肾脏黏液性管状和梭形细胞癌的临床病理特征

目的 探讨肾脏黏液性管状和梭形细胞癌(MTSCC)的临床病理特征、组织发生和预后.方法 收集5例肾MTSCC进行组织病理学、组织化学、免疫组织化学(EnVision)及电镜观察,随访3～52个月,并复习相关文献.结果 5例患者女性4例,男性1例.3例因腰酸、腰部不适而就诊,2例系体检偶然发现.大体观察:肿瘤与周围组织分界清楚,切面实性,灰白灰黄或灰红色,1例高级别MTSCC切面可见明显坏死.镜下观察:低级别MTSCC主要由小管状结构、梭形细胞及黏液样基质构成,细胞形态较温和,核分裂象稀少.1例高级别MTSCC细胞呈梭形或多边形,未见小管状及黏液样基质,细胞明显异形,核分裂象易见.免疫组织化学染色显示,波形蛋白(5/5)、CKpan(5/5)、CK7(5/5)、CK19(5/5)、34βE12(1/5)、上皮细胞膜抗原(EMA,5/5)、E-cadherin(3/5)、CD10(1/5)、P504S(5/5)、CAM5.2(5/5)有阳性表达;Ki-67阳性指数4例小于或等于5%,1例高级别者达15%.超微结构显示管腔内可见短的微绒毛,局部核膜有内陷.4例随访3～52个月,未见肿瘤复发和转移.结论 肾MTSCC好发于女性,是一种罕见的肾肿瘤,临床多为低级别MTSCC,预后良好;对高级别MTSCC患者因其预后较差,手术后须密切随访观察.

Abstract：

Objective To investigate the clinicopathological features, histogenesis and prognosis of mucinous tubular and spindle cell carcinoma (MTSCC). Methods Five MTSCCs were studied with histochemical, immunohistochemical staining, electron microscopy, and review of the related literatures. Results Four cases of MTSCC were females and one was male. Three patients presented with flank discomfort and two were incidentally found with health examination. In gross examination, the tumors were circumscribed. The cut surface was solid, gray-white, yellow or red. Necrosis was present in one case of high-grade MTSCC. Microscopically, low-grade MTSCC was mainly consisted of tubular, spindle cell and mucinous stroma with relatively bland morphology, and mitoses were rare. While in the high-grade area of one case, the cells were spindle or polymorphic with severe atypia and high mitotic activity, without mucinous stroma and tubular structure. Mucin was positive for Alcian blue. The neoplastic cells were positive for vimentin (5/5), CKpan (5/5), CK7 (5/5), CK19 (5/5), 34βE12 (1/5), EMA (5/5), E-cadherin (3/5),CD10 (1/5), P504S (5/5), and CAM5.2 (5/5). The Ki-67 index was low (≤5%) in the low-grade component, while it was high (15%) in the high-grade component. Ultrastructural study showed short microvilli along glandular lumens. The nuclear membrane was focally invaginated. Four cases were followed up for 3 to 52 months, and recurrence and metastasis were not found.Conclusions MTSCC occurs predominantly in females and it is a rare kidney neoplasm. Most of MTSCCs are low-grade and the prognosis is relatively good. However, the patients of high-grade MTSCC should be closely followed up.     

肾黏液样小管状和梭形细胞癌的临床病理学分析

目的：探讨肾黏液样小管状和梭形细胞癌（ mucinous tubu1ar and spind1e ce11 carcinoma,MTSCC）的临床病理学特征、免疫表型及鉴别诊断。方法回顾性分析2例MTSCC的临床病理学特征,并复习相关文献。结果 MTSCC临床表现无特异性,CT、MRI等影像学检查可辅助诊断,确诊需依赖病理检查。免疫表型：瘤细胞表达 CK7、CK18、CK19、P504S、EMA、vimentin、CK34βE12、E-cadherin,不表达 CD15、CD10、CD56、Syn、CgA、CD117、C-erbB-2及 vi11in。结论MTSCC是一种罕见的低级别恶性肿瘤,需与集合管癌、乳头状肾细胞癌、肾髓质癌等鉴别,CT、MRI及免疫组化检查有助于诊断。     

肾黏液样小管状和梭形细胞癌临床病理分析

目的：探讨肾黏液样小管状和梭形细胞癌(mucinous tubular and spindle cell carcinoma,MTSCC)的临床病理学特征、诊断和鉴别诊断,提高对MTSCC的认识和诊断水平。方法：对2例MTSCC标本进行临床病理分析,并复习相关文献。结果：肿瘤与周围肾组织分界清楚,肿瘤由紧密排列的、小而狭长的小管构成,小管间为淡染的黏液样间质。肿瘤细胞呈立方形和梭形,肿瘤细胞胞质嗜酸性,细胞核圆形或卵圆形,异型性小,核仁不明显,核分裂像少见,在黏液性间质中可见散在淋巴细胞、浆细胞。免疫组织化学显示2例均表达细胞角蛋白(cytokeratin,CK)7,CK18, CK8/18和波形蛋白,Ki-67增殖指数<5%。结论：MTSCC是一种较罕见的低度恶性肿瘤,具有独特的组织学和免疫组织化学特征,明确该肿瘤的形态学特征、诊断和鉴别诊断有重要意义。     

骨软骨瘤继发肉瘤变临床病理分析

Objective To investigate the clinicopathologic,radiological and immunohistochemical characteristics of osteochondroma with malignant transformation.Methods The clinical data,radiological imagings and hematoxylineosin stained histologic sections were reviewed in 463 cases of osteochondroma diagnosed in Shanghai 6th Hospital from 1991 to 2008,including 11 cases with malignant transformation.Immunohistochemical two-step staining was used to detect CK,vimentin,S-100 protein,p53 and c-myc expression in seven cases of osteochondroma with malignant transformation and 10 cases without malignant transformation.The relevant literature was reviewed.Results Among the 11 cases with malignant transformation,five were single osteochondroma(5/408,1.2%),and six were multiple osteochondromas (6/55,10.9%).The male to female ratio was 10:1.These 11 cases were derived from femur(4 cases),tibia(3 cases),ilium(3 cases),shoulder bone(1 case)and pubis(1 case).There was one case that showed malignant transformation in both the femur and ilium.The mean ages for the malignant and nonmalignant cases were 39.8 years and 20.4 years respectively.All the malignant cases showed large sized lesions with prominent calcification in the thick cartilage caps.The malignant component was low grade,peripheral chondrosarcoma(grade Ⅰ-Ⅱ).In some areas the tumor cells infiltrated the peripheral soft tissue and bone marrow.Of the seven cases with malignant transformation that had immunohistochemical staining,all were positive for vimentin and S-100 protein;p53 protein was positive in 2 of 7 cases.Conclusions Malignant transformation of osteochondroma was usually encountered in multiple lesions.Most patients were more than 30 years old with a long clinical history and with a male predominance.These tumors showed thick cartilage caps with prominent calcification.The lobulated nature of the tumors was evident and they infiltrated the surrounding soft tissue.The sarcomatoid component was peripheral type,well differentiated chondrosarcoma.p53 mutation may explain part of the molecular mechanism in the malignant transformation.     

肾黏液样小管状和梭形细胞癌与乳头状肾细胞癌的免疫表型及遗传学改变

目的 研究肾脏黏液样小管状和梭形细胞癌(mucinous tubular and spindle cell carcinoma, MTSCC)及乳头状肾细胞癌(papillary renal cell carcinoma, PRCC)的免疫表型及遗传学特征,探讨它们在肿瘤鉴别诊断中的意义.方法 应用免疫组化方法分别检测7例MTSCC及10例PRCC中 vimentin、RCC、CK7、CK19、34βE12、AMACR、CD10及CD117的表达,同时应用荧光原位杂交(fluorescence in situ hybridization, FISH)技术检测两种肿瘤中7号染色体的遗传学改变.结果 (1) vimentin、RCC、CK7、CK19、AMACR、CD117及34βE12在两种肿瘤中的表达均无明显差别;CD10在50% (5/10例)的PRCC中呈阳性表达,而在MTSCC 中均呈阴性反应.(2) FISH结果显示:10例 PRCC均存在7号染色体三倍体,而在MTSCC中未检测到这一遗传学改变.结论 MTSCC是一种罕见的低级别多形性肿瘤,其某些组织学特征及免疫表型与PRCC相似,极易误诊.7号染色体的异常改变对MTSCC和PRCC的鉴别诊断有着十分重要的意义.     

肾黏液样小管状和梭形细胞癌伴Xp11.2易位/TFE3基因融合相关性肾癌1例并文献复习

目的探讨肾黏液样小管状和梭形细胞癌(mucinous tubular and spindle cell carcinoma,MTSCC)伴Xp11.2易位/TFE3基因融合相关性肾癌(renal carcinomas associated with Xp11.2 translocations/TFE3 gene fusions,Xp11.2 RCC)的临床病理学特征、诊断及鉴别诊断。方法回顾性分析1例MTSCC伴Xp11.2 RCC的影像学、组织学及免疫表型特点,并复习相关文献。结果患者女性,60岁,增强CT检查示左肾中极见一直径约2.3 cm的圆形低密度区,界淸。镜下见肿瘤呈浸润性生长,由两种不同的形态组成:梭形细胞区和乳头状区。梭形细胞区肿瘤细胞梭形,胞质红染,可见紧密排列的小管结构;乳头状区肿瘤细胞乳头状排列,可见大量透明细胞及砂粒体。免疫表型:两种区域肿瘤细胞均表达vimentin、CK7、CK19、RCC、CD10,乳头状区肿瘤细胞表达TFE3。结论 MTSCC及Xp11.2 RCC均是临床少见的肿瘤,两种肿瘤组成的混合型肾细胞癌罕见。诊断依据组织学形态及免疫表型。     

Clinicopathologic features and prognostic factors of malignant phyllodes tumors北大核心CSCD

Objective: To study the clinicopathologic features of malignant phyllodes tumors (FT) by histopathologic analyses, immunohistochemical profiling and DNA content assay, and evaluation of the clinical outcome. Methods: Ten patients with malignant FT from 1999 to 2013 who were treated by surgery were enrolled in this study. The morphologic characteristics were studied under light microscope, standard two-step En Vision method of immunohistochemical staining was used to assess the expression of CK5/6, CKpan, 34βE12, desmin, p63, ER-α', PR, Ki-67, CD34, SMA, p53, p16, bcl-2 and CD117 in the tumors. The corresponding paraffin blocks were also used for flow cytometric DNA content assay. These data were correlated with the follow-up results. Results: The median age of onset was 46. 5 years old. The mean tumor size was 7. 4 cm (2. 0-25. 0 cm). At the end of the follow-up period (22 to 125 months), there were tumor recurrences in 3/8 patients and the median time of recurrence was 24 months. Metastasis occurred in 3/8 patients who all died of the tumors. FT had heterogeneous histology, with stromal overgrowth with leaflike projections, periductal stromal overgrowth, and most commonly, diffuse stromal overgrowth with sarcomatous differentiation. The mean positive index of Ki-67 was 11.4%. The stromal tumor cells were positive for CD34, SMA, p53, pl6, and bcl-2 in 3/10, 9/10, 6/10, 8/10, and 4/10 cases, respectively. CD117, ER-α and PR were negative. Interpretable DNA histograms were obtained in nine cases with triploidy in two cases. Conclusions: The diagnosis of malignant PT should be considered based on the diversity of growth patterns and heterogeneous histology. Ki-67 and CD34 are valuable diagnostic and prognostic factors in patients with malignant PT. Tumors with diffuse stromal overgrowth, heterologous elements, Ki-67≥20% or aneuploidy are more likely to metastasize.     

Survivin和Smac在卵巢黏液性肿瘤中的表达及意义

Objective To investigate the expressions and significances of Survivin and Smac in ovarian mucinous tumors. Methods A total of 55 paraffin-embedded specimens of primary ovarian mucinous tumors were collected. SABC was used to detect protein expression of Survivin and Smac genes.Immunoelectron microscopy using colloidal gold labeling was performed to determine the subcellular localization and patterns of Smac protein expression. Results (1) The cytoplasmic expression rates of survivin in benign, borderline and malignant ovarian mucinous tumors were 2/20, 12/15 and 20/20 respectively, which presents an improving trend. There were significant differences of survivin expression between benign vs. Borderline lesions (P < 0.01) , and benign vs. Malignant tumors (P < 0. 01) . The nuclear expression rates of survivin in benign, borderline and malignant ovarian mucinous tumors were 1/20,6/15 and 5/20, respectively, which presents a. Declining trend. There was significant difference of survivin expression between benign vs. Borderline tumors (P < 0. 05). The positive expression rates of Smac among the three groups were 19/20, 9/15 and 3/20, respectively. There was significant difference among the three groups (P<0. 01 or <0.05). There was a negative correlation between Survivin and Smac (r= -0. 153,P <0. 01). (2) Colloidal gold labeling study demonstrated that mitochondrion intramembranous storage of Smac granules in the three groups were 24. 1 ±7.2, 11. 1 ±1.9 and 5. 2±1. 7, respectively, and there were significant differences among the three groups (P < 0. 01 or < 0.05). The extramemebranous Smac granules were 4. 7±3. 0, 2. 9±1. 0 and 1. 7 ±1. 3, although without significant difference among the three groups (P >0. 05). Conclusions With the malignant development of ovarian mucinous tumors,the expressions of Survivin are up-regulated,and the expressions of Smac are down-regulated. Smac proteins exist mainly in an inactive intramembranous storage form inside of mitochondria.     

Survivin和Smac在卵巢黏液性肿瘤中的表达及意义

Objective To investigate the expressions and significances of Survivin and Smac in ovarian mucinous tumors. Methods A total of 55 paraffin-embedded specimens of primary ovarian mucinous tumors were collected. SABC was used to detect protein expression of Survivin and Smac genes.Immunoelectron microscopy using colloidal gold labeling was performed to determine the subcellular localization and patterns of Smac protein expression. Results (1) The cytoplasmic expression rates of survivin in benign, borderline and malignant ovarian mucinous tumors were 2/20, 12/15 and 20/20 respectively, which presents an improving trend. There were significant differences of survivin expression between benign vs. Borderline lesions (P < 0.01) , and benign vs. Malignant tumors (P < 0. 01) . The nuclear expression rates of survivin in benign, borderline and malignant ovarian mucinous tumors were 1/20,6/15 and 5/20, respectively, which presents a. Declining trend. There was significant difference of survivin expression between benign vs. Borderline tumors (P < 0. 05). The positive expression rates of Smac among the three groups were 19/20, 9/15 and 3/20, respectively. There was significant difference among the three groups (P<0. 01 or <0.05). There was a negative correlation between Survivin and Smac (r= -0. 153,P <0. 01). (2) Colloidal gold labeling study demonstrated that mitochondrion intramembranous storage of Smac granules in the three groups were 24. 1 ±7.2, 11. 1 ±1.9 and 5. 2±1. 7, respectively, and there were significant differences among the three groups (P < 0. 01 or < 0.05). The extramemebranous Smac granules were 4. 7±3. 0, 2. 9±1. 0 and 1. 7 ±1. 3, although without significant difference among the three groups (P >0. 05). Conclusions With the malignant development of ovarian mucinous tumors,the expressions of Survivin are up-regulated,and the expressions of Smac are down-regulated. Smac proteins exist mainly in an inactive intramembranous storage form inside of mitochondria.     

子宫上皮样滋养细胞肿瘤临床病理分析

Objective To study the clinicopathologic features,immunophenotype,differential diagnosis and prognosis of uterine epithelioid trophoblastic tumor(ETT).Methods From 2000 to 2007,5 ETTs cases were diagnosed in the affiliated Women's Hospital,School of Medicine,Zhejiang University.The pathologic characteristics and immunophenotype of the tumors were analyzed by histological examination and immunohistochemistry of CK18,p63,inhibin-α,HCG,HPL,PLAP and Ki-67.The clinical prognostic factors were evaluated based on a following-up data with a period of 11-SO months.Results The overall prevalence of ETT was 0.48% among all the gestational trophoblastic diseases patients received in the same period.Five ETT patients were in the reproductive ages with a median of 33 years.Histologically,the tumor showed an invasive,nodular growth consisting of uniform mononuclear trophoblastic cells.There were zones of hyaline material in the tumour nests.Necrosis was commonly seen with a characteristic geographic pattern.Immunohistochemically,all cases displayed a diffuse CK18 and p63 positivity,to be either positive focally or negative for HCG,HPL and PLAP staining.Inhibin-a staining was positive or negative either in the 5 cases.Two patients died of the tumour relapse:one died after 1 year with the tumor having a high mitotic activity(averagely 15 mitotic figures per 10 high-power fields),and the other died of lung metastasis 2 years after the diagnosis.Conclusions ETT is a rare trophoblastic disease with distinct clinicopathological features and immunostaining patterns.A high mitotic index and lung metastasis are indicators for an unfavorable prognosis.     

Mucinous tubular and spindle cell carcinoma of the kidney with prominent papillary component,a non-classic morphologic variant. A histologic,immunohistochemical, electron microscopic and fluorescence in situ hybridization study

Mucinous tubular and spindle cell carcinoma (MTSCC) is a rare type of kidney tumor with relatively indolent behavior. Non-classic morphological variants have not been well studied and rarely been reported. We report a challenging case MTSCC with a peculiar morphology in a 42-year-old man, arising in a background of end-stage renal disease (ESRD). Predominant areas with extensive papillary architecture, psammoma bodies and stromal macrophageal aggregates, reminiscent of a papillary renal cell carcinoma (papillary RCC), were intermixed with foci that transitioned into a MTSCC-like morphology exhibiting elongated tubules and a low grade spindle cell component in a background of mucinous stroma. Immunohistochemistry demonstrated diffuse positivity for P504s/AMACR and vimentin in tumor cells. Focal positivity for RCC, CD10 and CK7 was also noted. Kidney-specific cadherin, cytokeratin 34betaE12 and TFE3 stains were negative in the tumor. The major differential diagnostic considerations were papillary RCC, clear cell papillary RCC, and Xp11.2 translocation carcinoma. Negative FISH studies for trisomy 7 and 17 in both papillary and spindled components supported the diagnosis of MTSCC. The ultrastructural profile was not entirely indicative of the cellular origin of the tumor. Cytogenetic analysis should be performed in atypical cases of MTSCC for precise diagnosis.     

Fine needle aspiration biopsy of renal mucinous tubular and spindle cell carcinoma: Report of two cases

Mucinous tubular and spindle cell carcinoma (MTSCC) is a rare renal tumor. Here we report two cases of MTSCC which were initially evaluated by fine needle aspiration biopsy (FNAB) and followed by surgical resection of the tumors. The cytomorphologic features of MTSCC were characterized by aggregates of relatively uniform, predominantly oval to spindle cells intermixed with abundant metachromatic myxoid matrix. Only rare epithelioid tumor cells with vacuolated cytoplasm were present. Immunohistochemically, the tumor cells were positive for CK7, CK19, CD10, vimentin, E‐cadherin, alpha‐methyl CoA racemase, and negative for CK903 and CK20. EMA and carbonic anhydrase IX immunoreactivity was seen in one of the two cases. Multiple chromosomal losses involving chromosomes 1, 2, 17 and likely chromosome 7 were revealed by fluorescence in situ hybridization (FISH). These cytomorphologic, immunophenotypic, and cytogenetic features were helpful for including this entity in the differential diagnosis of renal cell carcinomas. Diagn. Cytopathol. 2010. © 2009 Wiley‐Liss, Inc.     

Mucinous tubular and spindle cell carcinoma of kidney without sarcomatoid change showing metastases to liver and retroperitoneal lymph node

Mucinous tubular and spindle cell carcinoma (MTSCC) is an uncommon, newly recognized tumor that in its classic histological form shows tightly packed, elongated tubules with transition into spindle cell areas and pale mucinous stroma. The current data suggest that the great majority of MTSCCs have a favorable prognosis; however, the follow-up data are limited and the full biologic potential of this tumor remains to be established. There are a few examples of MTSCCs metastatic to lymph nodes and rare cases with sarcomatoid differentiation associated with distant metastases. We report on a case of MTSCC of kidney with concurrent nodal and liver metastases. The metastatic nodules were well circumscribed and showed morphological and immunophenotypic features similar to those of the primary tumor. Extensive sampling revealed no evidence of sarcomatoid morphology. To our knowledge, this is the first case of MTSCC without sarcomatoid differentiation showing parenchymal metastasis.     

Mucinous tubular and spindle cell carcinoma of the kidney with neuroendocrine differentiation: report of two cases

We encountered 2 cases of mucinous tubular and spindle cell carcinoma (MTSCC) during a short time. In a 61-year-old man who had macroscopic hematuria for 1 month, the 14.5 x 14.0 x 12.0-cm resected tumor involved the right middle aspect of the renal parenchyma and compressed the renal pelvis. In an asymptomatic 47-year-old man, a renal tumor was found during an annual physical examination. The 3.5 x 3.0 x 2.0-cm tumor was located at the upper pole of the right kidney. The histologic findings in both cases were similar Tumors consisted of tightly packed, small, elongated tubules separated by pale mucinous stroma. The tumor cells were cuboidal to spindled with eosinophilic cytoplasm and low nuclear grade. Mitoses were few or nonexistent and without abnormal figures. Both tumors were immunoreactive for cytokeratin (CK) cocktail (AE1/AE3), high-molecular-weight CK (34betaE12), low-molecular-weight CK (35betaH11), CK7, epithelial membrane antigen, E-cadherin, and vimentin. The tumor cells also were reactive for neuron-specific enolase, chromogranin, and synaptophysin. The ultrastructure of the tumor cells contained abundant mitochondria, junctional complex, and dense-core neurosecretory granules. We present 2 additional cases of MTSCC showing typical morphologic features with neuroendocrine differentiation.     

Mucinous tubular and spindle-cell carcinoma of the kidney

4 cases (2 males and 2 females) of mucinous tubular and spindle-cell carcinoma of the kidney were studied. The patients' age was 43 to 87 years. Microscopically, this tumor comprised two major cell populations. One of them involved spindle cells with a sparse cytoplasm, which were arranged in the fascicular pattern, often resembling low-grade smooth muscle tumors. The other population was small cuboidal cells with round nuclei and an eosinophilic cytoplasm. The mitotic activity was low. Only one tumor had both cell components. The other three tumors were represented by the second type cells. Immunohistochemical staining with avidin-biotin was performed by using cytokeratin, vimentin, SMA, p53, Bcl 2, EMA, and CD10. All cases were stained positively with antibodies to the cytokeratins AE1-AE2 and vimentin. The expression of EMA, Bcl 2, and CK7 was varying in different tumors. Responses to smooth muscle actin, p53, CK20, and CD10 were negative in all cases. Mucinous tubular and spindle-cell carcinoma of the kidney should be differentiated from angiomyolipoma with the preponderance of a leiomyomatous component, renal smooth muscle neoplasms, metanephrotic adenoma, and renal cell carcinoma with sarcomatoid differentiation.     

Metastatic renal mucinous tubular and spindle cell carcinoma. Atypical behavior of a rare, morphologically bland tumor

Mucinous tubular and spindle cell carcinoma (MTSCC) is a rare, distinctive renal neoplasm characterized by an admixture of cuboidal cells in tubules and sheets of spindle cells, typically with low-grade nuclei and a myxoid or mucinous background. It is characteristically of low malignant potential, and only rare metastatic cases have been reported. We describe a case in which the patient presented with extensive regional and distant metastases, but both primary and metastatic tumor showed the typical histomorphology of bland cuboidal or spindle cells lacking pleomorphism, mitoses, and necrosis. Almost all previous cases of metastatic MTSCCs have shown nuclear atypia or sarcomatoid morphology of the primary tumor; and metastatic renal MTSCC in which the primary neoplasm does not display atypical features is exceptional, serving to highlight that these rare tumors can behave aggressively even with "indolent" histological appearances.     

肾脏黏液性管状和梭形细胞癌临床病理学观察

目的 探讨肾脏黏液性管状和梭形细胞癌的临床病理学特征、免疫表型及鉴别诊断。方法 应用常规病理、免疫组化方法观察1例肾脏黏液性管状和梭形细胞癌并复习相关文献。结果 肿物与周围肾组织分界清楚，组织学特点是排列呈管状的上皮样细胞，片状的梭形细胞和黏液性间质，无明显核的异型性，缺乏坏死。免疫组化：CK、EMA、vimentin阳性，SMA、desmin、S-100蛋白、HMB45等阴性。结论 肾脏黏液性管状和梭形细胞癌是WHO新确定的一类罕见的低度恶性肾上皮性肿瘤，预后较好，要与后肾腺瘤、肉瘤样癌和集合管癌等疾病相鉴别。     

Mucin poor mucinous tubular and spindle cell carcinoma of the kidney, with nonclassic morphologic variant of spindle cell predominance and psammomatous calcification

Mucinous tubular and spindle cell carcinoma (MTSCC) is a rare type of kidney tumor that has only been recently described. The classic MTSCCs are polymorphic renal neoplasms characterized by small, elongated tubules lined by cuboidal cells and/or cords of spindled cells separated by pale mucinous stroma. Nonclassic morphologic variants and features of MTSC have not been well studied and rarely been reported. We report a challenging case of mucin-poor MTSCC with marked spindle cell predominance and focal psammomatous calcification in a 39-year-old man and describe its histologic and immunohistochemical features. Pathologists must be aware of the histologic spectrum of MTSCCs to ensure their accurate diagnosis.     

小肠肉瘤样癌临床病理分析

目的探讨小肠肉瘤样癌的临床病理学特点及免疫组织化学表型。方法采用光镜结合免疫组化SP法对6例小肠肉瘤样癌标本进行观察分析。结果6例小肠肉瘤样癌患者中男性5例,女性1例;年龄43～72岁,平均61．3岁。肿瘤平均直径为5．5cm。呈菜花状或息肉状。光镜下可见3种肿瘤细胞：上皮样细胞、梭形细胞及间变性细胞,多呈片状或束状排列,偶呈巢状排列。免疫组化显示：6例vimentin、CKpan、EMA及CK7均为强阳性;CK20有两例弱阳性;CEA有1例弱阳性;NSE及Syn在6例均为阳性表达,CgA3例阳性,阳性强度为弱阳性～中等阳性;Ki-67阳性指数为37％～73％,平均值为51．6％。随访4例患者在术后2～7个月内死亡。结论小肠肉瘤样癌罕见,多发于老年男性,免疫组化证实有上皮及神经内分泌分化,预后很差,免疫组化在鉴别诊断中很重要。