Intramedullary spinal cord tumors and neurofibromatosis

Kushel, Yu V; Belova, Yu D; Tekoev, A R

doi:10.17116/neiro201780770-73

Cited by 5 publications

(4 citation statements)

References 6 publications

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“…Conti et al stated that IMS associates with NF; however, several studies showed a prevalence of 0-2% in spinal tumours [7,70,80,103,125]. Our review found NF in 11 of 166 cases (6.6%).…”

Section: Discussionmentioning

confidence: 44%

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Spinal intramedullary schwannomas—report of a case and extensive review of the literature

et al. 2020

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Intramedullary schwannomas (IMS) represent exceptional rare pathologies. They commonly present as solitary lesions; only five cases of multiple IMS have been described so far. Here, we report the sixth case of a woman with multiple IMS. Additionally, we performed the first complete systematic review of the literature for all cases reporting IMS. We performed a systematic review of the literature in PubMed, EMBASE and Cochrane Central Register of Controlled (CENTRAL) to retrieve all relevant studies and case reports on IMS. In a second step, we analysed all reported studies with respect to additional cases, which were not identified through the database search. Studies published in other languages than English were included. One hundred nineteen studies including 165 reported cases were included. In only five cases, the patients harboured more than one IMS. Gender ratio showed a ratio of nearly 3:2 (male:female); mean age of disease presentation was 40.2 years; 11 patients suffered from neurofibromatosis (NF) type 1 or 2 (6.6%). IMS are rare. Our first systematic review on this pathology revealed 166 cases, including the here reported case of multiple IMS. Our review offers a basis for further investigation on this disease.

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“…Conti et al stated that IMS associates with NF; however, several studies showed a prevalence of 0-2% in spinal tumours [7,70,80,103,125]. Our review found NF in 11 of 166 cases (6.6%).…”

Section: Discussionmentioning

confidence: 44%

“…Furthermore, they commonly present as solitary lesions. To date, only five cases of multiple intramedullary schwannomas (IMS) have been described [5][6][7][8][9].…”

Section: Introductionmentioning

confidence: 99%

Spinal intramedullary schwannomas—report of a case and extensive review of the literature

et al. 2020

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“…Genetic factors correlate with ITs. The syndromes associated with these tumors are neurofibromatosis 1 (NF1, 19% of patients), 2 (NF2, 33-53% of patients), and Von Hippel Lindau disease (VHL) [4,5]. These patients develop mostly astrocytoma in case of NF1, ependymoma in NF2, and hemangioblastoma in VHL.…”

Section: • Metastatic Tumorsmentioning

confidence: 99%

Intramedullary Spinal Tumors

Capo¹,

Vandenbulcke²,

Barrey³

2023

Central Nervous System Tumors - Primary and Secondary

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Intramedullary spinal tumors are uncommon intra-axial lesions, which can be either primary or metastatic. Primary tumors arise from cell of spinal cord and account for 2–4% of all intrinsic tumors of the central nervous system, being much less common of brain tumors. They are slow-growing tumors, so symptoms precede diagnosis by an average of 2 years. Metastatic lesions usually originate from lung and breast tumors and are usually diagnosed within 1 month from symptom onset. Pain and weakness are the most common presenting symptoms. Magnetic resonance imaging represents the gold standard technique to study the spinal cord tumors, and first-line treatment is surgical resection, but it is not always curative. In selected situations, watchful waiting can be considered. Chemotherapy and radiation are considered, but controversy exists. Novel treatment options must be developed to supplement partial resection and recurrence.

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“…Though rare, other histologies have been reported in NF1, such as gangliogliomas, primitive neuroectodermal tumors (PNETs), anaplastic astrocytomas, and even neurinomas have been reported anecdotally. [32][33][34] The surgical treatment for IMSCT consists in resection of as much tumor as possible guided by neurophysiological monitoring, to avoid further neurological deficits. Ependymomas and some pilocytic astrocytomas may be totally resected in some cases of focal and circumscribed tumors.…”

Section: Intramedullary Tumorsmentioning

confidence: 99%

Spinal Surgery in Patients with Type-1 Neurofibromatosis: A Comprehensive Review

Joaquim

2023

Arquivos Brasileiros de Neurocirurgia: Brazilian Neurosurgery

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Type-1 neurofibromatosis (NF1) is a neurocutaneous syndrome classically known as peripheral NF to distinguish it from type-2 NF (central NF). Its main characteristic is the high predisposition to the growth of multiple tumors, which specially arouses the interest of spinal surgeons due to the presence of spinal cord compression and spinal deformities.Considering this, we have performed a comprehensive review, with illustrative cases of the main manifestations of NF1, focusing on the perspective of the spine surgeon. Articles were grouped according to the following subjects: diagnosis, skeletal complications, spinal deformity, and spinal tumors. For all of them, a detailed discussion on pearls for practice was presented.The diagnosis of NF1 is based on the presence of at least two out of seven criteria. Cutaneous findings are very common in NF1, and the most usual tumor is cutaneous neurofibroma (NFB). Plexiform neurofibromas are also found and present a high risk of becoming malignant peripheral nerve sheath tumors (MPNSTs), reducing life expectancy. Astrocytomas, especially pilocytic astrocytomas, are the most common central nervous system tumor, including in the spinal cord. Surgery is necessary to resect as much as possible without adding new neurological deficits. Spinal deformities are also commonly found (in 30–70% of the cases), potentially associated with dystrophic changes, which may result in acute and rapid progression.In the present review, we discuss specific characteristics found in this group of patients which are of paramount importance to properly manage this challenging disease.

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Intramedullary spinal cord tumors and neurofibromatosis

Cited by 5 publications

References 6 publications

Spinal intramedullary schwannomas—report of a case and extensive review of the literature

Spinal intramedullary schwannomas—report of a case and extensive review of the literature

Intramedullary Spinal Tumors

Spinal Surgery in Patients with Type-1 Neurofibromatosis: A Comprehensive Review

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