Orbital hybrid peripheral nerve sheath tumors

Leung, Kai Ching Peter; Ko, Tak Chuen Simon

doi:10.4103/tjo.tjo_28_20

Cited by 5 publications

(11 citation statements)

References 17 publications

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“…1,7,[10][11][12] Neurofibroma-schwannoma features neurofibroma comprising a mixed population of fibroblasts, Schwann cells, and perineurial cells in a collagenous and occasionally myxoid background and cellular nodules composed of monomorphic Schwann cells in a palisaded Antoni A arrangement with Verocay body formation. 2,4,12,13 Hybrid neurofibroma-perineurioma, the least frequent HPNST subtype, has been described in association with NF1. This tumor is composed of a plexiform neurofibroma with areas of perineurial differentiation.…”

Section: Discussionmentioning

confidence: 99%

“…1 HPNST demonstrates a biphasic pattern containing mixed components of neurofibroma, schwannoma, and perineurioma with either a clear transition between cell types or an intermingling of cell types. 2 Several subtypes of HPNST have been identified, including neurofibroma-schwannoma, schwannoma-perineurioma, and neurofibroma-perineurioma. [2][3][4] Orbital involvement of HPNST is extremely rare with only 7 cases of all types reported in the literature.…”

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confidence: 99%

“…2 Several subtypes of HPNST have been identified, including neurofibroma-schwannoma, schwannoma-perineurioma, and neurofibroma-perineurioma. [2][3][4] Orbital involvement of HPNST is extremely rare with only 7 cases of all types reported in the literature. Due to its rarity, its clinical characteristics and prognosis remain largely unknown.…”

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confidence: 99%

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Hybrid Schwannoma-Perineurioma of the Orbit

Zhou

Milman

Rodríguez

et al. 2023

Ophthalmic Plastic &Amp; Reconstructive Surgery

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A 52-year-old woman presented with a 6-month history of progressive right proptosis associated with intermittent right retrobulbar and facial pain. MRI revealed a heterogeneously enhancing, well-circumscribed, ovoid, soft tissue mass in the intraconal space near the right orbital apex displacing the optic nerve medially. Excisional biopsy established the diagnosis of a schwannoma-perineurioma hybrid peripheral nerve sheath tumor (HPNST). This case represents only the second reported occurrence, to our knowledge, of an orbital schwannoma-perineurioma HPNST.

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Section: Discussionmentioning

confidence: 99%

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confidence: 99%

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Hybrid Schwannoma-Perineurioma of the Orbit

Zhou

Milman

Rodríguez

et al. 2023

Ophthalmic Plastic &Amp; Reconstructive Surgery

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“…In 1768, Akenside first published a scientific description of a patient with multiple tumors involving the peripheral nerves[ 1 ]. Over ten decades later, von Recklinghausen demonstrated that neurofibromas belong to a single nerve[ 2 ].…”

Section: Introductionmentioning

confidence: 99%

Imaging characteristics of orbital peripheral nerve sheath tumors: Analysis of 34 cases

Dai¹,

Wang²,

Wang³

et al. 2022

WJCC

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BACKGROUND Peripheral nerve sheath tumors (PNSTs), a rare group of neoplasms in the orbit, comprise only 4% of all orbital tumors. At present, there are very few studies detailing the features of these tumors identified using imaging technology. AIM To compare the differences in location, morphology, magnetic resonance imaging (MRI) signal intensity/computed tomography (CT) value, and enhancement degree of tumors of different pathological PNSTs types. METHODS Clinical, pathological, CT, and MRI data were analyzed retrospectively in 34 patients with periorbital sheath tumors diagnosed using histopathology from January 2013 to August 2021. RESULTS Among 34 cases of orbital peripheral nerve sheath tumors, 21 were schwannomas, 12 were neurofibromas, and 1 was a plexiform neurofibroma. Common clinical symptoms presented by patients with these types of tumors include eyelid swelling, exophthalmos, and limited eye movement. Schwannomas mostly occur in the intramuscular space with small tumor volume and rare bone involvement. Neurofibromas develop in the extrapyramidal space with larger tumor volume and more bone involvement. Radiologically, schwannomas and neurofibromas are characterized by regular morphology and uneven density and signal. One case of plexiform neurofibroma showed tortuous and diffuse growth along the nerve, with a worm-like appearance on imaging. CONCLUSION Different pathological types of orbital peripheral nerve sheath tumors have unique imaging characteristics. Comprehensive consideration of the patient's clinical and imaging manifestations is of great value in the diagnosis of orbital peripheral nerve sheath tumors.

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“…1 They rarely arise from the orbit. [1][2][3] This video shows the microsurgical resection of HNST involving both the orbit and lateral wall of the cavernous sinus. A 43-yr-old woman presented with 1-yr history of a slowly growing painless mass in her left medial eyelid.…”

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Microsurgical Resection of Hybrid Nerve Sheath Tumor Involving the Orbit and Lateral Wall of the Cavernous Sinus: 2-Dimensional Operative Video

Matsuo¹,

Higaki²,

Matsukado³

2021

Operative Surg.

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Surgical removal of the tumor involving both the orbital and cavernous sinus remains challenging. Hybrid nerve sheath tumors (HNSTs) are defined as benign peripheral nerve sheath tumors with combined features of more than one type of benign peripheral nerve sheath tumors, including schwannoma, neurofibroma, and perineurinoma.1 They rarely arise from the orbit.1-3 This video shows the microsurgical resection of HNST involving both the orbit and lateral wall of the cavernous sinus. A 43-yr-old woman presented with 1-yr history of a slowly growing painless mass in her left medial eyelid. Neuroimaging examinations demonstrated a multilobulated and well-demarcated mass with a heterogeneous enhancement located in the extraconal region, just above the levator muscle, and extending from the eyelid to the middle fossa through the superior orbital fissure. Based on the neuroimaging features, we diagnosed it as trigeminal schwannoma involving the orbit and lateral wall of the cavernous sinus preoperatively. We performed left frontotemporal craniotomy with superolateral orbitotomy to gain access to the tumor. The tumor was grayish yellow and relatively firm. No intact nerve fibers of the frontal and ophthalmic nerves could be identified, making the diagnosis of the tumor originating from both nerves. Finally, we completely resected the tumor, which was confirmed by postoperative magnetic resonance images. Her postoperative course was uneventful, except for transient diplopia and expected left forehead hypesthesia. The pathology returned to HNST containing schwannoma and neurofibroma with no malignancy. The patient consented to the procedure prior to the surgery. And, the patient consented to the publication of their image.

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Orbital hybrid peripheral nerve sheath tumors

Cited by 5 publications

References 17 publications

Hybrid Schwannoma-Perineurioma of the Orbit

Hybrid Schwannoma-Perineurioma of the Orbit

Imaging characteristics of orbital peripheral nerve sheath tumors: Analysis of 34 cases

Microsurgical Resection of Hybrid Nerve Sheath Tumor Involving the Orbit and Lateral Wall of the Cavernous Sinus: 2-Dimensional Operative Video

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