24‐Year‐Old Woman with An Internal Auditory Canal Mass

Heras, Facundo Las; Martuza, Robert L.; Caruso, Paul; Rincon, Sandra; Stemmer‐Rachamimov, Anat

doi:10.1111/bpa.12055

Cited by 10 publications

(9 citation statements)

References 9 publications

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“…6,11,15,19 One tumor arising in the internal auditory canal showed almost identical features to our case 15, showing a biphasic pattern of schwannoma and perineurioma with formation of pseudoonion bulbs. 15 Three other tumors, one of which occurred in the mediastinum, were also composed of distinct schwannoma and perineurioma areas. 6,11,19 Interestingly, a spinal nerve tumor reported by Hayashi et al 14 showed complex microscopic features: schwannoma and perineurioma components were independently present among mixed cellular areas, suggesting the transitional form of mixed cellular and combined types of hybrid tumors.…”

Section: Discussionsupporting

confidence: 75%

“…5 Others including our case 15 had an intraneural perineurioma component and arose in other sites, such as the mediastinum. 11,15 Furthermore, our case 15 was associated with neurofibromatosis type 2, whereas the mixed cellular type lacks neurofibromatoses. 7,13 To avoid confusion in the nomenclature, this type of HSP could be renamed combined (or composite) schwannoma and perineurioma.…”

Section: Discussionmentioning

confidence: 71%

“…This publication was followed by additional reports describing BPNST expressing hybrid features of schwannoma, neurofibroma, and perineurioma in various combinations, that is, hybrid schwannoma/perineurioma (HSP), hybrid neurofibroma/schwannoma, hybrid neurofibroma/perineurioma, and hybrid schwannoma/neurofibroma/perineurioma. [5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21] The current "WHO classification of tumors of soft tissue and bone" and "WHO classification of tumors of the central nervous system" include "hybrid nerve sheath tumor" as a distinctive entity in the category of peripheral nerve tumors. 2,3 Among these hybrid tumors, a HSP reported by Hornick et al 7 is characterized by mixed proliferation of Schwann cells and perineurial cells.…”

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confidence: 99%

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Hybrid Schwannoma/Perineurioma: Morphologic Variations and Genetic Profiles

Hirose¹,

Kobayashi

Nobusawa

et al. 2020

Applied Immunohistochemistry &Amp; Molecular Morphology

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To clarify the morphologic spectrum and molecular profiles of hybrid schwannoma/perineurioma (HSP), we investigated 15 tumors clinicopathologically and cytogenetically. HSP was classified into 2 morphologic types: mixed cellular and combined tumor types. The former comprising of 14 tumors mostly arose in the subcutaneous tissue of the extremities and the trunk of middle-aged adults. They were well-circumscribed and composed of elongated spindle-shaped tumor cells arranged in storiform and whorl patterns. Immunostaining revealed a mixed cellular proliferation of S-100 protein-positive and SOX10-positive Schwann cells and epithelial membrane antigenpositive, claudin 1-positive, and GLUT1-positive perineurial cells. During follow-up, no tumors were found to have recurred in any cases. In contrast, in the combined tumor type arising in the mediastinum of a young male with neurofibromatosis type 2, the intraneural perineurioma-like areas, characterized by small whorl-like structures, were present in plexiform schwannoma-like areas. No recurrence was noted in the case. Molecular analyses (array comparative genomic hybridization and fluorescence in situ hybridization) revealed LOH 22q in 2 tumors of 5 studied: one each of the mixed cellular and combined tumor types. Although the same diagnostic term, HSP, has been applied to both mixed and combined types, they should be separated from each other.

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Section: Discussionsupporting

confidence: 75%

Section: Discussionmentioning

confidence: 71%

mentioning

confidence: 99%

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Hybrid Schwannoma/Perineurioma: Morphologic Variations and Genetic Profiles

Hirose¹,

Kobayashi

Nobusawa

et al. 2020

Applied Immunohistochemistry &Amp; Molecular Morphology

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“…The patient was a 36-year-old male with a black tumor on his arm since birth. Also in 2013, Las Heras et al [28] reported the first ever case of a hybrid perineurioma/schwannoma in a cranial nerve. The patient was a 24-year-old female with an internal auditory canal mass.…”

Section: Discussionmentioning

confidence: 99%

Hybrid peripheral nerve sheath tumors: report of five cases and detailed review of literature

et al. 2017

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BackgroundHybrid peripheral nerve sheath tumors (PNSTs) have been recognized recently and were first included in the 4th edition of World Health Organization (WHO) Classification of Tumors of Soft tissue and Bone, published in 2013. These tumors show combined features of more than one type of conventional benign peripheral nerve sheath tumors. The most common combinations are those of schwannoma/perineurioma followed by combinations of neurofibroma/schwannoma and neurofibroma/perineurioma. A detailed literature review of published cases is presented.We have discussed the types and etiology, epidemiology and sites of localization, gross and microscopic appearances and immunohistochemical features of hybrid PNSTs and association of these tumors with tumor syndromes.Case presentationWe have included five cases which were diagnosed in our department as we believe that publication of these new cases is relevant for the improved understanding of these specific tumors. Four of our five patients were males, mean age was 24 years. There was wide variation in the location of these tumors. Mean size of excised tumors was 5.5 cms in the greatest dimensions. Three out of five cases represented hybrid schwannoma/perineurioma histologically. No significant nuclear atypia, mitotic activity or necrosis seen. All five cases were completely excised. All five patients are alive and well at the time of writing with no recurrence.ConclusionHybrid PNSTs are distinct tumors and are usually benign. However, rare case reports have described local recurrence and at least two recent case reports have described malignant transformation in these tumors. Further studies on large number of cases are required to determine the exact pathogenetic basis of these tumors.

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“…Typically, HNST consists of a painless mass in subcutaneous tissue or dermis [ 48 ]; when large peripheral nerves or spinal nerves are involved, the tumor may be associated with pain or neurological impairment. The most common site of HNST are the fingers [ 49 ], but some rare cases arise from cranial nerves [ 50 , 51 , 52 ]. Hybrid schwannomas/perineuriomas are sporadic [ 52 ], while hybrid neurofibroma/schwannomas are strongly associated with NF1 and 2, and schwannomatosis [ 53 ].…”

Section: Epidemiology and Clinical Featuresmentioning

confidence: 99%

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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24‐Year‐Old Woman with An Internal Auditory Canal Mass

Cited by 10 publications

References 9 publications

Hybrid Schwannoma/Perineurioma: Morphologic Variations and Genetic Profiles

Hybrid Schwannoma/Perineurioma: Morphologic Variations and Genetic Profiles

Hybrid peripheral nerve sheath tumors: report of five cases and detailed review of literature

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

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