scholarly journals Plexiform Schwannoma of the Tongue in a Pediatric Patient with Neurofibromatosis Type 2: A Case Report and Review of Literature

2018 ◽  
Vol 2018 ◽  
pp. 1-4
Author(s):  
Samir M. Amer ◽  
Aijan Ukudeyeva ◽  
Harold S. Pine ◽  
Gerald A. Campbell ◽  
Cecilia G. Clement
Keyword(s):  
Pediatric Patient ◽  
Histopathological Examination ◽  
Smooth Muscle Actin ◽  
Neurofibromatosis Type ◽  
Neurofibromatosis Type 2 ◽  
Case Presentation ◽  
Plexiform Schwannoma ◽  
S 100 ◽  
History Of

Introduction. Plexiform schwannoma is a rare variant of schwannoma that accounts for only 5% of all schwannomas. Herein, we present a rare case of plexiform schwannoma of the tongue in a pediatric patient with neurofibromatosis type 2 (NF2). Case Presentation. A 13-year-old female presented with a growing left-sided tongue mass. The patient has a past medical history of NF2. The tongue mass was excised and histopathological examination revealed a spindle cell tumor with multinodular growth pattern, with Verocay bodies' formation. Tumor cells were strongly positive for S-100 protein and negative for smooth muscle actin (SMA), and EMA highlighted perineural fibroblasts surrounding tumor nodules. These findings were diagnostic of plexiform schwannoma. Conclusion. Plexiform schwannoma of the tongue is an extremely rare tumor seen in patients with NF2. Clinical examination and histopathological evaluation are important for diagnosis of plexiform schwannoma.

scholarly journals Combined translabyrinthine, anterior petrosal approach for resection of collision vestibular schwannoma and petrous apex meningioma in neurofibromatosis type 2, with auditory brainstem implant placement

2021 ◽  
Vol 5 (2) ◽  
pp. V17
Author(s):  
Usman A. Khan ◽  
Jillian H. Plonsker ◽  
Rick A. Friedman ◽  
Marc S. Schwartz
Keyword(s):  
Vestibular Schwannoma ◽  
Neurofibromatosis Type ◽  
Auditory Brainstem ◽  
Neurofibromatosis Type 2 ◽  
Petrous Apex ◽  
Surgical Decision ◽  
Implant Placement ◽  
History Of ◽  
Sporadic Tumors

The natural history of neurofibromatosis type 2 (NF2) is profound bilateral hearing loss. The decision to pursue microsurgery may be more complicated in NF2 than with sporadic tumors. Schwannomas in NF2 often occur with other skull base tumors. Treatment should be tailored to preserve auditory perception for as long as possible. The authors present the case of a man with NF2 and a vestibular schwannoma who has poor hearing on the same side as a large petrous apex meningioma, both opposite to a well-hearing ear. This case highlights surgical decision-making and technical nuances during resection of collision tumors in NF2. The video can be found here: https://stream.cadmore.media/r10.3171/2021.7.FOCVID21130

scholarly journals Multifocal granulocytic sarcoma of the spine mimicking neurofibromatosis Type 2: case report

2016 ◽  
Vol 25 (4) ◽  
pp. 523-527
Author(s):  
Gregory P. Lekovic ◽  
Marc S. Schwartz ◽  
John L. Go
Keyword(s):  
Granulocytic Sarcoma ◽  
Neurofibromatosis Type ◽  
Rapid Onset ◽  
Neurofibromatosis Type 2 ◽  
Neurological Deficits ◽  
Lumbosacral Spine ◽  
Imaging Features ◽  
History Of ◽  
Patient’S History

In this report the authors report on a patient with a very indolent course of granulocytic sarcoma, characterized by steroid-induced remission of spinal and cranial tumors and recurrence over a period of several years. This 24-year-old man with history of leukemia presented with rapid-onset quadriparesis secondary to multiple extraaxial masses of the cervicothoracic spine, from C-5 to T-3, and lumbosacral spine, from L-5 to the coccyx. Although the imaging features were highly suggestive of neurofibromatosis Type 2, the patient's history and clinical course were consistent with granulocytic sarcoma; repeat imaging and, later, needle biopsy definitively established the diagnosis of granulocytic sarcoma. Laminectomy and surgical decompression of the spine were not required and, arguably, could have posed unnecessary risk to the patient. This case illustrates that the successful management of a patient presenting with profound neurological deficits due to intradural spinal cord tumors may sometimes be nonsurgical.

A pediatric case of neurofibromatosis type 2 presenting as a plexiform schwannoma of the intraparotid facial nerve

2006 ◽  
Vol 1 (3) ◽  
pp. 220-225 ◽  
Author(s):  
Jennifer Guillemaud ◽  
Hamdy El-Hakim ◽  
Nitin Chauhan ◽  
Mark Lee ◽  
Michelle Noga ◽  
...  
Keyword(s):  
Facial Nerve ◽  
Neurofibromatosis Type ◽  
Neurofibromatosis Type 2 ◽  
Pediatric Case ◽  
Plexiform Schwannoma

Natural history of neurofibromatosis type 2 with onset before the age of 1 year

Neurogenetics ◽  
2013 ◽  
Vol 14 (2) ◽  
pp. 89-98 ◽  
Author(s):  
Martino Ruggieri ◽  
Anna Lia Gabriele ◽  
Agata Polizzi ◽  
Vincenzo Salpietro ◽  
Francesco Nicita ◽  
...  
Keyword(s):  
Natural History ◽  
Neurofibromatosis Type ◽  
Neurofibromatosis Type 2 ◽  
History Of

scholarly journals Natural History of Vestibular Schwannoma Growth and Hearing Decline in Newly Diagnosed Neurofibromatosis Type 2 Patients

2014 ◽  
Vol 35 (1) ◽  
pp. e50-e56 ◽  
Author(s):  
Scott R. Plotkin ◽  
Vanessa L. Merker ◽  
Alona Muzikansky ◽  
Fred G. Barker ◽  
William Slattery
Keyword(s):  
Natural History ◽  
Vestibular Schwannoma ◽  
Neurofibromatosis Type ◽  
Neurofibromatosis Type 2 ◽  
Newly Diagnosed ◽  
History Of

Gastric plexiform schwannoma in association with neurofibromatosis type 2

2016 ◽  
Vol 9 (6) ◽  
pp. 352-357 ◽  
Author(s):  
Satoru Kudose ◽  
Michael Kyriakos ◽  
Michael Magdi Awad
Keyword(s):  
Neurofibromatosis Type ◽  
Neurofibromatosis Type 2 ◽  
Plexiform Schwannoma

Plexiform schwannoma of the esophagus in a child with neurofibromatosis type 2

2009 ◽  
Vol 44 (7) ◽  
pp. 1458-1461 ◽  
Author(s):  
Giuseppe Retrosi ◽  
Lorenzo Nanni ◽  
Riccardo Ricci ◽  
Carlo Manzoni ◽  
Claudio Pintus
Keyword(s):  
Neurofibromatosis Type ◽  
Neurofibromatosis Type 2 ◽  
Plexiform Schwannoma

scholarly journals Multiple CNS Tumors in a Patient With Neurofibromatosis Type 2: Classical Presentation of a Rare Disease

2020 ◽  
Author(s):  
Aamer Ubaid ◽  
Farishta Waheed ◽  
Awais Naeem
Keyword(s):  
Rare Disease ◽  
Lower Limb ◽  
Neurofibromatosis Type ◽  
Neurofibromatosis Type 2 ◽  
Lumbar Region ◽  
Nerve Paralysis ◽  
Function Tests ◽  
Left Lower Limb ◽  
History Of

Neurofibromatosis type 2 is a genetic autosomal dominant disorder caused by a spontaneous mutation in the gene located on chromosome 22 q11-13.1, which usually emerges in adolescence or early adulthood and is characterized by the development of bilateral vestibular schwannoma. We hereby report the classical case of Neurofibromatosis type 2 in a 25-year-old young male with multiple tumors associated with the disease. This patient presented to us with 3 years history of multiple painless nodules on his skin, facial weakness, left-sided progressive hearing loss, and 20 days history of weakness in the left lower limb. On Examination, he was vital with a GCS of 15/15. He was anemic with no jaundice. He had left inguinal lymphadenopathy along with multiple subcutaneous nodules on different areas, including the scalp, face, left mid-axillary line over the abdomen. He also had Right-sided facial palsy and horizontal nystagmus. CNS examination revealed an upgoing plantar on the left side, right facial nerve palsy, and bilateral vestibulocochlear nerve paralysis. Spine examination revealed spinal tenderness in the lower lumbar region. Superficial abdominal reflexes were absent. Upper limb and right lower limb power, tone, and reflexes were normal while the tone and power in the left lower limb were reduced power being ⅗. Reflexes were also exaggerated in the left lower limb. The right ankle showed swelling, most probably a plexiform neuroma. On investigations, he had normochromic normocytic anemia with mild leucocytosis. Platelets were normal. The rest of the biochemical investigations, including serum electrolytes, liver function tests, and renal function tests, were also normal.MRI brain and spine confirmed bilateral acoustic neuroma and multiple cranial and peripheral nerve tumors i.e., classical presentation of a rare disease neurofibromatosis. He was referred to the neurology unit for further assessment and treatment.

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