Intraneural neurofibromas involving the posterior interosseous nerve

Lallemand, R. C.; Weller, Roy O.

doi:10.1136/jnnp.36.6.991

Cited by 45 publications

(15 citation statements)

References 14 publications

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“…Several spindle cell neoplasms may grow inside the nerve trunks, including perineurioma 31,32 and peripheral nerve sheath tumor. [33][34][35] SS with a biphasic component may be easier to diagnose, but the monophasic presentation becomes a diagnosis by exclusion, and needs more sophisticated techniques, particularly molecular biology, to reach a proper diagnosis with certainty. The spindle cell tumor most often encountered in nerve trunk is a peripheral nerve sheath tumor (PNST), including both the benign neurofibroma or schwannoma and the malignant peripheral nerve sheath tumor (malignant schwannoma).…”

Section: Discussionmentioning

confidence: 99%

Intraneural synovial sarcoma: two cases

et al. 2004

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We report two cases of intraneural synovial sarcoma. The first patient is a 46-year-old female who presented for several months with soft-tissue mass in the right infra-auricular region. The second patient is a 11-year-old girl who fell and then presented with pain in the area innervated by the right C7 spinal root and a nodule identified in the nerve root foramina. Both lesions were of small size and presented with features of synovial sarcoma. A biphasic variant was found in case 1 and a monophasic variant was present in case 2. Immunohistochemical studies were performed to confirm the diagnosis, excluding the main differential diagnoses, namely schwannoma and malignant peripheral nerve sheath tumor. Ultrastructural study was performed in case 2 allowing exclusion of other possible diagnoses. Molecular studies were performed on paraffin-embedded tissue in both cases and revealed the known characteristic t(X;18)(SYT-SSX) translocation.

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Section: Discussionmentioning

confidence: 99%

Intraneural synovial sarcoma: two cases

et al. 2004

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“…In chronic neuropathies it is believed that the formation of "onion bulbs" involves an initial loss of myelinated and non-myelinated axons which results in proliferation of Schwann cells with a change in their surface contours [ 151. In human peripheral nerve tumours "onion bulb-like'' structures are thought to be formed from basal lamina clad cells that differ from normal human Schwann cells [6]. They probably represent a reactive change to slow progressive nerve degeneration [ 81.…”

Section: Discussionmentioning

confidence: 99%

The Ultrastructure of Bovine Peripheral Nerve Sheath Tumours

Canfield

1978

Vet Pathol

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Abstract. Bovine peripheral nerve sheath tumours from 30 cattle were similar ultrastructurally to human schwannomas and neurofibromas. Bovine neurofibromatous tissue had large amounts of extracellular material. primarily collagen and electron lucent granular material. The principal cells had basal laminae and a disorganized proliferation of the plasmalemma. Axons were consistently seen and were surrounded by the plasmalemma of principal cells. The principal cells seemed to be Schwann cells or variants of them. Bovine schwannomas had arcas similar to Antoni type A tissuc with sparse extracellular material, few, if any, axons. and an apparent organized layering of cytoplasmic processes clad in a basal lamina. Cell nuclei often formed palisades. The principal cells in bovine schwannomas might bc derived either from Schwann cells or perineurial cells. Bovine schwannomas appeared together with bovine neurofibromatous tissue in affected nerves.

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“…Intraneural perineurioma (also referred to as localized hypertrophic neuropathy / mononeuropathy, hypertrophic neurofibromatosis, hypertrophic interstitial neuritis, or intraneural neurofibroma) typically presents in adolescents or young adults as a slow‐growing, painless mononeuropathy and causes progressive loss of motor function, and uncommonly, sensory disturbance 19,20 . Perineurioma is considered as a perineural cell neoplasm; histologically it features pseudo onion‐bulbs composed of EMA‐positive, S‐100‐negative perineurial cells surrounding myelinated or non‐myelinated fibers 21–23 …”

Section: Discussionmentioning

confidence: 99%

“…19,20 Perineurioma is considered as a perineural cell neoplasm; histologically it features pseudo onion-bulbs composed of EMA-positive, S-100-negative perineurial cells surrounding myelinated or non-myelinated fibers. [21][22][23] Neurofilamentous axonal swellings can be seen in a number of degenerative and toxic disorders of the nervous system. Giant axonal neuropathy (GAN) is an autosomal recessively inherited disease of childhood, [24][25][26] with axonal swelling and accumulation of neurofilaments in giant axons.…”

Section: Discussionmentioning

confidence: 99%

Familial motor neuron disease with prominent onion-bulb-like structures and axonal swelling restricted to the spinal ventral root: autopsy findings in two siblings

Tokuyama¹,

Yagishita

Ryo

et al. 2010

Neuropathology

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We report autopsy cases of two siblings who developed muscular atrophy and dementia, clinically considered to be familial motor neuron disease (MND). They presented with motor neuron signs predominantly in the distal limbs without sensory impairment. At autopsy, severe neuronal loss in the anterior horn consistent with MND was found, but histopathological hallmarks like Bunina bodies and skein-like inclusions were absent. Surprisingly, numerous huge axonal swellings (about 30 microm in diameter) and onion-bulb-like structures were found in the spinal ventral roots. These changes were not observed in spinal dorsal roots or peripheral nerves. However, obvious segmental demyelination of the ventral root was not found. In addition, neurofibrillary tangles (NFTs) and neuritic plaques were present in the frontal cortex, temporal cortex and hippocampus, and to a lesser degree, in the amygdala, substantia nigra and thalamus. Our two cases are a hitherto unreported type of MND, which shows focal giant axonopathy and prominent formation of onion-bulb-like structures due to Schwann cell proliferation restricted to the spinal ventral roots.

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Intraneural neurofibromas involving the posterior interosseous nerve

Cited by 45 publications

References 14 publications

Intraneural synovial sarcoma: two cases

Intraneural synovial sarcoma: two cases

The Ultrastructure of Bovine Peripheral Nerve Sheath Tumours

Familial motor neuron disease with prominent onion-bulb-like structures and axonal swelling restricted to the spinal ventral root: autopsy findings in two siblings

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