[1]张颖,卢阳,冯林,等.Ⅰ型神经纤维瘤病恶变伴骨破坏1例并文献复习[J].中国皮肤性病学杂志,2019,(07):808-810.[doi:10.13735/j.cjdv.1001-7089.201809092]
 ZHANG Ying,LU Yang,FENG Lin,et al.Malignant Transformation of Neurofibromatosis Type Ⅰ with Bone Destruction:A Case Report and Literature Review[J].The Chinese Journal of Dermatovenereology,2019,(07):808-810.[doi:10.13735/j.cjdv.1001-7089.201809092]
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Ⅰ型神经纤维瘤病恶变伴骨破坏1例并文献复习
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《中国皮肤性病学杂志》[ISSN:1001-7089/CN:61-1197/R]

卷:
期数:
2019年07期
页码:
808-810
栏目:
病例报告
出版日期:
2019-06-16

文章信息/Info

Title:
Malignant Transformation of Neurofibromatosis Type Ⅰ with Bone Destruction:A Case Report and Literature Review
文章编号:
1001-7089(2019)07-0808-03
作者:
张颖卢阳冯林江阳王思平
重庆市中医院(重庆市第一人民医院)皮肤科,重庆 400011
Author(s):
ZHANG YingLU YangFENG LinJIANG YangWANG Siping
(Department of Dermatology,Chongqing First People's Hospital,Chongqing 400011,China(Department of Dermatology,Chongqing First People's Hospital,Chongqing 400011,China
关键词:
神经纤维瘤皮肤 恶性神经鞘瘤 骨破坏
Keywords:
Cutaneous neurofibiromatosis Malignant peripheral nerve sheath tumor Skeleton destruction
分类号:
R 739.5
DOI:
10.13735/j.cjdv.1001-7089.201809092
文献标志码:
B
摘要:
患者女,62岁,全身散在咖啡斑62年,结节渐多48年,增大2年。皮肤科情况:全身散在多个直径1~4 cm大小咖啡色斑,泛发直径0.5~5 cm大小皮色丘疹、结节,部分带蒂,结节皮温正常,质软,无压痛; 左背部可见一直径约15 cm肿块,质韧,局部皮温显著升高,无压痛,表面无糜烂、溃疡; 左髋部可触及直径约20 cm皮下结节,质韧,无压痛。PET-CT扫描:左侧骶骨溶骨性破坏,伴软组织块影。皮损组织病理示:表皮大致正常; 真皮及皮下组织见大量梭形细胞,分为细胞疏松区域及密集区域,梭形细胞胞浆少而淡染,核细长、波浪状,密集区域细胞呈紧密交织排列,全部区域均可见核大深染的异型细胞。免疫组织化学:Vimentin(+)、NSE(+)、p53(+)、S-100(灶性+)、EMA(-)、SMA(-)、Melan-A(-)、CK(-)、Myo(-)、Ki-67指数约15%。最终诊断神经纤维瘤病伴恶性外周神经鞘瘤伴骨破坏。患者放弃治疗出院,10个月后死亡。
Abstract:
A 62-year-old female presented with cafe-au-lait spots scattered all over the body for 62 years,and increased nodules for 48 years,which was enlarged for 2 years.Physical examination revealed cafe-au-lait spots scattered all over the body measuring 1~4 cm in diameter,and extensive skin-colored soft papules and nodules measuring 0.5~5 cm in diameter,some of which were pedicled.The skin temperature of the nodules was normal,and the nodules were soft with no tenderness.One huge tough nodule was on the left side of back with a diameter of about 15 cm,with significantly increased local skin temperature,no tenderness,no erosion or ulcer on the surface.There was a tough,nontender subcutaneous nodule with a diameter of about 20 cm on left hip.The positron emission tomography computed tomography(PET-CT)scan revealed osteolytic destruction of the left sacrum,with soft tissue mass shadow.The histopathologic examination showed normal epidermis,and extensive spindle cell in dermis and subdermis,with pale plasma and slender wave-shaped nucleus.There were areas with rare cells and areas with intensive cells.The cells in the dense area were closely intertwined,and heterotypic cells with deep-dyed big nucleolus could be seen in all areas.Immunohistochemistry showed that the tumor cells were stained positive for Vimentin,NSE,p53,and partly positive for S-100,and negative for EMA,SMA,Melan-A,CK and Myo,with Ki-67 positvie for 15% of the tumor cells.The diagnosis was cutaneous neurofibiromatosis with malignant peripheral nerve sheath tumor(MPNST)and bone destruction.The patient gave up treatment and died 10 months later.

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备注/Memo

备注/Memo:
[作者单位] 重庆市中医院(重庆市第一人民医院)皮肤科,重庆 400011
[通讯作者] 王思平,E-mail:56450536@qq.com
[Corresponding author] WANG Siping,E-mail:56450536@qq.com
更新日期/Last Update: 2019-06-15