Dataset Information

Submacular sclerosing capillary hemangioblastoma.

ABSTRACT:

Purpose

To report a case of submacular cystic lesion that turned out to be a sclerosing capillary hemangioblastoma. The retinal capillary hemangioblastoma may arise as a part of von Hippel-Lindau syndrome ¹ however, they may occur as an isolated entity ², but submacular capillary hemangioblastoma has never been reported.

Observations

A case of a 56-year-old female who presented with a right yellow submacular lesion. The mass was excised via pars-plana vitrectomy and histopathological assessment revealed a submacular sclerosing capillary hemangioblastoma.

Conclusion

and importance: The capillary hemangioblastoma is a benign vascular tumor that may arise submacularly and is not necessarily associated with von Hippel-Lindau disease. It is difficult to be differentiated clinically from other submacular lesions but the possibility should be considered.

SUBMITTER: Hamza HS

PROVIDER: S-EPMC6040628 | biostudies-literature | 2018 Sep

REPOSITORIES: biostudies-literature

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Publications

Submacular sclerosing capillary hemangioblastoma.

Hamza Hany S HS Elhusseiny Abdelrahman M AM

American journal of ophthalmology case reports 20180602

<h4>Purpose</h4>To report a case of submacular cystic lesion that turned out to be a sclerosing capillary hemangioblastoma. The retinal capillary hemangioblastoma may arise as a part of von Hippel-Lindau syndrome <sup>1</sup> however, they may occur as an isolated entity <sup>2</sup>, but submacular capillary hemangioblastoma has never been reported.<h4>Observations</h4>A case of a 56-year-old female who presented with a right yellow submacular lesion. The mass was excised via pars-plana vitrect ...[more]

PMID: 30003174

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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

Submacular sclerosing capillary hemangioblastoma.

Purpose

Observations

Conclusion

Publications

Submacular sclerosing capillary hemangioblastoma.

Similar Datasets

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