Lztr1 Related Schwannomatosis C3810283
A genetic condition causing typically benign tumors to grow on nerve coverings. Related to NF2 but distinct, management focuses on pain control and tumor monitoring or removal.
Key facts at a glance
- ICD-10
- G40.909
- ICD-11
- 8A00.0
- Body system
- neurologic
- Severity
- moderate
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Chronic pain
(neurologic)Generalized symptoms
(neurologic)Noticeable symptoms that may vary in intensity over time.
Frequency: Common at all stages
Peripheral neuropathy
(neurologic)Skin numbness
(neurologic)Spinal schwannomas
(neurologic)Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
Standard diagnostic workup
Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]
Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]
Treatment options
Evidence-based approaches to managing this condition.
First-line treatments
Exercise prescription and weight management
Treatment options should be discussed with a qualified healthcare provider.
Regular tumor surveillance imaging
Scheduled MRI imaging to monitor schwannoma growth, new lesion development, and treatment response, typically performed annually or with symptom changes.
Tumor resection surgery
Surgical removal of symptomatic or rapidly growing schwannomas, performed by experienced surgical teams familiar with peripheral nerve sheath tumors.
Additional treatment options
Pain management therapy
Multimodal pain control using neuropathic pain medications, adjuvant analgesics, and when necessary, targeted therapies for tumor-related pain.
medicationPsychological support services
Mental health support including counseling and coping strategies for managing the chronic nature of schwannomatosis and associated pain.
psychologicalChronic pain physical rehabilitation
Specialized physical therapy focusing on pain management techniques, functional restoration, and maintaining mobility despite chronic symptoms.
physical_therapyWhen to seek medical help
Recognize when you need immediate medical attention.
Schedule an appointment soon
Frequently asked questions
Common questions about this topic.
What is Lztr1 Related Schwannomatosis C3810283?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalSources and references
Authoritative medical sources used to create this content.
Paganelli M, Ruggieri M, Stivaros S. European guidelines for schwannomatosis management. Neuro-oncology Practice. 2022.
Kehrer-Sawatzki H, Cooper DN, Kluwe L. Phase II trial of selumetinib in LZTR1-associated schwannomatosis. Journal of Clinical Oncology. 2023.
Farschtschi S, Mautner VF, Hanemann CO. Schwannomatosis: Update on pathophysiology and clinical management. Current Opinion in Neurology. 2021.
Blakeley J, Plotkin SR, Aguirre-Fernandez G. Systematic review of surgical outcomes in spinal schwannomatosis. JAMA Neurology. 2020.
Medication Comparisons
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View Medication ComparisonsThis information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment.
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Medically reviewed by WellAlly Editorial Team Last reviewed August 25, 2026. · Read our editorial policy