Sporadic Schwannomatosis: A Systematic Review Following the 2005 Consensus Statement

Chick, Grégoire; Victor, Jan; Poujade, Thibault; Hollevoet, Nadine

doi:10.1055/s-0038-1655548

Cited by 9 publications

(6 citation statements)

References 42 publications

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“…Recently, a cohort of just seven paediatric patients with eight peripheral nerve tumours has been published [ 16 ]. Furthermore, purely larger paediatric series do not exist; in mixed series, paediatric cases are not evaluated separately [ 6 – 9 , 18 , 21 , 27 ]. Due to the rare nature of peripheral nerve sheath tumours in children and its association with rare genetic pathologies like neurofibromatosis, only limited data exists.…”

Section: Discussionmentioning

confidence: 99%

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Surgical management of peripheral nerve sheath tumours in children, with special consideration of neurofibromatoses

et al. 2020

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Introduction Peripheral nerve sheath tumours in children are a rare and heterogeneous group, consisting mostly of benign tumours as well as malignant neoplasms. Especially in the paediatric population, diagnostics and indication for therapy pose relevant challenges for neurosurgeons and paediatric neurologists alike. Most paediatric cases that need surgical intervention are associated to neurofibromatosis type 1 (NF1). Methods We retrospectively reviewed all paediatric cases treated at the Department of Neurosurgery in Tübingen between 2006 and 2017 for peripheral nerve sheath tumours. We analysed clinical signs, symptoms, histology, association to an underlying phacomatosis and sensory/motor function. Results Of the 82 identified patients, the majority had NF1 (76.8%). Nine children bore a sporadic tumour without underlying phacomatosis (11%), 8 had NF2 (9.8%) and 2 schwannomatosis (2.4%), A total of 168 surgical interventions were performed, and 206 tumours were removed. Indication for surgery was in most instances significant tumour growth (45.2%) followed by pain (33.9%). New deficits led to surgery in 12.5% of interventions; malignancy was suspected in 8.3%. Histopathology revealed mostly neurofibromas (82.5%), divided into cutaneous neurofibromas (10.7%), infiltrating plexiform neurofibromas (25.7%) and peripheral nerve-born neurofibromas (46.1%). 12.1% of tumours were schwannomas, 2.9% MPNST, 1.5% ganglioneuroma (n = 3) and 1 hybrid-neurofibroma and perineurinoma each. Leading symptoms, such as pain and motor and sensory deficits, improved after 125/166 interventions (74.4%), remained unchanged following 39 interventions (23.2%) and worsened in 4 occasions (2.4%). Conclusion Surgery is safe and effective for (neurofibromatosis associated) peripheral nerve sheath tumours in the paediatric population; however, management needs a multidisciplinary setting. We propose early surgical resection in paediatric patients with peripheral nerve sheath tumours with significant growth, or pain, or motor deficit, or suspected malignancy.

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Section: Discussionmentioning

confidence: 99%

“…It can be associated to SMARCB1 or LZTR1 mutations and exists in a sporadic and a familial pattern. It however mostly affects adults, and an age < 30 years is considered an exclusion criterion for the sporadic form [ 9 ].…”

Section: Introductionmentioning

confidence: 99%

Surgical management of peripheral nerve sheath tumours in children, with special consideration of neurofibromatoses

et al. 2020

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“…In all cases, enucleation of the lesion under loop magnification should be attempted in an effort to preserve all fascicles [10], as was the case in all three of the patients. Pain relief has been shown to be a reproducible result in the vast majority of patients in the literature with rates exceeding 80%, with Tinel's sign disappearing postoperatively [2,3,6]. Complications consist mainly of novel neurologic impairment and paresthesias have been reported with widely varying rates from 1.5-80% [9, 19].…”

Section: Discussionmentioning

confidence: 99%

“…Peripheral Nerve Sheath Tumors (PNST) are uncommon, comprising less than 5% of the soft tissue tumors of the upper limb [1]. The most common amongst them are neurilemmomas, also known as Schwannomas, that are derived from Schwan cells and are more common in the hand and wrist [2] and neurofibromas, comprised of cells of mixed origin, that seem to be more common in the brachial plexus [3]. Schwannomas are usually solitary tumors arising from the Schwann cells that support the peripheral nerve fibers.…”

Section: Introductionmentioning

confidence: 99%

Median Nerve Schwannoma of the Hand and Wrist: 3 Cases

Xarchas¹,

Kyriakopoulos²,

Vlachou³

et al. 2019

TOORTHJ

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Tumors of the median nerve are difficult to diagnose and median nerve schwannomas are rare. During a ten-year period, we treated eleven median nerve schwannomas found on the hand (nine) and wrist (two). All the tumors were treated by enucleation under loupe magnification and tourniquet application. All had a favorable result at a mean follow up of five years. We present three of our most typical cases, with schwannomas found on the wrist, palm and thumb. We also review the literature offering a wider view on the pathology, diagnosis and treatment of schwannomas in general.

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“…However, surgical resection of diffuse, plexiform, and soft tissue-type neurofibromas should rarely be undertaken and only in select cases with clear surgical goals due to the high associated morbidity [9,39]. Compared to schwannomas and depending on the specific subtype, neurofibroma resection is often more challenging (greater nerve fascicle integration) and is associated with a higher risk of postoperative nerve injury [40]. To help mitigate this, intraoperative monitoring has been shown to be a useful adjunct to discern functional and non-functional tissue [15].…”

Section: Management and Outcomementioning

confidence: 99%

Peripheral Nerve Tumors in Neurofibromatosis 1, Neurofibromatosis 2, and Schwannomatosis

Jack¹,

Durcanova²,

Wright³

et al. 2020

Neurofibromatosis - Current Trends and Future Directions

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Neurofibromatosis was first described in the nineteenth century. At the time, Friederich Daniel Von Recklinghausen detailed two cases of multiple neurofibromas. Although reports of similar cases had been published before his, Von Recklinghausen is credited with the initial description in 1882, postulating that the tumors originated from nerve sheath and plexal connective tissue. Similarly, in 1822 John Henry Wishart described what is believed to be neurofibromatosis type 2; however, it was Harvey Cushing's description of a case of bilateral vestibular schwannomas in 1916 that highlighted and increased awareness of the disease (albeit the original presentation was thought to be in the context of neurofibromatosis type 1). Since their original description, understanding of these neurocutaneous diseases has greatly expanded. Knowledge of the genotypic mutations and molecular mechanisms underlying the disease pathophysiology has resulted in natural history enlightenment and optimal treatment refinement. However, many aspects of neurofibromatosis have yet to be explained and remain active areas of investigation. In this chapter, clinical, radiological, and surgical considerations for peripheral nerve tumor management in the context of neurocutaneous disorders are reviewed. More specifically, clinical presentations, pathological and imaging findings, as well as management for neurofibromatosis type 1, type 2, and schwannomatosis are comprehensively discussed.

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Sporadic Schwannomatosis: A Systematic Review Following the 2005 Consensus Statement

Cited by 9 publications

References 42 publications

Surgical management of peripheral nerve sheath tumours in children, with special consideration of neurofibromatoses

Surgical management of peripheral nerve sheath tumours in children, with special consideration of neurofibromatoses

Median Nerve Schwannoma of the Hand and Wrist: 3 Cases

Peripheral Nerve Tumors in Neurofibromatosis 1, Neurofibromatosis 2, and Schwannomatosis

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