Spinal schwannomatosis in the absence of neurofibromatosis: A very rare condition

Landi, Alessandro; D, De Santis; Marotta, Nicola; Mancarella, Cristina; Delfini, Roberto

doi:10.1016/j.ijscr.2010.12.001

Cited by 17 publications

(13 citation statements)

References 11 publications

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“…Although spinal schwannomas are common in schwannomatosis (74% as reported 26 ), and they are more likely to cause functional disabilities than peripheral schwannomas, their clinical features have not been fully studied except for several cases or small series reports. 8,13,15,22,33 In this report, we first describe the detailed clinical features of spinal schwannomas in schwannomatosis and compare them with solitary schwannomas and NF2 in a large cohort of patients.…”

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confidence: 99%

Clinical features of spinal schwannomas in 65 patients with schwannomatosis compared with 831 with solitary schwannomas and 102 with neurofibromatosis Type 2: a retrospective study at a single institution

Zhao

Zhang

et al. 2016

SPI

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OBJECT The aim of this study was to evaluate the clinical features of spinal schwannomas in patients with schwannomatosis and compare them with a large cohort of patients with solitary schwannomas and neurofibromatosis Type 2 (NF2). METHODS The study was a retrospective review of 831 patients with solitary schwannomas, 65 with schwannomatosis, and 102 with NF2. The clinical, radiographic, and pathological data were extracted with specific attention to the age at onset, location of tumors, initial symptoms, family history, and treatment outcome. RESULTS The male-to-female ratio of patients with schwannomatosis (72.3% vs 27.7%) was significantly higher than that of patients with solitary schwannomas (53.3% vs 46.7%) and NF2 (54.0% vs 46.0%), respectively (chi-square test, p = 0.012). The mean age at the first spinal schwannoma operation of patients with NF2 (24.7 ± 10.2 years) was significantly younger than that of patients with solitary schwannomas (44.8 ± 13.2 years) and schwannomatosis (44.4 ± 14.1 years; 1-way ANOVA, p < 0.001). The initial symptoms were similar among the 3 groups, with pain being the most common. The distribution of spinal tumors among the 3 groups was significantly different. The peak locations of spinal schwannomas in patients with solitary schwannomas were at C1–3 and T12–L3; in schwannomatosis, the peak location was at T12-L5. A preferred spinal location was not evident for intradural-extramedullary tumors in NF2. Only a slight prominence in the lumbar area could be observed. The patients in the 3 groups obtained similar benefits from the operation; the recovery rates in the patients with solitary schwannomas, NF2, and schwannomatosis were 50.1%, 38.0%, and 53.9%, respectively. The prognosis varied among spinal schwannomas in the patients with schwannomatosis. Up until the last date of follow-up, most patients with schwannomatosis (81.5%) had undergone a single spinal operation, but 12 patients (18.5%) had undergone multiple spinal operations. Patients with nonsegmental schwannomatosis or those with early onset disease seemed to have a poor prognosis; they were more likely to undergo multiple spinal operations. Small cauda equina nodules were common in patients with schwannomatosis (46.7%) and NF2 (86.9%); these small schwannomas appeared to have relatively static behavior. Two patients suspicious for schwannomatosis were diagnosed with NF2 with the detection of constitutional NF2 mutations; 1 had unilateral vestibular schwannoma, and the other had suspicious bilateral trigeminal schwannomas. CONCLUSIONS The clinical features of spinal schwannomas vary among patients with solitary schwannomas, NF2, and schwannomatosis. Spinal schwannomas of patients with NF2 appear to be more aggressive than those in patients with solitary schwannomas and schwannomatosis. Spinal schwannomas of schwannomatosis predominate in the lumbar area, and most of them can be treated successfully with surgery. The prognosis varies among spinal schwannomas of schwannomatosis; some patients may need multiple operations due to newly developed schwannomas. Sometimes, it is difficult to differentiate schwannomatosis from NF2 based on clinical manifestations. It is prudent to perform close follow-up examinations in patients with undetermined schwannomatosis and their offspring.

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Clinical features of spinal schwannomas in 65 patients with schwannomatosis compared with 831 with solitary schwannomas and 102 with neurofibromatosis Type 2: a retrospective study at a single institution

Zhao

Zhang

et al. 2016

SPI

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“…1). Regarding the level of evidence, the nine case reports [8][9][10][11][12][13][14][15][16] were categorized as level 4, and the six case series were categorized as level 3, 3,4,17 4, 18 or 5. 19,20 Thirty-eight individuals met the inclusion criteria for sporadic schwannomatosis (►Table 2).…”

Section: Resultsmentioning

confidence: 99%

Sporadic Schwannomatosis: A Systematic Review Following the 2005 Consensus Statement

Chick

Victor

Poujade

et al. 2018

J Neurol Surg A Cent Eur Neurosurg

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This systematic review quantified the number of individuals who meet the criteria for sporadic schwannomatosis and better described this population to facilitate the classification of neurofibromatosis in regard to the 2005 consensus statement. Unilateral or segmental distribution of nerve tumors are key aspects when dealing with multiple noncutaneous schwannomas without involvement of the vestibular nerve.

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“…Surgery is indicated for symptomatic lesions [9]. Excision of painful tumours provides satisfactory results with improvement of neurological status and histological diagnosis [10][11][12]. The first report of multiple spinal schwannomas in the absence of neurofibromatosis was done by Daras et al [5].…”

Section: Discussionmentioning

confidence: 99%

“…Then there were reports of multiple spinal schwannomas without neurofibromatosis in lumbar and sacral vertebrae, in cervical and lumbar vertebrae, and in the cauda equina [2,6,15,16]. There are 2 reports of multiple schwannomas in the dorsal and lumbar spine without neurofibromatosis: one at the and the other at the D11 and L1 levels [10,12]. The case described here is the third such report of multiple schwannomas in the dorso-lumbar region, with two discrete lesions, one at the D12-L2 level and the other at the L3-L5 level, without any intervening connection (Tab.…”

Section: Discussionmentioning

confidence: 99%

Segmental Schwannomatosis of the Spine: Report of a Rare Case and Brief Review of Literature

Baruah

Bora

Haque

2016

Ortop Traumatol Rehabil

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Spinal schwannomatosis in the absence of neurofibromatosis: A very rare condition

Cited by 17 publications

References 11 publications

Clinical features of spinal schwannomas in 65 patients with schwannomatosis compared with 831 with solitary schwannomas and 102 with neurofibromatosis Type 2: a retrospective study at a single institution

Clinical features of spinal schwannomas in 65 patients with schwannomatosis compared with 831 with solitary schwannomas and 102 with neurofibromatosis Type 2: a retrospective study at a single institution

Sporadic Schwannomatosis: A Systematic Review Following the 2005 Consensus Statement

Segmental Schwannomatosis of the Spine: Report of a Rare Case and Brief Review of Literature

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