Most patients present to the doctor because of chronic pain in adulthood (median age at onset of symptoms 30 years, median age of diagnosis 40 years). During the clinical or imaging examination, the schwannomas, which can compress or displace the nerve due to their location and cause pain, become apparent. However, the localization of pain and the location of the schwannomas do not always correlate well. Also, the tumor burden does not always explain pain intensity and frequency. Although pain is the most common and usually the only complaint, schwannomas can cause other neurological symptoms such as numbness, paresthesia, bladder and rectal dysfunction, visual disturbances, and headaches, depending on their location.<\/p>\n
A specific form of schwannomatosis can present clinically with symptoms of intracranial pressure, such as headaches and fasting vomiting (vomiting at night or early in the morning upon waking). In these cases, meningiomas are identified as the cause. Neurological deficits arise depending on the tumor’s location. This form is also known as meningiomatosis.<\/p>\n<\/div>
Clinical Diagnostic Criteria<\/h4><\/div><\/div><\/div>
\n- Evidence of more than 2 schwannomas not located in the skin, at least one of which has been confirmed by tissue examination
\nand<\/strong>
\nwithout evidence of bilateral vestibular schwannomas (a benign tumor of the nerve sheath of the VIII cranial nerve, the auditory balance nerve) in imaging from the head or brain. cranial nerve sheath, the auditory balance nerve) on imaging of the head
\nor<\/em><\/li>\n- Existence of a schwannoma confirmed by tissue examination or an intracranial meningioma (usually a benign tumor of the meninges)
\nand<\/strong>
\na first-degree relative (biological parents or children) with schwannomatosis<\/li>\n<\/ul>\nHowever, if there are more than 2 schwannomas not located in the skin without tissue examination confirmation, the suspected diagnosis of schwannomatosis is obvious, especially if there is chronic pain.<\/p>\n
Segmental schwannomatosis describes the occurrence of schwannomas in only one extremity or limited to 2 spinal segments (30% of patients have segmental schwannomatosis).<\/p>\n<\/div><\/div><\/div><\/div><\/div>