Diagnosis,Surgical Treatment And Literature Analysis Of A Case Of Type Ⅰ Neurofibroma

Background: Type I Neurofibromatosis(NF1)is an autosomal genetic disease with systemic manifestations caused by abnormal changes in the neuroectodermal lobes,The clinical variability is very high and instability.The main clinical manifestations were neurofibroma,Lisch nodules,milk-coffee spots and abnormal pigmentation.With the iterative update of genetics and multidisciplinary surgical treatment,the methods of diagnosis and treatment of neurofibromatosis have been diversified,but systematic retrospective analysis of the disease is still rare.Objective: This paper intends to retrospectively analyze the etiology,clinical manifestations,preoperative and postoperative diagnostic modes,treatment methods and epidemiology of NF1 by recording the diagnosis and treatment process of a typical case of large type I neurofibroma,so as to provide reliable experience for clinicians in diagnosis and surgical treatment.Methods: According to the case specifications of the First Affiliated Hospital of Guangxi Medical University,a total of 11 months from May 2020 to April 2021 were collected and followed up for 1 patient with extremely large NF1.The preoperative and postoperative ultrasound,CT and MRI examinations were completed,and the size,location,degree of invasion and pathology of the tumor were evaluated.Finally,the patients underwent 5 stages of surgical resection of the giant NF1 tumor and the literature was analyzed.Results: After 5 stages of operation for nearly 11 months,the huge neurofibroma of buttock,back and scrotum was successfully removed.From the beginning of the patient was bedridden and unable to walk normally to the end of the patient is able to walk normally.We retained bilateral testicles during the operation,and the patient’s skin incisions healed well.The patient’s mental satisfaction was high,which greatly improved the patient’s quality of life.The patients were followed up for nearly 11 months without recurrence.Conclusion: 1.The clinical manifestations of NF1 have a high degree of variation,presenting different symptoms in different periods,with time specificity.Early diagnosis,early detection and early treatment are the most effective intervention measures for the treatment of NF1,which can avoid aggravation and deterioration.2.In the auxiliary examination and diagnosis of NF1,gene diagnosis technology is the most reliable,and MRI has the best applicability in imaging examination,which is better than CT and ultrasound examination;3.In the pathological diagnosis of NF1,Ag staining of nerve fibers supported the highest diagnostic support under the premise of histomorphology,but the positive rate was low,followed by Ki-67#(+1%),and finally positive for Vimentin and S-100.4.The improvement of the preoperative multidisciplinary diagnosis and treatment plan for NF1 surgery can greatly avoid the surgical design and the risk of intraoperative and postoperative complications;5.During surgical resection of NF1,we can make full use of the advantage of normal tissue to stop bleeding.The use of anatomical layers to bypass the tumor to reduce damage,as far as possible exposed deep ligation for hemostasis,can effectively avoid the risk of intraoperative bleeding;6.After 11 months of postoperative follow-up,no recurrence was observed and the surgical effect was good.Therefore,staging surgery and multidisciplinary surgical treatment is one of the effective methods for the treatment of giant type I neurofibroma at present.7.Considering the low malignant change rate of NF1 patients,radical surgery may not be the best,and palliative surgical treatment can also be a good treatment method,on the premise of improving the quality of life.