A hybrid tumor with schwannoma‐perineurioma‐neurofibroma morphology

McLaughlin, Colin T.; Kaffenberger, Benjamin H.; Gru, Alejandro A.

doi:10.1111/cup.12544

Cited by 8 publications

(5 citation statements)

References 13 publications

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“…In this scenario, the differential diagnosis in these tumors could include low-grade malignant peripheral nerve sheath tumor. 16 However, malignant transformation in hybrid peripheral nerve sheath tumors can be assumed to be extremely rare 8 and the degree of atypia in hybrid tumors is commonly degenerative. 6,17 In addition, they usually present a low proliferation index with immunohistochemistry for Ki-67.…”

Section: Discussionmentioning

confidence: 99%

Hybrid Neurofibroma/Schwannoma of the Oral Cavity: A Rare Case Report and Literature Review

et al. 2022

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Hybrid peripheral nerve sheath tumors show combined features of more than one type of conventional benign peripheral nerve sheath tumors. There are few cases reported of hybrid peripheral nerve sheath tumors in the head and neck region. A 68-year-old female patient was referred for evaluation of an oral swelling lasting five years. Intraoral examination revealed a small mobile nodule located in the lower vestibule. The patient underwent excisional biopsy and microscopic evaluation showed typical features of neurofibroma enclosing areas with palisading nuclei compatible with Antoni A pattern, which are seen in schwannomas. These regions showed strong and diffuse immunoreactivity for S100 protein and moderate positivity in the neurofibroma area. CD34 was positive in the neurofibroma area and entrapped axons were positive for neurofilament. The final diagnosis was oral hybrid neurofibroma-schwannoma tumor. Hybrid peripheral nerve sheath tumors, although extremely rare, may arise within the oral cavity. To the best of our knowledge, this is the first neurofibroma-schwannoma tumor reported in the oral cavity. Recognizing hybrid peripheral nerve sheath tumors as a distinct clinicopathological entity is important because they may also be associated with syndromic disorders.

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Section: Discussionmentioning

confidence: 99%

Hybrid Neurofibroma/Schwannoma of the Oral Cavity: A Rare Case Report and Literature Review

et al. 2022

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“…This tumor presented as a slowly enlarging, painful nodule in the upper thoracic region. It attained a size of nearly 8 cm in largest diameter over a course of five years [37]. …”

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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“…The hallmark of conventional schwannoma, such as the variable admixture of cellular Antoni A and hypocellular Antoni B areas or the formation of Verocay body was absent. Since most schwannoma-like components in HPNSTs reported so far demonstrated characteristic of conventional schwannoma [8, 11–14],the epithelioid schwannoma-like areas observed in the present case seemed to be rare. The other two cases of HPNSTs with such morphologic features involved in the superficial dermis [15, 16].…”

Section: Discussionmentioning

confidence: 64%

Primary intraosseous hybrid epithelioid schwannoma/perineurioma in the proximal tibia: a case report of benign hybrid neoplasm with local hypercellularity

et al. 2019

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Background As a new entity included in the 4th edition of the WHO classification of tumours of soft tissue and bone in 2013, hybrid peripheral nerve sheath tumours are benign composite neoplasms that demonstrate features of more than one type of nerve sheath tumour, with a wide age distribution and a predilection for superficial location. Those involving deep sites are relatively rare. To the best of our knowledge, only one case of primary intraosseous hybrid peripheral nerve sheath tumours has been documented. In this article, we report another case of hybrid peripheral nerve sheath tumours occurring in bone with different clinical, radiological and pathological features from those in the previously reported cases. Case presentation A 28-year-old female presented with a painful nodule in the right tibia. Radiological examination revealed an oval eccentric osteolytic lesion in the proximal tibia. Histologically, the circumscribed but unencapsulated lesion demonstrated biphasic cellular differentiation. Bland, small epithelioid cells arranged in clusters in the myxoid or collagenous stroma and inconspicuous spindle cells scattered in the hypercellular areas were suggested to originate from Schwann cells according to the detection of S100. Both the elongated spindle cells with thin, wavy nuclei and the spindle cells in fascicular or storiform pattern in hypercellular areas showed a positive immunoreaction for epithelial membrane antigen, indicating perineurial differentiation. Based on histological and immunochemical examinations, the patient was diagnosed with hybrid epithelioid schwannoma/perineurioma. The lesion was resected and has not recurred for 8 months since resection. Conclusion The present case is the second primary intraosseous hybrid peripheral nerve sheath tumour to be reported. This is also the first reported intraosseous tumour composed of epithelioid schwannoma and perineurioma with hypercellularity, indicating diverse involvement sites and a wide range of histological features among hybrid peripheral nerve sheath tumours. Awareness of such diversity is critical for accurate diagnoses. The morphological overlap with other spindle and epithelioid cell neoplasms, especially pure peripheral nerve sheath tumours, requires that immunochemical and molecular examinations be used as objective tools to provide the necessary information for a differential diagnosis. Electronic supplementary material The online version of this article (10.1186/s13000-019-0829-x) contains supplementary material, which is available to authorized users.

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A hybrid tumor with schwannoma‐perineurioma‐neurofibroma morphology

Cited by 8 publications

References 13 publications

Hybrid Neurofibroma/Schwannoma of the Oral Cavity: A Rare Case Report and Literature Review

Hybrid Neurofibroma/Schwannoma of the Oral Cavity: A Rare Case Report and Literature Review

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

Primary intraosseous hybrid epithelioid schwannoma/perineurioma in the proximal tibia: a case report of benign hybrid neoplasm with local hypercellularity

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