A Case of Occipital Malignant Peripheral Nerve Sheath Tumor with Neurofibromatosis Type 1

The prognosis of malignant peripheral nerve sheath tumor (MPNST) with neurofibromatosis type 1 (NF-1) is worse than that of a solitary MPNST, because of the tumor size and location difficult to resect completely. We experienced a case of MPNST in the occipital region with NF-1.

A 59-year-old woman presented with NF-1 and an MPNST of the occipital region. We performed wide excision involving the occipital bone, and reconstructed with a titanium plate and a free latissimus dorsi muscle flap. Despite three operations and postoperative radiotherapy, the tumor locally recurred after each surgery; further complicated by lung and mediastinal metastasis. Adjuvant therapy was insufficient to control local recurrence, which was observed intracranially, resulting in sagittal sinus invasion. Thirty months after the initial surgery, the patient died of respiratory failure due to lung metastasis.

MPNST with NF-1 has poorer prognosis than that of a solitary lesion. Recently, it is reported in several literature that combination therapy with surgery and radiotherapy improve survival rates. But as we presented, when the complete local resection with free margin could not achieved due to the size and anatomical location of the tumor, the effectiveness of radiotherapy is not sufficient to control local recurrence.