Multiple schwannomas: schwannomatosis or neurofibromatosis type 2?

Seppälä, Matti; Sainio, Markku; Haltia, Matti; Kinnunen, J.; Setälä, Kai; Jääskeläinen, Juha E.

doi:10.3171/jns.1998.89.1.0036

Cited by 83 publications

(65 citation statements)

References 22 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Most patients with schwannomatosis (81.5%) underwent a single spinal operation; other patients (18.5%) underwent multiple spinal operations. These variations in schwannomatosis have also been reported in several previous studies; 13,33 some patients may develop new symptomatic tumors every few years, while others do not. In our cohort, patients with nonsegmental schwannomatosis or patients with early onset appeared to have a poor prognosis and were more likely to undergo multiple spinal operations.…”

Section: Discussionmentioning

confidence: 58%

“…Patients with schwannomatosis appeared to have a similar clinical course as patients with solitary schwannomas. Chen et al 6 and Seppälä et al 33 also found that patients with schwannomatosis were similar in age at operation as patients with solitary schwannomas. However, Gonzalvo et al 13 found that the patient age at onset of schwannomatosis tended to be younger than that of solitary schwannomas.…”

Section: Discussionmentioning

confidence: 89%

“…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.…”

mentioning

confidence: 99%

See 2 more Smart Citations

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

View full text Add to dashboard Cite

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.

show abstract

Section: Discussionmentioning

confidence: 58%

Section: Discussionmentioning

confidence: 89%

mentioning

confidence: 99%

See 1 more Smart Citation

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

View full text Add to dashboard Cite

show abstract

“…Patients diagnosed with schwannomatosis constitute a small fraction of all patients treated surgically for schwannomas. In material presented by Seppälä et al patients with schwannomatosis accounted for 3.7% and in materi al by Huang for 4.6% of all patients who underwent operative treatment [9,28]. In material presented by Wang et al 5 cases of segmental schwannomatosis constituted 1.4% of all treated patients [39].…”

Section: Discussionmentioning

confidence: 99%

Case report Multiple schwannomas of the digital nerves and superficial radial nerve: two unusual cases of segmental schwannomatosis

Gosk

Gutkowska²,

Kuliński³

et al. 2015

View full text Add to dashboard Cite

show abstract

“…When multiple schwannomas are present without any other stigmata of neurofibromatosis, then it can be defined as schwannomatosis. 8 …”

Section: Discussionmentioning

confidence: 99%

Cervical Vagal Schwannoma- A Rare Differential Diagnosis of Benign Solid Neck Mass

Haldar¹,

Khondaker²,

Chattopadhyay³

2018

jemds

View full text Add to dashboard Cite

Multiple schwannomas: schwannomatosis or neurofibromatosis type 2?

Abstract: The clinical course, long-term outcome, and genetic mechanism of schwannomatosis differ from that of NF2.

Cited by 83 publications

References 22 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

Case report Multiple schwannomas of the digital nerves and superficial radial nerve: two unusual cases of segmental schwannomatosis

Cervical Vagal Schwannoma- A Rare Differential Diagnosis of Benign Solid Neck Mass

Contact Info

Product

Resources

About