Intraosseous schwannoma in schwannomatosis

Kashima, Takeshi; Gibbons, M R J P; Whitwell, Duncan; Gibbons, C. L. M. H.; Bradley, Kevin M.; Ostlere, S; Athanasou, Nicholas A.

doi:10.1007/s00256-013-1712-6

Cited by 15 publications

(13 citation statements)

References 14 publications

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“…If the types of mutations are different, NF2 can be ruled out. 20 Detecting mutations of SMARCB1 and LZTR1 can also help with the diagnosis of schwannomatosis. Additionally, it is very difficult to definitively diagnose 1 patient with schwannomatosis.…”

Section: Discussionmentioning

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

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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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Section: Discussionmentioning

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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“…Tumor growth is slow and insidious, with long periods of evolution. [1][2][3] Radiographically, these tumors manifest as well-circumscribed lytic lesions, possibly with a sclerotic halo, which sometimes tapers or destroys the cortical layer. Adjacent soft tissues are rarely invaded.…”

Section: Discussionmentioning

confidence: 99%

“…There is no calcification or bone tissue formation within the lesion. [1][2][3] Nuclear magnetic resonance is a better imaging method to assess cortical and soft tissue involvement when compared with CT. Schwannoma lesions present hypointense signal on T1-weighted images, heterogeneous signal on T2-weighted images and diffuse paramagnetic contrast enhancement. 3 Histopathological findings do not usually differ from other soft tissue neoplasms.…”

Section: Discussionmentioning

confidence: 99%

“…Schwannoma intraosseous presentation is rare, corresponding to 0.2% of primary bone tumors. [1][2][3][4][5][6] It mainly affects the mandible, the sacrum, vertebral bodies, the ulna, the humerus, the femur, the tibia, the patella, the scapula, the ribs and small bones of the hand. 1 After reviewing the literature, we found only four reported cases of calcaneal intraosseous schwannoma.…”

Section: Introductionmentioning

confidence: 99%

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Schwannoma intraósseo: Relato de caso e revisão da literatura

et al. 2019

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ResumoSchwannomas são tumores benignos oriundos de células da bainha neural cuja apresentação intraóssea é rara. Estima-se que os schwannomas intraósseos representem 0,2% de todos os tumores ósseos. Schwannomas podem acometer diferentes ossos do esqueleto, como a mandíbula, o sacro, corpos vertebrais, a ulna, o úmero, o fêmur, a tíbia, a patela, a escápula, costelas e ossos da mão. Apenas quatro casos que acometeram o calcâneo foram descritos na literatura. No presente trabalho, os autores relatam o caso de um paciente masculino de 49 anos com dor no retropé direito e com exames de imagem que evidenciaram lesão osteolítica no calcâneo. O estudo histopatológico confirmou o diagnóstico de Schwannoma intraósseo. O tratamento de escolha foi ressecção intralesional com adjuvância local e substituição por polimetilmetacrilato e fixação com parafusos canulados. O paciente evoluiu de forma satisfatória no pós-operatório e, após 1 ano de evolução, encontra-se assintomático, com boa resposta funcional e sem evidência de doença. Com esse relato, os autores desejam chamar atenção para essa patologia rara, sua apresentação clínica, radiológica e patológica, além de reiterar a importância de incluir o schwannoma intraósseo dentre os diagnósticos diferenciais das lesões osteolíticas do calcâneo.

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“…Reported locations include the mandible, maxilla, spine, petrous apex and, uncommonly, the long bones of the extremities. [3][4][5][6][7][8][9][10][11][12][13][14][15][16][17] Of 165 IOS cases reviewed by Suzuki et al, 18 only 10 cases (6%) were found in the sacrum.…”

Section: Introductionmentioning

confidence: 99%

Intraosseous schwannomas involving the sacrum: Characteristic imaging findings and review of the literature

et al. 2018

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Background and purpose Sacral intraosseous schwannomas represent a rare subset of schwannomas. The existing literature detailing the radiographic appearance of intraosseous schwannomas is limited. The aim of this study is to formally characterize the radiological appearance of sacral intraosseous schwannomas to differentiate them from other lytic lesions. Materials and methods Imaging studies of 13 pathologically proven intraosseous schwannomas were reviewed from multiple institutions by fellowship-trained radiologists. A PubMed search was performed and identified four papers pertaining to the imaging characteristics of sacral intraosseous schwannomas. The results of these papers were compared to findings from our cases. Results All tumors had heterogeneous signals and were predominately solid but cystic components with fluid-fluid levels were present. The tumors caused a mass effect but none infiltrated the surrounding soft tissues. Post-contrast T1-weighted images revealed heterogeneous enhancement in all 13 tumors and four possessed non-enhancing cysts. A literature review identified 16 other cases of sacral intraosseous schwannomas forming a total of 29 cases examined. Conclusions Sacral intraosseous schwannomas should be considered in the differential diagnosis for both radiologists and pathologists when dealing with large expansile, lytic lesions, with well-defined sclerotic margins involving the sacrum. This is particularly important in middle-aged adults presenting with pathology centered around S2-3.

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Intraosseous schwannoma in schwannomatosis

Cited by 15 publications

References 14 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

Schwannoma intraósseo: Relato de caso e revisão da literatura

Intraosseous schwannomas involving the sacrum: Characteristic imaging findings and review of the literature

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