Clinical features of and treatment strategies for primary retroperitoneal neurofibromas: clinical analysis of 7 cases / 中华普通外科杂志

ABSTRACT

Objective To investigate the clinical features,diagnosis and treatment strategies for primary retroperitoneal neurofibromas.Methods The clinical data of 7 patients with primary retroperitoneal neurofibromas admitted to Cancer Institute & Hospital,Chinese Academy of Medical Sciences,from Jan 2000 to Jul 2017,were retrospectively analyzed.Results The average age was (42 ± 11) years and six were female.6 cases were with solitary tumor and 1 case was with multiple tumors.Clinical symptoms and imaging were of no help in determining tumor type.All patients underwent surgical resection.Postoperative pathology confirmed retroperitoneal neurofibroma in all seven patients,including 1 case with neurofibromatosis type Ⅰ and retroperitoneal malignant peripheral nerve sheath tumor.On immunohistochemistry all of the tumors were S-100 protein positive.At the end of the follow-up period ranging from 14 months to 166 months,sevent patients were alive and two patients experienced tumor recurrence.The longest disease-free survival time was 166 months.Conclusion Primary retroperitoneal neurofibromas are a rare type of primary retroperitoneal tumors that require diagnosis at pathology.Clinical symptoms and imaging of primary retroperitoneal neurofibromas patients were found to be ineffective at determining tumor type.Patients had a good prognosis after tumor resection.