Annals of medicine and surgery (2012) · 2024
A rare case of schwannomatosis with meningioma: a case report.
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Neupane Saurav, Kashyap Ashutosh, Paudel Shreeram, Bhattarai Ganesh, Kharel Santosh K, Adhikari Asim, Ghimire Ashim, Pokharel Simankan, Yadav Pratibha, Yadav Krishna K
Аннотация
INTRODUCTION: Schwannomatosis is characterized by multiple schwannomas without vestibular schwannomas or any other stigmata of neurofibromatosis type 2 (NF2). Schwannomatosis is a rare disorder, with a reported incidence ranging from 1 in 40 000 to 1 in 1.7 million. Meningioma is also associated with schwannomatosis in around 5% of cases.
CASE PRESENTATION: We describe a case of a 20-year-old female presenting with progressive weakness of the right lower limb for 7 months with a tingling sensation and numbness of the same limb for 6 months and was found to have schwannomatosis with multiple spinal and right cerebellopontine angle (CPA) (9th/10th cranial nerve) schwannomas and left anterior cranial fossa meningioma.
DISCUSSION: Schwannomas in schwannomatosis are seen along the cranial, spinal, and peripheral nerves but not along the vestibular nerve, as is characteristically seen in NF2. The occurrence of meningiomas is about 5% in individuals with schwannomatosis, and the patient in our case also had an associated meningioma. The tumor was confirmed to be a schwannoma based on features on an MRI examination and histological examination.
CONCLUSION: It is of great significance to identify the entire spectrum of the disease in a patient with schwannomatosis, and to differentiate it from related conditions in order to track and surgically manage the patient appropriately based on symptomatology and imaging findings.
CASE PRESENTATION: We describe a case of a 20-year-old female presenting with progressive weakness of the right lower limb for 7 months with a tingling sensation and numbness of the same limb for 6 months and was found to have schwannomatosis with multiple spinal and right cerebellopontine angle (CPA) (9th/10th cranial nerve) schwannomas and left anterior cranial fossa meningioma.
DISCUSSION: Schwannomas in schwannomatosis are seen along the cranial, spinal, and peripheral nerves but not along the vestibular nerve, as is characteristically seen in NF2. The occurrence of meningiomas is about 5% in individuals with schwannomatosis, and the patient in our case also had an associated meningioma. The tumor was confirmed to be a schwannoma based on features on an MRI examination and histological examination.
CONCLUSION: It is of great significance to identify the entire spectrum of the disease in a patient with schwannomatosis, and to differentiate it from related conditions in order to track and surgically manage the patient appropriately based on symptomatology and imaging findings.