A Rare Case of Clival Hemangioma Simulating Chordoma

Abstract

Primary intraosseous hemangiomas are rare, benign, vascular malformations that account for ∼1% of all primary bone neoplasias. A 59-year-old female patient with unknown comorbidities had a history of headache, visual impairment and dizziness that led to the diagnosis of a clivus tumor. Two resections were attempted through transcranial and transnasal transsphenoidal approach in the last two years in another hospital. The initial MRI scan showed an expansive lesion with T2 hyperintense signal and diffuse, heterogenic contrast enhancement. Clival chordoma was the main diagnostic hypothesis done. A CT scan was performed to evaluate the extent of clival invasion, the sinus anatomy, and the clival destruction - all simulating clival chordoma. The interdisciplinary tumor board decided to proceed with endoscopic endonasal tumor resection. There were no postoperative complications and the histopathological analysis revealed a primary intraosseous haemangioma. Skull base intraosseous hemangiomas are rare entities, with a limited number of case reports found after literature reviews, especially in the clival region. The clinical pattern and imaging characteristics can vary widely according to the tumor extension and development, simulating some other common tumors found at this topography. We present a case report of a clival intraosseous hemangioma presenting as an isolated abducens paresis with a positive outcome after intranasal endoscopic resection after two years of follow-up.

Resumo

Os hemangiomas intraósseos primários são malformações vasculares benignas raras que representam cerca de ∼1% de todas as neoplasias ósseas primárias. Uma paciente de 59 anos do sexo feminino com comorbidades desconhecidas história de cefaleia deficiência visual e tontura que levou ao diagnóstico de tumor de clivus. Duas ressecç ões foram tentadas por via transcraniana e transesfenoidal transnasal nos dois anos anteriores ao presente estudo em outro hospital. A ressonância magnética inicial mostrou lesão expansiva com sinal hiperintenso em T2 e realce difuso e heterogêneo pelo contraste. Cordoma clival foi a principal hipótese diagnóstica. Uma tomografia computadorizada foi realizada para avaliar a extensão da invasão clival a anatomia do seio e a destruiç ão clival - todas simulando cordoma clival. A comissão interdisciplinar do tumor decidiu prosseguir coma ressecç ão endoscópica do tumor endonasal. Não houve complicaç ões pós-operatórias e a análise histopatológica revelou hemangioma intraósseo primário. Os hemangiomas intraósseos da base do crânio são entidades raras com número limitado de relatos de casos encontrados após revisões da literatura principalmente na região clival. O padrão clínico e as características de imagem podem variar amplamente de acordo com a extensão e desenvolvimento do tumor simulando alguns outros tumores comuns encontrados nesta topografia. Apresentamos um relato de caso de hemangioma clival intraósseo apresentando-se como uma paresia isolada do abducente com evoluç ão positive após ressecç ão endoscópica intranasal e dois anos de acompanhamento.

Keywords

chordoma - hemangioma - neurosurgery - tumor

Palavras-chave

cordoma - hemangioma - neurocirurgia - tumor

Note

This article was developed in collaboration between the departments of Neurosurgery from the Federal University of Rio Grande do Sul (Porto Alegre, RS, Brazil), and the University of Sao Paulo (São Paulo, SP, Brazil).

Disclosure

The authors have no disclosures.

Publication History

Received: 09 October 2020

Accepted: 09 March 2021

Article published online:
02 May 2024

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