长骨骨纤维结构不良型釉质上皮瘤临床病理分析

目的探讨长骨骨纤维结构不良型釉质上皮瘤的临床病理特点、鉴别诊断及生物学特征。方法对2例长骨骨纤维结构不良型釉质上皮瘤进行病理学、免疫组织化学观察,结合临床资料进行分析并复习相关文献。结果2例长骨骨纤维结构不良型釉质上皮瘤患者均为年轻人,临床以小腿疼痛或无痛性肿胀为主,病程较长,影像学均示胫骨骨干病变。光镜下肿瘤主要由梭形细胞和散在的新生编织骨组成,梭形细胞密度较高,呈束状或编织状排列,纤维组织间见少数巢团状或裂隙状的上皮样细胞巢,梭形细胞和上皮样细胞均无明显异型性。免疫表型:上皮细胞巢表达CKpan、CK14和CK19,但不表达CK8、CK18。EGFR阳性表达主要见于上皮样细胞,Ki-67在上皮样细胞和梭形细胞均有少量散在阳性表达。结论长骨骨纤维不良型釉质上皮瘤是一种罕见的好发于年轻人胫骨骨干的低度恶性肿瘤,主要根据影像学、光镜及免疫组化确诊,应与骨纤维结构不良、转移癌、滑膜肉瘤等相鉴别。     

上皮样肉瘤临床病理特征并文献复习

目的:探讨上皮样肉瘤的临床病理学特点及诊断要点。方法:对2例上皮样肉瘤进行临床资料、病理形态学及免疫组化观察,并结合文献对其诊断及鉴别诊断进行探讨。结果:镜下肿瘤由上皮样细胞或梭形细胞构成,呈结节状排列,浸润性生长;免疫组化示肿瘤细胞Vimentin、CK、EMA、CD34呈阳性。结论:上皮样肉瘤,临床少见,因此缺乏对其认识,从而易与其他肿瘤混淆导致误诊。提高对上皮样肉瘤的认识,对避免误诊是至关重要的。     

特殊类型肾盂尿路上皮癌8例临床病理分析

目的探讨特殊类型的肾盂尿路上皮癌的临床病理特点。方法对2000年～2006年间收集的8例具有特殊临床病理特征的肾盂癌作光镜和免疫组化染色观察,并行随访。结果肾盂尿路上皮癌特殊的组织学表现为：肉瘤样癌（3例）,鳞癌和伴鳞癌（2例）,微乳头癌（1例）,透明细胞癌（1例）,伴有横纹肌样及印戒样细胞特征（1例）。其中2例肉瘤样癌肉眼观呈弥漫浸润型,组织形态最具多样性,瘤细胞呈短梭形束状或假血管肉瘤样或弥漫多角形排列,间质伴假肉瘤样反应或黏液变性等。病理分期：1例高分化鳞癌为PT2,余7例均为PT2-PT4。8例均见尿路上皮乳头状癌或原位癌。免疫组化：微乳头癌、透明细胞癌表达CK7、CK20、CD15、CEA,肉瘤样细胞表达AE1／AE3、CK7、vimentin。横纹肌样及印戒样细胞表达AE1／AE3、CK7、3413E12、vimentin。随访3—6I）个月（平均29．3个月）,1例高分化鳞癌至今存活,1例伴有鳞癌的低分化尿路上皮癌术后至今8个月存活,但伴广泛转移,余6例于术后2—28个月死于肿瘤转移。结论肾盂尿路上皮癌多为低分化、高分期,类似于膀胱尿路上皮癌,具有多种细胞形态特征,易发生广泛浸润及转移,预后较差。其特殊的形态学变异和免疫组化特点,为其鉴别诊断提供了依据。     

43例食管基底样鳞状细胞癌临床病理分析

目的探讨食管基底样鳞状细胞癌（basaloid squamous cell carcinoma,BSCC）临床病理特点。方法对43例食管BSCC进行组织形态学及免疫组化观察,并对其临床病理特点及随访资料进行分析。结果BSCC由类似于鳞状上皮基底细胞样的细胞组成,细胞排列呈实性巢状、小梁状、假腺样或筛状结构,巢周边瘤细胞常呈栅栏状排列,巢中央可见粉刺样坏死。24例（55.8％）与普通鳞癌或腺鳞癌并存,11例（25.6％）出现局灶鳞化,28例（65．1％）见到脉管内癌栓。免疫组化染色CKpan、AE1、AE3均呈不同程度的阳性表达,p53、Ki-67、PCNA呈中至强阳性表达,Syn、CgA分别有1例呈弱阳性,S-100蛋白、SMA、CEA均为阴性。结论BSCC是食管一种少见的鳞状细胞癌变异型,具有独特的形态特点和明显的侵袭性行为,预后较差。免疫组化无特异性,诊断主要依靠形态学特点。     

乳腺导管内乳头状肿瘤的形态学和免疫表型特征

目的观察乳腺导管内乳头状肿瘤（intraductal papillary neoplasms,IDPN）的形态学和免疫表型特征,并探讨其诊断。方法根据WHO分类（2003）和Tavassoli等（1999、2003）有关标准,筛选出乳腺导管内乳头状肿瘤41例,对上述病例进行HE形态观察和免疫组化检测,选用的一抗有CK5、CK34βE12、CK8、CD34、SMA、p63、CD10。结果（1）导管内乳头状瘤19例,镜检为扩张的导管或小管内含纤维-脉管轴心的乳头状病变,由腺（系）上皮和肌上皮覆盖轴心形成双层构型。其中16例伴腺（系）上皮普通性增生,2例〈10％的乳头覆盖上皮呈不典型增生（AH）改变。腺（系）上皮CK5 17例（89．5％）中～强阳性;CK34βE1216例（84．2％）中～强阳性。肌上皮SMA、p63、CD10均阳性。（2）非典犁导管内乳头状瘤4例,3例伴发于囊内乳头状癌,1例作为主要诊断。表现为≥10％而〈90％的乳头覆盖上皮呈AH改变。其AH区域CK5均阴性,3例CK34βE12阴性～弱阳性。（3）导管内乳头状癌21例,其中①2例仅表现为≥90％的乳头区肌上皮层完全缺乏;②1例仪表现为〉90％的乳头覆盖上皮呈低级导管原位癌（DCIS）样改变;③8例兼具上两种特征;④10例乳头覆盖上皮呈中～高级别DCIS样改变。符合②及③者CK5均阴性,CK34βE12 6例（66．7％）阴性～弱阳性;符合④者CK5、CK34βE12 8例（80％）均阴性～弱阳性。结论IDPN是一组良恶性不同的病变,各自的HE形态和免疫表型均有差异,应采用HE形态和免疫组化相结合的综合标准进行诊断。     

表达CD10的非特殊类型乳腺肉瘤3例报道并文献复习

目的 探讨表达CD10的非特殊类型乳腺肉瘤的病理形态学、免疫表型特点以及诊断与鉴别诊断.方法 应用光镜观察、免疫组织化学染色等方法对3例表达CD10的非特殊类型乳腺肉瘤进行分析,并复习文献.结果 肿瘤境界清楚,主要由梭形-胖梭形细胞组成,与数量不等的胶原束交错呈流水样或席纹状排列,胞质红染或空淡,核梭形,染色体粗颗粒状,部分有细小核仁.1例伴大片坏死,1例伴出血及破骨细胞样巨细胞反应.免疫组化染色3例均CD10(+),2例EGFR(+),上皮标记物cK(AEl/AE3)、CK5/6、CK7、CKl4、CK8/18、3413E12及其他肌上皮标记物SMA、p63、ealponin等则(一).结论 表达CD10的非特殊类型乳腺肉瘤是一种罕见而具有独特免疫表型的肉瘤,诊断时须借助免疫组化与化生性癌、叶状肿瘤等鉴别.     

乳腺上皮样型管周间质肉瘤病理特征及与叶状肿瘤比较观察

目的 探讨乳腺上皮样型管周间质肉瘤的临床病理特点及与叶状肿瘤的关系。方法 采用HE、特殊染色、免疫组化染色(CK，EMA，S-100蛋白，SMA，Vim，Des，MG，CD34，CD99，CD117，PR，HMB45)对1例乳腺上皮样型管周间质肉瘤与5例叶状肿瘤(良性、交界性各1例，恶性3例)做比较性观察。结果 乳腺管周间质肉瘤(上皮样型)有独特的镜下图像：①显著的多角形(上皮样)细胞绕导管或小管的上皮肌上皮层呈间质性增生，无叶状结构；②组织学模式有袖套状、花冠状、菊形团状、结节状、融合结节状和片状浸润；③瘤细胞形态有：多角形(大、小)、柱状和梭形。多角形细胞呈上皮样形态，异型明显，核分裂象多见(10～30个／10HPF，个别区域达6个／1HPF)，病理性核分裂象易见，在浸润灶内见肿瘤性坏死；④瘤细胞Vim弥漫阳性、EMA灶性阳性、CD99和CD117灶性弱阳性、CD34少数阳性，CK、SMA、S-100蛋白、Des、MG、PR、HMB45均阴性。5例叶状肿瘤均具备叶状结构、间质过度增生、细胞密集(异质性分布)、核分裂象2～10个／10HPF等诊断要素。在3例恶性叶状肿瘤中，2例有极少的上皮样袖套状病灶，2例有梭形细胞袖套状病灶。结论 乳腺上皮样型管周间质肉瘤是一种极罕见的恶性纤维上皮肿瘤亚型，它可能是恶性叶状肿瘤的最早期病变，也可能是一种独特的类型。     

阴茎假血管肉瘤样鳞状细胞癌1例及文献复习

目的探讨阴茎假血管肉瘤样鳞状细胞癌(SCC)的诊断、组织学和免疫组化特征以及鉴别诊断。方法通过HE、免疫组化及特殊染色观察1例阴茎原发假血管肉瘤样鳞状细胞癌，并复习文献。结果镜下可见显著的棘细胞松解，肿瘤由排列呈腔隙状、网状结构的长梭形细胞组成，细胞异型性明显，核分裂象易见，胞质大多红染，局部区域弥漫出血坏死。免疫表型：CK、Vim阳性，CD34、CD31、SMA、FⅧRAg阴性。网状纤维染色不显示血管外形。结论假血管肉瘤样SCC是一种罕见的易与敌国管肉瘤混淆的特殊形态的SCC．需与血管肉瘤、癌肉瘤等鉴别。     

原发性甲状腺上皮样血管肉瘤1例报告

目的:观察1例原发性甲状腺上皮样血管肉瘤患者的临床病理特征,并结合相关文献进行讨论。方法和结果:本例原发性甲状腺上皮样血管肉瘤患者行术中快速冰冻切片及常规石蜡切片,HE染色和网状纤维染色、并行免疫组化检查。镜下见,肿瘤主要由实性片状或巢状排列的上皮样细胞组成,肿瘤细胞核较大,呈空泡状,核仁明显,部分肿瘤细胞胞质内可见含有红细胞的空泡或小管腔形成;可见迷路状血管,网状纤维染色能清晰显示这些管腔结构。免疫组化染色,肿瘤细胞CD31、CD34、AE1/AE3阳性,TG、TTF-1、calcitonin、CK19、S-100、EMA和HMB45阴性,Ki-67高表达。结论:原发性甲状腺上皮样血管肉瘤是一种少见的恶性肿瘤,致死率高。确诊主要依靠组织学和免疫组化检查,临床要与未分化癌、低分化癌及上皮样血管内皮瘤等鉴别。     

原发性子宫颈恶性黑色素瘤6例临床病理分析

目的 探讨原发性子宫颈恶性黑色素瘤(primary malignant melanoma of the cervix,PMMC)的临床病理学特征、诊断、鉴别诊断、治疗和预后。方法 回顾性分析6例PMMC临床资料,采用HE、免疫组化EnVision两步法染色,镜下观察其组织学形态、免疫表型,并复习相关文献。结果 6例患者发病年龄38～61岁,中位年龄53.5岁,临床表现均为阴道不规则出血,大体见子宫颈结节状或菜花状病灶,病灶最大径1～3.5 cm。镜下见肿瘤细胞弥漫分布,排列呈肉瘤样、巢状或结节状,细胞呈上皮样、梭形细胞样,部分呈较一致的小圆细胞样,细胞质丰富,红染或淡染,少数呈空泡样,核形态多样,可见嗜酸性大核仁,常见坏死及病理性核分裂象,4例可见色素,2例几乎不见色素。免疫组化标记MelanA(6/6)、vimentin(6/6)、HMB-45(5/6)、S-100 (5/6)阳性。1例失访,1例FIGOⅠ期患者确诊后28个月仍健在,4例FIGOⅡ期及以上患者确诊后3～16个月死亡。结论 PMMC恶性程度高,病灶超出子宫颈提示患者预后不良;无色素性PMMC需结合组织学形态及免疫组化与分化差的鳞状细胞癌、腺癌、横纹肌肉瘤、间质肉瘤、淋巴瘤等进行鉴别。     

Papillary renal cell carcinoma with prominent spindle cell stroma - tumor mimicking mixed epithelial and stromal tumor of the kidney: Clinicopathologic,morphologic, immunohistochemical and molecular genetic analysis of 6 cases

Papillary renal cell carcinoma (PRCC) is currently a well-studied type of RCC. In addition to PRCC type 1, there are a number of other subtypes and variants of PRCCs which have been reported. We describe a series of 6 PRCCs with papillary, micropapillary and/or tubulopapillary architecture and prominent spindle cell stroma, resembling stroma in mixed epithelial and stromal tumor of the kidney (MESTK) or sarcomatoid RCC.Clinicopathologic, morphologic, immunohistochemical and molecular features were analyzed.All patients were males with an age range of 44–98 years (mean 65.3, median 65.5 years). Tumor size ranged from 2.4–11.4 cm (mean 5.8, median 4.5 cm). Follow-up data were available for 4 patients, ranging from 3 to 96 months (mean 42.75, median 36 months). Epithelial cells were mostly cylindrical with eosinophilic cytoplasm, showing nuclear grade 2 and 3 (ISUP/WHO).In all cases, loose to compact prominent stroma composed of spindle cells, without malignant mesenchymal heterologous elements was detected. No atypical mitoses were found, while typical mitoses were rare in both epithelial and stromal components.Epithelial cells were positive for CK7, AMACR, and vimentin in all cases, while negative for TFE3, HMB45, desmin, CD34, and actin. The stroma was positive for vimentin, actin and focally for CD34, while negative for CK7, AMACR, TFE3, HMB45, and desmin. Estrogen and progesterone receptors were completely negative. FH and SDHB expression was retained in all analyzable cases. Proliferative index was barely detectable in stromal component and low in epithelial component, ranging 0 to 5% positive stained cells/high power field.Copy number variation was variable with no distinct pattern. No mutations in CDKN2A, BAP1, MET were detected.PRCC with MESTK-like features is a distinct variant of PRCC mimicking MESTK. Our findings add to the body of literature on ever expanding variants of PRCCs. Both epithelial and stromal components lacked true Müllerian features, which was also proven by immunohistochemistry.     

Chromophobe renal cell carcinoma: a morphologic and immunohistochemical study of 45 cases

The aim of this study was to evaluate the morphological spectrum of chromophobe renal cell carcinoma (CRCC) and diagnostic utility of a panel of three immunohistochemical stains. All cases of CRCC reported between 2002 and 2012 in the Section of Histopathology, Aga Khan University Hospital, were retrieved. A total of 45 cases were identified. Slides were reviewed and immunohistochemical stains (CK7, CD117, and vimentin) were performed. Ages ranged from 18 to 90 years (mean, 48.5 years). Male-to-female ratio was 0.8:1. The tumor was located in the left kidney in 24 patients and the right kidney in 20 patients. The tumor size ranged from 3.5 to 22 cm (mean 10 cm). Histologically, 4 were classic, 22 were eosinophilic, 16 were mixed, and 3 were sarcomatoid type. Morphologic patterns included broad alveolar, solid, nested, tubular, tubulocystic, trabecular, papillary, and microglandular. Binucleation and perinuclear halos were seen in all cases. Nuclear grooves and pseudoinclusions were seen in 17 and 6 cases, respectively. Multinucleated cells were seen in 19 cases. Mitoses ranged from 1 to 11/10 HPFs (mean 3/10 HPFs). Hyalinized stroma was seen in 38 cases and calcification in 26 cases. Necrosis was seen in 18 cases. Palisading of smaller cells around the broad alveolar pattern was noted in 5 cases. The Furhman’s nuclear grade was I (11), II (26), III (5), and IV (3). Hale’s colloidal iron was positive in all cases. Immunohistochemical stain CK7 and CD117 were positive in 100% and 95.5% of cases respectively. Vimentin was negative in all cases, except in the sarcomatoid areas of 3 cases. In conclusion, chromophobe renal cell carcinoma has certain unique morphological features and immunohistochemical profile which help to distinguish it from conventional renal cell carcinoma and oncocytoma. We identified nuclear pseudoinclusions, microglandular pattern and palisading of smaller cells, which have not been reported earlier.     

Sarcomatoid carcinoma of the small intestine: Histologic, immunohistochemical and ultrastructural features of three cases and its differential diagnosis

Three cases of sarcomatoid carcinoma of the small intestine are presented. One of them was found accidentally in the duodenum of a patient with a well differentiated adenocarcinoma and a malignant lymphoma that were limited to the stomach. The other two cases arose from the lleum. All of the tumors were whitish, soft and ulcerated with focal hemorrhage and necrosis and showed expansive growth. Each tumor consisted of a mixture of polygonal and spindle shaped anaplastic neoplastic cells arranged in sheet, short fas-cicular or haphazard fashion, with no finding suggesting epithelial differentiation. Special stains demonstrated intra-cellular mucin in only a small number of tumor cells in two cases, but not in the other case. Immunohistochemically, the tumor cells of two cases at both primary and metastatic sites showed a positive immunoreaction for cytokeratin and epithelial membrane antigen. In the other case, only a few tumor cells at the metastatic site, but not at the primary site, showed cytokeratin positivity. Various numbers of tumor cells positive for vimentin, α-1-antitrypsin (AAT), α-1-antichy-motrypsin (ACT) and KP-1 were detected in each case. Ultrastructurally, some populations of tumor cells possessed various amounts of tonofilaments with a few Intercellular connections between adjacent tumor cells. These cases should be classified as sarcomatoid carcinoma of the small intestine, despite partial or complete loss of epithelial features, and distinguished from the various sarcomas.     

肾脏黏液性管状和梭形细胞癌的临床及病理学特点

目的 探讨肾脏黏液性管状和梭形细胞癌的临床病理学特点.方法 分析7例肾脏黏液性管状和梭形细胞癌的临床特点、组织形态及免疫表型特点[CD10、CK7、CK18、CK19、Villin、上皮细胞膜抗原(EMA)、P504S和波形蛋白],并复习相关文献.结果 7例黏液性管状和梭形细胞癌中,男性3例,女性4例,平均年龄48.2岁(39～61岁).均为体检时发现肿瘤,肿瘤最大径平均5.5 cm(4.0～9.0 cm),术后随访18～51个月,得到随访资料的5例均无复发及转移.肿瘤大体切面均为实性、灰白色,无包膜,但与周围肾组织分界清晰.镜下观察肿瘤细胞主要由两种形态构成:均一的由立方细胞构成的紧密排列的小管状结构和梭形细胞成分.两种成分比例或多或少,交错分布,其中5例伴有黏液样基质,3例见到明显的透明细胞区域,1例可见灶状肉瘤样区域,1例见乳头状结构及泡沫细胞.免疫组织化学染色显示,7例肿瘤CK7均呈阳性表达,EMA、CK18和P504S在染色的5例中全部呈阳性表达,CK19在染色的5例中有4例表达,而CD10、Villin和波形蛋白表达差异较大.结论 肾脏黏液性管状和梭形细胞癌是一种低度恶性的多形性肿瘤,组织形态学谱系较宽,不典型的病例可以主要由两种成分之一构成,并缺少黏液,有些病例可见到透明细胞、乳头状结构,少数可见肉瘤样形态及坏死.免疫表型上对于从近曲小管到集合管的标志物均有表达.

Abstract：

Objective To investigate the clinical and pathological features of the mucinous tubular and spindle cell carcinoma (MTSCC) of the kidney. Methods Seven cases of MTSCC were analyzed by gross examination and light microscopy. Immunostaining was performed to detect the expression of CD10, CK7, CK18, CK19, Villin, EMA, P504S and vimentin. The literature on this tumor was reviewed to discuss the histological features of MTSCC and its clinical behavior. Results Three of 7 cases were male and the other 4 were female. The mean age of the patients was 48.2 years old, with a range from 39 to 61 years. All the patients presented no symptom and their tumors were found by health examination. Tumors averaged 5.5 cm in greatest dimension (range from 4.0 cm to 9.0 cm). The tumors were well-circumscribed without capsules, and the cut surfaces were solid and soft with white-tan color. By light microscopy, tumors were composed of tightly packed, small, elongated tubules with transitions to spindle cell components. Five cases had mucinous stroma. Clear cell clusters, focal sarcomatoid differentiation, papillations and foamy macrophages were seen in several cases. Immunohistochemically, all 7 cases showed positive for CK7, five of 5 cases positive for EMA, CK18 and P504S, four of 5 cases positive for CK19, but heterogeneous for CD10, villin and vimentin expression. No evidence of local recurrence or distant metastases was identified in the 5 patients with follow-up information. Conclusions The mucinous tubular and spindle cell carcinoma is a low-grade and polymorphic neoplasm. The morphology of these tumors may not be uniform with a wide histological spectrum. The tumors can be tubular predominant or spindle cells predominant with scant to abundant mucinous stroma, which coupled with the presence of other unusual features such as clear cells, papillations, foamy macrophages, necrosis and sarcomatoid differentiation. Immunohistochemically, MTSCC can express the markers from the proximal convoluted tubules to collecting tubules.     

乳腺上皮-肌上皮性肿瘤4例临床病理学分析

目的：探讨乳腺上皮-肌上皮性肿瘤(epithelial-myoepithelial tumor of breast)的临床病理学特点、免疫表型、诊断及鉴别诊断。方法：对4例乳腺上皮-肌上皮性肿瘤的临床特点、组织形态学及免疫组织化学结果进行分析,并复习相关文献。结果：患者：男性1例,女性3例,平均年龄51岁(27~63岁)。4例肿瘤直径1.5~3.0 cm(平均2.0 cm),无包膜,切面灰白色。显微镜下可见肿瘤由双相增生的肌上皮细胞和腺上皮细胞构成,肌上皮细胞环绕腺上皮细胞构成特征的套管结构。免疫组织化学染色,腺上皮细胞表达CK8/18、CK7,肌上皮细胞表达p63、Calponin、CK5/6。1例诊断为腺肌上皮瘤(adenomyoepithelioma,AME),3例诊断为伴有癌的腺肌上皮瘤(恶性腺肌上皮瘤, malignant adenomyoepithelioma,MAME)。结论：乳腺上皮–肌上皮性肿瘤是少见的肿瘤类型,需与导管内乳头状瘤、化生性癌等鉴别。     

Sarcomatoid/metaplastic carcinoma of the breast: a clinicopathological study of 12 cases

AIMS: To analyse the clinical and pathological features with long-term follow-up of a series of 12 cases of sarcomatoid carcinoma of the breast. methods and results: The cases were selected from the surgical files of the Department of Pathology, University of Edinburgh, between 1977 and 1988. The following clinical parameters were recorded: the age of the patients, size of tumour, presence or absence of lymph node or distant metastases, and patient survival. Pathological assessment included: the type of epithelial and mesenchymal components, the proportion of monophasic to biphasic tumours and the presence of adjacent in-situ carcinoma/atypical epithelial proliferation. The mean age of the patients was 61 years with a median of 64 and range 46-82 years. The mean size of the tumour was 52 mm (range 22-100 mm). None of the patients had distant metastasis at presentation and only one case had local lymph node metastasis which had a carcinomatous appearance. Five women were still alive after a minimum 12-year follow-up period. Four patients died of their disease (three with lung metastasis only and one with lung and bone metastases), one died of carcinoma of the cervix and two patients were lost to follow-up. Pathologically, four cases (33.3%) had no or almost undetectable epithelial structures by light microscopy, i.e. "monophasic sarcomatoid carcinoma". The remaining cases revealed varying proportions of both epithelial and mesenchymal elements, i.e. "biphasic sarcomatoid carcinoma". Of the epithelial component, six (50%) tumours had predominantly carcinoma of no special type, one lobular and one tubular carcinoma. The mesenchymal component was fibromatosis/nodular fasciitis-like, malignant fibrous histiocytoma-like (MFH), osteosarcoma-like and fibrosarcoma-like in five (42%), four (33%), two (17%) and one (8%) tumours, respectively. In 3/4 monophasic tumours, the mesenchymal component was of a low-grade fibromatosis/nodular fasciitis type. In 6/12 (50%) of the cases there was associated in-situ atypical epithelial proliferation (five ductal carcinoma in situ (DCIS) and one atypical ductal hyperplasia). CONCLUSIONS: From this small series it appears that sarcomatoid carcinoma is an uncommon tumour, which is large in size and tends to lack local or distant metastasis at presentation. Pathologists should be alert to the presence of the bland monophasic sarcomatoid carcinoma which has a pure mesenchymal appearance on light microscopy, but epithelial components demonstrated by cytokeratin immunohistochemistry. These showed metastases on long-term follow-up, similar to other histological patterns of sarcomatoid carcinoma.     

Anaplastic and sarcomatoid carcinoma of the small intestine: a clinicopathologic study

Carcinomas involving the jejunum and ileum are rare tumors. During a review of small intestinal neoplasms, six primary carcinomas of jejunum or ileum with an anaplastic and sarcomatoid histology were identified. At presentation, three of the patients had symptoms related to metastatic disease and three had symptoms referable to the local tumor. The tumors were large (greater than 4.5 cm in diameter), usually endophytic masses composed of large cells with eosinophilic cytoplasm, anaplastic nuclei, and prominent nucleoli. In many areas, the cells had a spindled configuration. Mucin positivity was identified in all six cases. Electron microscopic findings in two cases were indicative of epithelial differentiation. The tumors behaved aggressively; all five patients for whom there was clinical follow-up died of metastases within 40 months. The six anaplastic and sarcomatoid carcinomas were compared with 29 typical adenocarcinomas arising in the jejunum or ileum. Only two of the latter group had symptoms referable to distant metastases at presentation. These tumors also tended to be smaller at presentation (11 tumors were less than 4 cm in greatest dimension). Of 25 patients with typical adenocarcinomas who had acceptable follow-up, 18 (72%) died of disease and five (20%) were alive with no evidence of disease after 5 years. We conclude that anaplastic and sarcomatoid carcinoma is a rare variant of small intestinal carcinoma with an aggressive clinical course.     

Basaloid carcinoma of the breast: a review of 9 cases, with delineation of a possible clinicopathologic entity

Basaloid carcinoma of the breast (BCB) is an unusual neoplasm composed of basal-type neoplastic cells similar to those found in adenoid cystic carcinoma (ACC), although lacking distinctive features such as a cribriform pattern, a dual neoplastic population (epithelial-myoepithelial/basaloid), and stromal deposits of basement membrane-like material. In this article, we present 9 cases of breast cancer showing overall/predominant basaloid morphology. Patients' ages ranged from 47 to 75 years (mean, 61.4 years). Surgical treatment included mastectomy or quadrant excision with or without axillary dissection. Most tumors had a circumscribed outline and ranged in size from 1.3 to 5.5 cm (mean, 2.5 cm). Microscopically, they featured sheets, nests, and cords of proliferating basaloid tumor cells with ovoid, hyperchromatic nuclei with inconspicuous nucleoli and scant cytoplasm. No foci with characteristics of ACC were found in any of the tumors. Transition into pleomorphic basaloid carcinoma with foci of high-grade ductal carcinoma in situ plus infiltrating ductal carcinoma (IDC) and admixture with grade 3 ductal and sarcomatoid carcinoma was seen in 2 cases. Tumor cells were positive for wide-spectrum keratins and epithelial membrane antigen (9/9) and high-molecular-weight keratins (7/9). They were negative for smooth muscle actin, p63, calponin, and CD10 in all tested cases. Estrogen receptor, progesterone receptor, and HER-2 were negative. Axillary lymph node metastases were seen in 3 cases. At follow-up (range, 10-169 months), 5 patients were alive, 1 with evidence of contralateral breast cancer. Three patients died: one of disseminated BCB metastases, another of liver cirrhosis, and one of disseminated estrogen receptor/progesterone receptor-positive contralateral IDC. One patient was lost to follow-up. We concluded that BCB has some phenotypic and immunohistochemical features enabling its distinction from ACC or IDC. It appears to be a morphological and possibly a clinical entity. Compared with ACC, BCB appears to be more aggressive and may entail a more guarded prognosis.     

Pure sarcomatoid carcinoma of maxillary sinus and nasal cavity simulating malignant fibrous histiocytoma

Although a few cases of sinonasal carcinoma with focal sarcomatous differentiation have been reported, pure sarcomatoid carcinoma has not been reported in the English literature. Imaging studies and gross inspection in a 60-year-old man with left-sided face pain revealed a mass in the left maxillary sinus and nasal cavity. A large incisional biopsy specimen from the nasal cavity revealed proliferation of malignant spindle and round cells with a malignant fibrous histiocytoma (MFH) pattern. Tumor giant cells were scattered, and there were areas of a vague storiform pattern. Mitotic figures were numerous. Carcinomatous component was not recognized. The histologic diagnosis was storiform-pleomorphic MFH. Tumor cells were positive for pancytokeratins AE1/3, KL-1, and CAM5.2 and cytokeratin (CK) 18, vimentin, CD68, p53, Ki-67 (labeling, 90%), α?-antitrypsin, and α?-antichymotrypsin and negative for pancytokeratin WSS, CK 34βE14, CK7, CK8, CK14, CK19, CK20, epithelial membrane antigen, S-100 protein, desmin, α-smooth muscle actin, CD34, HMB45, chromogranin, synaptophysin, myoglobin, CD45, CD30, and CD15. Because keratins were positive in tumor cells, a diagnosis of sarcomatoid carcinoma simulating MFH was made. The patient was treated with chemoradiation without significant effect and died 9 months after initial examination.