NEUROFIBROMATOSIS TYPE 2: A REVIEW OF PAIN MANAGEMENT OPTIONS

Pain Management Case Reports ◽

10.36076/pmcr.2019/3/63 ◽

2019 ◽

pp. 63-68

Author(s):

Devang Padalia

Keyword(s):

Chronic Pain ◽

Pain Management ◽

Treatment Options ◽

Disease Process ◽

Neurofibromatosis Type ◽

Neurofibromatosis Type 2 ◽

Successful Outcome ◽

Management Options ◽

Novel Treatment

Neurofibromatosis is a chronic and painful disease process that can cause significant morbidity. The development of central and peripheral lesions is the primary source of acute and chronic pain. The pain associated with this condition is difficult to treat, but there are a number of options to make the lives of these patients more comfortable. A review of current literature and research data was used to compile a detailed list of treatment options for patients with neurofibromatosis type 2 (NF2). Key points on physiology and pain, as well as current and potential treatment options, are discussed. There are a number of articles, case reports, and studies regarding the treatment options for pain in patients with NF2. However, there is a lack of well-controlled randomized trials that demonstrate the superiority of one treatment modality. There are novel treatment plans being tailored to work at a genetic level, but these still require further research before implementing in daily practice. The management of chronic pain in NF2 is a difficult challenge for the medical community. Novel treatment options are currently being researched to improve the quality of life in these patients. Using a multimodal approach to pain control will improve the chances of a successful outcome. Key words: Neurofibromatosis type 2, schwannomatosis, neurofibromatosis type 1, chronic pain, pain management, neurofibroma, analgesics

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New Developments in Neurofibromatosis Type 2 and Vestibular Schwannoma

Neuro-Oncology Advances ◽

10.1093/noajnl/vdaa153 ◽

2020 ◽

Author(s):

Yin Ren ◽

Divya A Chari ◽

Sasa Vasilijic ◽

D Bradley Welling ◽

Konstantina M Stankovic

Keyword(s):

Treatment Options ◽

Neurofibromatosis Type ◽

Suppressor Gene ◽

Severe Form ◽

Neurofibromatosis Type 2 ◽

Future Research ◽

Autosomal Dominant Disorder ◽

Management Options ◽

Stereotactic Radiation

Abstract Neurofibromatosis type 2 (NF2) is a rare autosomal dominant disorder characterized by the development of multiple nervous system tumors due to mutation in the NF2 tumor suppressor gene. The hallmark feature of the NF2 syndrome is the development of bilateral vestibular schwannomas (VS). Although there is nearly 100% penetrance by 60 years of age, some patients suffer from a severe form of the disease and develop multiple tumors at an early age, while others are asymptomatic until later in life. Management options for VS include surgery, stereotactic radiation, and observation with serial imaging; however, currently there are no FDA-approved pharmacotherapies for NF2 or VS. Recent advancements in the molecular biology underlying NF2 have led to a better understanding of the etiology and pathogenesis of VS. These novel signaling pathways may be used to identify targeted therapies for these tumors. This review discusses the clinical features and treatment options for sporadic- and NF2-associated VS, the diagnostic and screening criteria, completed and ongoing clinical trials, quality of life metrics, and opportunities for future research.

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Resection of 2 Intradural Extramedullary Cervical Spine Tumors in a Patient With Neurofibromatosis Type 2: 3-Dimensional Operative Video

Operative Neurosurgery ◽

10.1093/ons/opy149 ◽

2018 ◽

Vol 16 (2) ◽

pp. 274-274

Author(s):

Simone E Dekker ◽

Chad A Glenn ◽

Thomas A Ostergard ◽

Osmond C Wu ◽

Fernando Alonso ◽

...

Keyword(s):

Spinal Cord ◽

Cervical Spine ◽

Treatment Options ◽

Tumor Resection ◽

Neurofibromatosis Type ◽

Cord Compression ◽

Neurofibromatosis Type 2 ◽

3 Dimensional ◽

Intradural Extramedullary

Abstract This 3-dimensional operative video illustrates resection of 2 cervical spine schwannomas in a 19-yr-old female with neurofibromatosis type 2. The patient presented with lower extremity hyperreflexity and hypertonicity. Magnetic resonance imaging (MRI) demonstrated 2 contrast-enhancing intradural extramedullary cervical spine lesions causing spinal cord compression at C4 and C5. The patient underwent a posterior cervical laminoplasty with a midline dural opening for tumor resection. Curvilinear spine cord compression is demonstrated in the operative video. After meticulous dissection, the tumors were resected without complication. The dural closure was performed in watertight fashion followed by laminoplasty using osteoplastic titanium miniplates and screws. Postoperative MRI demonstrated gross total resection with excellent decompression of the spinal cord. The postoperative course was uneventful. The natural history of this disease, treatment options, and potential complications are discussed.

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