Journal article
Expression of somatostatin receptors in hemangioblastomas associated with von Hippel-Lindau disease as a novel diagnostic, therapeutic, and follow-up opportunity: A case report and literature review
Archives of endocrinology and metabolism, Vol.68, p.e230181
05/17/2024
DOI: 10.20945/2359-4292-2023-0181
PMID: 38788146
Abstract
Hemangioblastomas associated with von Hippel-Lindau (VHL) disease are frequently multiple and recur during prolonged follow-up. Currently, no systemic treatment is available for these tumors. Recent studies have shown the expression of somatostatin receptors in these types of hemangioblastomas. Notably, increased somatostatin receptor expression in a tumor, as determined by peptide-receptor radionuclide imaging, is a predictive factor of response to treatment with somatostatin analogs and peptide-receptor radionuclide therapy. The aim of this study was to describe the case of a patient with increased expression of somatostatin receptors in a suprasellar hemangioblastoma associated with VHL disease and conduct a literature review on somatostatin receptor expression in patients with VHL-associated hemangioblastomas. We describe herein the case of a 51-year-old man with VHL disease who had a suprasellar hemangioblastoma detected on magnetic resonance imaging. Peptide-receptor radionuclide imaging using gallium-68-DOTATOC (68Ga-DOTATOC) identified increased expression of somatostatin receptors in the suprasellar hemangioblastoma, along with multiple pancreatic neuroendocrine tumors and bilateral pheochromocytomas. The patient was treated for 1 year with lanreotide, a somatostatin analog. A repeat 68Ga-DOTATOC 1 year after starting lanreotide revealed decreased radiotracer uptake by the hemangioblastoma, consistent with a metabolic response. The presence of somatostatin receptors in hemangioblastomas associated with VHL disease is a novel finding. The decreased expression of these receptors after treatment with a somatostatin analog, as described in the present case, positions the somatostatin receptor as a new target for novel diagnostic, therapeutic, and follow-up opportunities in patients with VHL disease.
Details
- Title: Subtitle
- Expression of somatostatin receptors in hemangioblastomas associated with von Hippel-Lindau disease as a novel diagnostic, therapeutic, and follow-up opportunity: A case report and literature review
- Creators
- Eloá Pereira Brabo - D’Or Institute for Research and EducationSergio Altino de Almeida - Instituto D'Or de Pesquisa e Ensino Departamento de Radiologia Departamento de Radiologia, Instituto D'Or de Pesquisa e Ensino (IDOR), Rio de Janeiro, RJ, BrasilPatrícia Piazza Rafful - D’Or Institute for Research and EducationPaulo Henrique Rosado-de-Castro - Universidade Federal do Rio de JaneiroLeonardo Vieira - Hospital Universitário Clementino Fraga Filho
- Resource Type
- Journal article
- Publication Details
- Archives of endocrinology and metabolism, Vol.68, p.e230181
- DOI
- 10.20945/2359-4292-2023-0181
- PMID
- 38788146
- NLM abbreviation
- Arch Endocrinol Metab
- ISSN
- 2359-3997
- eISSN
- 2359-4292
- Publisher
- Sociedade Brasileira de Endocrinologia e Metabologia
- Language
- English
- Date published
- 05/17/2024
- Academic Unit
- Radiology
- Record Identifier
- 9985143124102771
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