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  11. 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

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

Eloá Pereira Brabo1,2, Sergio Altino de Almeida3, Patrícia Piazza Rafful4,5

  • 1Serviço de Oncologia, Hospital Universitário Clementino Fraga Filho, Universidade Federal do Rio de Janeiro, Rio de Janeiro, RJ, Brasil.

Archives of Endocrinology and Metabolism
|May 24, 2024

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View abstract on PubMed

Summary
This summary is machine-generated.

Von Hippel-Lindau (VHL) disease patients with hemangioblastomas show somatostatin receptor expression. Treatment with somatostatin analogs reduced receptor expression, indicating a new therapeutic target for VHL-associated tumors.

Area of Science:

  • Endocrinology
  • Oncology
  • Radiology

Background:

  • Von Hippel-Lindau (VHL) disease frequently causes multiple, recurring hemangioblastomas for which no systemic treatment exists.
  • Recent research indicates somatostatin receptor expression in VHL-associated hemangioblastomas.

Observation:

  • A 51-year-old man with VHL disease presented with a suprasellar hemangioblastoma.
  • Peptide-receptor radionuclide imaging (68Ga-DOTATOC) revealed increased somatostatin receptor expression in the hemangioblastoma, alongside other VHL-related tumors.
  • The patient received a year of lanreotide treatment, a somatostatin analog.

Findings:

  • Post-treatment imaging showed decreased radiotracer uptake in the hemangioblastoma, signifying a metabolic response.
  • This indicates that somatostatin receptors are present in VHL-associated hemangioblastomas and their expression can be modulated by treatment.

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Implications:

  • Somatostatin receptors represent a novel diagnostic and therapeutic target for hemangioblastomas in patients with VHL disease.
  • This finding opens new avenues for managing VHL disease, potentially improving patient outcomes through targeted therapies.