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Adrenal hemangioblastoma.

ABSTRACT: Hemangioblastoma (HB) is a rare benign tumor that most commonly occurs in the cerebellum. HB is composed of neoplastic stromal cells and abundant small vessels. However, the exact origin of stromal cells is controversial. Extraneural HBs have been reported in a small series, and peripheral HBs arising in the adrenal gland are extremely rare. Herein, we report a case of sporadic adrenal HB in a 54-year-old woman. The tumor was a well-circumscribed, yellow mass measuring 4.2 cm in diameter. Histologically, the tumor was composed of small blood vessels and vacuolated stromal cells with clear cytoplasm. On immunohistochemical stain, the stromal cells were positive for S-100 protein, neuron-specific enolase, and synaptophysin. The tumor did not reveal mutation of VHL alleles. We herein present a case of HB of the adrenal gland and review of the literature.

SUBMITTER: Koo JY

PROVIDER: S-EPMC9119805 | biostudies-literature | 2022 May

REPOSITORIES: biostudies-literature

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Adrenal hemangioblastoma.

Koo Joo-Yeon JY Lee Kyung-Hwa KH Choi Joon Hyuk JH Chung Ho Seok HS Choi Chan C

Journal of pathology and translational medicine 20220228 3

Hemangioblastoma (HB) is a rare benign tumor that most commonly occurs in the cerebellum. HB is composed of neoplastic stromal cells and abundant small vessels. However, the exact origin of stromal cells is controversial. Extraneural HBs have been reported in a small series, and peripheral HBs arising in the adrenal gland are extremely rare. Herein, we report a case of sporadic adrenal HB in a 54-year-old woman. The tumor was a well-circumscribed, yellow mass measuring 4.2 cm in diameter. Histol ...[more]

PMID: 35209700

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Project description:BackgroundHemangioblastomas are benign (World Health Organization Grade I), highly vascular neoplasms commonly associated with Von Hippel-Lindau (VHL) disease.[2] The VHL tumor-suppressor gene, located on chromosome 3, is implicated in sporadic cases and cases associated with VHL disease. Hemangioblastomas most commonly arise in the posterior fossa; however, they may also be found supratentorially or within the spinal cord.[3] Surgical intervention is indicated for symptomatic lesions with a goal of complete resection of the enhancing nodule.[1].Case descriptionWe demonstrate the case of a 69-year-old man with a history of multiple hemangioblastomas who had undergone two previous craniotomies and Gamma-Knife radiosurgery (Video https://drive.google.com/file/d/1lUwsb80NLmIW2Enp-DVdtM9_Oqbid3Ih/view?usp=sharing). He presented with progressive imbalance and diplopia and was found to have a new lesion within the suprasellar cistern. Digital subtraction angiography (DSA) and magnetic resonance imaging (MRI) characteristics were typical of hemangioblastoma. Surgery was determined to be indicated, with a goal of vision preservation. Preoperative embolization was not possible because preoperative DSA demonstrated vascular supply by only small perforators directly from the internal carotid artery. Hypopituitarism was identified preoperatively, although diabetes insipidus was not present. The patient underwent a right orbitozygomatic craniotomy and extradural anterior clinoidectomy for access. The tumor was noted to encapsulate the infundibulum, which necessitated its sacrifice. Postoperatively, the patient remained at his neurologic baseline. He had a positive triphasic diabetes insipidus response and was discharged home on maintenance desmopressin. Postoperative MRI demonstrated complete lesion resection.The patient gave informed consent for treatment and video recording. Institutional review board approval was deemed unnecessary.ConclusionThis video highlights a safe and effective surgical technique for suprasellar lesions as well as the complex anatomy observed through an orbitozygomatic approach.

Dataset Information

Adrenal hemangioblastoma.

Publications

Adrenal hemangioblastoma.

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