Yonsei Med J.  2006 Apr;47(2):264-270. 10.3349/ymj.2006.47.2.264.

Intraosseous Nerve Sheath Tumors in the Jaws

• Che Z
• Nam W
• Park WS
• Kim HJ
• Cha IH
• Kim HS
• Yook JI
• Kim J
• Lee SH

• KMID: 1110753
• DOI: http://doi.org/10.3349/ymj.2006.47.2.264

Abstract

Although the head and neck region is recognized as the most common location for peripheral nerve sheath tumors, central involvement, particularly in the jaw bones, is quite unusual. Neurofibroma is one of the most common nerve sheath tumors occurring in the soft tissue and generally appears in neurofibromatosis 1 (NF1 or von Recklinghausen's disease). Malignant peripheral nerve sheath tumors (MPNSTs) are uncommon sarcomas that almost always arise in the soft tissue. Here, we report four cases of intraosseous peripheral nerve sheath tumors occurring in the jaw bones and compare the clinical, radiologic, and pathologic findings in order to make a differential diagnosis.

Keyword

Nerve sheath tumor; malignant peripheral nerve sheath tumor (MPNST); jaw; neurofibroma; neurofibromatosis

MeSH Terms

X-Rays
Sarcoma/*diagnosis
Neurofibromatoses/pathology
Neurofibroma/*pathology
Nerve Sheath Neoplasms/*diagnosis
Male
Jaw/*radiography
Humans
Female
Diagnosis, Differential
Child
Bone Neoplasms/*diagnosis
Adult
Adolescent

Figure

• 

  Fig. 1 Preoperative panoramic images (A) and CT scan (B, and C) show the expansile radiolucent intraosseous lesions in the mandible (indicated by arrows in A & B) and maxilla (indicated by arrow heads in A & C). The elongated tumor cells in the interlacing bundles with surrounding collagen bundles can be seen in H-E staining (D) and they are positive for the S-100 protein (E).

• 

  Fig. 2 Preoperative panoramic (A), CT scan (B) and dental periapical image (C) shows an ill-defined radiolucent lesion (indicated by arrows) in the mandible with root resorption of the adjacent tooth. The tumor cells do not show a palisading arrangement of the nuclei (D and E). However, there are multinucleated giant cells (indicated by arrow heads) with abnormal mitosis (indicated by arrow).

• 

  Fig. 3 A clinical intraoral picture (A) shows a bony depression, gingival recession and root exposure (indicated by arrow) on the right maxillary incisors. A dental radiographic image (B) and maxillary CT (C) reveal an extensive osseous defect and external root resorption of the right maxillary incisors and canine. The elongated tumor cells in the interlacing bundles with surrounding collagen bundles and multinucleated giant cells with abnormal mitoses are seen in H-E staining (D) and they are positive for S-100 protein (E).

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