婴幼儿孤立性肌纤维瘤病1例并文献复习

目的探讨婴幼儿孤立性肌纤维瘤病的临床病理学特点、免疫表型和鉴别诊断。方法对1例婴幼儿孤立性肌纤维瘤病进行光镜观察和免疫组化标记,并复习相关文献。结果肿瘤呈血管外皮瘤样结构,主要由呈结节状或束状排列的梭形细胞构成。瘤内血管丰富,部分血管内皮下生长。灶区呈侵袭性生长,侵犯周边纤维脂肪组织,未见坏死和核异型;免疫组化:肿瘤细胞CD34、vimentin、梭形细胞SMA均(+),desmin、Calponin、h-Caldesmon、S-100蛋白均(-)。结论婴幼儿孤立性肌纤维瘤病是一种少见的先天性病变,病理形态学特点和免疫组化染色结果有助于明确诊断。     

胃炎性肌纤维母细胞肿瘤的临床病理观察

目的探讨胃炎性肌纤维母细胞肿瘤(inflammatory myofibroblastic tumor,IMT)的临床病理学特征、诊断及鉴别诊断。方法对4例胃IMT进行免疫组化和原位杂交检测,并复习相关文献。结果 4例胃IMT中男性2例,女性2例,年龄21~51岁,肿瘤直径1.5~6.5 cm。镜下见肿瘤由多少不等的梭形肿瘤细胞及淋巴细胞、浆细胞、嗜酸性粒细胞构成,伴有黏液样或胶原化间质,甚至可出现钙化及骨化。大部分肿瘤细胞形态温和,部分细胞轻度不典型性,核分裂象1~2个/10 HPF。免疫表型:4例SMA均阳性,2例ALK阳性,1例CD34局灶阳性,S-100、desmin、CD68、CD117和DOG1均阴性。4例胃IMT患者随访24~66个月,均无复发及转移。结论胃IMT是一种比较少见的具有独特病理学改变的交界性肿瘤,需与多种梭形细胞肿瘤相鉴别,少数病例具有复发倾向及恶变潜能。     

儿童炎性肌纤维母细胞肿瘤临床病理特点

目的 探讨儿童炎症性肌纤维母细胞性肿瘤(inflammatory myofibroblastic tumor,IMT)的临床、病理学特点及鉴别诊断.方法 对3例儿童IMT进行了组织形态学、免疫组化检查,结合文献复习进行分析并随访.结果 其中例1、2症状均为反复咳嗽、发热、呼吸困难,年龄分别为8岁、5岁,胸片均为支气管旁实性肿块.例3年龄为11岁,因乏力、食欲不振、贫血就诊,B超示盆腔实质性肿块.镜下可见肿瘤由肌纤维母细胞性梭形细胞、浆细胞、淋巴细胞、嗜酸性粒细胞等炎症细胞构成.免疫表型:梭形细胞胞质内vimentin、desmin、MSA、SMA灶性或弥漫性阳性,myogenin、myoglobin、S-100和CD117为阴性,其中1例ALK阳性.结论 IMT是一种罕见的中间型肿瘤,原发于儿童和青少年的软组织和内脏,需与肉芽组织、结节性筋膜炎、平滑肌瘤、纤维组织细胞瘤或韧带样型纤维瘤病等鉴别.     

钙化性纤维性肿瘤4例临床病理学分析

目的 探讨钙化性纤维性肿瘤(calcifying fibrous tumor,CFT)的病理形态学特点、免疫组化表达及鉴别诊断.方法 对4例CFT进行免疫组化检测并文献复习.结果 本组患者年龄19～33岁,发病部位分别为胃、纵膈、大网膜及肠系膜根部.镜下可见在大量胶原化的纤维组织内伴有钙化或砂砾体形成,淋巴细胞、浆细胞散在浸润.免疫组化标记瘤细胞表达vimentin,而CD117、CD34、CD99、desmin、S-100蛋白、SMA、actin、NFP、ALK1、ER、PR、CK、BCL-2及Ki-67均阴性.结论 CFT是一种极少见的良性纤维性肿瘤,其诊断需要结合年龄、组织学形态及免疫组化等特点,还需与钙化性腱膜纤维瘤、钙化性肉芽肿、纤维瘤病、胃肠道间质瘤、炎性肌纤维母细胞瘤、结节性筋膜炎和淀粉样瘤等相鉴别.     

丛状纤维组织细胞瘤3例临床病理观察

目的 探讨丛状纤维组织细胞瘤临床病理特点及鉴别诊断要点.方法 对3例丛状纤维组织细胞瘤进行临床资料及光镜和免疫组化标记观察.结果 组织学特点:纤维结缔组织把肿瘤细胞分隔成丛状或结节状.结节则由单核或多核组织细胞样细胞构成,结节外周围绕短梭形的纤维母/肌纤维母细胞样细胞.部分结节则主要由纤维母细胞样细胞组成,不见多核巨细胞.免疫组化染色显示:单核或多核组织细胞样细胞表达CD68、α-ACT和溶菌酶,梭形细胞表达Vim和SMA.结论 丛状纤维组织细胞瘤是一种低度恶性的软组织肿瘤,其诊断主要依靠组织病理学和免疫组化标记.     

钙化性纤维性肿瘤32例临床病理学分析

目的探讨钙化性纤维性肿瘤(CFT)的临床病理学特征、免疫表型及鉴别诊断。方法收集河南省人民医院病理科(22例)及解放军陆军特色医学中心病理科(10例)2009年6月至2019年2月期间诊治的32例CFT患者的临床及病理学资料。采用免疫组织化学染色检测波形蛋白、CD34、间变性淋巴瘤激酶(ALK)、CD117、S-100蛋白等的表达情况;采用逆转录聚合酶链反应(RT-PCR)方法行C-KIT、PDGFRA检测;荧光原位杂交(FISH)法检测是否有ALK基因重排或MDM2基因扩增。结果患者年龄范围15~63岁,平均40.8岁,男性12例,女性20例,11例发生于胃,4例发生于腹膜后,4例位于卵巢,阴囊、纵隔、头颈部各2例,胸腔、肺、肾上腺、肾脏、乙状结肠、附睾和输卵管系膜各1例。大体均表现为界限清楚的实性肿块,肿瘤最大径0.6~10.0 cm。镜下特点均为玻璃样变的胶原纤维组织内伴有钙化或沙砾体形成,纤维母细胞稀疏,间质内散在或成片淋巴细胞、浆细胞浸润。免疫组织化学示梭形细胞恒定表达波形蛋白,9.4%(3/32)病例表达CD34,而calponin、平滑肌肌动蛋白、结蛋白、S-100蛋白、SOX10、STAT6、β-catenin、ALK、CD117、DOG1、广谱细胞角蛋白、上皮细胞膜抗原均呈阴性。全部病例行FISH检测ALK均无重排,11例发生于胃、4例发生于腹膜后及1例发生于乙状结肠的病例行C-KIT、PDGFRA分子检测均未见突变,4例腹膜后病例FISH检测MDM2均无扩增。结论CFT是一种少见的良性纤维母细胞性肿瘤,其诊断主要依靠组织形态及免疫表型。不同部位CFT应注意与其他良恶性梭形细胞间叶性肿瘤相鉴别。     

伴有肌样/肌纤维母细胞性分化的隆突性皮纤维肉瘤的临床病理分析

目的 探讨隆突性皮纤维肉瘤（ＤＦＳＰ）中肌样／肌纤维母细胞性分化的本质及其临床病理学意义。方法 采用常规ＨＥ切片对１２４例ＤＦＳＰ进行筛选,对6例伴有肌样／肌纤维母细胞性分化的ＤＦＳＰ病例进行免疫组织化学标记,其中２例加做电镜检测。结果 肌样／肌纤维母细胞性分化多出现在纤维肉瘤型ＤＦＳＰ（ＦＳ－ＤＦＳＰ）中,表现为肿瘤周边部或肿瘤内散在性分布的深嗜伊红色小结节或短要束,由梭形细胞组成,细胞多无异型性,核分裂象也罕见,形态上似平滑肌细胞或肌纤维母细胞。免疫组织化学标记显示肌样区域细胞表达α－平滑肌肌动蛋白和肌物质特异性肌动抗原,不表达ＣＤ３４;电镜观察证实细胞含有质膜下微丝束、局灶性致密体及微胸饮囊泡样结构,与肌纤维母细胞相一致,结论 ＤＦＳＰ中的肌样／肌纤维母细胞性分化可能是间质中肌纤维母细胞增生的结果,并非代表了瘤细胞的真性肌纤维母细胞性分化。     

钙化性纤维性肿瘤的临床病理学研究及其组织学发生的再评价

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。     

乳腺纤维母细胞/肌纤维母细胞性肿瘤临床病理分析

目的 探讨乳腺纤维母细胞/肌纤维母细胞性肿瘤(breast fibroblastic/ myofibroblastic tumors BF/MFT)的临床病理、免疫表型特点及相关鉴别诊断.方法 应用HE染色、免疫组化标记对7例BF/MFT进行形态学观察并进行文献复习.结果 7例患者均为女性,2例为孤立性纤维性肿瘤;2例为肌纤维母细胞瘤;1例为纤维瘤病;1例为炎性肌纤维母细胞瘤;1例为低度恶性肌纤维母细胞肉瘤.结论 BF/MFT非常少见,不同的类型肿瘤生物学行为不同,它们的鉴别诊断主要是组织学及免疫组化.     

胃钙化性纤维性肿瘤的临床病理特征

目的 探讨胃钙化性纤维性肿瘤(calcifying fibrous tumour,CFT)的临床病理特征及鉴别诊断.方法 对1例CFT进行病理组织学和免疫组织化学观察并复习相关文献.结果 肉眼观察见黏膜下结节,最大径1 cm.镜下表现为胶原背景内散在分布少量梭形细胞,伴钙化或砂砾体形成,间质伴淋巴细胞、浆细胞浸润.免疫表型:vimentin强阳性,SMA、desmin、CD34、CD117、ALK、S-100、GFAP均阴性.结论 胃CFT是一种罕见的良性纤维性病变,需与胃其他间叶源性肿瘤鉴别.     

Calcifying fibrous tumor of small intestine

Calcifying fibrous tumor (CFT) is a rare benign tumor with a predilection for children and young adults that usually arises in the subcutaneous and deep soft tissues, pleura, or peritoneum. It presents histologically as a well-circumscribed mass consisting of hyalinized, hypocellular lamellar collagen, bland spindle cells, chronic inflammatory cell infiltrates, and psammomatous or dystrophic calcifications. Calcifying fibrous tumor of the gastrointestinal tract is exceedingly rare and therefore prone to confusion with other spindle cell lesions more commonly encountered in this location. We describe 4 cases of calcifying fibrous tumor arising in the terminal ileum, one of which caused the heretofore unreported complication of intestinal intussusception, and discuss the differential diagnosis with other common and uncommon spindle cell lesions.     

Calcifying fibrous pseudotumor versus inflammatory myofibroblastic tumor: a histological and immunohistochemical comparison.

Calcifying fibrous pseudotumor (CFP), a recently described lesion, is characterized by a predominantly lymphoplasmacytic infiltrate with abundant hyalinized collagen and psammomatous or dystrophic calcifications. The cause and pathogenesis are unclear, but it has been postulated that CFP may represent a sclerosing end stage of inflammatory myofibroblastic tumor (IMT). We compared the histological and immunohistochemical profiles of seven cases diagnosed as CFP and seven as IMT. Histologically, the CFP demonstrated varying degrees of calcifications in addition to fibroblastic proliferation admixed with inflammatory cells composed of lymphocytes, eosinophils, and mast cells. The IMTs rarely contain calcifications and had a myofibroblastic proliferation varying from hyalinized acellular collagen to florid fibroblastic proliferations simulating sarcoma. The inflammatory component was composed primarily of plasma cells and lymphocytes, sometimes arranged as lymphoid aggregates with germinal centers. All CFP cases were diffusely positive for factor XIIIa and negative for smooth muscle actin, muscle-specific actin, and CD34. All IMTs demonstrated diffuse positivity for actin, variable positivity for CD34, and focal positivity for Factor XIIIa. This study demonstrates certain distinct histologic, immunohistochemical, and electron microscopic features between IMTs and CFPs.     

腹腔脏器炎性肌纤维母细胞瘤的临床病理观察

目的探讨腹腔脏器炎性肌纤维母细胞瘤(inflammatorymyofibroblastictumor,IMT)的临床病理特征、组织发生和预后。方法分析10例IMT的临床病理学资料,所有病例行HE染色和免疫组化染色。结果腹腔脏器IMT的组织学分为三型:(1)黏液样-血管型,(2)梭型细胞密集型,(3)少细胞纤维瘢痕型。肿瘤细胞表达vimentin、SMA、MSA、CKpan和ALK,其阳性率分别为100%、90%、90%、60%和20%。10例IMT中有5例做了根治性切除术,另有5例单纯肿瘤切除,随访无1例复发。结论腹腔脏器IMT易被临床医师误诊为晚期癌。免疫组化表型是该肿瘤与其他软组织肿瘤鉴别的重要依据。腹腔脏器IMT一般具有良性病变的生物学行为,根治性手术可能治愈。     

Inflammatory myofibroblastic tumor with bone marrow involvement. A case report and review of the literature

Inflammatory myofibroblastic tumor, also referred to as inflammatory fibrosarcoma, is a rare tumor composed of myofibroblastic spindle cells of uncertain etiology and disputed nosology. We report a case of inflammatory myofibroblastic tumor of the omentum with involvement of the bone marrow in an 18-year-old man. Histologic and immunohistochemical studies of the abdominal mass and bone marrow were consistent with inflammatory myofibroblastic tumor. Additionally, fluorescence in situ hybridization using a probe specific for the ALK gene showed disruption of the gene. The literature is reviewed with emphasis on the ability of inflammatory myofibroblastic tumor to recur, metastasize, and cause mortality.     

Splenic inflammatory myofibroblastic tumor (inflammatory pseudotumor): a clinicopathologic and immunophenotypic study of 12 cases

CONTEXT: Inflammatory pseudotumor is an uncommon and enigmatic lesion. The spindle cells found in this tumor have features of myofibroblasts. Because of the indefinite relationship of these lesions with inflammatory fibrosarcoma and their indefinite biologic behavior, inflammatory pseudotumor is currently classified as inflammatory myofibroblastic tumor (IMT). To date, only case reports or small series have been published on these tumors, which are primary in the spleen. DESIGN: In this study, we describe the clinical, morphologic, and immunophenotypic findings of 12 cases of splenic IMT and examine their relationship to Epstein-Barr virus (EBV). RESULTS: The patients included 8 women and 3 men, ranging from 19 to 77 years of age (mean, 53 years; median, 60 years). Demographic data were unavailable for 1 patient. Patients generally presented with abdominal pain (n = 5) and fever (n = 4). Associated lesions included renal cell carcinoma (n = 2), colonic adenocarcinoma (n = 1), and cholecystitis (n = 1). All tumors were composed of a bland spindle cell proliferation in association with a variable mixed inflammatory component. There were 2 growth patterns, namely, a cellular spindle cell pattern and a hypocellular fibrous pattern. An immunohistochemical panel confirmed the myofibroblastic nature of the spindle cells. The spindle cells of 2 cases were immunoreactive for EBV latent membrane protein 1, whereas 6 of 10 cases were positive for EBV-encoded RNA using in situ hybridization. Follow-up was available for 8 patients; 6 were alive with no evidence of recurrence and 2 were dead of other causes. CONCLUSION: Splenic IMTs are uncommon lesions that can be distinguished from other conditions using a combination of clinical, histologic, and immunophenotypic findings. Epstein-Barr virus may play a role in the pathogenesis of splenic IMT, and there may be an association of splenic IMT with concomitant disease or malignancy. Most splenic IMTs have an excellent long-term prognosis.     

A successfully treated inflammatory myofibroblastic tumor of the mandible with long-term follow-up and review of the literature

Inflammatory myofibroblastic tumor (IMT) of the oral cavity is an extremely rare clinical and pathological disease entity. It was originally described in the lung but has recently been reported in various anatomic sites. We report such a case of inflammatory myofibroblastic tumor of the mandible in a 14-year-old girl. The patient presented with an aggressive ulcerative soft tissue mass of 3 months duration in the mandibular molar gingiva. Histologically, the lesion was composed of fibroblastic or myofibroblastic spindle cell proliferations with infiltrative margins in an inflammatory background. Immunohistochemically, the fibroblastic or myofibroblastic spindle cells were positive for vimentin, α-smooth muscle actin, and Ki-67 (MIB-1) but negative for desmin, pan-cytokeratin, S-100 protein, CD34, CD68, CD99, bcl-2, β-catenin, estrogen receptor, progesterone receptor, ALK-1, and p53. These spindle cells were focally and weakly Ki-67- (MIB-1-) positive. The MIB-1 labeling index was 5%. The results of in situ hybridization for Epstein-Barr virus-encoded-RNA were negative. The ratio of IgG4+/IgG+ plasma cells was about 10%. A pathological diagnosis of inflammatory myofibroblastic tumor was made. The postoperative course was uneventful, and the patient has had no recurrence in the 10-year follow-up period. Although no evidence of oral inflammatory myofibroblastic tumor recurrence or malignant transformation has been reported, it has been observed that in inflammatory myofibroblastic tumors of other regions, a prolonged follow-up is necessary after surgical resection. No other case of an IMT patient under 20 years of age has appeared in either the English or the Japanese literature.     

Soft tissue tumors of the urinary bladder, Part I: myofibroblastic proliferations, benign neoplasms, and tumors of uncertain malignant potential

Most bladder tumors arise from the urothelium. However, there are several uncommon but significant bladder lesions that must be differentiated from urothelial carcinomas. These include both benign and malignant spindle cell lesions. The first half of this 2-part review will describe benign myofibroblastic proliferations including inflammatory myofibroblastic tumor and postoperative spindle cell nodule; benign neoplasms including leiomyoma, hemangioma, neurofibroma, and schwannoma; and tumors of uncertain malignant potential including paraganglioma, granular cell tumor, and perivascular epithelioid cell tumor. Common clinical presentations, morphological characteristics, and immunohistochemical features are described to aid the practicing pathologist in the identification of these entities. This review also describes current theories as to the pathogenesis of inflammatory myofibroblastic tumor and postoperative spindle cell nodule and details the current molecular markers identifying several of these lesions.     

Calcifying fibrous pseudotumor of mesentery presenting with acute peritonitis: case report with immunohistochemical study and review of literature

Calcifying fibrous pseudotumor (CFP) is a benign soft tissue lesion composed of thick collagen bundles, scattered fibroblasts, and psammomatous and dystrophic calcifications, located most commonly in the extremities and trunk of children and young adults. The present case in a 36-year-old woman is to the best of our knowledge the first report of a large CFP confined to the mesentery, which, because of torsion, led to acute peritonitis and emergency laparotomy. The typical histologic features were accompanied by a prominent myofibroblastic proliferation along with inflammatory response at the periphery of the lesion. The spindle cells of the lesion were positive for vimentin and focally for CD34 and smooth-muscle actin. Review of the literature and discussion of differential diagnosis in this report focuses on abdominal CFP and other intraabdominal soft tissue lesions, some of which may be precursors of CFP. Int J Surg Pathol 9(3):249-253, 2001