Malignant schwannoma is an aggressive
tumor that carries a poor prognosis despite wide excision,
chemotherapy, and
radiotherapy.
Malignant schwannoma of the parapharyngeal space is an uncommon finding; to our knowledge, only four cases have been described in the literature during the past 30 years, and only one of them involved a patient who had clinical evidence of
neurofibromatosis type 1. In this article, we describe a new case of
malignant schwannoma of the parapharyngeal space in a patient who had clinical evidence of
neurofibromatosis type 1. Following resection of the
tumor and a total parotidectomy, the diagnosis was made on the basis of histology and immunohistochemistry. The patient underwent postoperative
chemotherapy with
carboplatin and UP16. However, 5 months following surgery, the
tumor recurred and metastasized. The patient was then placed on a different polychemotherapeutic regimen, which was made up of 3 g/m2 of
ifosfamide, 1.5 mg/m2 of
vincristine, and 1.5 mg/m2 of
doxorubicin (IVA2 protocol). The IVA2 regimen slowed
tumor growth, but 13 months after the initiation of
therapy, the patient died of neoplastic
cachexia. Although
chemotherapy is generally ineffective in most cases of
malignant schwannoma, we did experience some positive results with the IVA2 protocol. Therefore, we recommend that this combination be considered as a first-line adjuvant
therapy following surgery or as a first-line
therapy for patients with inoperable
tumors.