Fibroma of tendon sheath.

A series of nine cases of fibroma of tendon sheath is described including details of the ultrastructural features of two cases. The series was composed of lesions from six males and three females with a mean age of 38 yr. The most common site of involvement was the hand (including fingers) and the mean greater diameter was 19 mm. Typically the tumours were lobulated and microscopically there was a collagenous stroma with spindle and stellate cells in a moderate degree of cellularity. One recurrence was noted in the series. The lesion was distinguished from circumscribed fibromatosis, nodular fasciitis, neurofibroma, leiomyoma, scar tissue, giant cell tumour of tendon sheath (localised nodular tenosynovitis) and fibrous histiocytoma. Ultrastructural studies revealed that the large majority of cells present in the two cases studied were myofibroblasts and fibroma of tendon sheath is therefore the third instance of a benign tumour containing these cells (the other two being dermatofibroma and giant cell fibroma of the oral mucosa).     

Fibroma of the tendon sheath. Description of a case

One case of fibroma of tendon sheath is described. This tumor is a distinct entity with a characteristic location and histological appearance. The lesion must be distinguished from giant cell tumor of tendon sheath, nodular fasciitis and fibrous histiocytoma. Recurrence rate is 24%. Local excision is the treatment of choice for primary and recurrent lesions.     

Fibroma of tendon sheath in the knee: A report of three cases and literature review

Fibroma of tendon sheath (FTS) is a rare and benign soft-tissue tumor. It is predominantly found in the fingers, hands, and wrists of young, adult males. We describe a series of three cases all presenting in the atypical knee location. We also review the literature on FTS, located both in the knee and elsewhere, looking for common clinical, imaging, and histologic patterns to help differentiate it from similar knee lesions. FTS typically presents as a painless, slow-growing, solid nodule. In the knee though, 71% of lesions present with pain/discomfort and 31% present with a palpable mass. Physical exam for knee FTS commonly reveals painful range of motion (50%), decreased range of motion (42%), and a palpable non-tender mass (33%). MRI of FTS usually reveals a well-defined soft-tissue mass, with low signal on T1, variable signal on T2, and variable enhancement. Histologically, the tumors are composed of a dense fibrocollagenous stroma with scattered spindle-shaped fibroblasts and narrow slit-like vascular spaces. Most FTS are removed by marginal excision, with 24% of lesions subsequently recurring. No lesion has ever metastasized. Despite its rarity, this lesion should be included in the differential diagnosis of a knee mass on physical exam or imaging, especially if it is painful, benign appearing, and present in a middle-aged male.     

Giant cell tumor of tendon sheath. An immunohistochemical study of 28 cases

An immunohistochemical study was carried out on 28 cases of giant cell tumor of tendon sheath. Although this tumor has been considered to be of histiocytic origin on the basis of light and electron microscopic findings, there remains some debate about the histogenesis of the tumor. To clarify this point, by using the PAP method, each surgical specimen was stained for alpha 1-antitrypsin, alpha 1-antichymotrypsin, lysozyme, ferritin, neuron specific enolase, and S-100 protein. Tumor cells in fifteen out of 28 cases were positively stained for alpha 1-antitrypsin, 19 for alpha 1-antichymotrypsin, 23 for lysozyme, 22 for ferritin, 22 for neuron specific enolase, but no case for S-100 protein. These results suggest that this tumor is composed of cells with histiocytic character. In addition, from the immunohistochemical point of view, at least two types of giant cells seem to exist in this disease.     

Fibroma of the tendon sheath. Diagnosis and differential diagnosis

Ten cases of fibroma of the tendon sheath were examined by means of histological, topochemical, and immunohistochemical methods. Investigations were aimed at re-evaluation of histological variability and definition of diagnostic criteria. Microscopic observations suggested the following features to be typical of fibroma of the tendon sheath: Primitive mesenchymal or fibroblastic cells without further differentiation, slit- and cleft-like spaces with lining cells decorated by endothelial markers, and collagenous, cartilaginous or myxoid basic substance at least in areas rich with sulphated glycosaminoglycans. Differential diagnostic distinction of tendon sheath fibroma from myxoid cartilage tumors, fasciitis nodularis, and myxoid fibromatosis as well as from fibrohistiocytic lesions and giant cell tumors of tendon sheath is discussed with reference to the above definition.     

Fibroma of tendon sheath arising from the radio-ulnar joint

A report of a 65-year-old male with a tumor arising from synovial tissue of the radio-ulnar joint. On magnetic resonance imaging, the tumor was demonstrated as a heterogeneous and lobulated mass with a low signal intensity both in T1- and T2-weighted images. Histological findings of the tumor were identical to those of fibroma of the tendon sheath. In the peripheral villous synovial tissue, several small and fibrous nodules were observed, and their histological features were identical to those of the main tumor. Immunohistochemically, the tumor cells showed diffuse and intense reactivity to vimentin, muscle actin and S-100. These results indicated that the tumor might be a fibromatous analog of synovial chondromatosis.     

Extrapleural solitary fibrous tumor: a clinicopathologic study of 24 cases.

Solitary fibrous tumors (SFTs), rare in extrapleural sites, can present difficulties in diagnosis at these locations. From the files of the M. D. Anderson Cancer Center, we accessed 24 cases of extrapleural SFT (14 females, 10 males, ages 19 to 85 yr) obtained for clinical, histologic, immunohistochemical, and follow-up findings. Tumor locations included the head and neck (n = 12), the abdomen (n = 10), and the extremities (n = 2). Tumors were 2 to 25 cm in greatest dimension (mean, 8.2 cm) and were well circumscribed or encapsulated. Histologic features were typical of their pleural counterparts, e.g., bland spindle cells with some hypercellular areas and myxoid to hyalinized backgrounds. A hemangiopericytic vascular pattern was present in 19 cases and prominent in 11. Mitotic activity ranged from 0 to 4 counts per 10 high-power fields. Necrosis was seen in two cases. Focally increased cellularity was seen in seven cases and margins were positive in another seven cases. Spindle cells were positive for vimentin (19 of 19) and CD34 (18 of 20), and negative for cytokeratin (0 of 19). Fibroblastic differentiation was present in the three cases studied by electron microscopy. Flow cytometry in three cases revealed diploid cell populations. Follow-up for 19 cases (9 to 99 mo) revealed no evidence of metastasis, although one patient had residual tumor after an incomplete excision, and one patient died of unrelated causes. Histologic findings such as mitotic counts, necrosis, cellularity, and marginal status were not associated with outcome. We conclude that extrapleural SFTs are clinically and histologically similar to their pleural counterparts. Although the length of clinical follow-up was short (mean follow-up, 41.4 mo), recognizing these lesions is important because they typically follow an indolent clinical course if completely excised. Although CD34 is nonspecific, it is usually positive in SFTs and may aid in their diagnosis.     

钙化性纤维性肿瘤的临床病理学分析

目的探讨钙化性纤维性肿瘤(calcifying fibrous tumor,CVF)的临床病理学特征、免疫表型和鉴别诊断。方法回顾性分析7例CFT的临床资料、病理学形态和免疫组化标记结果。结果患者中1例为少年,6例均为成年人,年龄14—50岁,平均37岁。5例临床表现为局部缓慢性生长的无痛性肿块,2例为术中偶然发现。肿瘤分别位于腹腔／盆腔(3例)、颈部(2例)、左腹股沟(1例)和左小腿(1例)。眼观：肿块境界清楚,卵圆形或结节状,质地坚韧。镜检：肿瘤由大量胶原化的纤维结缔组织组成,其间夹杂少量梭形细胞。特征性形态学表现为在胶原化的纤维组织间可见散在的钙化灶或砂砾小体,间质内伴有多少不等的淋巴细胞和浆细胞浸润灶,部分病例中可见生发中心形成。免疫组化标记显示,梭形细胞主要表达vimentin,不表达CD34、S-100蛋白、actin、desmin、h-caldesmon和ALK1等标记。随访6例,均无复发。结论CFT是一种不同于炎性肌纤维母细胞瘤的良性纤维母细胞性肿瘤。CFT不仅好发于儿童和青少年,也可发生于成年人。组织学上应与伴有钙化的纤维母细胞／肌纤维母细胞性病变相鉴别。     

Giant cell tumor of tendon sheath: a light and electron microscopic study

A neoformation has been surgically withdrawn from third finger extensor tendon of the right hand of a 52 year male subject. Light (LM) and electron microscope (EM) observations from a number of tissue fragments allowed the identification of tumor nature, which appeared a giant cell tendon sheath. Moreover, some structural patterns have been described and compared to the previously reported cases. In areas of major cell density, parenchyma does not show lobular or gland-like organization; on the other hand, wide zones characterized by an amorphous matrix, progressively replacing collagen and containing elongated cells, are present. Giant multinucleated cells, mostly localized close to collagen bundles, can also be revealed. Unexpectedly, no foam cells appear and no phagocytized cell debris can be identified in giant multinucleated cells. Engulfed crystals are, differently, evidentiated by electron microscopy, both in mono- and multinucleated cell cytoplasm. Their electron density and their localization within cytoplasmic vacuoles suggest the presence of calcium. A correlation between giant cells and osteoclasts is then proposed. Multiple variously oriented centrioles support the possible mitotic genesis of multinucleated giant cells, which never show, on the other hand, fusion features. Siderosomes and residual bodies are also present. An unusual, diffuse thickening of nuclear lamina, only interrupted at nuclear pore level, is described and discussed.     

上皮样恶性周围性神经鞘瘤临床病理分析

目的 探讨上皮样恶性周围性神经鞘瘤(epithelioid malignant peripheral nerye sheath tumor,EMPNST)的临床病理特征及鉴别诊断.方法 收集9例EMPNST的临床病理资料,行光镜和EnVision法免疫组化观察,并复习文献.结果 9例EMPNST,女性4例;年龄20～67岁,中位年龄37.5岁;病变主要位于四肢,上肢3例,下肢4例,右季肋部和咽隐窝各1例;>5 cm 7例,其中1例>10 cm;<5 cm 2例,平均6.2 cm,无包膜.深在型8例,浅在型1例,组织学,纯上皮样型5例,其中2例见节细胞样或横纹肌样瘤样区域,4例混合型伴有梭形细胞区.免疫表型S-100蛋白及NSE 9例均呈阳性反应,纯上皮样型5例S-100蛋白呈强阳性,4例混合型呈灶性阳性,8例PGP 9.5阳性,7例MBP阳性,5例EMA灶性或弱阳性,4例vimentin阳性,3例CD57灶性阳性,而HMB-45、desmin、CD34、CK阴性.结论 EMPNST是恶性周围性神经鞘瘤的一种少见亚型,形态学上缺乏特征性,易与其他软组织上皮样肿瘤混淆.S-100蛋白及PGP9.5阳性是诊断EMPNST有价值的指标,但缺乏特异性,因此诊断时必须结合临床、组织形态和免疫表型的结果,综合判断以免引起误诊.     

Myxoid solitary fibrous tumor: a clinicopathologic study of three cases

While focal myxoid areas are occasionally observed in solitary fibrous tumors, neoplasms of this type exhibiting extensive myxoid change are considered exceedingly uncommon. Due to their rarity, the biologic behavior of myxoid solitary fibrous tumor has not been determined. Three cases of myxoid solitary fibrous tumor are described in order to better characterize the clinical and pathologic features of this uncommon variant of solitary fibrous tumor. The tumors occurred in one man and two women, with ages of 37, 47, and 58 years, respectively. Sites of involvement included the retroperitoneum, pelvis, and soft tissue of the neck. Histologically, all cases were characterized predominantly by the presence of myxoid stroma comprising 70% to 100% of the tumor. The tumor cells were predominantly spindled in all cases, and arranged randomly, in loose fascicles, or in anastomosing strands imparting a microcystic/reticular appearance. The lesional cells had a bland cytologic appearance and low mitotic count. All tumors lacked necrosis and areas of increased cellularity. By immunohistochemistry, all cases were positive for CD34, CD99, and bcl-2, and negative for keratin, epithelial membrane antigen, desmin, actin, smooth muscle actin, and S-100 protein. To date, all cases have followed a benign course without evidence of recurrence or metastasis with a follow-up duration ranging from 50 to 87 months. The data suggest that myxoid solitary fibrous tumors are associated with an indolent clinical course and favorable prognosis.     

胸部原始神经外胚层瘤5例临床病理分析

目的 探讨胸部原始神经外胚层瘤(PNET)的临床病理特征、免疫学表型及其鉴别诊断。方法 对5例发生于胸部的PNET进行光镜观察和免疫组化研究。结果 5例PNET中4例为女性,1例男性,年龄12～52岁,平均27．0岁。肿瘤体积较大,平均直径11．4cm,无包膜或包膜不完整。镜下：肿瘤由小圆细胞构成,细胞胞质少,部分区域肿瘤细胞胞质透亮,可见Homer-Wwright菊形团和假菊形团。免疫表型：5例CD99(MIC2)、NSE阳性,4例synaptophysin阳性,3例vimentin阳性,2例S-100蛋白阳性。结论 胸部PNET是较少见的高度恶性软组织肿瘤,其诊断主要依据病理形态学特征及免疫组化标记。     

Cannibalism in a benign soft tissue tumor (giant-cell tumor of the tendon sheath, localized type): a study of 66 cases

Cellular cannibalism refers to a phenomenon where a living cell is phagocytosed into a tumoral cell, where it eventually dies. With the exception of cells in suspension, cellular cannibalism has only been observed with malignant tumors. The finding of occasional images of cannibalism in our daily biopsies of giant cell tumors of the tendon sheath led us to examine this phenomenon further in a retrospective study of 66 cases from our archives. In each case, four morphological features were evaluated: evidence of giant cells, cannibalism, xanthomatous cells, and hemosiderin deposits. Five cases were randomly selected for further immunohistochemical study with the following antibodies: CD68, vimentin, leukocytary common antigen (LCA), Bcl-2 oncoprotein, p53, caspase-3, and Bax. Patients included 35 (53.03%) females and 31 (46.97%) males. Mean age was 50.73 years (range from 14 to 75 years). Giant cells were found in all cases but one (98.48%). Cannibalism was found in 56 cases (84.34%) and this phenomenon was graded as 1 in 35 cases, 2 in 13 cases, and 3 in six cases. The internalized cells frequently appeared apoptotic. Immunohistochemical analysis revealed that the internalized cells as well as the cannibal cells expressed CD68.     

Diffuse type of giant-cell tumor of tendon sheath: an ultrastructural study of two cases with cytogenetic support

Two cases of the diffuse type of giant-cell tumor of the tendon sheath (GCTTS) are described. Both tumors arose in the vicinity of large joints of the lower extremity, showing similar clinical and radiological features. Histologically, a proliferation of polygonal mononuclear cells was seen, together with osteoclastlike giant cells, foam cells, and siderophages. The tumors were poorly delineated, displaying an infiltrative pattern into the neighboring soft tissues. Immunohistochemically, strong expression of vimentin, neuron-specific enolase, A 1 -antitrypsin, and CD68 was found in both mono- and multinucleated tumor cells. At the ultrastructural level, mononuclear cells revealed a diverse morphology, displaying features of histiocytelike and fibroblastlike cells, with the former being more numerous. Scarce neurosecretorylike granules, made up of electrondense membrane-bound material, were found in the cytoplasm of the mononuclear cells. Cytogenetic analysis of one case shows the presence of a clonal population with 47 chromosomes and two different translocations, t(2;3) and der(8) t(8;12). Present findings provide further support regarding the neoplasic nature of this tumoral entity.     

膀胱炎性肌纤维母细胞肿瘤的临床病理观察

目的探讨膀胱炎性肌纤维母细胞肿瘤的临床病理特点、诊断及鉴别诊断。方法采用HE、免疫组织化学（En Vision法）染色方法,观察5例膀胱炎性肌纤维母细胞肿瘤的组织学特点和免疫学表型,并对其中4例进行了随访。结果男性3例,女性2例,年龄10～53岁,平均年龄35岁;肿瘤位于膀胱顶底部3例,左侧壁2例,临床上表现为尿痛、血尿等症状。组织学表现为黏液丰富区和富于细胞区,梭形和星芒状肿瘤细胞散在或束状排列,间质可见大量纤细扩张的小血管网形成,各种炎细胞包括浆细胞、嗜酸性粒细胞、淋巴细胞及中性粒细胞的浸润。免疫组织化学检测显示瘤细胞AE1／AE3、波形蛋白、平滑肌肌动蛋白（SMA）、Calponin全部阳性,3例钙结合蛋白、4例结蛋白和4例间变性淋巴瘤激酶（ALK1）阳性。随访4例目前均存活,无复发。结论膀胱炎性肌纤维母细胞肿瘤是一种具有特殊临床病理特征的少见的中间型具恶性潜能的肿瘤。     

Warthin's tumor with multiple granulomas: a clinicopathologic study of six cases

Granulomatous inflammation occurring in Warthin's tumor is a rare phenomenon. It has been suggested that prior fine-needle aspiration (FNA) might be a triggering factor. We studied cases of Warthin's tumor with granulomatous inflammation to analyze the possible relationship with prior FNA. Granulomatous inflammation was noted in 6 cases (1.6%) of 382 cases of Warthin's tumor in a medical center. Clinical history, histology, and cytology slides were reviewed. Special stains for detection of infective agents were performed. All 6 cases showed typical features of Warthin's tumor accompanied by multiple granulomas. No specific microbiologic infective agents were revealed in these cases. There were 2 cases with history of preoperative FNA; both already had granulomas in Warthin's tumor in cytologic specimens taken by aspiration. The pathogenesis of granulomatous inflammation in Warthin's tumor remains speculative. Our observation of granulomas in the cytology specimens did not support the hypothesis of prior FNA being the cause.     

促结缔组织增生性小圆细胞肿瘤的临床病理学研究

目的探讨促结缔组织增生性小圆细胞肿瘤（DSRCT）的细胞学和组织学形态、免疫学表型以及在石蜡包埋组织中检测EWS-WT1融合基因的可行性。方法回顾性复习15例DSRCT的临床资料、1例细胞学形态、14例组织学形态和15例免疫学表型,采用逆转录聚合酶链反应（RT-PCR）法检测1例石蜡包埋组织中的EWS-WT1融合性mRNA,经测序证实并确定融合类型。结果13例为男性,2例为女性,年龄范围12～38岁,平均23．8岁。临床上多表现为腹部不适、腹胀、腹痛和腹部包块,伴有呕心、便秘和体重减轻等症状。体检显示,多数病例于中下腹可触及质硬肿块,境界不清,活动度差。影像学检查显示腹腔或盆腔内多个或单个结节状肿块,直径为3—25cm,平均8．6cm。细胞学涂片显示,在出血性的背景中可见散在、成簇的小圆细胞,核染色深,核仁不清,核分裂象易见,胞质稀少。组织学上,肿瘤由深染的小圆细胞、卵圆形细胞及短梭形细胞组成,呈大小不一、外形不规则的巢状排列,大的瘤巢中央可见坏死,瘤巢之间为大量增生的纤维结缔组织,可伴有玻璃样变性。所有病例均弥漫强阳性表达AE1／AE3、波形蛋白、结蛋白和神经特异性烯醇化酶,部分病例尚表达CAM5．2、上皮膜抗原、CD57、嗜铬粒素A、突触素和WT1,不表达肌生成素、CK5／6、CD117、钙（视）网膜蛋白和CD99。RT-PCR检测出EWS-WT1融合性mRNA,测序结果显示由EWS基因的7号外显子与WT1基因的8号外显子融合所产生,融合性基因含有KTS序列。结论（1）DSRCT是一种好发于青少年男性腹腔和盆腔内、具有多向性分化的高度恶性小圆细胞肿瘤。（2）瘤细胞特征性的波形蛋白和结蛋白核旁点状染色,在DSRCT的诊断和鉴别诊断中具有十分重要的价值。（3）在石蜡包埋的DSRCT组织中能检测出EWS-WT1融合基因的转录产物,RT-PCR可作为DSRCT一项实用的分子遗传学诊断手段。     

胰腺实性-假乳头状瘤2例临床病理分析

目的 探讨胰腺实性-假乳头状瘤(SPT)的临床病理学特征及诊断、鉴别诊断。方法 对2例SPT行临床病理学分析及免疫组化研究。结果 2例患者均有腹部包块及腹痛症状。组织学：单形性肿瘤细胞构成实性及假乳头状结构，常伴有出血、囊性变，间质不同程度硬化。免疫表型：Vim( )、NSE( )、αl-AT( )、ER( )、PR( )；α-ACT灶性( )；上皮性标志(-)、Syn(-)、CgA(-)、CD68(-)。结论 胰腺SPT是较少见的、预后较好的交界性肿瘤，其诊断与鉴别诊断主要依据临床资料、组织学形态特征及免疫组化标记。     

恶性外周神经鞘瘤52例临床病理分析

目的观察恶性外周神经鞘瘤的临床病理学特征、诊断及鉴别诊断。方法回顾性分析52例恶性外周神经鞘瘤的临床病理学及免疫表型特征并复习相关文献。结果 52例患者中,男女发病率为1∶1,年龄4~71岁,头颈部18例(35%),四肢12例(23%),躯干9例(17%),深部组织8例(15%),椎管内4例(8%),生殖道1例(2%)。镜下肿瘤组织呈束状或漩涡状排列,瘤细胞短纺锤形、卵圆形、梭形,核分裂象易见。免疫表型:瘤细胞局灶表达S-100蛋白,Ki-67增殖指数10%~70%。结论恶性外周神经鞘瘤罕见,侵袭性高,预后差,其组织形态复杂多样,需与滑膜肉瘤、纤维肉瘤、血管外皮瘤、富于细胞性神经鞘瘤、纤维型脑膜瘤以及平滑肌肉瘤等鉴别。