现代泌尿外科杂志
現代泌尿外科雜誌
현대비뇨외과잡지
Journal of Modern Urology
2015年
8期
570-573
,共4页
张立进%张士伟%赵晓智%刘光香%孟凡青%郭宏骞
張立進%張士偉%趙曉智%劉光香%孟凡青%郭宏鶱
장립진%장사위%조효지%류광향%맹범청%곽굉건
Ⅰ型神经纤维瘤病%恶性外周神经鞘瘤%恶性嗜铬细胞瘤
Ⅰ型神經纖維瘤病%噁性外週神經鞘瘤%噁性嗜鉻細胞瘤
Ⅰ형신경섬유류병%악성외주신경초류%악성기락세포류
neurofibromatosis I%malignant peripheral nerve sheath tumor%malignant pheochromocytoma
目的 探讨Ⅰ型神经纤维瘤病合并恶性外周神经鞘瘤与恶性嗜铬细胞瘤的临床、病理学特征,提高对此类疾病的诊疗认知水平.方法 回顾性分析我院收治的1例Ⅰ型神经纤维瘤病合并不同性质恶性肿瘤的临床资料,总结归纳其临床及病理特征,并结合相关文献,分析此类疾病的相关特征及治疗预后.结果 本例患者成功施行双侧腹膜后恶性肿瘤切除术,术后左侧提示恶性外周神经鞘瘤,免疫组化示:S-100(+ ),Ki-67(+ ),SMA(-),HMB45(-),右侧示恶性嗜铬细胞瘤,免疫组化示:Syn(+ ),CgA(+ ),Ki-67(+ ),皮肤结节病理提示神经纤维瘤.术后1月后给予IVP化疗方案化疗6个疗程,随访半年余,未见肿瘤复发或转移.结论 Ⅰ型神经纤维瘤病同时合并恶性外周神经鞘瘤与恶性嗜铬细胞瘤为临床十分罕见病例,恶性程度相对较高,预后较差.治疗以手术治疗为主,辅以化疗等相关治疗具有一定疗效,但具体疗效仍需相关临床随访观察.
目的 探討Ⅰ型神經纖維瘤病閤併噁性外週神經鞘瘤與噁性嗜鉻細胞瘤的臨床、病理學特徵,提高對此類疾病的診療認知水平.方法 迴顧性分析我院收治的1例Ⅰ型神經纖維瘤病閤併不同性質噁性腫瘤的臨床資料,總結歸納其臨床及病理特徵,併結閤相關文獻,分析此類疾病的相關特徵及治療預後.結果 本例患者成功施行雙側腹膜後噁性腫瘤切除術,術後左側提示噁性外週神經鞘瘤,免疫組化示:S-100(+ ),Ki-67(+ ),SMA(-),HMB45(-),右側示噁性嗜鉻細胞瘤,免疫組化示:Syn(+ ),CgA(+ ),Ki-67(+ ),皮膚結節病理提示神經纖維瘤.術後1月後給予IVP化療方案化療6箇療程,隨訪半年餘,未見腫瘤複髮或轉移.結論 Ⅰ型神經纖維瘤病同時閤併噁性外週神經鞘瘤與噁性嗜鉻細胞瘤為臨床十分罕見病例,噁性程度相對較高,預後較差.治療以手術治療為主,輔以化療等相關治療具有一定療效,但具體療效仍需相關臨床隨訪觀察.
목적 탐토Ⅰ형신경섬유류병합병악성외주신경초류여악성기락세포류적림상、병이학특정,제고대차류질병적진료인지수평.방법 회고성분석아원수치적1례Ⅰ형신경섬유류병합병불동성질악성종류적림상자료,총결귀납기림상급병리특정,병결합상관문헌,분석차류질병적상관특정급치료예후.결과 본례환자성공시행쌍측복막후악성종류절제술,술후좌측제시악성외주신경초류,면역조화시:S-100(+ ),Ki-67(+ ),SMA(-),HMB45(-),우측시악성기락세포류,면역조화시:Syn(+ ),CgA(+ ),Ki-67(+ ),피부결절병리제시신경섬유류.술후1월후급여IVP화료방안화료6개료정,수방반년여,미견종류복발혹전이.결론 Ⅰ형신경섬유류병동시합병악성외주신경초류여악성기락세포류위림상십분한견병례,악성정도상대교고,예후교차.치료이수술치료위주,보이화료등상관치료구유일정료효,단구체료효잉수상관림상수방관찰.
Objective To explore the clinical and pathological features of neurofibromatosis I combined with malignant peripheral nerve sheath tumor (MPNST) and malignant pheochromocytoma,as to improve the level of diagnosis and treatment of this disease.Methods Clinical data of 1 case of neurofibromatosis I with two different malignant tumors were retrospec-tively analyzed and relevant literature was reviewed.Results The operation was performed and bilateral retroperitoneal tumors were successfully removed.The pathological results showed that the left tumor was MPNST.Immunohistochemical stai-ning showed:S-100(+ ),Ki-67(+ ),SMA(-),HMB45(-).The right tumor was malignant pheochromocytoma.Immuno-histochemical staining showed:Syn(+ ),CgA(+ ),Ki-67(+ ).The skin nodules were proved to be neurofibroma.Six sessions of chemotherapy regimens of IVP were given to the patient a month later after the surgery.No tumor recurrence or metastasis was detected.Conclusions NeurofibromatosisⅠcombined with MPNST and malignant pheochromocytoma is a rare event.An appropriate surgery with adjuvant chemotherapy might be effective to some extent.But the prognosis of this disease is poor for it's high degree of malignancy.More relevant studies are needed for further observation.
