中华病理学杂志
中華病理學雜誌
중화병이학잡지
Chinese Journal of Pathology
2011年
10期
683-688
,共6页
毛荣军%熊晓平%史健%李启明%房惠琼
毛榮軍%熊曉平%史健%李啟明%房惠瓊
모영군%웅효평%사건%리계명%방혜경
软组织肿瘤%钙质沉着症%病理学,外科%组织学,比较%免疫球蛋白G
軟組織腫瘤%鈣質沉著癥%病理學,外科%組織學,比較%免疫毬蛋白G
연조직종류%개질침착증%병이학,외과%조직학,비교%면역구단백G
Soft tissue neoplasms%Calcinosis%Pathology,surgical%Histology,comparative%Immunoglobulin G
目的探讨钙化性纤维性肿瘤(CFT)的临床病理学特征及其组织学发生机制。方法对11例CFT的临床表现、组织学形态及免疫组织化学表型进行分析。结果11例CFT中男性5例，女性6例，年龄从25至52岁，平均38岁，位于盆腹腔6例、皮下软组织4例、阴囊内1例。临床上表现为缓慢增大的无痛性肿块，5例伴随其他病症或既往有炎性改变、外伤或手术史，4例病变为偶然发现，肿瘤多为单发，切除后未见复发。影像学显示病变为孤立性或多发性实性软组织肿块，境界清楚无包膜，实质内散在大小不等、数量不一的高密度钙化灶。大体上，肿瘤呈灰黄色，质硬，边清，圆形、卵圆形、分叶状或不规则形，最大径为0.5 ～20.0 cm，切面散在浅黄色斑点状钙化灶，切开时具有沙砾感。显微镜下显示肿瘤实质主要由玻璃样变的胶原纤维及厚壁血管构成，其中散在少量梭形细胞、单核炎性细胞、沙砾体及营养不良性钙化。此外，少数肿瘤边缘区局灶性中性粒细胞呈带状浸润，另见少量神经束及脂肪组织内陷。不同病例肿瘤实质外周区局灶性具有类似于孤立性纤维瘤、纤维瘤病、瘢痕疙瘩及炎性肌纤维母细胞瘤样形态学改变。沙砾体及营养不良性钙化分别形成于透明变性的血管及玻璃样变的胶原纤维。肿瘤组织内浸润的单核炎性细胞主要为淋巴浆细胞，局部区域可形成淋巴滤泡样结构。免疫组织化学染色显示所有受检的肿瘤组织内梭形细胞弥漫性表达波形蛋白，少数局灶性表达CD34、第八因子相关抗原及β-caltenin，其他标记为阴性。具有特征性的是，与炎性病变相比，CFT组织中浸润的浆细胞显著表达IgG及IgG4，且IgG4+/IgG+＞50％，IgG1及IgG3表达的细胞较少。结论CFT具有较为特征性的组织病理学表现，但其发病机制尚未明确。由于CFT与IgG4相关的硬化性疾病具有相似的组织学及免疫组织化学表型，因此，推测CFT可能为IgG4相关的硬化性疾病家族谱系中一种新的独立实体。该病变的发展呈良性经过，炎性改变及创伤可能为该病变的重要诱因，手术切除后罕见复发。
目的探討鈣化性纖維性腫瘤(CFT)的臨床病理學特徵及其組織學髮生機製。方法對11例CFT的臨床錶現、組織學形態及免疫組織化學錶型進行分析。結果11例CFT中男性5例，女性6例，年齡從25至52歲，平均38歲，位于盆腹腔6例、皮下軟組織4例、陰囊內1例。臨床上錶現為緩慢增大的無痛性腫塊，5例伴隨其他病癥或既往有炎性改變、外傷或手術史，4例病變為偶然髮現，腫瘤多為單髮，切除後未見複髮。影像學顯示病變為孤立性或多髮性實性軟組織腫塊，境界清楚無包膜，實質內散在大小不等、數量不一的高密度鈣化竈。大體上，腫瘤呈灰黃色，質硬，邊清，圓形、卵圓形、分葉狀或不規則形，最大徑為0.5 ～20.0 cm，切麵散在淺黃色斑點狀鈣化竈，切開時具有沙礫感。顯微鏡下顯示腫瘤實質主要由玻璃樣變的膠原纖維及厚壁血管構成，其中散在少量梭形細胞、單覈炎性細胞、沙礫體及營養不良性鈣化。此外，少數腫瘤邊緣區跼竈性中性粒細胞呈帶狀浸潤，另見少量神經束及脂肪組織內陷。不同病例腫瘤實質外週區跼竈性具有類似于孤立性纖維瘤、纖維瘤病、瘢痕疙瘩及炎性肌纖維母細胞瘤樣形態學改變。沙礫體及營養不良性鈣化分彆形成于透明變性的血管及玻璃樣變的膠原纖維。腫瘤組織內浸潤的單覈炎性細胞主要為淋巴漿細胞，跼部區域可形成淋巴濾泡樣結構。免疫組織化學染色顯示所有受檢的腫瘤組織內梭形細胞瀰漫性錶達波形蛋白，少數跼竈性錶達CD34、第八因子相關抗原及β-caltenin，其他標記為陰性。具有特徵性的是，與炎性病變相比，CFT組織中浸潤的漿細胞顯著錶達IgG及IgG4，且IgG4+/IgG+＞50％，IgG1及IgG3錶達的細胞較少。結論CFT具有較為特徵性的組織病理學錶現，但其髮病機製尚未明確。由于CFT與IgG4相關的硬化性疾病具有相似的組織學及免疫組織化學錶型，因此，推測CFT可能為IgG4相關的硬化性疾病傢族譜繫中一種新的獨立實體。該病變的髮展呈良性經過，炎性改變及創傷可能為該病變的重要誘因，手術切除後罕見複髮。
목적탐토개화성섬유성종류(CFT)적림상병이학특정급기조직학발생궤제。방법대11례CFT적림상표현、조직학형태급면역조직화학표형진행분석。결과11례CFT중남성5례，녀성6례，년령종25지52세，평균38세，위우분복강6례、피하연조직4례、음낭내1례。림상상표현위완만증대적무통성종괴，5례반수기타병증혹기왕유염성개변、외상혹수술사，4례병변위우연발현，종류다위단발，절제후미견복발。영상학현시병변위고립성혹다발성실성연조직종괴，경계청초무포막，실질내산재대소불등、수량불일적고밀도개화조。대체상，종류정회황색，질경，변청，원형、란원형、분협상혹불규칙형，최대경위0.5 ～20.0 cm，절면산재천황색반점상개화조，절개시구유사력감。현미경하현시종류실질주요유파리양변적효원섬유급후벽혈관구성，기중산재소량사형세포、단핵염성세포、사력체급영양불량성개화。차외，소수종류변연구국조성중성립세포정대상침윤，령견소량신경속급지방조직내함。불동병례종류실질외주구국조성구유유사우고립성섬유류、섬유류병、반흔흘탑급염성기섬유모세포류양형태학개변。사력체급영양불량성개화분별형성우투명변성적혈관급파리양변적효원섬유。종류조직내침윤적단핵염성세포주요위림파장세포，국부구역가형성림파려포양결구。면역조직화학염색현시소유수검적종류조직내사형세포미만성표체파형단백，소수국조성표체CD34、제팔인자상관항원급β-caltenin，기타표기위음성。구유특정성적시，여염성병변상비，CFT조직중침윤적장세포현저표체IgG급IgG4，차IgG4+/IgG+＞50％，IgG1급IgG3표체적세포교소。결론CFT구유교위특정성적조직병이학표현，단기발병궤제상미명학。유우CFT여IgG4상관적경화성질병구유상사적조직학급면역조직화학표형，인차，추측CFT가능위IgG4상관적경화성질병가족보계중일충신적독립실체。해병변적발전정량성경과，염성개변급창상가능위해병변적중요유인，수술절제후한견복발。
Objective To study the clinicopathologic features and histogenesis of calcifying fibrous tumor (CFT). Methods The clinical manifestations, histopathologic characteristics and immunophenotype were analyzed in 11 cases of CFT. Results The male-to-female ratio was 5∶6, with a mean age of 38 years and age range of 25 to 52 years. The sites of involvement included abdominopelvic cavity ( n = 6), soft tissue (n =4) and scrotum (n = 1 ). Most patients presented with a gradually enlarging and painless mass.Nearly half of the cases were associated with other diseases or history of inflammation, trauma or surgical intervention. One third of the tumors represented incidental findings and showed no recurrence after resection. Imaging revealed a solitary solid soft tissue mass or multiple nodules with clear borders and associated high-density calcifications. Macroscopically, the tumors were well-circumscribed but non-encapsulated. They ranged from 0.5 to 20.0 cm in diameter and were tan-greyish, round to oval, lobulated or irregular and solid with rubbery consistency. The cut surface was whitish to tan-yellowish, gritty and showed scattered spotty yellowish discoloration corresponding to the foci of dystrophic calcifications.Histologically, CFT was composed of hyalinized fibrous tissue and thickened vessel walls with interspersed bland spindly fibroblastic cells, scattered psammomatous calcifications, dystrophic calcification and lymphoplasmacytic infiltration. In addition, focal cloak-like polymorph infiltration at the tumor periphery and entrapment of adipocytes and nerves were demonstrated in some cases. Foci resembling solitary fibrous tumor, fibromatosis, keloid or inflammatory myofibroblastic tumor were observed. Immunohistochemical study showed that the tumor cells were diffusely positive for vimentin and focally positive for CD34, factor Ⅷ-related antigen and beta-catenin. The admixed plasma cells were notably IgG positive, with more than 50％ being IgG4 positive. Conclusions CFT has characteristic histopathologic manifestations and shows morphologic and immunohistochemical overlaps with known IgG4-related sclerosing diseases. It is possible that CFT may represent another example of IgG4-related diseases. It often runs a benign clinical course, with rare recurrence after surgical resection. Previous inflammation and trauma may be the precipitating factors of CFT.
