• Journal of Korean Neurosurgical Society
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• v.30 no.sup1
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• pp.140-143
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• 2001

We report a case of huge trigeminal schwannoma in a 10-year-old boy with neurofibromatosis type II, extending into the three spaces of the left middle, posterior, and infratemporal fossa. Initially we thought the child had a solitary trigeminal schwannoma, and most of the mass was resected successfully through one-stage operation, cranio-orbito-zygomatic intradural approach. However during the follow-up of the patient we found radiographically other multiple intracranial tumors of bilateral acoustic schwannomas, right trigeminal schwannoma, and foramen magnum tumor. Eventually the patient was diagnosed as neurofibromatosis type II presenting multiple intracranial tumors. We think childhood trigeminal schwannoma, even though in the case of solitary tumor, should be considered as possible initial manifestation of neurofibromatosis type II and that careful follow-up for the possibility of occurrence of other brain tumors such as schwannomas or meningiomas is necessary.

• Korean Journal of Head & Neck Oncology
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• v.39 no.2
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• pp.45-48
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• 2023

Malignant peripheral nerve sheath tumor (MPNST) is one of the soft tissue sarcoma believed to originate from neural crest cells. The patients with neurofibromatosis type I (NF1) have about 8-13% of the lifetime risk of the malignant transformation. Neurofibroma on patients with NF1 can be surgically resected and has good prognosis if approach to the tumor is possible. We experienced a case of a 50-year-old woman with NF who had incompletely resected neurofibroma, which presented a rapid malignant transformation to MPNST, 3 months after the first surgery. We reported this case with a brief review of literature.

• Archives of Plastic Surgery
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• v.33 no.6
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• pp.761-763
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• 2006

Purpose: Malignant peripheral nerve sheath tumors most often arise from the anatomically discernible peripheral nerve or neurofibroma. Methods: A 55-year-old man had a rapidly growing pedunculated large mass on the sacrolumbar junction for 2 years. He has congenital neurofibromatosis type I. He had multiple caf-au-lait spots and multiple neurofibromas on the entire body. The mass developed from a subcutaneous nodule on the sacrolumbar junction and grew rapidly. The preoperative punch biopsy revealed a malignant peripheral nerve tumor. The mass was completely excised with 1 cm free margin above the deep fascial plane. Results: There was no evidence of recurrence of tumor for 19 months of follow-up examination. Conclusion: Malignant peripheral nerve sheath tumor is very rare and has unique feature. We report a successful case of malignant peripheral nerve sheath tumor with the review of the literatures.

• Journal of Chest Surgery
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• v.36 no.3
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• pp.175-181
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• 2003

Primary chest wall tumors originate from soft tissue, bone or cartilage of the chest wall and it comprises 1∼2% of all primary tumors. Resection of tumor is often indicated for chronic ulceration or pain, and long-term survival might be achieved after surgery depending on the histology and the surgical procedure. Material and Method: Retrospective study of 125 primary chest wall tumors (86 benign, 39 malignant) operated between Sep. 1976 to Mar 2001 were reviewed and their clinical outcomes were analyzed. Follow-up data were collected at the outpatient clinic. Result: All patients with benign tumors were treated by excision without recurrence or death, and most malignancies were treated by wide resection. Malignant fibrous histiocytoma and chondrosarcoma constituted 46.2% of the malignant neoplasm. There was no operative death. The overall 3-year survival for patients with primary malignant neoplasm was 76.0%, and the 10-year survival was 60.5%. All deaths were disease-related and the tumor recurred in 11 patients. There was no significant difference in survival between patients with resection margins less than 4 cm and those with resection margins greater than 4 cm. Conclusion: Chest wall resection offers excellent results for benign chest wall tumors and substantial long-term survival for malignant diseases. Safe resection margin of 4 cm or more did not correlate with the survival rate although the tumor recurrence correlated with poor survival.