PMC4960925_01
From left to right: Cafe-au-lait spot in the right upper limb; neurofibromatosis multiple nodules in the lumbar region; neurofibromatosis nodules in the abdomen
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F1
Upon first checkup, the patient had several cafe-au-lait spots in variable sizes, larger than 15 mm, and several neurofibromas, in particular on his thorax, arms, and face [Figure 1].
(a) Sagittal section: Herniation of the cerebellar tonsils of 4 mm; (b) Sagittal section: signs of cervical- thoracic syringomyelia; (c) axial section: Spinal cord syringomyelia; (d) axial section: Tonsillar herniation
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F2
Although there were no changes in the CT of the skull, the MRI showed hydro/syringomyelia in the cervical spine area [Figure 2].
Surgical incision: Occipital craniotomy
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F3
In this case, the patient went to a subocciptal craniectomy with duroplasty and partial laminectomy of c2, along with tonsillectomy and expansion of cistern magna due to intense arachnoid thickening and obliteration of the obex by the cerebellar tonsils [Figure 3].
Histology: Hematoxylin-and-eosin stained sections show a dermal proliferation of spindle cells with wavy nuclei and inconspicuous nucleoli; presence of neurofibromas confirms the diagnosis
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F4
The histology confirmed neurofibromas [Figure 4].