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Submandibular nodal schwannoma: where did it come from?

Abdul Razak SF, Gendeh HS, Idris A

BMJ Case Rep, 2023 Aug 27;16(8).
PMID: 37640417 DOI: 10.1136/bcr-2022-253868

SummarySchwannoma, a type of benign neurological tumour arising from Schwann cells, is commonly seen in the head and neck region. However, the incidence of cervical lymph node schwannoma is extremely rare, with only two cases reported in the literature. Schwannomatosis is a disease characterised by a constellation of multiple schwannomas seen in a patient, guided by at least one confirmational histological diagnosis. This is a case of a painless progressively enlarging submandibular swelling which is confirmed as a schwannoma based on histology. The patient is also diagnosed with schwannomatosis as the patient has multiple neurological tumours. Nodal schwannoma has a good prognosis and no documentation of recurrence in the literature. It may arise from sympathetic nerves located within the lymph node.

Matched MeSH terms: Neurofibromatoses*
Severe Cervicothoracic Kyphoscoliosis in Neurofibromatosis - A Failure Of Posterior-Only Instrumentation: A Case Report

Soh E, Muhamad-Ariffin MH, Baharudin A

Malays Orthop J, 2020 Mar;14(1):88-91.
PMID: 32296489 DOI: 10.5704/MOJ.2003.015

Treatment of severe spinal deformities associated with neurofibromatosis has proven to be challenging. An 11-year-old girl, with neurofibromatosis and severe cervicothoracic kyphoscoliosis, was initially treated with posterior instrumentation and fusion. Implant failure developed within a year, requiring an anterior stabilisation and fusion with a non-vascularised fibular strut graft for better stability and increased likelihood of achieving union. The posterior instrumentation was removed due to its prominence and wound breakdown. Following the removal of the posterior implant, the fibular graft fractured. The patient was maintained on a cervical collar until union was achieved. Posterior spinal fusion alone in severe spinal deformities in neurofibromatosis has a high risk of failure. A combined anterior and posterior fusion may increase the chance of success, with better stability and union rate.

Matched MeSH terms: Neurofibromatoses
A case of neurofibromatosis with elephantiasis neuromatosa, bone changes, and coincident pulmonary tuberculosis

MCDOUGALL C

Med J Malaya, 1955 Jun;9(4):265-75.
PMID: 13253126
Matched MeSH terms: Neurofibromatoses/complications*
Fulltext Correction of severe thoracolumbar spondylolisthesis (grade 4) secondary to neurofibromatosis with posterior spinal instrumented fusion alone. A case report

Muhammad MT, Kwan MK, Chan CY, Lim BS, Goh DW

Med J Malaysia, 2012 Dec;67(6):633-5.
PMID: 23770965 MyJurnal

A 15-year-old teenager with Type 1 Neurofibromatosis presented with grade 4 spondylolisthesis over T12/L1 junction resulting paraparesis (Frankel D). Radiograph showed a Cobb angle of 88 degrees. Computed tomography scan showed dysplastic vertebral bodies, pedicles and facet joints of T11, T12 and L1 vertebra with complete T12/L1 facets dislocation. Magnetic resonance imaging confirmed presence of spinal cord compression. He underwent posterior instrumentation and posterolateral fusion (T8 to L4) using hybrid instrumentation. Extensive corticotomy of the posterior elements was followed by the use of large amount of bone graft. Post operatively, his neurology improved markedly back to normal. Radiographs showed a good correction of the deformity. He was immobilized in a thoracolumbar orthosis for six months. A solid posterior fusion was achieved at six months follow up. At 36-month follow up, he remained asymptomatic. This case report illustrates a successful treatment of a grade 4 thoracolumbar spondylolisthesis secondary to neurofibromatosis with posterior spinal fusion alone.

Matched MeSH terms: Neurofibromatoses
Fulltext Two-stage surgery for a large cervical dumbbell tumour in neurofibromatosis 1: a case report

Mohd Ariff S, Joehaimey J, Ahmad Sabri O, Abdul Halim Y

Malays Orthop J, 2011 Nov;5(3):24-7.
PMID: 25279032 MyJurnal DOI: 10.5704/MOJ.1111.003

Spinal neurofibromas occur sporadically and typically occur in association with neurofibromatosis 1. Patients afflicted with neurofibromatosis 1 usually present with involvement of several nerve roots. This report describes the case of a 14- year-old child with a large intraspinal, but extradural tumour with paraspinal extension, dumbbell neurofibroma of the cervical region extending from the C2 to C4 vertebrae. The lesions were readily detected by MR imaging and were successfully resected in a two-stage surgery. The time interval between the first and second surgery was one month. We provide a brief review of the literature regarding various surgical approaches, emphasising the utility of anterior and posterior approaches.

Matched MeSH terms: Neurofibromatoses
Limb-length discrepancy in children : treatment by growth arrest

Ibrahim, S.

Malaysian Journal of Child Health, 1997;9(2):178-181.
MyJurnal

Percutaneous epiphysiodesis for arresting growth is a useful method for equalising moderate lower limb-length discrepancy. This paper reviews the result of this procedure performed at the Royal Hospital for Sick Children in Glasgowfrom 1989 to 1993. There were 8 children (5 girls and 3 boys) ranging in age (chronological) from 10 years 3 months to 13 years 3 months. Limb-length discrepancies (LLD) at the time of surgery were between 2.5 cm to 5.5 cm. The predicted LLD at maturity was obtained using the Moseleys graph. This ranged from 3.9 cm to 8.3 cm. The cause of discrepancy were hemihypertrophy (5), neurofibromatosis (1), AV malformation (1) and neonatal osteomyelitis (1). Bone age in 3 patients corresponded to their chronological age. In 2 patients their bone age were less than their chronological age by 1 year. In the remaining 3 patients their bone were less than their chronological age by 1 to 3 years. All patients had percutaneous epiphysiodesis of both the distal femur and proximal tibia. All patients except one had afinal LLD of 1 cm or less. There were 2 unsatisfactory results: one girl had a valgus knee due to asymmetric physeal closure. Another boy had a final LLD of 3.3 cm as the procedure was done near skeletal maturity. This technique is useful even with LLD greater than 5 cm in children whose predicted height is above average. The difference between chronological age and bone age ranged from I to 3 years.

Matched MeSH terms: Neurofibromatoses
Fulltext Gastrointestinal stromal tumors (GIST) in a patient with Neurofibromatosis: A case report

Zailani, M.H., Naqiyah, I., Rohaizak, M., Siti Aishah, M.A.

International Medical Journal Malaysia, 2007;6(1):1-5.
MyJurnal

Type 1 Neurofibromatosis (von Reckinghausen’s disease) has been associated with several gastrointestinal tumor complications, either benign or malignant. We report a case of a middle age man who had Type 1 Neurofibromatosis and presented with acute intestinal obstruction. Laparotomy revealed multiple nodular lesions along the small intestine with two larger nodules which were resected. Histopathological examination of the nodules confirmed it as gastrointestinal stromal tumors (GIST) with malignant potential. The association between neurofibromatosis and GIST is discussed.

Matched MeSH terms: Neurofibromatoses
Fulltext A Rare Presentation of Spinal Schwannoma Causing Conus Medullaris Syndrome: A Case Series on Surgical Outcome

Khan ESKBM, Thean CAP, Zakaria ZB, Awang MSB, Karupiah RK, Awang MB

J Orthop Case Rep, 2020;10(2):101-105.
PMID: 32953668 DOI: 10.13107/jocr.2020.v10.i02.1718

Introduction: Spinal schwannoma can occur anywhere along the spinal cord but is predominantly seen in the cervical and thoracic region.It composes mainly of well-differentiated schwann cell and is benign in nature. It is typically seen in the peripheral nerves and is commonly associated with neurofibromatosis. Up to 80% of cases, spinal schwannoma is reported to be intradural in location and 15% of cases have both intradural and extradural components. Spinal schwannoma rarely causes conus medullaris syndrome.
Case Report: In this case series, all three female patients in their 4th and 5th decades of life presented with conus medullaris syndrome. Lower back pain, radiculopathy, lower limb weakness, and urinary incontinence are their main clinical presentation. Magnetic resonance imaging shows a well-defined intradural, extramedullary mass compressing onto the conus medullary region. These patients undergone microscopic assisted excision of the tumor and had remarkably good early outcome despite the advanced presentation of neurological deficit.
Conclusion: Despite the late presentation with significant neurological deficit, surgical excision of spinal schwannomas carries a good prognosis postoperatively due to their benign nature and extramedullary location.

Matched MeSH terms: Neurofibromatoses
A rare case of cervical hypoglossal nerve neurofibromas in a patient with type 1 neurofibromatosis

Lum SG, Baki MM, Yunus MRM

Braz J Otorhinolaryngol, 2021 Feb 25.
PMID: 33707119 DOI: 10.1016/j.bjorl.2021.01.006

BACKGROUND: Neurofibromas are benign peripheral nerve sheath tumours. Hypoglossal nerve neurofibromas in cervical region are relatively rare, bilateral occurrence is extremely rare.
METHODS: A 32-year-old man with type 1 neurofibromatosis presented with bilateral neck masses. Magnetic resonance imaging showed parapharyngeal masses consistent with neurogenic tumours, most likely neurofibromas.
RESULTS: Surgical exploration through lateral cervical approach revealed unexpected finding of the tumour that arose from the hypoglossal nerve. The tumour had totally engulfed the nerve with no normal nerve fascicles identifiable, thus resected in toto. In the postoperative course, the patient developed right hypoglossal palsy and vocal fold palsy treated with augmentation of the paralysed vocal fold with temporary injection material.
CONCLUSIONS: The authors described a patient with type 1 neurofibromatosis with neurofibroma originating from cervical part of hypoglossal nerve. This paper discussed this rare condition and the management on how to improve the treatment outcome.

Matched MeSH terms: Neurofibromatoses
Fulltext Bilateral Mirror Image Cervical Neurofibroma in an Adult with Neurofibromatosis Type 1

Pandey S, Singh K, Sharma V, Khan MT, Ghosh A, Santhosh D

Malays J Med Sci, 2017 Mar;24(1):117-120.
PMID: 28381935 DOI: 10.21315/mjms2017.24.1.13

Neurofibromatosis type 1 (NF1) is an autosomal dominant disorder characterised by various phenotypic features like hyperpigmented spots, neurofibromas, Lisch nodules, skeletal abnormalities and tendency to develop neoplasms. Only few cases of Non-Familial Spinal Neurofibromatosis-1 (Non-FSNF1) have been described in literature with tumors involving the spinal roots at every level being even rarer. We reported an interesting case of bilateral symmetrical cervical neurofibroma with multiple spinal neurofibromas appearing as mirror image on CT, associated with non familial NF-1 as a rare presentation in a 25-year-old adult male.

Matched MeSH terms: Neurofibromatoses
Neurofibromatosis Type 2 Presenting with Oculomotor Ophthalmoplegia and Distal Myopathy

Tevaraj JM, Li Min ET, Mohd Noor RA, Yaakub A, Wan Hitam WH

Case Rep Ophthalmol Med, 2016;2016:1701509.
PMID: 27738538

Neurofibromatosis type 2 usually presents with bilateral acoustic schwannomas. We highlight the rare presentation of neurofibromatosis initially involving third nerve. A 23-year-old Malay female presented with left eye drooping of the upper lid and limitation of upward movement for 8 years. It was associated with right-sided body weakness, change in voice, and hearing disturbance in the right ear for the past 2 years. On examination, there was mild ptosis and limitation of movement superiorly in the left eye. Both eyes had posterior subcapsular cataract. Fundoscopy showed generalised optic disc swelling in both eyes. She also had palsy of the right vocal cord, as well as the third and eighth nerve. There was wasting of the distal muscles of her right hand, with right-sided decreased muscle power. Pedunculated cutaneous lesions were noted over her body and scalp. MRI revealed bilateral acoustic and trigeminal schwannomas with multiple extra-axial lesions and intradural extramedullary nodules. Patient was diagnosed with neurofibromatosis type 2 and planned for craniotomy and tumour debulking, but she declined treatment. Neurofibromatosis type 2 may uncommonly present with isolated ophthalmoplegia, so a thorough physical examination and a high index of suspicion are required to avoid missing this condition.

Matched MeSH terms: Neurofibromatoses