PMC3173921_01
(a) Cafe-au-lait pigmentation on stomach area. (b) Cafe-au-lait pigmentation on the trunk area
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F1
Multiple "Cafe-au-lait" spots were seen in the trunk region [Figure 1].
Mild diffuse facial swelling on the right side of the face
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F2
Extraorally, a solitary, diffuse, smooth-surfaced swelling was present on the right side of the face, which extended anterioposteriorly from cheek to temple area [Figure 2].
Intraoral swelling involving the right maxilla
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F3
Intraorally, a solitary, well-defined ovoid swelling measuring 6 3 cm was present on the right side, extending from the distal aspect of upper right canine to alveolar ridge in relation to the unerupted upper right second molar, and from upper buccal sulcus up to 0.5 cm from the midline of palate [Figure 3].
OPG revealing thinning of coronoid process, highly marked coronoid notch and hypoplastic condyle on the right side
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F4
Orthopantomogram (OPG) revealed thinning of coronoid process, highly marked coronoid notch and hypoplastic condyle on the right side [Figure 4].
(a) CT scan showing massive tumor of maxilla. (b) CT scan showing tumor extending upward toward the base of the skull
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F5
A contrast-enhanced computed tomography (CT) impression was that of a large plexiform neurofibroma within the right masticatory space, extending into right orbit, infratemporal and middle cranial fossa [Figure 5].
PMC3173921_02
Photomicrograph showing epithelium and connective tissue occupied by a lesion of neural origin (H and E, x4)
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F6
Biopsy revealed oral mucosa covered with stratified squamous epithelium and underlying dense connective tissue which was occupied by a lesion of neural origin [Figure 6].
Photomicrograph showing compact nerve bundles in the stroma (H and E, x4X). (b) Nerve bundle interspersed in the fibrous tissue (H and E, x10X)
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F7
The neural bundles were made up of spindle-shaped cells with wavy nuclei suspended in a delicate background of collagen fibers [Figure 7].
(a) Photomicrograph showing S-100 positivity in the neural cells (HRP, x10X). (b) S-100 positivity in the neural bundle (HRP, x10X)
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F8
An immunohistochemical test for the S-100 protein was positive in 60% of the cells (detection system used was the HRP polymer), confirming the neural origin of the tumor [Figure 8].