submit manuscript

Abstract

Von Hippel-Lindau (VHL) disease is a dominantly inherited familial cancer syndrome with a variety of benign and malignant tumors such as retinal and central nervous system hemangioblastomas, endolymphatic sac tumors, renal cysts and tumors, pancreatic cysts and tumors, pheochromocytomas, and epididymal cystadenomas. Cross-sectional modalities (computed tomography and magnetic resonance imaging) as well as ultrasound play a major role in the initial evaluation and follow-up of the various manifestations of VHL disease. Ga-68-labeled somatostatin receptor analogs already have a significant role in the diagnosis, staging, and therapy management of neuroendocrine neoplasms and neural crest tumors. Herein, we report a case presenting a variety of malignancies in VHL and showing the usefulness of Ga-68 somatostatin receptor PET/CT as a one-stop-shop imaging modality in the management of VHL disease.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Subscribe and save

Springer+ Basic
EUR 32.99 /Month
  • Get 10 units per month
  • Download Article/Chapter or Ebook
  • 1 Unit = 1 Article or 1 Chapter
  • Cancel anytime
Subscribe now

Buy Now

Price includes VAT (United Kingdom)

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3

References

  1. Lonser RR, Glenn GM, Walther M, Chew EY, Libutti SK, Linehan WM, et al. von Hippel-Lindau disease. Lancet. 2003;361:2059–67.

    Article  PubMed  CAS  Google Scholar 

  2. Leung RS, Biswas SV, Duncan M, Rankin S. Imaging features of von Hippel-Lindau disease. Radiographics. 2008;28:65–79. quiz 323.

    Article  PubMed  Google Scholar 

  3. Davenport MS, Caoili EM, Cohan RH, Hoff CN, Ellis JH. Pancreatic manifestations of von Hippel-Lindau disease-effect of imaging on clinical management. J Comput Assist Tomogr. 2010;34:517–22.

    Article  PubMed  Google Scholar 

  4. Hes FJ, Feldberg MA. Von Hippel-Lindau disease: strategies in early detection (renal, adrenal, pancreatic masses). Eur Radiol. 1999;9:598–610.

    Article  PubMed  CAS  Google Scholar 

  5. Prasad V, Ambrosini V, Alavi A, Fanti S, Baum RP. PET/CT in neuroendocrine tumors: evaluation of receptor status and metabolism. PET Clin. 2007;2:351–75.

    Article  Google Scholar 

  6. Rufini V, Calcagni ML, Baum RP. Imaging of neuroendocrine tumors. Semin Nucl Med. 2008;36:228–47.

    Article  Google Scholar 

  7. Melmon KL, Rosen SW. Lindau's disease, review of literature and study of a large kindred. Am J Med. 1964;36:595–671.

    Article  PubMed  CAS  Google Scholar 

  8. Seizinger BR, Rouleau GA, Ozelius LJ, Lane AH, Farmer GE, Lamiell JM, et al. Von Hippel-Lindau disease maps to the region of chromosome 3 associated with renal cell carcinoma. Nature. 1988;332:268–9.

    Article  PubMed  CAS  Google Scholar 

  9. Latif F, Tory K, Gnarra J, Yao M, Duh FM, Orcutt ML, et al. Identification of vol Hippel-Lindau disease tumor suppressor gene. Science. 1993;260:1317–20.

    Article  PubMed  CAS  Google Scholar 

  10. Karsdorp N, Elderson A, Wittebol-Post D, Hene RJ, Vos J, Feldberg MA, et al. Von Hippel-Lindau disease: new strategies in early detection and treatment. Am J Med. 1994;97:158–68.

    Article  PubMed  CAS  Google Scholar 

  11. Marcos HB, Libutti SK, Alexander HR, Lubensky IA, Bartlett DL, Walther MM, et al. Neuroendocrine tumors of the pancreas in von Hippel-Lindau disease: spectrum of appearances at CT and MR imaging with histopathologic comparison. Radiology. 2002;225:751–8.

    Article  PubMed  Google Scholar 

  12. Woodward ER, Maher ER. Von Hippel-Lindau disease and endocrine tumour susceptibility. Endocr Relat Cancer. 2006;13:415–25.

    Article  PubMed  CAS  Google Scholar 

  13. Kaji P, Carrasquillo JA, Linehan WM, Chen CC, Eisenhofer G, Pinto PA, et al. The role of 6-[18F]fluorodopamine positron emission tomography in the localization of adrenal pheochromocytoma associated with von Hippel-Lindau syndrome. Eur J Endocrinol. 2007;156:483–7.

    Article  PubMed  CAS  Google Scholar 

  14. Kok J, Lin M, Wong V, Campbell P, Lin P. [18F]FDG PET/CT in pancreatic neuroendocrine tumours associated with von Hippel Lindau Syndrome. Clin Endocrinol (Oxf). 2009;70:657–9.

    Article  Google Scholar 

  15. Ambrosini V, Campana D, Allegri V, Opocher G, Fanti S. 68Ga-DOTA-NOC PET/CT detects somatostatin receptors expression in von Hippel-Lindau cerebellar disease. Clin Nucl Med. 2011;36:64–5.

    Article  PubMed  Google Scholar 

  16. Kroiss A, Putzer D, Uprimny C, Decristoforo C, Gabriel M, Santner W, et al. Functional imaging in phaeochromocytoma and neuroblastoma with 68Ga-DOTA-Tyr 3-octreotide positron emission tomography and 123I-metaiodobenzylguanidine. Eur J Nucl Med Mol Imaging. 2011;38:865–73.

    Article  PubMed  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Nuclear Medicine, Chonnam National University Hwasun Hospital, Hwasun, South Korea

    Jong-Ryool Oh & Jung-Joon Min

  2. Department of Nuclear Medicine and Center for PET/CT, Zentralklinik Bad Berka, 99437, Bad Berka, Germany

    Harshad Kulkarni, Cecilia Carreras, Georg Schalch & Richard P. Baum

Authors
  1. Jong-Ryool Oh
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Harshad Kulkarni
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Cecilia Carreras
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Georg Schalch
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Jung-Joon Min
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Richard P. Baum
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Richard P. Baum.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Oh, JR., Kulkarni, H., Carreras, C. et al. Ga-68 Somatostatin Receptor PET/CT in von Hippel-Lindau Disease. Nucl Med Mol Imaging 46, 129–133 (2012). https://doi.org/10.1007/s13139-012-0133-0

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s13139-012-0133-0

Keywords

Search

Navigation