Cutaneous Neurofibromas
Conditions
Keywords
Neurofibromatosis Type 1, Morbus Recklinghausen, cutaneous neurofibromas, Erbium-YAG-Laser, Vaporisation, Thermal Necrosis
Brief summary
The purpose of this study is to determine whether multiple cutaneous neurofibromas in patients with neurofibromatosis type 1 can be removed with an erbium-YAG-laser.
Detailed description
With a prevalence of 1 in 3-5000 births, neurofibromatosis Type I is one of the most common genetic defects. The mode of inheritance is autosomal dominant and affects a gene (NF1), which is responsible for the production of the tumor suppressor protein neurofibromin. The consequence is an uninhibited expansion of neural tissue which leads amongst others to cosmetic disfigurement of the patients. In comparison to the plexiform neurofibromas the cutaneous tumors do not undergo malignant transformation. Excision and CO2 laser vaporisation were established as standard treatment but cause unattractive scars. In one operation more than 500 neurofibromas can be removed with an Erbium: YAG laser (2940 nm). The tumors are dissected by shooting holes into the skin and vaporising the neurofibromas in-between or underneath. Specimen are harvested after Er: YAG-, CO2 - and electrosurgical treatment to evaluate the difference of thermal necrosis histologically and photographs are taken to assess the cosmetic results.
Interventions
PROCEDUREErbium-YAG laser vaporization
spot size 2-5 mm diameter, applying about 1,2 -2 J at a frequency of 6-12 Hz
Sponsors
Medical University of Vienna
Study design
Allocation
NA
Intervention model
SINGLE_GROUP
Primary purpose
TREATMENT
Masking
NONE
Eligibility
Sex/Gender
ALL
Healthy volunteers
No
Inclusion criteria
* Patients with multiple cutaneous neurofibromas with the request for tumor removal
Exclusion criteria
* Pregnancy
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| The aim of this study is to enhance the quality of life (measured by the Nottingham Health Profile the Rosenberg's Self-Esteem Scale and the SF-36 health survey) of patients suffering from disfigurement due to thousands of cutaneous neurofibromas. | 6 months | The standardized questionnaires are done before and 6 month after the operation in which the tight tumor cluster are phased down and thereby an almost normal skin appearance is achieved. |
Secondary
| Measure | Time frame |
|---|---|
| With this new method we want to decrease the painfulness, accelerated the time till wound healing as well as reduce the stay in hospital and the incidence of side effects. | 6 months |
Countries
Austria
Contacts
Primary ContactWolfgang Happak, Prof. MD
Backup ContactLukas Kriechbaumer, MD
Outcome results
None listed