J Korean Assoc Oral Maxillofac Surg.  2004 Jun;30(3):228-233.

Malignant peripheral nerve sheath tumor on palate: A case report

• Kwon MS
• Lee HS
• Kim HC
• Ko SO
• Shin HK

Abstract

SUMMARY: The malignant peripheral nerve sheath tumor(MPNST) is an aggressive neoplasm and can either arise independently or result from malignant change in preexisting neurofibromatosis (von Recklinghausen's disease). Its histologic characteristics remain controversial, but currently it is believed that the schwann cell is the origin of the peripheral nerve sheath tumors. MPNST is an uncommon neoplasm of the head and neck region, and its presentation in the oral cavity is quite rare. In this study, we report a patient with a rare case of a MPNST involving the maxilla. A CASE REPORT: A 29-year-old female presented with a chief complaint of painless swelling with bleeding tendency on the left maxillary tuberosity area 2 months ago. Clinical examination showed a 5.0*3.0 cm2 sized, indurative swelling on the site. Conventional radiographs showed a relatively well-defined soft tissue mass involving the left maxillary sinus, and destruction of the anterior, posterolateral walls of the left maxillary sinus. Subtotal maxillectomy and split-thickness skin graft from thigh were undertaken. In histochemical and immunohistochemical studies, the specimen revealed positive reactivities to Vimentin and S-100 protein. Final diagnosis was made as MPNST.

Keyword

Malignant peripheral nerve sheath tumor; MPNST; Subtotal maxillectomy

MeSH Terms

Adult
Diagnosis
Female
Head
Hemorrhage
Humans
Maxilla
Maxillary Sinus
Mouth
Neck
Nerve Sheath Neoplasms
Neurilemmoma
Neurofibromatoses
Palate*
Peripheral Nerves*
S100 Proteins
Skin
Thigh
Transplants
Vimentin
S100 Proteins
Vimentin