Schwannomatosis patient who was followed up for fifteen years: A case report

Table 1 From 2006 to 2018, the patient underwent 5 operations in our department, as shown in the above table

Time	Cardinal symptom	Therapeutic method	Postoperative follow-up
September 12, 2006	Pain in the back, neck and chest for 5 yr with walking dysfunction for 1 mo	Posterior cervical and thoracic vertebral canal tumor resection was performed	After the operation, the patient's pain and lower limb muscle strength improved, recovered, and the patient was discharged from hospital, and lived normally
January 5, 2011	Lumbago with numbness of both lower limbs for 1 mo	Posterior cervical and thoracic vertebral canal tumor resection was performed	After the operation, the patient's pain and lower limb muscle strength improved, recovered, and the patient was discharged from hospital and lived normally
March 9, 2013	Low back pain with numbness and fatigue of both lower limbs for 3 mo	Thoracolumbar intraspinal tumor resection was performed	After operation, the symptoms did not improve, and the muscle strength of both lower limbs gradually decreased, accompanied by persistent pain in chest, waist and both lower limbs
August 2, 2013	Persistent pain in chest, waist and lower limbs for 5 mo	Thoracic and lumbar posterior tumor resection + pelvic anterior tumor resection + thoracoscopic thoracic tumor resection wereperformed	The pain and muscle strength were improved after the operation, which could meet the needs of his daily life
October 17, 2018	Repeated neck and upper limb pain for 2 mo with paraplegia of both lower limbs	Posterior cervical spinal cord tumor resection	After the operation, his neck and upper limb pain was relieved, but the muscle strength of lower limbs was not significantly improved. After the neck incision had healed, the patient was discharged from hospital

Table 2 Diagnosis of schwannomatosis based on molecular and/or clinical diagnostic criteria according to Plotkin et al[18]

Clinical diagnosis	Combined molecular and clinical diagnosis	Exclusion criteria
≥ 2 nonintradermal schwannomas, 1 pathologically confirmed schwannoma and absence of bilateral vestibular schwannomas Or 1 pathologically confirmed schwannoma or intracranial meningioma and 1 affected first-degree relative	≥ 2 pathologically confirmed schwannomas or meningiomas; ≥ 2 tumors with 22q LOH and 2 different somatic NF2 mutations Or 1 pathologically confirmed schwannoma or meningioma; Germline SMARCB1 or LZTR1 pathogenic mutation	Germline pathogenic NF2 mutation; Diagnostic criteria for NF2 fulfilled; First-degree relative with NF2; Schwannomas occur exclusively in a region of previous radiation therapy