2002 CTOS Annual Meeting Posters — Pathology

A PREVIOUSLY UNREPORTED CASE OF BONE METASTASIS IN MALIGNANT PERIPHERAL NERVE SHEATH TUMOR IN A PATIENT WITH TYPE I NEUROFIBROMATOSIS (VON RECKLINGHAUSEN’S DISEASE)
[Abstract ID: 60]

Category: Pathology

Authors: John Christopher Herzog¹, Manjula Bansal², Yehuda Kleinman¹, Timothy Shane Shaw¹, John R. Denton¹

Author Institutions: ¹Saint Vincents Catholic Medical Centers, NY, United States; ²Hospital for Special Surgery, NY, United States

Presenter: John Christopher Herzog
JHerzog@pol.net

Correspondent: John Christopher Herzog
JHerzog@pol.net
Elmhurst NY United States 11373
Ph: 718 639 0969
Fax: 718 639 4433

Objectives: Malignant peripheral nerve sheath tumors arise directly from nerves or from pre-existing neurofibromas. Histologically, MPNST appears as a spindle cell sarcoma. Frequently they occur in the proximal area of the extremities, and commonly involve the brachial plexus or sciatic nerve. Patients with neurofibromatosis are at increased risk for malignant degeneration which occurs in up to 5-30% of cases. A painful mass arising in a patient with neurofibromatosis warrants careful consideration. It may represent a malignant peripheral nerve sheath tumor. Although metastases do occur, no cases have been reported with metastasis to bone.

Methods: This is a case report of a 37 year old male with Type I Neurofibromatosis who presented with a painful right groin mass arising from the femoral nerve in the proximal thigh. A biopsy, followed by excision was performed which revealed a large 10cm x 7 cm mass with histology consistent with a MPNST which was confirmed with immunohistochemical stains. The patient later developed a large mass on his sternum, and began to have pelvic pain. Radiographic studies confimed multiple bony metastases including the sternum, spine, pelvis, as well as a pathologic fracture of the intertrochanteric area of the right hip which was treated surgically. Specimen obtained during the open reduction and internal fixation of the hip revealed a similar lesion of MPNST.

Results: This appears to be the first case of a malignant peripheral nerve sheath tumor with metastasis to bone.

Conclusions: Clinicians should be alert that bony metastasis is possible in patients with malignant peripheral nerve sheath tumors.