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Adrenal hemangioblastoma.

Joo-Yeon Koo1, Kyung-Hwa Lee1, Joon Hyuk Choi2

  • 1Department of Pathology, Chonnam National University Research Institute of Medical Science, Bio-Medical Sciences Graduate Program (BMSGP), Chonnam National University Hwasun Hospital and Medical School, Hwasun, Korea.

Journal of Pathology and Translational Medicine
|February 25, 2022
PubMed
Summary
This summary is machine-generated.

This study reports an extremely rare case of adrenal hemangioblastoma (HB) in a 54-year-old woman. The findings contribute to understanding rare peripheral hemangioblastomas.

Keywords:
Adrenal glandsHemangioblastomaImmunohistochemistrySequencingvon Hippel-Lindau disease

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Area of Science:

  • Oncology
  • Pathology
  • Endocrinology

Background:

  • Hemangioblastoma (HB) is a rare, typically benign tumor predominantly found in the cerebellum.
  • The precise origin of HB stromal cells remains a subject of debate.
  • Extraneural and peripheral HBs are exceptionally uncommon, with adrenal gland involvement being exceedingly rare.

Purpose of the Study:

  • To document and characterize an extremely rare case of sporadic hemangioblastoma originating in the adrenal gland.
  • To contribute to the limited literature on peripheral hemangioblastomas.
  • To provide histopathological and immunohistochemical details of an adrenal hemangioblastoma.

Main Methods:

  • Case report of a 54-year-old woman with an adrenal mass.
  • Gross pathological examination of the tumor.
  • Histological analysis including immunohistochemical staining (S-100 protein, neuron-specific enolase, synaptophysin).
  • Genetic analysis for VHL (von Hippel-Lindau) gene mutations.

Main Results:

  • A 4.2 cm well-circumscribed, yellow adrenal mass was identified.
  • Histology revealed small blood vessels and vacuolated stromal cells with clear cytoplasm.
  • Immunohistochemistry showed stromal cell positivity for S-100 protein, neuron-specific enolase, and synaptophysin.
  • No VHL allele mutations were detected in the tumor.

Conclusions:

  • This case represents one of the rare instances of hemangioblastoma occurring in the adrenal gland.
  • The immunohistochemical profile supports the neoplastic nature of the stromal cells.
  • The absence of VHL mutations suggests a sporadic origin for this adrenal hemangioblastoma.
  • Further research is needed to elucidate the origins of peripheral hemangioblastomas.