Plexiform neurofibromatosis of the spine

Case contributed by Bahman Rasuli
Diagnosis certain


A known patient with cutaneous cafe au lait patches underwent thoracic spine surgery for removal of the huge left paraspinal mass.

Patient Data

Age: 28 years
Gender: Female

 Severe spinal deformity as kyphoscoliosis of the thoracolumbar spine

Opacity within the heart shadow due to posterior thoracic mass.

Multiple bilateral neurofibromas in the thoracic spinal canal, in the neural foramina and paravertebral regions dominantly on the left side

Numerous similar small intramuscular and subcutaneous plexiform neurofibroma on the left side

Most of the lesions show hypointense signal on T1, high signals on T2 with central hypointense central foci giving target appearance.

After surgical resection


Surgical resection of most parts of the left thoracic paraspinal and intramuscular mass

Fluid equivalent signal rim enhancing area along with left posterior thoracic region at the site of surgery

Left-sided pleural effusion.

Pathology proved the case of plexiform neurofibroma associated with neurofibromatosis type I.

Case Discussion

A case of neurofibromatosis type I with plexiform neurofibromas of the spine, kyphoscoliosis, and cafe au lait spots.

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