Hybrid Neurofibroma-Schwannoma of the Orbit

Youens, Kenneth E.; Woodward, Julie; Wallace, Dana; Cummings, Thomas J.

doi:10.1080/01676830802009804

Cited by 29 publications

(21 citation statements)

References 3 publications

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“…In 2008, Shelekhova et al [17] reported another case of hybrid neurofibroma/perineurioma in an extradigital site. The fact that these hybrid tumors can arise in even more unusual sites, which was demonstrated by Youens et al [18] by reporting a hybrid neurofibroma/schwannoma in the orbit of a 51-year-old female. In 2009, Hornick et al [4] published a large series of 42 hybrid schwannoma/perineuriomas.…”

Section: Discussionmentioning

confidence: 90%

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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Section: Discussionmentioning

confidence: 90%

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

et al. 2017

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“…Since the first report of an hybrid peripheral nerve sheath tumor in 1998 , a few cases have been described, with the great majority arising in peripheral nerves of the trunk and extremities . Uncommon locations, such as colon, nasal cavity, orbit and lymph node, have also been reported . However, there is no report to date, of an intracranial hybrid tumor arising from a cranial nerve.…”

Section: Discussionmentioning

confidence: 99%

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

et al. 2013

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Benign peripheral nerve sheath tumors are divided into schwannomas, neurofibromas and perineuriomas. In recent years, tumors with hybrid features, composed of multiple, discrete areas of different histological types, were described. These tumors may represent a diagnostic challenge. A 24-year-old woman with multiple sclerosis was found to have a 1.3 cm TV × 0.7 cm AP T2 intermediate lesion within the left internal auditory canal. Gross examination revealed a tan-white, well circumscribed mass. Histologic examination demonstrated a well demarcated, cellular, solid neoplasm with a biphasic pattern. Most of the tumor was composed of spindle cells arranged in fascicles with focal Verocay body formation and diffuse S100 positivity. A second, minor area showed concentric proliferation of neoplastic spindle cells around one or more axons. Tumor cells in this area were positive for perineurial markers, claudin-1 and Glut-1, and focally immunopositive for CD34.We present here a case of a benign peripheral nerve sheath tumor with histological and immunohistochemical features consistent with a dual pattern of differentiation of schwannoma and perineurioma, in the VIIIth cranial nerve. This is, to our knowledge, the first case of a hybrid perineurioma/schwannoma reported in a cranial nerve.

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“…[3][4][5] Interestingly, there are several recent reports describing hybrid peripheral nerve sheath tumors showing a biphasic component of neoplastic cells. These combinations include a mixture of neurofibroma and schwannoma, [6][7][8][9][10][11][12] schwannoma and perineurioma, [13][14][15][16][17][18] neurofibroma and perineurioma, 14,[19][20][21] and perineurioma and granular cell tumor. 22 Only 1 described case included a triphasic combination of neurofibroma, schwannoma, and perineurioma.…”

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A Benign Cutaneous Plexiform Hybrid Tumor of Perineurioma and Cellular Neurothekeoma

Requena¹,

Sitthinamsuwan²,

Fried³

et al. 2013

American Journal of Surgical Pathology

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There are several recent reports describing hybrid peripheral nerve sheath tumors showing a biphasic component of neoplastic cells. These combinations include a mixture of neurofibroma and schwannoma, schwannoma and perineurioma, neurofibroma and perineurioma, and perineurioma and granular cell tumor. A case of a triphasic combination of neurofibroma, schwannoma, and perineurioma has also been described. We describe the clinicopathologic and immunohistochemical characteristics of 9 cases of a benign cutaneous plexiform nerve sheath tumor located on the lips and exhibiting hybrid features of perineurioma and cellular neurothekeoma. Clinically, lesions were solitary dome-shaped papules located on the lips. Histopathologically, the neoplasms consisted of well-circumscribed but uncapsulated dermal nodules with a plexiform pattern. They were composed of nests or rounded aggregations of neoplastic cells embedded in a slightly myxoid stroma. Within the aggregates, cells were distributed in a storiform and lamellar pattern. Immunohistochemically, most neoplastic cells expressed strong immunoreactivity for S100A6, MiTF, NKI/C3, PGP9.5, EMA, and NSE, whereas variable, focal, and weaker positivity for CD34, claudin-1, and Glut-1 was seen in some cases. On the basis of these findings, we believe that this neoplasm is a distinctive benign cutaneous plexiform nerve sheath tumor with histopathologic and immunohistochemical hybrid features of perineurioma and cellular neurothekeoma.

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Hybrid Neurofibroma-Schwannoma of the Orbit

Abstract: Hybrid neurofibroma-schwannomas may arise within the orbit.

Cited by 29 publications

References 3 publications

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

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

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

A Benign Cutaneous Plexiform Hybrid Tumor of Perineurioma and Cellular Neurothekeoma

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