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Posterior fossa melanocytic schwannoma extending to the cervicothoracic spinal cord: A clinical rarity

BACKGROUND: Melanocytic schwannomas (MSs) are rare, malignant peripheral nerve sheath tumors with only 200 cases reported to date. These pose imaging and pathological challenges for definitive diagnosis. CASE DESCRIPTION: A 25-year-old lady presented at our center with a prolonged history of gait di...

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Detalles Bibliográficos
Autores principales: Fazal, Zoha Zahid, Kazmi, Syeda Maryam, Bajwa, Mohammad Hamza, Khan, Ahsan Ali
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Scientific Scholar 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9479506/
https://www.ncbi.nlm.nih.gov/pubmed/36128142
http://dx.doi.org/10.25259/SNI_183_2022
Descripción
Sumario:BACKGROUND: Melanocytic schwannomas (MSs) are rare, malignant peripheral nerve sheath tumors with only 200 cases reported to date. These pose imaging and pathological challenges for definitive diagnosis. CASE DESCRIPTION: A 25-year-old lady presented at our center with a prolonged history of gait disturbance, left ear tinnitus, headaches, and drowsiness. MRI findings showed a midline cystic lesion in the posterior cranial fossa extending caudally to the D1 vertebral body, with marked central hypointensity, and peripheral hyperintensity on T1-weighted images. A suboccipital craniotomy and debulking of the lesion were performed, showing a hyperpigmented, infiltrative tumor adherent to the surrounding structures. This was confirmed as a melanocytic schwannoma on histopathological analysis. CONCLUSION: Posterior fossa MSs involving cervicomedullary region and extending distally to cervicothoracic spinal cord are rare and complex cases, particularly with regard to difficulty diagnosing preoperatively and surgical resection.