Treatment of patients with giant tumors in the course of Recklinghausen disease - own experience.

UNLABELLED: 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.