Intracranial Malignant Peripheral Nerve Sheath Tumors Not Associated with a Cranial Nerve: Systematic Review and Illustrative Case

Mackel, Charles E.; Medeiros, Isabela Novais; Moore, Brian E.; Zhao, Qing; Jha, R. Tushar

doi:10.1016/j.wneu.2021.09.072

Cited by 4 publications

(9 citation statements)

References 59 publications

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“…MINST are extremely unusual tumours. 2 Similar to its MPNST counterpart, the diagnosis of MINST can be difficult and necessitates a thorough investigation of clinical, radiological and histopathological findings. The cell of origin of these tumours is uncertain.…”

Section: Discussionmentioning

confidence: 99%

“…Awareness of this tumour, its confirmatory histological examination and extensive surgical extirpation are needed in the definitive management. 2 They have poor prognoses and benefit from aggressive resection, multimodal treatment, and close follow-up. Next-generation sequencing can be helpful for potential targeted therapy.…”

Section: Discussionmentioning

confidence: 99%

“…The male-to-female ratio is 1.5:1 with a mean age of 29.7 ± 21.8 years. 2 There are 71% of cases of sporadic and 23% cases of NF type 1 related MINST. 3 These tumours have few risk factors like the presence of NF1 syndrome, along with a history of ionizing radiation exposure.…”

Section: Introductionmentioning

confidence: 99%

“…3 These tumours have few risk factors like the presence of NF1 syndrome, along with a history of ionizing radiation exposure. 2 Schwannomas, the benign counterpart are also usually associated with neurofibromatosis. 4 It is very rare to find this tumour within the brain or cerebellar parenchyma.…”

Section: Introductionmentioning

confidence: 99%

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Malignant intracerebral nerve sheath tumour of the brain presenting as a recurrent meningioma

Zaheer¹,

Aden

Ranga³

2023

JDPO

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A malignant intracerebral nerve sheath tumour (MINST) is a variant of MPNST arising inside the brain. They are very rare tumours that are aggressive and have strong metastatic potential. We report a case of a recurrent lesion in the brain in a 39 years male who presented as recurrent meningioma. It was finally reported as MINST with histopathology and immunohistochemistry. Surgical excision is the treatment of choice. The patient is on follow-up after an uneventful surgical excision. To the best of our knowledge, very few cases of malignant intracerebral nerve sheath tumours have been reported so far in the literature. This case is presented not only for its rarity but also to highlight that this aggressive tumour needs to be kept in mind while diagnosing such lesions with these histomorphological findings.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Malignant intracerebral nerve sheath tumour of the brain presenting as a recurrent meningioma

Zaheer¹,

Aden

Ranga³

2023

JDPO

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“…What’s more, it is of great clinical significance to distinguish between benign and malignant Intracranial schwannoma. Intracranial malignant schwannoma usually presents with a large mass with irregular margins, mass effect, frequently accompanied by cystic degeneration, necrosis, calcification, and bleeding, and obviously uneven strengthening on enhancement scan[ 36 ].…”

Section: Discussionmentioning

confidence: 99%

CT and MRI findings of intra-parenchymal and intra-ventricular schwannoma: a series of seven cases

Zhang

Mo²,

Zhang

et al. 2022

BMC Med Imaging

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Objective To analyze the computed tomography (CT) and magnetic resonance imaging (MRI) features of patients with intra-parenchymal and intra-ventricular schwannoma. Methods The CT and MRI features of seven cases with intra-parenchymal and intra-ventricular schwannoma were analyzed retrospectively. Results There were four men and three women (median age, 25 years; range, 12–42 years) in this study. The median tumor size was 4.4 cm (range, 3.1–6.5 cm). The mass was, respectively, round in four cases (57.1%), lobulated in two cases (28.6%) and oval in one case (14.3%). All tumors were well-circumscribed. Septa in the mass could be observed in three cases (42.9%), and nodular calcification was observed in two cases (28.6%), which peritumoral edema (n = 3, 42.9%) and hydrocephalus (n = 3, 42.9%) could be observed. Most of these lesions (n = 6) presented iso-hypointensity on T1-weighted images and iso-hyperintensity on T2-weighted images, except one lesion showing low intensity on T2WI. In addition, a fluid–fluid level was observed in one case. After contrast agents’ injection, all masses illustrated heterogeneously moderate to marked enhancement. Conclusions A well-defined solid and cystic mass with calcification and moderate to marked delayed enhancement may be an objective account of intra-parenchymal or intra-ventricular schwannoma.

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Diagnosis and Treatment of Peripheral and Cranial Nerve Tumors with Expert Recommendations: An EUropean Network for RAre CANcers (EURACAN) Initiative

Pellerino

Verdijk

Nichelli

et al. 2023

Cancers

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The 2021 WHO classification of the CNS Tumors identifies as “Peripheral nerve sheath tumors” (PNST) some entities with specific clinical and anatomical characteristics, histological and molecular markers, imaging findings, and aggressiveness. The Task Force has reviewed the evidence of diagnostic and therapeutic interventions, which is particularly low due to the rarity, and drawn recommendations accordingly. Tumor diagnosis is primarily based on hematoxylin and eosin-stained sections and immunohistochemistry. Molecular analysis is not essential to establish the histological nature of these tumors, although genetic analyses on DNA extracted from PNST (neurofibromas/schwannomas) is required to diagnose mosaic forms of NF1 and SPS. MRI is the gold-standard to delineate the extension with respect to adjacent structures. Gross-total resection is the first choice, and can be curative in benign lesions; however, the extent of resection must be balanced with preservation of nerve functioning. Radiotherapy can be omitted in benign tumors after complete resection and in NF-related tumors, due to the theoretic risk of secondary malignancies in a tumor-suppressor syndrome. Systemic therapy should be considered in incomplete resected plexiform neurofibromas/MPNSTs. MEK inhibitor selumetinib can be used in NF1 children ≥2 years with inoperable/symptomatic plexiform neurofibromas, while anthracycline-based treatment is the first choice for unresectable/locally advanced/metastatic MPNST. Clinical trials on other MEK1-2 inhibitors alone or in combination with mTOR inhibitors are under investigation in plexiform neurofibromas and MPNST, respectively.

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Intracranial Malignant Peripheral Nerve Sheath Tumors Not Associated with a Cranial Nerve: Systematic Review and Illustrative Case

Cited by 4 publications

References 59 publications

Malignant intracerebral nerve sheath tumour of the brain presenting as a recurrent meningioma

Malignant intracerebral nerve sheath tumour of the brain presenting as a recurrent meningioma

CT and MRI findings of intra-parenchymal and intra-ventricular schwannoma: a series of seven cases

Diagnosis and Treatment of Peripheral and Cranial Nerve Tumors with Expert Recommendations: An EUropean Network for RAre CANcers (EURACAN) Initiative

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