Clinicoradiological features of intraneural perineuriomas obviate the need for tissue diagnosis

Wilson, Thomas J.; Howe, Benjamin M.; Stewart, Shelby; Spinner, Robert J.; Amrami, Kimberly K.

doi:10.3171/2017.5.jns17905

Cited by 36 publications

(17 citation statements)

References 14 publications

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“…6 In addition, Wilson et al developed the following Perineurioma Diagnostic Criteria: no cancer history, unifocal disease, moderate to severe hyperintensity on T2-weighted MR images, moderate to severe contrast enhancement, homogeneous contrast enhancement, fusiform shape, enlargement of the involved nerves, and age 40 years. 9 Therefore, in their opinion, biopsy samples are not necessary. Based on our experience, there are rare cases (see Illustrative Cases a and b), such as chronic inflammatory demyelinating polyneuropathy (CIDP) 6 in which MRI findings are similar to perineurioma, but their treatment differs.…”

Section: Discussionmentioning

confidence: 99%

Perineurioma: A Rare Entity of Peripheral Nerve Sheath Tumors

Brand

Pedro

Paľa

et al. 2021

J Neurol Surg A Cent Eur Neurosurg

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Objective Intraneural perineurioma is a rare tumor entity. It is a benign, very slow growing peripheral nerve sheath tumor that typically occurs in children and young adults. Motor deficits and muscle atrophy are classic presenting symptoms, while sensory deficits are rare at the onset of the disease. Recommended treatment strategies are lacking. We have evaluated the clinical follow-up and our experience with treatment of this rare entity. Methods A total of 30 patients with intraneural perineuriomas were assessed retrospectively. Demographic data, clinical symptoms, diagnostic examinations, therapy strategies, and clinical outcome were analyzed. Descriptive statistical methods were used for evaluation. Results The mean age was 22 years. Eleven women and 19 men were affected. The lesion occurred in the area of the upper extremity in 16 patients and in the area of the lower extremity in 14 patients. The most frequently affected nerve was the sciatic nerve, followed by the radial nerve. All patients showed a motor deficit to some extent. Seventy percent (n = 21) revealed atrophy, 43.3% (n = 13) had sensitive deficits, and 17% (n = 5) suffered of pain. Fascicle biopsies were performed in 26 patients (87%). In four patients (13%), the tumor was completely resected and then reconstructed via nerve grafts. Seventy percent of the patients (n = 21) received a magnetic resonance imaging (MRI) within 5 years postoperatively, in which no progress was shown. Conclusions To diagnose perineurioma, it is essential to take a biopsy of an enlarged, nonfunctional fascicle. Furthermore, a long-distance epineuriotomy to decompress the hypertrophic fascicle is reasonable. To preserve the nerves' residual function, a complete resection is not recommended. Results after grafting are poor. One reason for this might be residual tumor cells along the nerve that cannot be visualized. Malignant transformation is not yet reported and tumor growth is stable for years.

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Section: Discussionmentioning

confidence: 99%

Perineurioma: A Rare Entity of Peripheral Nerve Sheath Tumors

Brand

Pedro

Paľa

et al. 2021

J Neurol Surg A Cent Eur Neurosurg

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“…On MRI, PNs typically present as a fusiform enlargement of the nerve fascicles, isointense on T1-weighted and moderate to strong hyperintensity on T2-weighted MR images with moderate to strong homogenous contrast enhancement after intravenous gandolinium application [9,32]. On HRNS examinations, a nerve enlargement over several centimetres with hyperechoic perineurial tissue can be detected.…”

Section: Discussionmentioning

confidence: 99%

Modern treatment of perineuriomas: a case-series and systematic review

et al. 2020

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Background: Perineuriomas are rare benign peripheral nerve sheath tumours of perineurial cell origin and can be classified into intraneural and extraneural perineuriomas. They most commonly present a mononeuropathy of gradual onset and slow progression, resulting in progressive neurological deficits like hypoesthesia or motor weakness. Therapy is still variable. Aim of the study was to compare our surgical treatment and our follow-up regime including high-resolution nerve sonography with the current literature to evaluate best treatment of perineuriomas. Methods: Retrospective analysis of our dataset "peripheral nerve lesion" to identify patients suffering from perineuriomas between 01.01.2012 until 31.12.2018. Surgical treatment and the follow-up examination of three patients were described. Additionally, a systematic review including PubMed, the Cochrane Collaboration Library, Scopus and Google Scholar was performed for literature published between January 1, 1990 and October 31, 2019 independently by 2 authors. Results: In the first case, the left ulnar nerve was affected. In the second case, the left peroneal nerve and in the third case the right median nerve was affected. High-resolution nerve sonography was performed in each case. All patients underwent interfascicular neurolysis combined with a targeted fascicular biopsy under electrophysiological monitoring. Neurological deficits improved subsidized by rehabilitation. Surgical therapy and the neurological outcome were compared with literature. Systematic review revealed 22 articles, which met the inclusion criteria. Therefore, demographics, surgical treatment and neurological outcome of 77 patients were analysed. Conclusions: Perineuriomas are rare benign nerve sheath tumours with a slow progression, sometimes difficult to diagnose. Decompression and neurolysis may improve neurological deficits. High resolution nerve sonography might serve as a helpful additional diagnostic tool in this process.

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“…), sparing the patient of the risk and costs of these medications. Based on our extensive experience with these conditions and the lessons we have learned from common clinicoradiologic features, we do not biopsy "routine" cases of CIDP (Amrami et al, 2010;Pinto et al, 2019), intraneural perineuriomas (Wilson et al, 2018a), or nerve hamartomas/choristomas (see below).…”

Section: "Tissue Is the Issue"mentioning

confidence: 99%

A novel mechanism for the formation and propagation of neural tumors and lesions through neural highways

Spinner

2021

Clinical Anatomy

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By recognizing anatomic and radiologic patterns of rare and often misdiagnosed peripheral nerve tumors/lesions, we have defined mechanisms for the propagation of neural diseases. The novel concept of the nervous system serving as a complex system of "highways" driving the neural and perineural spread of these lesions is described in three examples: Intraneural dissection of joint fluid in intraneural ganglion cysts, perineural spread of cancer cells, and dissemination of unknown concentrations of neurotrophic/inhibitory factors for growth in hamartomas/choristomas of nerve. Further mapping of these pathways to identify the natural history of diseases, the spectrum of disease evolution, the role of genetic mutations, and how these neural pathways interface with the lymphatic, vascular, and cerebrospinal systems may lead to advances in targeted treatments.

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Clinicoradiological features of intraneural perineuriomas obviate the need for tissue diagnosis

Cited by 36 publications

References 14 publications

Perineurioma: A Rare Entity of Peripheral Nerve Sheath Tumors

Perineurioma: A Rare Entity of Peripheral Nerve Sheath Tumors

Modern treatment of perineuriomas: a case-series and systematic review

A novel mechanism for the formation and propagation of neural tumors and lesions through neural highways

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