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Characterization of spinal hemangioblastomas in patients with and without von Hippel-Lindau, and YAP expression.
Calderón-Garcidueñas, Ana-Laura; Piña-Ballantyne, Steven-Andrés; Espinosa-Aguilar, Eunice-Jazmín; de Jesús Ramos-Sánchez, Rebeca.
Afiliación
  • Calderón-Garcidueñas AL; Neuropathology Department, Instituto Nacional de Neurología y Neurocirugía Manuel Velasco Suárez, 14269 Mexico City, Mexico. Electronic address: ana.calderon@innn.edu.mx.
  • Piña-Ballantyne SA; Neuropathology Department, Instituto Nacional de Neurología y Neurocirugía Manuel Velasco Suárez, 14269 Mexico City, Mexico.
  • Espinosa-Aguilar EJ; Internal Medicine Department, Clínica-Hospital Mérida, 97314 Yucatan, Mexico.
  • de Jesús Ramos-Sánchez R; Neuroradiology Department, Instituto Nacional de Neurología y Neurocirugía Manuel Velasco Suárez, 14269 Mexico City, Mexico.
Rev Esp Patol ; 57(3): 160-168, 2024.
Article en En | MEDLINE | ID: mdl-38971615
ABSTRACT

INTRODUCTION:

Hemangioblastoma (HB) is a benign tumor of the central nervous system, associated with von Hippel-Lindau disease (VHL), or sporadic. The aim of this study was to compare and examine the clinical-pathological profile of patients with spinal hemangioblastoma and YAP expression.

METHODS:

A retrospective, descriptive, comparative study. All patients who underwent surgery for spinal HB between 2016 and 2023 were included. Clinical and radiological data were collected and analyzed. An immunohistochemistry panel including NeuN, neurofilaments (NF), and YAP-1, was performed.

RESULTS:

Nine patients were studied, six women and three men. Four patients had previously diagnosed VHL. The tumor location included four cervical (44.44%), two thoracic (22.22%), two pontine with cervical extension (22.22%) and one patient with two lesions, one cervical and one thoracic (11.11%). Non-significant clinical differences were identified between VHL and sporadic patients. Imaging evidenced seven extramedullary and three intramedullary tumors. Histologically, intra-tumoral and perivascular axonal tracts were observed in all cases. One third of the tumors (two with VHL and one sporadic) presented extramedullary hematopoiesis. Seven cases (77.8%) expressed nuclear YAP (three with VHL and four sporadic HBs). The surgical outcome was good and only one patient with VHL undergoing subtotal resection had recurrence.

CONCLUSIONS:

Spinal HBs can be associated with VHL or be sporadic. To the best of our knowledge, this is the first study to describe YAP expression in HB. It is important to investigate the involvement of the Hippo pathway in HBs as a possible therapeutic target.
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Factores de Transcripción / Hemangioblastoma / Proteínas Señalizadoras YAP / Enfermedad de von Hippel-Lindau Límite: Adult / Aged / Female / Humans / Male / Middle aged Idioma: En Revista: Rev Esp Patol Año: 2024 Tipo del documento: Article Pais de publicación: España
Texto completo
Añadir a Mi BVS
Imprimir
XML
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Factores de Transcripción / Hemangioblastoma / Proteínas Señalizadoras YAP / Enfermedad de von Hippel-Lindau Límite: Adult / Aged / Female / Humans / Male / Middle aged Idioma: En Revista: Rev Esp Patol Año: 2024 Tipo del documento: Article Pais de publicación: España