Clinical Retrospective Study Of Neurofibromatosis Type 1

Object:Neurofibromatosis type? is a disease involving the skin,muscles,peripheral nervous system and even the central nervous system,visceral system and other systemic diseases.Patients mostly come for treatment with peripheral nervous system fibroma,recurrence is easy after operation and have a certain genetic tendency.We now summarize the medical records of NF1 patients in the department of hand and foot surgery in the first Hospital of Jilin University and make a retrospective clinical study on the pathogenesis,imaging examination,clinical manifestation,treatment and outcome of the disease was carried out in combination with domestic and foreign literature,to investigate the effect of surgical resection range and tumor nature on the recurrence rate of NF1 patients after resection.Methods:Clinical data of 36 patients with NFI treated in the frist hospital of jilin university' hand and foot surgery department and the branch courts' hand and foot surgery department from March 2007 to March 2017 were selected.These involves 17 males and 19 females;16 sporadic cases and 20 withf amily medical history;aged from 0.6-70 years old,with an average age of 35.9 years old.All the patients had clinical symptoms caused by peripheral nervous system tumors and had been treated with surgical indications,the routine examination before operation,ultrasound X-ray of tumor and MRI if necessary were all perfect.According to the scope of surgical resection recorded in the medical records,the patients were divided into simple resection group and extended resection group,according to postoperative tumor pathology,the patients were divided into neurofibroma group and(Malignant peripheral nerve sheath tumor,MPNST)group,and then spss software was used to test the accuracy of Fisher in 32 patients who received effective follow-up on the difference of the effect of surgical resection range and tumor nature on the recurrence rate.Results:36 patients underwent tumor resection,32 cases have effective follow-up after operation,4 people lost their follow-up(three of them were malignant.),the follow-up rate is 88.9%.Of the 32 patients with NFI who were followed up effectively,23 cases underwent direct closure of the wound after resection and 5 cases received free skin grafting after resection.4 cases underwent skin flap transfer after resection of the tumor,all patients had good appearance after operation.Of the 14 patients with neurological impairment,12 had sensory recovery,which could meet the needs of daily life,the function of tumor resection in 2 cases was not as good as that before operation because of repeated resection.Of 29 cases with neurofibroma,18 cases were treated with simple resection of tumor,11 cases were treated with extended excision and 3 cases with MPNST were treated with expanded resection.Twenty-nine patients with neurofibroma were divided into groups according to the range of surgical resection,then SPSS software was used to detect the recurrence rate of two kinds of resection areas by Fisher exact probability method,P > 0.05,with no statistical significance.The patients with neurofibroma who were also treated with extended resection and those with MPNST were grouped according to the nature of the tumor,among them,there were 11 cases of neurofibroma group;2 cases of recurrence;3 cases of group MPNST;3 cases of recurrence,then using spss software to test the postoperative recurrence rate of two groups of tumors with different nature by Fisher accurate probability method,P < 0.05,with statistical significance.Conclusions:Neurofibromatosis type? combined with peripheral neurofibroma is the most important factor for patients to come to see the doctor;imaging examination has an important guiding role in the diagnosis,classification and treatment of NF1;surgical treatment is the most important treatment for NF1 at present,excellent microsurgical techniques and suitable repair methods after tumor resection can make the patients obtain satisfactory appearance and function;there is no difference in the local recurrence rate of NF1 patients with tumor operation due to the difference of surgical resection range(simple resection and extended resection);the difference of the tumor nature affected the recurrence rate of the patients with NF1.