{"title":"神经纤维瘤病的一种不寻常表现-外阴肿块1例报告","authors":"Shivraj M. Ingole, Ayantika Maity","doi":"10.25259/crcr_54_2023","DOIUrl":null,"url":null,"abstract":"Neurofibromatosis (NF) of the female genitalia is an uncommon manifestation. Selective involvement of the labium majus is quite rare. We describe a rare manifestation of a vulval plexiform neurofibroma without clitoral involvement in a post-pubertal girl. The patient had multiple café-au-lait spots over the neck, back, buttocks and thighs, a patch of pigmentation over the pelvis and inguinal region, axillary freckling, and a large hanging left vulval mass. On ultrasound, it showed hypoechoic nodules on a hyperechoic background with significant vascularity. Magnetic resonance imaging revealed T1-weighted (T1W) hypointense and T2-weighted (T2W) hyperintense, diffuse infiltrating lesion with few T2W hyperintense tubular and nodular areas, presence of “target sign,” and intense post-contrast enhancement. A biopsy confirmed it to be neurofibroma. The patient was diagnosed with NF type 1 and underwent surgical resection.","PeriodicalId":419021,"journal":{"name":"Case Reports in Clinical Radiology","volume":"65 1","pages":"0"},"PeriodicalIF":0.0000,"publicationDate":"2023-06-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"An unusual manifestation of neurofibromatosis – A case report of a vulval mass\",\"authors\":\"Shivraj M. Ingole, Ayantika Maity\",\"doi\":\"10.25259/crcr_54_2023\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"Neurofibromatosis (NF) of the female genitalia is an uncommon manifestation. Selective involvement of the labium majus is quite rare. We describe a rare manifestation of a vulval plexiform neurofibroma without clitoral involvement in a post-pubertal girl. The patient had multiple café-au-lait spots over the neck, back, buttocks and thighs, a patch of pigmentation over the pelvis and inguinal region, axillary freckling, and a large hanging left vulval mass. On ultrasound, it showed hypoechoic nodules on a hyperechoic background with significant vascularity. Magnetic resonance imaging revealed T1-weighted (T1W) hypointense and T2-weighted (T2W) hyperintense, diffuse infiltrating lesion with few T2W hyperintense tubular and nodular areas, presence of “target sign,” and intense post-contrast enhancement. A biopsy confirmed it to be neurofibroma. The patient was diagnosed with NF type 1 and underwent surgical resection.\",\"PeriodicalId\":419021,\"journal\":{\"name\":\"Case Reports in Clinical Radiology\",\"volume\":\"65 1\",\"pages\":\"0\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2023-06-20\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Case Reports in Clinical Radiology\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.25259/crcr_54_2023\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"\",\"JCRName\":\"\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Case Reports in Clinical Radiology","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.25259/crcr_54_2023","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
An unusual manifestation of neurofibromatosis – A case report of a vulval mass
Neurofibromatosis (NF) of the female genitalia is an uncommon manifestation. Selective involvement of the labium majus is quite rare. We describe a rare manifestation of a vulval plexiform neurofibroma without clitoral involvement in a post-pubertal girl. The patient had multiple café-au-lait spots over the neck, back, buttocks and thighs, a patch of pigmentation over the pelvis and inguinal region, axillary freckling, and a large hanging left vulval mass. On ultrasound, it showed hypoechoic nodules on a hyperechoic background with significant vascularity. Magnetic resonance imaging revealed T1-weighted (T1W) hypointense and T2-weighted (T2W) hyperintense, diffuse infiltrating lesion with few T2W hyperintense tubular and nodular areas, presence of “target sign,” and intense post-contrast enhancement. A biopsy confirmed it to be neurofibroma. The patient was diagnosed with NF type 1 and underwent surgical resection.