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- Title
Endolymphatic sac tumor: An urgent case presenting acute intracranial hypertension successfully treated with suboccipital decompressive craniectomy - 8 years of follow-up.
- Authors
Gonçalves Bastos Torres, João Antonio; Costa Ferreira-Pinto, Pedro Henrique; Coelho de Oliveira Ferreira, Domênica Baroni; Lannes Simões, Elington; Gonçalves de Carvalho, Felipe; Gonçalves Brito, Ana Carolina; Almeida Filho, José Alberto; Cavalcante de Sousa, Bruna; Viveiros de Castro, Maria Eduarda; Ribeiro Carvalho de Gouvea, Pedro Luiz; Novaes da Silva, Wellerson; Mendes Correa, Eduardo; Zanon Cruz, Thainá; Nigri, Flavio
- Abstract
Background: Endolymphatic sac tumor (ELST) is a rare lesion. It may be sporadically or associated with Von Hippel-Lindau syndrome. Progressive audiovestibular symptoms characterize the typical clinical presentation. Here, we report a unique case of ELST with acute intracranial hypertension (IH) due to tumor compression, successfully treated with an urgent suboccipital decompressive craniectomy (SDC). Case Description: A 33-year-old woman previously underwent a biopsy and ventriculoperitoneal shunt. The histopathological finding revealed an ELST. One year later, she developed headache, vomiting, and somnolence due to brainstem compression. An urgent SDC was performed. One month later, preoperative endovascular embolization and partial tumor resection were carried out. After 6 months adjuvant radiotherapy (RT) therapy was administered. She has been under follow-up for 8 years since the last surgical procedure, and the tumor remains stable. Conclusion: ELST generally has a progressive clinical course. This is a unique case with acute IH due to tumor compression. The tumor's high vascularity and the unavailability of endovascular embolization precluded its resection. SDC was an alternative approach. The final treatment included tumor embolization, surgical resection, and RT. No progression was observed for 8 years after the last procedure, and long-term follow-up is warranted.
- Subjects
VON Hippel-Lindau disease; DECOMPRESSIVE craniectomy; INTRACRANIAL tumors; TUMOR surgery; CEREBROSPINAL fluid shunts
- Publication
Surgical Neurology International, 2024, Vol 15, p1
- ISSN
2229-5097
- Publication type
Article
- DOI
10.25259/SNI_34_2024