Artikel
Neurosurgical treatment of peripheral nerve sheath tumours (PNST)
Neurochirurgische Behandlung peripherer Nervenscheidentumore
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Veröffentlicht: | 8. Mai 2006 |
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Objective: PNST are rare. Estimated incidence amongst tumours of the upper extremity ranges from 1-5%. From 1/99 until 11/2005 we operated on 169 PNST at our department (72 Schwannomas, 86 Neurofibromas, 11 malignant PNST). Our series was evaluated for frequency of tumour type, clinical presentation (pain, neurological deficits), functional outcome and recurrence.
Methods: Patients that had been operated until 12/2002 with a follow-up of at least 2 years were retrospectively analysed.
Results: Schwannomas – In 32 patients and 38 operations 50 tumors were enucleated. Eight of these patients had known neurofibromatosis. Thirty tumours were painful, 6 led to paresis, and 6 produced hypaesthesia.
Neurofibromas – In 17 patients and 21 operations 51 tumours were removed. Three of these patients did not exhibit signs of systemic neurofibromatosis (spontaneous mutations). Thirteen patients exhibited preoperative pain, 2 showed paresis, one a sensory deficit.
Malignant PNST – Seven patients were operated, 2 had systemic neurofibromatosis. All patients suffered from preoperative pain, 6 had sensory deficits and/or paresis. Tumour size ranged from 3 to 15 cm. Across all types of tumour, pain was the symptom best to improve. This also applied to malignant PNST.
Among schwannoma patients, there were 15 cases with new, minor sensory deficits (5 transient, 7 improved) vs. 5 new cases with sensory deficits in the neurofibroma group (3 transient). Improvement of preoperative paresis in schwannoma- (S) and neurofibroma patients (NF) occurred in 7 of 8 cases (S: out of 6 palsies, 4 vanished, one improved; NF: out of 2 palsies one vanished, one improved).
Recurrent tumours occurred in one schwannoma- and one neurofibroma patient. All seven malignant PNST recurred; six of these patients died within 40 months.
Conclusions: Applying microsurgical techniques, most PNST can be completely removed without creating relevant, new functional deficits. In benign PNST, preoperative motor deficits improve in the postoperative course. By tumour removal pain alleviation can frequently be expected. Treatment of malignant PNST remains dismal.