Clinical Analysis Of 34 Cases Of Extramammary Paget’s Disease And A Case Of Giant Neurofibroma

Background:Extramammary Paget’s disease is a rare skin malignant tumor.It originates from the skin or skin appendages.It often occurs in areas with abundant distribution of apocrine sweat glands,such as vulva,perianal,scrotum,penis.A few cases occur in groin,axilla,anterior chest,buttock,abdomen and so on.Most of them occur in men over 50 years old.Its clinical manifestations are not typical.Early feature are erythema,pruritus,pain and so on.The rate of misdiagnosis is high.Clinicians usually do not know enough about the disease and early diagnosis is relatively difficult,so the treatment was deleyed.Currently,the main treatment method is surgical resection,but the recurrence rate after resection is high,and the prognosis of the disease is not clear.Objective:To analyze and summarize the basic information,clinical characteristics,diagnosis and treatment of extramammary Paget’s disease,and to explore the related factors on the prognosis.Method:The clinical data of 34 patients with extramammary Paget’s disease diagnosed by pathology in Qilu Hospital of Shandong University from 2010.01 to 2019.01 were reviewed.The clinical characteristics were summarized,and the relationship between each factor and prognosis was analyzed statistically.Result:A total of 34 patients,aged range from 30 to 86 years,with an average age of 63.88.26 males(average age of 66.81)and 8 females(average age of 54.38)were included in the study.Among the male patients,22 lesions occurred in the penis and scrotum,4 in the perianal region,and female patients only have vulva involvement.Among 34 patients,the smallest lesion area was 0.5 cm2,the largest lesion area was 150 cm2,and the average lesion area was 29.35cm2.Early clinical manifestations were erythema and pruritus,including 27(79.41%)case of erythema,31(91.18%)cases of pruritus,11(32.35%)cases of ulceration and exudation,3(8.82%)case of repeated scabbing,2(5.88%)cases of "Desquamation",and 1(2.94%)cases of verrucous hyperplasia.All patients underwent extended resection to the subcutaneous adipose layer.Postoperative pathology showed that tumor cells were confined to the epidermis in 10 cases and involved the dermis in 14 cases.Among the patients with dermal cortex involvement,7 cases involved subdermal sweat glands and hair follicle tissue,2 cases were associated with sweat ductal carcinoma,and 1 case was associated with poorly differentiated adenocarcinoma.The mean follow-up time was 42.06 months,with 8 cases of recurrence and 9 cases of death.The overall 5-year survival rate was 64.3%,and the 5-year survival rate was reduced in patients with disease duration longer than 24 months(P=0.047)and disease size greater than 20cm2(P=0.005).Tumor cells with lesion area greater than 20cm2(P=0.008)in patients were more likely to involve the dermis,and tumor cells involving the dermis were more likely to relapse than those confined to the epidermis(P=0.045),with a higher mortality rate(P=0.025).Conclusion:Extramammary Paget’s disease is more common in elderly men,often involving the penis,scrotum,early manifestations to skin erythema with pruritus,tumor cells involved in the dermis than limited to the epidermis are more likely to relapse,poor prognosis.The 5-year survival rate of patients with the course of disease over 24 months and the lesion greater than 20cm2 was reduced.Patients suspected of the disease were confirmed by pathological biopsy as soon as possible,and early diagnosis and treatment were achieved.Background:Neurofibroma is a benign peripheral neurogenic tumor,which can be divided into isolated and multiple.Systemic multiple neurofibromatosis is called neurofibromatosis,and can be divided into type Ⅰ(NF1)and type Ⅱ(NF2).NF1 is one of the common diseases in plastic surgery,while NF1 with giant neurofibroma is relatively rare.Large tumors droop for a long time,often appear as sacks with ulceration and bleeding,which hinders the function of adjacent tissues and organs.It not only becomes a psychological burden of patients,but also seriously affects the normal life of patients.Surgical resection is still the most effective treatment for this disease,but due to the specificity of the tumor tissue,it is difficult to hemostasis and the amount of bleeding is abundant.Serious cases can threaten life.Controlling the amount of bleeding during operation is still a difficult problem.Objective:To explore the diagnosis of NF1 with giant neurofibroma and the feasibility of comprehensive treatment for the control of intraoperative hemorrhage volume.Method:Retrospectively analyzing a case of NF1 with giant neurofibroma involving the right lower extremity,buttock and vulva that was diagnosised and treated in November 2018.Result:Combined with the patient’s medical history,physical examination,imaging examination and pathological findings,the diagnosis of NF1 with giant neurofibroma was established.The main nutrient arteries were embolized by angiography 3 days before the operation,and the amount of hemorrhage in the course of treatment was significantly reduced by the operation combined with the circumferential ligation of the pedicle of the tumor.Up to now,there is no recurrence of the tumors,and the shape of the buttocks,right lower limbs and vulva has been improved.Conclusion:Preoperative interventional embolization of nutrient vessels and intraoperative circumferential ligation of the pedicle of the tumor can achieve ideal hemorrhage control effect and improve the operation efficiency and quality of life of patients.Two or more comprehensive hemostasis measures can be generalized.Regularly visit and strengthen health education for patients should be carried out in the later period.