Segmental Schwannomatosis of the Spine: Report of a Rare Case and Brief Review of Literature.

UNLABELLED: To report a case of segmental schwannomatosis involving the dorsal and lumbar spine and describe its excision as well as review of literature on schwannomatosis involving the spine.

SUMMARY OF BACKGROUND DATA: Schwannomas are nerve sheath tumours which usually occur as solitary lesions. Presence of multiple schwannomas suggests a genetic predisposition to tumorogenesis and possible association with neurofibromatosis. However, in very rare cases multiple schwannomas exist without typical features of neurofibromatosis and constitute a clinically and genetically distinct rare syndrome termed schwannomatosis. A 31-year-old female presented with low back pain with left lower limb radiculopathy and sensory deficit over the L4-L5 dermatome. Auditory and ophthalmologic examinations were normal. MRI showed two discrete intradural masses at D12-L2 and L3-L5. MRI of the brain was negative for any vestibular schwannoma. The tumours were excised discretely through a single midline incision to improve the symptoms. HPE of both the tumours revealed them to be schwannomas. Karyotyping from lymphocyte DNA revealed no abnormality.

CONCLUSION: This is the 3rd case of schwannomatosis involving the dorsal and lumbar spine, in which excision of the tumours led to resolution of symptoms.