浅表肢端纤维黏液瘤的临床病理特征

目的 探讨浅表肢端纤维黏液瘤(SAF)的临床病理学特点、免疫表型和鉴别诊断.方法 对1例发生于左手中指末端SAF的临床表现、组织形态和免疫学表型进行回顾性分析,并复习文献.结果 患者男,62岁.因左手中指背侧末端肿块伴疼痛就诊,曾有外伤史.术中见肿块近甲床,并深达骨膜.大体观察,肿块周界不清,直径约2 cm,切面呈灰白色,实性,质韧.镜下观察,肿瘤位于真皮层内,略呈分叶状.瘤细胞由梭形至星形纤维母细胞样细胞组成,呈杂乱状分布于黏液样基质内,局部区域可呈条束状或疏松的席纹状排列.黏液样基质内含有丰富的纤细血管,并可见较多散在的肥大细胞.瘤细胞异型性不明显或仅显示轻度的异型性,核分裂象罕见.肿瘤内也未见坏死.免疫组织化学标记显示,梭形和星形细胞表达波形蛋白、CD34和CD99,灶性表达CD10,不表达上皮细胞膜抗原、肌动蛋白、结蛋白和S-100蛋白.结论 SAF好发于成年人指趾末端.熟悉其临床病理特点则有助于与其他发生于指趾的软组织黏液性肿瘤相鉴别.临床上宜将SAF作完整性切除,以预防局部复发.     

巨细胞血管母细胞瘤的临床病理学研究

目的 探讨巨细胞血管母细胞瘤(GCAB)的临床病理学特征、影像学特点、免疫学表型及其鉴别诊断.方法 对1例15个月的男患儿发生于左侧胫骨、腓骨中上段的GCAB进行影像学、光镜观察和免疫组织化学标记.结果 X线片及CT片显示左侧胫骨、腓骨中上段骨病变,以胫骨改变明显,病变骨干明显增粗变形,髓腔内密度增高不均匀,骨皮质不规则增厚,骨内缘局部皮质缺损,邻近的软组织肿胀.镜下病变组织位于骨小梁之间,肿瘤组织呈结节状、梁索状或丛状聚集,瘤细胞卵圆形至梭形,散在胞质丰富、核仁明显的单核组织细胞样巨细胞及多核巨细胞;肿瘤组织内可辨认出数量不等的无或含有少量红细胞的微血管腔隙;部分瘤细胞呈同心圆样围绕在微血管腔隙周围并与周围间质胶原共同形成洋葱皮样外观.肿瘤间质疏松,其间为少量梭形纤维母细胞样细胞、星形间质细胞、单个核炎性细胞及散在的肥大细胞.免疫组织化学标记显示,卵圆形至梭形瘤细胞及巨细胞弥漫性强阳性表达波形蛋白;多数瘤细胞显著表达CD31及CD34,少数弱阳性表达第八因子相关抗原(FⅧRAg);单核组织细胞样巨细胞及多核巨细胞强阳性表达CD68,偶见个别巨细胞胞质内局灶性表达FⅧRAg;肿瘤实质内小血管腔隙周围瘤细胞显著表达α-平滑肌肌动蛋白(SMA),其他部分瘤细胞呈弱阳性表达.结论 GCAB是一种罕见的发生于婴幼儿并具有独特形态学特征及局部具有侵袭性行为的血管源性肿瘤,不仅可发生于皮肤、黏膜、皮下及深部软组织,还可发生于骨内.     

增生性筋膜炎临床病理分析

目的：探讨增生性筋膜炎的临床病理特点。方法：例1为男性患者,74岁,发现右颌下区包块1周;例2为女性患者,71岁,发现左颈部包块1周,迅速长大3 d。观察2例增生性筋膜炎的组织学及免疫表型并复习相关文献。结果：2例患者镜下见肿块由大量形态不规则的梭形细胞组成,呈致密区与疏松区交替排列。致密区中梭形细胞排列成编织状、席纹状。疏松区梭形细胞呈束带状排列于胶原和黏液样间质为主的间质中,其间可见神经节样大细胞。免疫组织化学显示：例1梭形细胞的波形蛋白（vimentin）、平滑肌肌动蛋白（smooth muscle actin,SMA）、钙调蛋白结合蛋白和肌动蛋白结合蛋白呈阳性表达,不表达可溶蛋白-100（soluble protein-100,S-100）和细胞分化簇（cluster of differenation 34,CD34）;例2梭形细胞的vimentin和SMA呈阳性表达,不表达细胞角蛋白（cytokeratin,CK）、S-100、人黑色素相关抗体（human melanoma-related antibody 45,HMB45）和CD34,两例Ki-67阳性率均低于2%。结论：增生性筋膜炎是一种罕见的良性增生性病变,手术切除是主要治疗手段。因其临床及病理特征,易被误诊为恶性肿瘤而予以后续的放射治疗和化学治疗,故应加强对该病的认识,避免误诊和漏诊。     

指趾纤维黏液瘤3例临床病理分析

目的探讨指趾纤维黏液瘤(digital fibromyxoma, DF)的临床病理学特征、诊断及鉴别诊断。方法回顾性分析3例DF的临床资料、病理学形态及免疫表型特征,并复习相关文献。结果 3例DF中男性2例,女性1例,年龄30～57岁,平均44岁,且病变均发生于手指。肿瘤最大径1.5～2 cm。镜下见肿瘤细胞呈梭形和星形纤维母细胞样,束状分布于黏液样基质内;黏液样基质富含纤细的血管,瘤细胞异型性不明显或仅轻度异型,核分裂象罕见。免疫表型:梭形和星形细胞表达CD34、CD10和CD99,灶性表达EMA,不表达MUC4、desmin和S-100蛋白,Ki-67增殖指数1%～2%。3例患者手术切除后均痊愈。结论 DF属少见的良性肿瘤,为防止局部复发,临床上应行肿瘤完整切除术。     

脾脏硬化性血管瘤样结节性转化临床病理分析

目的 探讨脾脏硬化性血管瘤样结节性转化的病理形态特征、形成原因及鉴别诊断.方法 对10例脾脏硬化性血管瘤样结节性转化病例进行病理形态观察,EnVision法行免疫组织化学染色,并行网状纤维染色和PAS染色,1例行电子显微镜观察.结果 脾脏硬化性血管瘤样结节性转化有其特征性血管瘤样结节,免疫组织化学显示结节内内皮细胞异质性表达,即小叶状分布毛细血管免疫表型为CD34~+/CD31~+/CD8~-,窦岸样细胞免疫表型为CD8~+/CD31~+/CD34~-,小静脉样血管免疫表型为CD31~+/CD8~-/CD34~-,结节内梭形细胞混合表达CD31、平滑肌肌动蛋白(SMA)和CD68,结节间梭形细胞混合表达SMA和CD68.网状纤维染色显示结节轮廓和结节内血管,PAS染色显示结节周围胶原沉积物.电镜显示淋巴窦扩大,胶原丰富等特征.结论 该病是一种少见的脾脏良性病变,可能是一种反应性病变,与血管瘤出血坏死机化关系密切.尤其需要与脾脏交界性或恶性肿瘤鉴别.     

十二指肠神经节细胞性副神经节瘤3例临床病理分析

目的探讨神经节细胞性副神经节瘤(gangliocytic paraganglioma,GP)临床病理特征、诊断、鉴别诊断、治疗及预后。方法回顾性分析3例十二指肠GP的临床病理学特征、免疫表型、治疗及预后,并复习相关文献。结果3例患者内镜下均表现为十二指肠黏膜下病变,隆起于肠腔;组织学上肿瘤由3种不同类型的细胞混合组成:梭形的施万细胞、上皮样神经内分泌细胞和神经节样细胞。免疫表型:梭形细胞表达SOX-10、NF、NSE、CD56、Syn、S-100蛋白,上皮样细胞表达CKpan、CAM5.2、NSE、CD56、Syn、CgA、PR,神经节样细胞表达NSE、CD56、Syn、CgA、Calretinin、PR、NF。结论GP属于罕见的神经内分泌肿瘤,多发于十二指肠,内镜提示十二指肠黏膜下病变时应考虑该病可能,确诊依赖于组织病理学特征及免疫表型,治疗以内镜下切除和外科手术切除为主,患者预后较好。     

树突状纤维黏液脂肪瘤6例临床病理分析

目的 探讨树突状纤维黏液脂肪瘤(dendritic fibromyxolipoma, DFML)的临床病理学特征。方法 回顾性分析6例DFML的临床病理学特征、免疫表型、诊断及鉴别诊断等,并复习相关文献。结果 6例中男性4例,女性2例,年龄6～57岁,平均40岁;肿瘤直径1～7.5 cm,平均3.3 cm;肿瘤发生于腋窝、腿等体表部位。肿块多为类圆形,切面灰白、灰黄色,胶冻或黏液样。镜下形态学大致相同,均表现为在黏液样间质及绳索样(瘢痕样)胶原纤维背景中见混杂不同比例的小梭形细胞、星芒状细胞或成熟脂肪细胞。免疫表型：梭形细胞和星芒状细胞vimentin、CD34均强阳性,BCL-2弥漫/灶阳性,部分成熟的脂肪细胞S-100阳性,散在的肥大细胞CD117阳性,CK、desmin、SMA、HMB-45、MDM2、CDK4、p16、EMA、β-catenin、STAT6、CD56、NSE均阴性。Ki-67增殖指数均<1%。结论 DFML是一种良性脂肪瘤,是梭形细胞脂肪瘤的黏液样亚型,病理检查结合免疫表型有助于确诊,患者肿块切除后多可治愈。     

异位错构瘤性胸腺瘤的临床病理学观察和免疫组织化学研究

目的探讨异位错构瘤性胸腺瘤的临床病理学特征、免疫学表型和组织发生。方法对2例异位错构瘤性胸腺瘤进行临床病理学分析,采用免疫组织化学LSAB法行AE1/AE3、细胞角蛋白(CK)5、CK7、CK8、CK20、上皮膜抗原(EMA)、波形蛋白、CD5、CD10、α-平滑肌肌动蛋白(SMA)、calponin、结蛋白、CD34、S-100蛋白、CD57、胶质纤维酸性蛋白(GFAP)、甲状腺转录因子(TTF)-1和CD99标记。结果两例均发生于男性,年龄分别为20岁和40岁,因发现胸骨柄上缘肿块6个月和左锁骨上肿块2个多月就诊。肿块呈球形和卵圆形,边界清楚,最大直径分别为5cm和3cm,切面呈灰白色,质地柔软。组织学上,肿瘤由梭形细胞、上皮细胞和成熟的脂肪细胞3种成分混合组成。梭形细胞成分在2例中分别占到85%和70%,形态上类似纤维母细胞,多呈束状、编织状或席纹状排列。上皮细胞成分在两例中均占10%左右,形态上以非角化性鳞状上皮为主,可呈实性的小岛屿状、类似造釉细胞瘤的条索状和扩张的囊肿样排列,部分区域显示腺样分化,可见腺管形成。上皮细胞和梭形细胞在形态上有移行。成熟的脂肪组织呈不规则性分布,在两例肿瘤中所占的比例分别为<5%和20%。免疫组织化学标记显示,上皮细胞表达AE1/AE3、CK5、CK7、CK8和EMA,梭形细胞除CK外还表达波形蛋白、CD10和CD34,并部分表达α-SMA和calponin。两种细胞成分均不表达CK20、CD5、TTF-1、结蛋白、S-100蛋白、CD57、GFAP和CD99。结论异位错构瘤性胸腺瘤是一种好发于中青年男性锁骨上和胸锁关节附近软组织内的良性肿瘤,是一种由上皮和肌上皮组成的混合性肿瘤,可能起自于His颈窦,采用鳃原基混合瘤来命名或许更为合适。     

腹腔内淋巴结外滤泡树突细胞肉瘤的临床病理学分析

目的探讨腹腔内结外滤泡树突细胞肉瘤的临床病理学特征、免疫表型及与EB病毒感染的相关性。方法分析4例腹腔内结外滤泡树突细胞肉瘤的临床和形态特点,采用CD21、CD23、CD35、波形蛋白、S-100蛋白、上皮细胞膜抗原（EMA）、CD68、CD34、CD117、HLA-DR、CD1a、结蛋白和d-平滑肌肌动蛋白等抗体进行免疫组织化学标记（EnVision法）,采用原位杂交检测EBER。结果患者发病年龄28-63岁（平均42岁）,男性3例,女性1例。临床上表现为腹部不适、腹痛和腹部肿块。影像学检查显示,1例患者同时含有2个病灶,分别位于胃黏膜下和肝左叶,另3例表现为腹腔内肿块,术后显示分别位于阑尾、空肠系膜和大网膜。2例在术前被诊断为胃肠道间质瘤。大体上,肿瘤呈结节状,平均直径为10．8cm,3例肿瘤内可见明显的坏死灶。镜下4例非肝脏性肿瘤均由胖梭形、卵圆形至上皮样细胞组成,可见散在的多核瘤细胞。瘤细胞主要呈席纹状、旋涡状或条束状排列,部分区域呈片状分布。肿瘤内可见散在的淋巴细胞,常围绕血管分布,并与瘤细胞形成双相细胞性形态。2例肿瘤显示明显的异型性,核分裂象易见（包括病理性）。3例肿瘤内可见凝固性坏死。1例肝脏肿瘤由大量的慢性炎性细胞和散在的异形大细胞组成,呈炎性假瘤样。免疫组织化学标记显示,瘤细胞均弥漫强阳性表达CD21、CD23和波形蛋白,部分表达CD35、S-100蛋白、CD68、HLA-DR和EMA,不表达CD34和CD117。2例EBER病毒检测均为阴性。结论发生于腹腔内的结外滤泡树突细胞肉瘤非常少见,熟悉其特征性的病理形态和免疫表型有助于与腹腔内其他类型梭形细胞肿瘤（特别是胃肠道间质瘤）的鉴别诊断。除经典型外,发生于肝脏者可呈炎性假瘤样形态,易被误诊。腹腔内非肝脏性滤泡树突细胞肉瘤与EB病毒感染的关系并不密切。     

乳腺结节性筋膜炎1例及文献复习

目的探讨乳腺结节性筋膜炎的临床病理特征。方法对1例乳腺结节性筋膜炎的临床表现、组织形态和免疫表型进行研究,并文献复习。结果该例肿块3cm×2cm,边界不清,向周围乳腺及脂肪组织浸润。肿块主要由梭形细胞组成,其中含少量破骨样巨细胞。梭形细胞表达Vim、SMA和MSA,破骨样巨细胞表达CD68(KP-1),未发现CK阳性细胞。结论结节性筋膜炎很少复发,也不转移,组织学形态易与一些良恶性肿瘤混淆,需做免疫组化检测以明确其性质。     

Nasal chondromesenchymal hamartoma of infancy: The first Japanese case report

The first Japanese case of nasal chondromesenchymal hamartoma, a rare infantile nasal neoplasm, is presented. A 4-month-old Japanese boy was referred to our Centre because of intranasal mass and oculomotor disturbance. Radiological images showed the intranasal tumor extending to the paranasal sinus, orbit, and anterior frontal fossa. Subtotal resection and radiation therapy to residual tumor were performed. There has been no recurrence of the tumor for 13 years. Histologically, the lesion demonstrated admixture of various mesenchymal elements, including cellular spindle cell stroma with occasional myxoid change, nodules of mature/immature cartilaginous tissue, focal osteoclast-like giant cells, and erythrocyte-filled spaces resembling aneurysmal bone cyst. The histology was consistent with the findings presented by McDermott et al. 1986. Immunohistochemically, the spindle cells were positive for vimentin and smooth muscle actin. Chondrocytes in the mature cartilaginous tissue were positive for S-100 and vimentin; chondrocytes in the immature cartilaginous tissue were positive for S-100, vimentin, and smooth muscle actin. Ultrastructurally, the spindle cells showed features of either fibroblast or myofibroblast.     

树突状纤维黏液脂肪瘤的临床病理学研究

目的探讨树突状纤维黏液脂肪瘤的临床病理特征,并讨论与其他易于混淆的软组织肿瘤的鉴别诊断。方法收集8例树突状纤维黏液脂肪瘤,观察和分析其临床和病理组织学特征,并通过免疫组织化学标记链霉素卵白素生物素(LSAB)法,分析其CD34、bcl-2、波形蛋白、细胞角蛋白(CK)、上皮膜抗原(EMA)、S-100、HHF35和平滑肌肌动蛋白(SMA)等的表达。结果该肿瘤主要见于中老年男性皮下软组织或肌筋膜;主要分布在颈、背、肩及小腿,也可见于面部和足部。大体上边界清楚或有薄包膜,切面部分呈黏液样或胶冻状。该肿瘤在组织形态学上以成熟脂肪组织、增生的梭形细胞或星状细胞、黏液样及纤维化间质等为主要成分,在不同区域以不同的比例混杂构成。梭形细胞或星状细胞伸出细长分枝状胞质突起是其形态特征之一。无细胞异型性和核分裂象。此外,该肿瘤常有较丰富的丛状小血管和毛细血管,间质有肥大细胞、小淋巴细胞及浆细胞浸润。免疫组织化学染色梭形细胞显示CD34、bcl-2和波形蛋白强阳性,而HHF35、SMA和S-100阴性,肿瘤中所有成分对CK和EMA呈阴性反应。结论树突状纤维黏液脂肪瘤在临床表现、病理组织学改变以及免疫组织化学反应等方面有其独自的特点。该肿瘤似乎是介于梭形细胞脂肪瘤和软组织孤立性纤维性肿瘤的中间类型。需注意与黏液性脂肪肉瘤和黏液性恶性纤维组织细胞瘤鉴别诊断。该肿瘤生物学行为为良性,只需局部手术切除。     

Angiomyofibroblastoma of the Male Perineum: An Unusual Location for a Rare Lesion

We report a case of angiomyofibroblastoma (AMF)-like tumor of the perineum in a 54-year-old man. The asymptomatic lesion measured 3 cm and appeared as a tan homogeneous mass. Histologically, it appeared as a circumscribed nodular spindle cell proliferation with alternating cellular and hypocellular areas. The spindle cells exhibited minimal nuclear pleomorphism and scanty mitotic activity. Focally, some cells were epithelioid. Large blood vessels were present, with perivascular fibrosis. The spindle cells expressed vimentin, but not desmin, actin, S100, or CD34. These features were similar to those of AMF as initially reported in the vulva. A perineal localion of this lesion in a male has not been reported in the literature, to the best of our knowledge. Int J Surg Pathol 8(1):79-82, 2000     

Calcifying fibrous tumor of the adrenal gland

Calcifying fibrous tumor is an uncommon entity with distinctive pathologic features. Most calcifying fibrous tumors involve the peripheral soft tissues or serosal surfaces, with reports of visceral examples of this lesion being extremely limited. We report the clinical and pathologic features of an unusual case of calcifying fibrous tumor occurring in the adrenal gland of a 32-year-old woman. Microscopically, the lesion was well circumscribed and composed of dense, poorly cellular collagenous tissue, scattered spindle cells, an inflammatory infiltrate consisting of plasma cells and lymphocytes, and dystrophic calcifications. The morphologic diagnosis of calcifying fibrous tumor was supported by diffuse positive immunoreactivity for factor XIIIa and absence of reactivity for muscle specific actin, smooth muscle actin, and anaplastic lymphoma kinase. Although rare, awareness that calcifying fibrous tumor may occur at this particular site is important so as not to confuse this lesion with other mesenchymal neoplasms of the adrenal gland.     

Low-grade periductal stromal sarcoma of the breast with myxoid features: Immunohistochemistry

A 52-year-old woman was admitted with a painful right breast tumor measuring more than 20 cm in largest diameter, which ulcerated the overlying skin. The lesion had appeared 4 years previously but the patient hesitated to seek medical care due to 'fear of cancer'. Microscopically, the tumor was composed of spindle cells that formed cuffs around multiple open tubules and ducts set in an abundant, myxoid stroma. The spindle cells had significant atypia with nuclear pleomorphism, occasional cytoplasmic vacuolation and moderate mitotic activity. The ducts and lobules surrounded by the proliferating tumor cells had minimal distortion, with a pericanalicular growth pattern devoid of the phyllodes pattern. The tumor had a multinodular growth pattern with coalesced and individual tumor nodules, the latter being found mostly at the periphery of the lesion. On immunohistochemistry the tumor cells were positive for smooth muscle actin, CD34, and vimentin, and focally positive for CD10. A diagnosis of low-grade periductal stromal sarcoma (PDSS) with myxoid features was established. PDSS is a distinct low-grade breast sarcoma, the appropriate diagnosis of which requires extensive tumor sampling and additional broad immunohistochemistry. PDSS should not be confused with other spindle cell breast tumors because they require different treatment.     

Angiomyolipoma of the large intestine: report of a case.

A case of an angiomyolipoma of the large intestine occurring in a 55-year-old man without evidence of tuberous sclerosis is reported. Endoscopically, the lesion resembled a sessile adenomatous polyp. The tumor measured 1 cm. Histologic examination revealed a lesion composed predominantly of spindle and epithelioid cells with significant nuclear atypia. Mitoses were rare. The tumor was strongly positive for HMB-45, CD68, vimentin, desmin, and smooth muscle actin. Rare scattered cells reacted with CD34. No residual tumor was found in the resected colon.     

Elastofibroma: a clinicopathologic and immunohistochemical study of seven cases and literature review

Elastofibroma is a rare fibrous lesion characterized by accumulated abnormal elastic fibers whose etiology remains largely unknown. In this study, we analyzed seven cases of elastofibroma to further explore the characteristics of its cellular composition. Immunohistochemistry was performed for mast cell tryptase, S-100 protein, vimentin, CD34, smooth muscle actin, desmin and collagen type IV. Histochemical staining methods for Gomori's trichrome and Verhoeff elastica-van Gieson were also evaluated. Histopathologically, a haphazard array of collagen, eosinophilic amorphous fibers, and globules in a fibrous tissue was seen. The elastic nature of the fibers was confirmed by elastic stain, and with Gomori's trichrome collagen fibers were also demonstrated. The interspersed spindle or stellate cells were almost consistently positive for vimentin and frequently positive for CD34. Mast cell tryptase-positive cells were present in five of the cases. Collagen type IV immunoreactivity was seen in two cases. No staining was observed with smooth muscle actin, desmin or S-100 protein. Our findings suggest that CD34-positive mesenchymal cells are an integral component of elastofibroma.     

Myopericytoma: a pleural-based spindle cell neoplasm off the beaten path

Myopericytoma is a recently described hemangiopericytoma-like neoplasm with myoid differentiation. These tumors are typically located in the subcutaneous and soft tissues of the extremities. The authors report a rare pleural-based pulmonary myopericytoma in a 58-year-old woman. The lesion was grossly homogeneous and well circumscribed. Microscopically, it was composed of densely packed spindle cells organized as whorls and short interlacing fascicles with a concentric perivascular distribution. Immunohistochemical reactions were positive for vimentin, smooth muscle actin (SMA), muscle-specific actin, and Bcl-2 and negative for desmin, h-caldesmon, cytokeratin, and CD34. Atypically, increased mitotic activity was noted, but no other malignant features were identified. The differential diagnoses are discussed with specific emphasis on solitary fibrous tumor of the pleura, which is the most common benign pleural-based spindle cell neoplasm and may be a diagnostic pitfall with potentially harmful consequences.     

Expression of desmin and smooth muscle myosin heavy chain in dermatofibromas

BACKGROUND: The histopathologic features of dermatofibroma vary remarkably, and this diversity may occasionally cause problems in differentiating between benign and malignant mesenchymal lesions, including smooth muscle neoplasms. Immunohistochemical stains are sometimes necessary to clarify the histogenesis of a lesion. OBJECTIVE: To evaluate dermatofibromas for expression of desmin and smooth muscle myosin heavy chain (SM-MHC) antigens, which are commonly used as evidence of smooth muscle differentiation. METHODS: We studied 100 consecutive cases of dermatofibroma using hematoxylin-eosin-stained sections and immunoperoxidase staining with antibodies against desmin, SM-MHC, and smooth muscle actin. RESULTS: We found focal positivity for desmin in 9 cases, and in 2 of these cases, at least 10% of lesional cells showed strong expression. We found focal staining for SM-MHC in 10 cases, and in 2 of these cases, at least 10% of the lesional cells were positive. Regions positive for desmin and/or SM-MHC did not show definite histologic features of myogenous differentiation on hematoxylin-eosin-stained sections. All dermatofibromas expressing desmin and SM-MHC were also strongly positive for smooth muscle actin. CONCLUSIONS: About 10% of dermatofibromas show focal expression of desmin and SM-MHC, and this expression may be present in up to 10% to 15% of lesional cells. Thus, in dermal spindle cell lesions, focal expression of these muscle antigens, like that of smooth muscle actin, is not diagnostic of a smooth muscle tumor.