Myoepithelial carcinoma of pharynx expressing KIT and PDGFRA

KIT and PDGFRA expression has rarely been examined in myoepithelial carcinoma (MC) of the salivary glands. An 89-year-old Japanese woman presented with a pharyngeal mass. Gross and imaging examinations revealed an elevated mass in the middle pharynx next to the oral cavity. A biopsy revealed atypical cells, and tumorectomy was performed. The tumor was composed of atypical epithelioid cells arranged in solid nests, cords, and vague acinar patterns. Mitotic figures were recognized in 3 per 50 high power fields. Immunohistochemically, the tumor cells were positive for myoepithelial markers including cytokeratin (CK) 14, α-smooth muscle antigen, S100 protein, and p63. They were also positive for KIT, PDGFRA, pancytokeratin AE1/3, CK34βE12, CK5/6, vimentin, p53, and Ki-67 (labeling=28%). They were negative for neuron-specific enolase, CD45, CD34, CD56, chromogranin, synaptophysin, melanosome, desmin, epithelial membrane antigen, CK18, CK20, pancytokeratin (CAM5.2). A pathologic diagnosis of myoepithelial carcinoma arising from minor salivary glands was made. No metastatic lesions were found by various imaging techniques. The patient is now receiving palliative radiation therapy 2 months after the operation. The present case showed that MC can express KIT and PDGFRA.     

Small cell carcinoma of the endometrium: a clinicopathological and immunohistochemical study

Objective: To investigate both clinicopathological and immunohistochemical characteristics of small cell carcinoma (SCC) of the endometrium, and to explore the diagnosis, treatment and prognosis of the disease. Methods: A retrospective analysis was performed on clinicopathological data of a patient with SCC of the endometrium in our hospital, who underwent comprehensive surgery of radical hysterectomy, bilateral adnexectomy, pelvic lymphadenectomy, omentectomy, appendectomy, and bilateral ovarian vein ligation. Paraffin blocks of formaldehyde-fixed tumor tissue were cut and stained for histological and immunohistochemical studies. Results: microscopic examination showed clusters of small-sized cells with scant cytoplasm, hyperchromatic nuclei and high mitotic activity. The patient was classified as endometrial SCC stage Ib according to the clinical staging of the International Federation of Gynecology and Obstetrics (FIGO). Immunohistochemical tests revealed positive staining for synaptophysin (Syn) and CD56, and the proliferation rate measured with the proliferative marker (Ki67) was approximately 70 percent of tumour cells. The patient was given 4 sessions of postoperative chemotherapy. Tumor markers were within normal range during follow-up examinations, and the patient had survived tumor free for two years. Conclusion: Primary SCC of the endometrium is extremely rare, and is reported to have a strong invasiveness and a poor prognosis. Immunohistochemistry technique is an important tool for clinical diagnosis of the disease. Comprehensive treatments composed of surgery, radiotherapy and chemotherapy may improve the prognosis.     

Pseudoangiosarcomatous carcinoma: a clinicopathological study of seven cases

Seven cases of carcinoma mimicking angiosarcoma occurring in skin (3 cases), breast (3) and lung (1) are described. The cutaneous, pulmonary and one of the breast carcinomas were poorly differentiated and squamous in type; the other two breast tumours were poorly differentiated ductal carcinomas with focal squamous differentiation. Histologically, the pseudoangiosarcomatous pattern was due to complex anastomosing channels and spaces lined by neoplastic cells. The spaces contained hyaluronic acid. The neoplastic cells exhibited cytokeratin positivity but yielded negative results with the endothelial cell markers, factor VIII-related antigen and CD 34 (QB-END/10). Two breast tumours showed binding of UEA-1. Ultrastructurally, unequivocal epithelial differentiation was demonstrated in six of the cases. Pathogenetically, these tumours appeared to be variants of acantholytic squamous cell carcinoma. Recognition of this unusual form of carcinoma is important, as an incorrect diagnosis of angiosarcoma may lead to inappropriate treatment and prognostication.     

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。     

Small cell carcinoma of the bladder: a contemporary clinicopathological study of 51 cases

AIMS: We present 51 cases of primary small cell carcinoma of the bladder in a clinicopathological study with emphasis on features that aid in the initial recognition and diagnosis of small cell carcinoma of the bladder. METHODS AND RESULTS: The patients were 40 men and 11 women between the ages of 39 and 87 years (mean age 67 years). Clinical data were available in 41 cases. The most common symptomatology was haematuria in 63% of the patients while dysuria was present in 12%. Thirty-eight patients were caucasians; seven patients were Hispanics; two patients were Asian; one patient was African-American; in the three additional patients no racial information was obtained. Biopsy material was obtained in all of the patients. Cystectomy was performed in 20 patients. At diagnosis, clinical stage was as follows: stage I in two (5%), stage II in 18 (44%), stage III in 10 (24%), and stage IV in 11 (27%). Histologically, urothelial carcinoma was present in 70% of the cases, adenocarcinoma in 8%, and squamous cell carcinoma in 10% of the cases. Small cell carcinoma was the only histology present in only 12% of the cases studied. Immunohistochemical studies using chromogranin, synaptophysin and chromogranin were positive in 30-70% of the cases. CONCLUSIONS: The present study highlights the unusual phenomenon of pure small cell carcinoma of the bladder and its association with other non-small cell carcinomas in that anatomical location. In addition, the study highlights the different modalities employed to treat patients in whom there is a component of small cell carcinoma of the bladder.     

胰腺腺泡细胞癌14例临床病理分析

目的探讨胰腺腺泡细胞癌的临床病理学特点及其鉴别诊断。方法复习14例胰腺腺泡细胞癌手术切除标本的病理切片并做相应的免疫组化染色,5例做电镜观察。结果患者平均年龄55岁,男性9例,女性5例。肿瘤平均大小为6cm×5cm×4.5cm,与周围组织境界清楚,切面呈灰褐色。镜检:肿瘤细胞排列呈腺泡状、条索状、小梁状或实性排列。细胞核大小一致,核仁清楚。细胞质丰富,呈颗粒状。免疫组化染色14例均弥漫表达抗胰蛋白酶和淀粉酶,神经内分泌标记物NSE和CgA少数细胞呈散在阳性,而胰岛素和胰高血糖素均为阴性。14例中6例于术后8～18个月内复发,4例发生肝脏、肺等远处转移。14例患者术后均死于肿瘤,存活时间13～36个月,平均24个月。结论胰腺腺泡细胞癌属于高度恶性肿瘤,在细胞学形态和免疫表型方面均与胰腺导管细胞肿瘤以及胰腺内分泌肿瘤不同。     

Extrapulmonary small cell carcinoma of the liver: clinicopathological and immunohistochemical findings

Patients with primary small cell carcinoma of the liver have rarely been described in medical literature. Knowledge of clinical, pathological and immunohistochemical properties remains limited. We described an 82-year-old female patient with primary small cell carcinoma of the liver. Histologically, the tumor showed typical morphology of a pulmonary small cell carcinoma. Immunohistochemically, the tumor revealed neuroendocrine differentiation; positive reaction for chromogranin, synaptophysin, CD56, and neuron specific enolase. The tumor was also positive for TTF-1 and c-kit but completely negative for hepatocyte, carcinoembryonic antigen, cytokeratin 7; 19; and 20. Herein, we discussed the clinical, pathological and immunohistochemical findings of extrapulmonary small cell carcinoma of the liver and reviewed the relevant literature.     

Invasive micropapillary carcinoma of the breast: clinicopathological and immunohistochemical study

Invasive micropapillary carcinoma (IMPCa) of the breast refers to a unique variant of invasive ductal carcinoma, but its biological behavior has not been elucidated well. We analyzed 16 IMPCa cases (10 pure type, six mixed type). The incidence of IMPCa was 1.0% of all primary breast carcinoma. High nuclear grade (75.0%), as well as poorly differentiated histological grade (81.3%), was frequently seen. Lymph node metastases were evident in 92.9% of the examined cases, and about half of them showed more than 10 positive nodes. Comparison between serially experienced invasive ductal carcinoma, not otherwise specified (IDC-NOS), revealed that both high nuclear grade and poor histological grade were significantly more frequent ( P  < 0001), there was a lower frequency of positive estrogen receptor/progesterone receptor ( P  < 0.05, P  < 0.01), a higher frequency of HER-2 overexpression ( P  < 0.025), and more frequent lymph node metastases ( P  < 0.05) in IMPCa. The comparison between lymph node positive IDC-NOS did not show any statistically significant differences in frequency for positive p53, matrix metalloproteinase protein-2 (MMP-2), vascular endothelial growth factor (VEGF) or E-cadherin. However, IMPCa showed a significantly increased number of blood vessels counted by CD34 immunostains ( P  < 0.05). These results suggest that IMPCa is, at least, the same or more aggressive than lymph node positive cases of IDC-NOS. Hence, not only the high incidence of lymph node metastases but also distant, blood-borne metastases may be important.     

Thymic carcinoma: a clinicopathological and immunohistological study of 19 cases

Aims:  To study 19 cases of primary thymic carcinoma in order to define the clinicopathological features and the precise histochemical profile of this rare and heterogeneous group of tumours of the anterior mediastinum.
Methods and results:  The study group consisted of 13 males and six females, with a mean age of 58.5 years (range 29–75 years). Superior vena cava syndrome and chest pain were the main presenting symptoms. Three patients were asymptomatic. No patient had myasthenia gravis. Six different histological types were identified: neuroendocrine tumours (six patients), epidermoid carcinoma (five patients), sarcomatoid carcinoma (three patients), lymphoepithelioma-like carcinoma (two patients), mucoepidermoid carcinoma, clear cell carcinoma, and undifferentiated carcinoma (one patient each). The clear cell carcinoma was associated with a thymic cyst. No association with thymoma was observed. Surgical resection, performed in 10 cases, was complete in two. Sixteen patients received thoracic radiation, and 11 received systemic chemotherapy. Follow-up information was available in 16 cases; 12 patients presented with local or metastatic relapse, and 10 patients died of their tumour. The overall 5-year survival was 14.5%.
Conclusion:  Primary thymic carcinoma is a very heterogeneous group of tumours of the anterior mediastinum with an aggressive clinical behaviour, and a poor overall prognosis.     

子宫内膜移行细胞癌临床病理学观察

目的观察子宫内膜移行细胞癌（transitional cell carcinoma of the endometrium,ETCC）的临床病理特点,探讨其病理诊断、组织发生、生物学行为及预后。方法采用光镜、免疫组化对3例ETCC进行病理观察,并复习相关文献。结果3例ETCC患者均表现为绝经后不规则阴道流血。大体上,肿瘤呈息肉状生长伴浅肌层浸润。镜下,肿瘤表现为乳头状结构,乳头表面覆盖多层异型上皮细胞,中央为纤维血管中心柱,其周围肿瘤细胞呈单层栅栏状排列,乳头表面细胞呈扁平状。乳头内可见腺样结构,部分乳头呈实性团状排列,中央见大片坏死。肿瘤细胞大小相对较一致,核呈圆形、卵圆形,核膜明显,染色质呈空泡状,未见核沟,核中度异型,核分裂象15～20／10HPF。浸润肌层的为分化差的巢状移行细胞成分。除了主要TCC成分外,2例分别混合有透明细胞癌及内膜样型腺癌。免疫组化染色结果示肿瘤细胞CK7＋、CK20-。结论ETCC是一种少见的子宫内膜癌的组织学亚型,形态学上具有尿路移行上皮分化特征,但保持了苗勒管的免疫表型CK7＋／CK20-。诊断ETCC的关键在于认识移行细胞的形态学特征。     

消化道小细胞癌的临床病理分析

目的 研究消化道小细胞癌临床病理和免疫表型特征。方法 对17例消化道小细胞癌作了临床病理形态学观察和免疫组织化学检测。结果光镜形态分为3型：小细胞型8例，中间细胞型4例，混合型5例。免疫表型：EMA12例、NSE10例、keratin9例、CgA6例、S-100蛋白3例呈阳性，vimentin均阴性。结论 EMA、NSE、keratin为小细胞癌的较为可靠的标记物，支持此瘤来源于内胚层全能干细胞。     

支气管上皮-肌上皮癌临床病理观察

目的探讨支气管上皮-肌上皮癌(epithelial-myoepithelial carcinoma,EMC)的临床病理学特点、诊断及鉴别诊断。方法采用HE、免疫组化SP法对4例支气管EMC的标本进行组织学观察及免疫组化标记,并分析病理组织学形态和免疫表型。结果 EMC由内层腺上皮及外层肌上皮构成,呈双套管结构。腺上皮成分CK、EMA均呈阳性,肌上皮成分Calponin、p63、CK、CD117、S-100、GFAP均呈阳性。结论原发于支气管的EMC临床罕见,多数位于大支气管内,诊断依靠组织学形态及免疫表型,应与黏液表皮样癌、腺样囊性癌、多形性腺瘤转移性透明细胞癌鉴别。EMC治疗以手术切除为主,但也可复发或转移。     

转移性透明细胞性肾细胞癌42例临床病理分析

目的探讨转移性透明细胞性肾细胞癌(clear cell renal cell carcinoma,CCRCC)的临床病理学特征、诊断及鉴别诊断。方法回顾性分析42例转移性CCRCC的临床病理资料,并复习相关文献。结果 42例转移性CCRCC中,男性35例,女性7例;转移部位依次为肺19例,骨8例,淋巴结及软组织各3例,肾上腺、皮肤及脑各2例,肝、小肠及鼻腔各1例。42例转移性CCRCC中,31例于肾原发灶手术切除后3周~11年出现转移,4例原发灶与转移灶同时发现;28例转移灶为单发,14例为多发性。34例转移性CCRCC仍具有原发性CCRCC的组织学形态,4例出现多灶状坏死,6例呈肉瘤样分化。Fuhrman细胞核分级:Ⅰ级4例、Ⅱ级13例、Ⅲ级18例、Ⅳ级7例。17例间质小血管呈簇状增生。免疫组化标记42例转移性CCRCC中CD10、vimentin、CAⅨ、PAX8、RCC和EMA阳性例数分别为39例(92.9%)、40例(95.2%)、38例(90.5%)、38例(90.5%)、31例(73.8%)和38例(90.5%)。结论转移性CCRCC具有独特的临床病理学特征,结合临床病史及肿瘤组织形态学特点,联合免疫组化标记vimentin、EMA、CD10、CAⅨ、PAX8等有助于诊断及鉴别诊断。     

Multilocular cystic renal cell carcinoma: a clinicopathological, immuno- and lectin histochemical study of nine cases

Multilocular cystic renal cell carcinoma (MCRCC) is an uncommon variant of renal neoplasm and its histogenesis is unclear. The aim of this study was to use immuno- and lectin histochemistry to delineate histochemical patterns which might indicate the histogenetic origin of MCRCC from a particular part or parts of the nephron. We present our experience with nine cases of MCRCC. Fifteen cases of renal cell carcinoma with cystic degeneration (RCC-CD) were selected for comparison with MCRCC. We carried out clinicopathological and immunohistochemical examinations of the MCRCC cases. Clinically, the prognosis of the patients was quite good, in that all nine patients are alive and without recurrence at the time of this report. The MCRCCs reacted strongly in a higher proportion of cases with the distal nephron markers, such as peanut agglutinin (PNA, 88.9%) and MUC1-core antibody (MUC1, 100%), but none reacted preferentially with proximal nephron markers such as vimentin, Leu M1 and Lotus tetragonolobus (LTA). The RCC-CD tumours reacted with vimentin (40%), Leu M1 (66.7%) and LTA (86.7%). Except for two cases, the RCC-CD tumours did not react with PNA or MUC1 core antibody. These results illustrate the different patterns of expression of MCRCC and RCC-CD and suggest that MCRCC originates from the distal nephron. Therefore, MCRCC should be differentiated from other types of renal cell carcinoma on the basis of the histogenesis of the tumour and the clinicopathological findings.     

Clear cell papillary renal cell carcinoma: a clinicopathological study emphasizing ultrastructural features and cytogenetic heterogeneity

Clear cell papillary renal cell carcinoma (CCPRCC) is a recently recognized renal neoplasm, which was initially described in end-stage renal disease (ESRD), but some cases have been reported in otherwise normal kidneys. We report a series of 11 CCPRCC (age range, 33-72 years; male-to-female ratio, 8:3). Follow-up was available for 8 patients. No patients developed local recurrence, distant or lymph-node metastasis, or cancer death. Histologically, all tumors exhibit morphologic features typical of CCPRCC including a mixture of cystic and papillary components, covered by small to medium-sized cuboidal cells with abundant clear cytoplasm. All 11 cases exhibited moderate to strong positivity for CK7, CA9, Vim, and HIF-1α, coupled with negative reactions for CD10, P504S, and RCC. We did not find any VHL gene mutations in all 11 cases. Losses of chromosomes 3 (monoploid chromosome 3) was detected in 3 cases. Ultrastructurally, the tumor cells composed of numerous glycogens with scanty cell organelles, reminiscent of clear cell renal cell carcinoma (CCRCC). In conclusion, the coexpression of CA9 and HIF-1α in the absence of VHL gene abnormalities in CCPRCC suggests activation of the HIF pathway by mechanisms independent of VHL gene mutation. Losses of chromosomes 3 (monosomies chromosome 3) was detected in 3 cases suggesting that at least some of these lesions have demonstrated abnormalities of chromosomes 3. Ultrastructurally, CCPRCC composed of numerous glycogens with scanty cell organelles, reminiscent of CCRCC suggesting the close pathogenesis relationship of CCPRCC with CCRCC.     

子宫浆液性乳头状癌24例临床病理分析

目的比较子宫浆液性乳头状癌(uterine papillary serous carcinoma,UPSC)和子宫内膜样癌(uterine endometrioid carcino-ma,UEC)的组织病理学和免疫组化表达,以了解两种肿瘤生物学行为的差异。方法对24例UPSC和54例UEC进行组织学复查和应用免疫组化SP法检测肿瘤的p53蛋白、ER和PR的表达。结果24例UPSC占子宫内膜癌的3·77%,平均年龄UP-SC组为60岁,UEC组为51·7岁(P<0·01),晚期癌(FIGOⅢ-Ⅳ)UPSC组占62·5%,UEC组占35·1%(P<0·025)。p53蛋白的表达UPSC组16例阳性(66·7%),UEC组10例阳性(18·5%),两组比较(P<0·01)。ER阳性表达UPSC组(8·3%),UEC组(42·5%),PR阳性表达UPSC组(12·5%),UEC组(35·2%),两组比较:ER(P<0·01),PR(P<0·05),差异有显著性。UPSC组p53蛋白表达与肿瘤分期、分级、及肌层浸润无明显关系,而UEC组Ⅲ~Ⅳ期肿瘤的p53蛋白的表达率高于Ⅰ-Ⅱ期(P<0·005)。UPSC的5年生存率为25%,UEC组5年生存率为80·9%(P<0.01),两组差异有显著性。结论UPSC为p53高表达,而缺乏雌激素和孕激素受体,为高度恶性的肿瘤。它的生物学行为不同于UEC,因而强调诊断时需和其他类型的子宫内膜癌相区别。     

气管上皮-肌上皮癌的临床病理学观察

目的观察气管上皮-肌上皮癌(epithelial-myoepithelial carcinoma,EMC)的临床病理学、影像学特点,探讨其免疫组化特征及鉴别诊断。方法采用组织学、免疫组化及组织化学技术对1例气管EMC进行光镜观察及免疫标记,并结合相关文献对其临床表现、影像学、组织形态和免疫组化特点等进行综合分析。结果患者男性,25岁,肿瘤病理组织学表现为分叶状生长,管状和实性区混合存在。瘤细胞由上皮和肌上皮细胞组成,呈双层腺管样结构。免疫组化染色示:上皮细胞CK、EMA均(+),肌上皮细胞SMA、S-100、p63、HHF35、GFAP均(+),管状结构周围PAS染色(+)。结论原发于气管的EMC十分罕见,影像学具有一定特征,免疫组化染色有助于该肿瘤的诊断,应与多形性腺瘤、肌上皮瘤、嗜酸细胞瘤、腺样囊性癌等鉴别;以手术切除为主的综合治疗预后较好。     

Glycogen-rich clear cell carcinoma of the breast: a solid variant with mucus. A light microscopic, immunohistochemical and ultrastructural study of a case

The light microscopic, immunohistochemical and ultrastructural features of a clear cell carcinoma of the breast have been studied. Both intraductal and invasive components were found. Histochemistry showed large amounts of intracytoplasmic glycogen and sparse neutral mucin in the tumour. The tumour cells were stained by antisera to carcinoembryonic antigen, keratin and epithelial membrane antigen, but not by antisera to alpha-lactalbumin, desmin or vimentin. Ultrastructurally, the epithelial derivation of the tumour was confirmed. Only a few intracytoplasmic lumina were demonstrated. The tumour was classified as a mucin-containing variant of glycogen-rich, clear cell carcinoma of the breast.