中华病理学杂志
中華病理學雜誌
중화병이학잡지
Chinese Journal of Pathology
2012年
7期
456-460
,共5页
软组织肿瘤%皮肤纤维肉瘤%诊断
軟組織腫瘤%皮膚纖維肉瘤%診斷
연조직종류%피부섬유육류%진단
Soft tissue neoplasms%Dermatofibrosarcoma%Diagnosis
目的 探讨黏液样隆突性皮纤维肉瘤(DFSP)的临床病理学特征及其鉴别诊断.方法 回顾性复习16例黏液样DFSP的临床资料、组织学形态和免疫学表型.结果 患者中男15例,女1例,平均和中位年龄分别为47和48岁,年龄范围为11 ～ 73岁.11例位于躯干,2例位于肩部,2例位于头皮,1例位于上臂.临床表现与经典型DFSP相似,表现为皮下缓慢性生长的结节,部分病例近期生长迅速.16例中的12例为原发性,4例为复发性.肿瘤直径为2.0 ～10.0 cm(平均5.0 cm).镜下观察:肿瘤境界不清,呈浸润性生长,其中7例完全呈黏液样,由短梭形和星状细胞、大量的黏液样基质和纤细的血管网组成,核分裂象范围为(0 ～ 10)/HPF;另9例除黏液样区域外,5例可见经典型DFSP区域,其中1例含有灶性的巨细胞纤维母细胞瘤样区域,另4例则呈纤维肉瘤样变.免疫组织化学标记显示,所有病例均表达CD34,但与经典型DFSP相比,阳性强度相对较弱.随访显示,4例复发性病例中1例发生肺转移和局部复发.结论 黏液样DFSP是DFSP的一种少见亚型,当肿瘤完全呈黏液样时容易被误诊为其他类型的黏液样软组织肿瘤,在诊断时应注意加以鉴别.
目的 探討黏液樣隆突性皮纖維肉瘤(DFSP)的臨床病理學特徵及其鑒彆診斷.方法 迴顧性複習16例黏液樣DFSP的臨床資料、組織學形態和免疫學錶型.結果 患者中男15例,女1例,平均和中位年齡分彆為47和48歲,年齡範圍為11 ～ 73歲.11例位于軀榦,2例位于肩部,2例位于頭皮,1例位于上臂.臨床錶現與經典型DFSP相似,錶現為皮下緩慢性生長的結節,部分病例近期生長迅速.16例中的12例為原髮性,4例為複髮性.腫瘤直徑為2.0 ～10.0 cm(平均5.0 cm).鏡下觀察:腫瘤境界不清,呈浸潤性生長,其中7例完全呈黏液樣,由短梭形和星狀細胞、大量的黏液樣基質和纖細的血管網組成,覈分裂象範圍為(0 ～ 10)/HPF;另9例除黏液樣區域外,5例可見經典型DFSP區域,其中1例含有竈性的巨細胞纖維母細胞瘤樣區域,另4例則呈纖維肉瘤樣變.免疫組織化學標記顯示,所有病例均錶達CD34,但與經典型DFSP相比,暘性彊度相對較弱.隨訪顯示,4例複髮性病例中1例髮生肺轉移和跼部複髮.結論 黏液樣DFSP是DFSP的一種少見亞型,噹腫瘤完全呈黏液樣時容易被誤診為其他類型的黏液樣軟組織腫瘤,在診斷時應註意加以鑒彆.
목적 탐토점액양륭돌성피섬유육류(DFSP)적림상병이학특정급기감별진단.방법 회고성복습16례점액양DFSP적림상자료、조직학형태화면역학표형.결과 환자중남15례,녀1례,평균화중위년령분별위47화48세,년령범위위11 ～ 73세.11례위우구간,2례위우견부,2례위우두피,1례위우상비.림상표현여경전형DFSP상사,표현위피하완만성생장적결절,부분병례근기생장신속.16례중적12례위원발성,4례위복발성.종류직경위2.0 ～10.0 cm(평균5.0 cm).경하관찰:종류경계불청,정침윤성생장,기중7례완전정점액양,유단사형화성상세포、대량적점액양기질화섬세적혈관망조성,핵분렬상범위위(0 ～ 10)/HPF;령9례제점액양구역외,5례가견경전형DFSP구역,기중1례함유조성적거세포섬유모세포류양구역,령4례칙정섬유육류양변.면역조직화학표기현시,소유병례균표체CD34,단여경전형DFSP상비,양성강도상대교약.수방현시,4례복발성병례중1례발생폐전이화국부복발.결론 점액양DFSP시DFSP적일충소견아형,당종류완전정점액양시용역피오진위기타류형적점액양연조직종류,재진단시응주의가이감별.
Objective To study the clinicopathologic features and differential diagnosis of myxoid dermafibrosarcoma protuberans(DFSP).Methods The clinical and pathologic features of 16 cases of myxoid DFSP were reviewed.Results There were altogether 15 males and I female.The age of the patients ranged from 11 to 73 years(mean =47 years and median =48 years).The commonest site of involvement was trunk(number =11),followed by shoulder(number =2),head and neck(number =2),and extremity(number =1).Similar to conventional DFSP,most patients presented with a slowly enlarging subcutaneous nodule which showed a rapid recent growth in some cases.Amongst the 16 cases studied,12 occurred de novo and 4 represented local recurrence.The tumors ranged from 2 to 10 cm in greatest dimension(mean =5 cm and median =4 cmm).Histologcically,they were poorly circumscribed and located in the dermis,with focal infiltration into the underlying subcutaneous tissue.Seven cases were purely myxoid and composed of spindly and stellate cells with delicate arborizing vascular meshwork.The remaining 9 cases were predominantly myxoid( ＞ 50％),with 5 cases containing cellular areas resembling conventonal D)FSP and 4 cases showing fibrosarcomatous transformation.In addition,foci of giant cell fibroblastoma-like areas were noted in 1 case.Immunohistochemical study showed that the tumors cells were positive for CD34.The staining was weak in the myxoid areas,as compared with conventional DFSP.Of the 4 recurrent cas,es,one patient developed lung metastases.Conclusions Myxoid DFSP represents a rare variant of DFSP and may pose important diagnostic pitfalls.It is especially so if the tumor purely consists of myxoid element.Familiarity with the histologic features helps to avoid misdiagnosis.
