Neurofibromatosis type 1 (NF-1, von Recklinghausen disease) is included in the group of diseases known as phacomatosis. Clinical diagnosis of Recklinghausen disease is based on the presence of 2 from 7 criteria developed by a panel of expert. So far effective strategy of treatment of patients with NF-1 is not developed. One method of treatment are surgical excisions. The aim of the study was to present own experience of treatment of patients with giant tumors in the course of Recklinghausen disease. Material and methods. The Department of Plastic, Reconstructive and Aesthetic Surgery of Medical University in Łódź has under care 34 patients with NF-1. Fifteen patients (8 females and 7 males) with giant tumors were included in this analysis. The patients age was from 6 to 57 years. In analyzed group a retrospective review of surgical treatment was carried out on the basis of patients’ medical documentation. Results. Ten from 15 analyzed patients were operated on in our Department and performed surgeries were aimed to partially reduce the tumors mass. Only one patient had his tumor excised in one-stage procedure, in rest of the patients the lesions were too extensive. Most patients in histopathological examinations were diagnosed with neurofibroma (8 patients), 1 - neurofibromaplexiforme and 1 - neurofibrolipoma. Conclusions. Unpredictable course of Recklinghausen disease causes that follow-up examinations are necessary. Treatment of such patients is particularly indicated when the tumor mass causes difficulty in patients daily habits or is life-threatening. Also it is worth to remember that delayed decision of surgical treatment unable excision of the whole tumor in one-stage procedure. This in turn causes the risk of tumor re-growing and/or developing of malignancy
In August 2003, a 15 year old male, with a giant tumor located on the whole left side of his face, was admitted for treatment. The changes in appearance first began to show in the 2nd month after birth and systematic enlargement lead to an overgrowth of the soft tissues and bones of the face. As a result of the pressure excerted by the tumor, the frontal bone and the surrounding protective structures became thickened and displaced posterio-inferiorly, causing a shrinking of the anterio-inferior part of the skull. Histological analysis indicated that the tumor was of neurological origin. The patient was operated on a year later because of the continued growth of the tumor. Another year later, the enlarged and protruding maxillar bone was filed down.A radical approach is important in such cases, not only from an aesthetic point of view, but most of all because it is the best way of counteracting any regrowth of the tumour and is the most effective means of oncological prophylaxis.
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