Malignant Peripheral Nerve Sheath Tumours - Report of 8 Cases and Review of the Literature

Stark, Andreas M.; Buhl, Ralf; Hugo, Heinz-Hermann; Mehdorn, H. M.

doi:10.1007/s007010170090

Cited by 95 publications

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“…In the literature, both malignancy and recurrence of the tumor mean reserved to poor prognosis (Stark et al 2001). Histologically, the lesions correspond to reports of cases of PNST in dogs (Hendrick et al 1998).…”

Section: Discussionsupporting

confidence: 82%

Retrospective canine skin peripheral nerve sheath tumors data with emphasis on histologic, immunohistochemical and prognostic factors

et al. 2015

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In this retrospective study was determined the frequency of canine skin peripheral nerve sheath tumors (PNST) in cases diagnosed by the Setor de Patologia Veterinária of the Universidade Federal do Rio Grande do Sul (SPV-UFRGS), Brazil, between the years 2000 and 2012. The canine profiles, as well as histological, immunohistochemical and prognostic aspects of the tumors were based on 70 samples, comprising 40 females, 29 males and one unspecified sample. Between 2000 and 2012, 2,984 skin tumors of dogs were diagnosed in the SPV-UFRGS, totaling 2.34% of skin neoplasms in dogs. Animals that comprised the largest amount of samples (43%) were those with no breed (SRD), followed by German Shepherds (10%). Females were more affected than males (40/70 - 57% and 29/70 - 41% respectively). Skin PNST of this research showed predominant localization on the limbs (40% in the forelimbs and 29% in the hindlimbs); affecting adult dogs, mostly aged between 8 and 11 years (54%). The samples were routinely processed for hematoxylin and eosin, and were also evaluated by toluidine blue and Masson's trichrome staining, and immunohistochemistry (IHC) anti-vimentin, -S-100, -GFAP, -actin, von Willebrand factor and neurofilament. Anisocytosis and anisokaryosis, mitotic index, intratumoral necrosis, invasion of adjacent tissues, tumor location, local recurrence and metastasis were related to the diagnosis of benign (49/70) or malignant tumor (21/70). The Antoni A histological pattern was observed more frequently in benign tumors. The immunohistochemistry helped to diagnose PNST, and anti-vimentin and anti-protein S-100 showed the highest rates of immunostaining. Throughout statistical analysis of animals with tumor recurrence, it was found that the chance of an animal with a malignant peripheral nerve sheath tumor to develop recurrence is 4.61 times higher than in an animal that had a benign tumor.

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Section: Discussionsupporting

confidence: 82%

Retrospective canine skin peripheral nerve sheath tumors data with emphasis on histologic, immunohistochemical and prognostic factors

et al. 2015

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“…9,12 The majority of these cases involve the trunk and extremities. 31 There is generally no sex or race predilection, but MPNSTs are more commonly found in older patients. 12 Although it is widely accepted that uncontrolled growth of Schwann cells is the cause of these tumors, it has been suggest ed that they occur de novo and do not arise as a result of malignant transformation from a benign schwanno ma.…”

Section: Malignant Peripheral Nerve Sheath Tumorsmentioning

confidence: 99%

“…12 Although it is widely accepted that uncontrolled growth of Schwann cells is the cause of these tumors, it has been suggest ed that they occur de novo and do not arise as a result of malignant transformation from a benign schwanno ma. 1,3,23,31,33 However, some reports have suggested that ra diation may play a role in the development of MPNSTs.…”

Section: Malignant Peripheral Nerve Sheath Tumorsmentioning

confidence: 99%

“…5 MPNSTs have also been shown to metastasize to the lungs, soft tissues, bone, liver, and/or brain nearly 65% of the time. 4,9,12,31 The 5-year survival rate for pa tients with these tumors ranges from 10% to 50%, and patients with a history of NF have an exceptionally poor prognosis. 1,5,9,12,22,31 When only MPNSTs of the head and neck are considered, the 5-year survival rate decreases to about 20%.…”

Section: Prognosis and Treatmentmentioning

confidence: 99%

“…Patients with NF also typically have a much younger age of onset. 9,12,31 Al though the presence of NF is known to greatly increase the odds of developing MPNSTs in general, it is interest ing to note that it has not been shown to play a significant role in MPNSTs of the cranial nerves. 1,33 Of the reports we identified for patients with a trigeminal MPNST, only one patient was described as having NF1 and one as hav ing NF2.…”

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confidence: 99%

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Malignant peripheral nerve sheath tumors of the trigeminal nerve: a systematic review of 36 cases

Schmidt¹,

Yick²,

Boghani³

et al. 2013

FOC

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Object Malignant peripheral nerve sheath tumors (MPNSTs) are a rare form of malignancy arising from the Schwann cells of peripheral nerves. MPNSTs of the trigeminal nerve are exceptionally rare, with only a handful of reports in the literature. These tumors are typically very aggressive, resulting in significant patient morbidity and a generally grim prognosis. Most current reports suggest that radical resection with radiation therapy offers the best benefit. In this study, the authors systematically reviewed the world English-language literature on MPNSTs of the trigeminal nerve to analyze the presentations, treatment options, and outcomes for patients with this disease. Methods A literature search for MPNSTs of the trigeminal nerve confined to nonanimal, English-language articles was conducted utilizing the PubMed database, with additional cases chosen from the references of selected articles. Only cases of confirmed MPNSTs of the trigeminal nerve or its peripheral branches, based upon surgical, pathological, or radiological analysis, were included. Results From the literature search, 29 articles discussing 35 cases of MPNSTs of the trigeminal nerve were chosen. With the addition of 1 case from their own institution, the authors analyzed 36 cases of trigeminal MPNSTs. The average age of onset was 44.6 years. These tumors were more commonly seen in male patients (77.1%). The gasserian ganglion was involved in 36.1% of the cases. Of the cases in which the nerve distribution was specified (n = 25), the mandibular branch was most commonly involved (72.0%), followed by the maxillary branch (60.0%) and the ophthalmic branch (32.0%), with 44.0% of patients exhibiting involvement of 2 or more branches. Altered facial sensation and facial pain were the 2 most commonly reported symptoms, found in 63.9% and 52.8% of patients, respectively. Mastication difficulty and diplopia were seen in 22.2% of patients, facial weakness was seen in 19.4%, and hearing loss was present in 16.7%. With regard to the primary treatment strategy, 80.6% underwent resection, 16.7% underwent radiation therapy, and 2.9% received chemotherapy alone. Patients treated with complete resection followed by postoperative radiation therapy had the most favorable outcomes, with no patients showing evidence of disease recurrence with a mean follow-up of 34.6 months. Patients treated with incomplete resection followed by postoperative radiation therapy had more favorable outcomes than patients treated with incomplete resection without radiation therapy or radiation therapy alone. Conclusions Trigeminal MPNSTs most commonly present as altered facial sensation or facial pain, although they exhibit a number of other clinical manifestations, including the involvement of other cranial nerves. While a variety of treatment options exist, due to their highly infiltrative nature, aggressive resection followed by radiation therapy appears to offer the greatest chance of recurrence-free survival.

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Nerve Tumors of Childhood and Infancy

Kvint

Ali

Jacques

et al. 2021

Diagnostic Assessment and Treatment of Peripheral Nerve Tumors

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Malignant Peripheral Nerve Sheath Tumours - Report of 8 Cases and Review of the Literature

Cited by 95 publications

References 0 publications

Retrospective canine skin peripheral nerve sheath tumors data with emphasis on histologic, immunohistochemical and prognostic factors

Retrospective canine skin peripheral nerve sheath tumors data with emphasis on histologic, immunohistochemical and prognostic factors

Malignant peripheral nerve sheath tumors of the trigeminal nerve: a systematic review of 36 cases

Nerve Tumors of Childhood and Infancy

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