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。     

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。     

Epithelial-myoepithelial carcinoma of the lacrimal gland: a rare case

Epithelial-myoepithelial carcinoma is a rare tumor of the salivary gland constituting only 1% of all tumors. It is a low-grade malignancy characterized by a classical biphasic morphology and immunophenotype. In the lacrimal gland, it is extremely rare with only 3 cases reported in the English medical literature. We describe the fourth case of epithelial-myoepithelial carcinoma, the first case in a female patient, and review the available literature.     

Mucoepidermoid carcinoma of the bronchus: a review

Although mucoepidermoid carcinoma of the salivary gland is relatively common, mucoepidermoid carcinoma arising from the mucous glands of the bronchus is rare. Bronchial mucoepidermoid carcinoma usually presents as an intraluminal mass producing luminal occlusion. Symptoms are airway obstruction and recurrent pneumonia. Macroscopically, mucoepidermoid carcinoma appears as an exophytic intrabronchial mass with intact or ulcerated bronchial mucosa. Microscopically, the tumors are located in the submucosa of the large bronchi. The tumors are usually well differentiated and contain a combination of mucus-secreting, squamous, and intermediate cells. The increased frequency of this tumor in the pediatric population suggests a genetic abnormality. Recent genetic studies have demonstrated reciprocal chromosomal translocations including t(1;11)(p22;q13), t(11;19)(q14-21;p12), and t(11; 19)(q21;p13). Chromosome 11 in the first translocation appears to have been altered resulting in up-regulation of the cyclin D1 gene and overexpression of cyclin D1. The t(11;19)(q21;p13) encodes a novel fusion product capable of disrupting the Notch signaling pathway.     

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。     

Epithelial-myoepithelial carcinoma of the parotid gland in a child.

A rare case of epithelial-myoepithelial carcinoma of the parotid gland, which occurred in a child, is reported. An 8-year-old boy presented with swelling of the right parotid gland. He underwent total parotidectomy followed by irradiation for a parotid gland tumor. Three years after the operation, a recurrent tumor invading the base of the skull and the brain and metastases in the lung were noted. The patient expired in spite of extirpation of the intracranial recurrent tumor. The resected tumor showed a characteristic histologic feature: double-layered tubular structures composed of inner dark cells (epithelial cells) and outer clear cells (myoepithelial cells). This patient may be the youngest one with the epithelial-myoepithelial carcinoma reported in the literature.     

Epithelial-myoepithelial carcinoma of salivary glands.

Four cases of epithelial-myoepithelial carcinoma of the salivary glands arose as painless masses in patients over 60 years old, three in the parotid and one in the submandibular gland. Histologically, all the tumours were composed of small ducts with a double cell lining surrounded by a basement membrane. The inner cells were epithelial and the outer cells myoepithelial, the latter usually possessing clear cytoplasm. There was a variable degree of intervening hyalinised stroma. All the tumours were partly encapsulated, but also displayed local invasiveness. One of the tumours also showed areas of dedifferentiation when it later recurred and metastasised. The other three were apparently cured by initial excision, with adjuvant radiotherapy in one instance. In the past this tumour has been described as clear cell adenoma, and it was only recently that its true malignant nature, albeit low grade, was recognised. Reports of epithelial-myoepithelial carcinoma are still relatively few, with only one case described from Britain. It is recommended that this histologically distinct neoplasm deserves wider recognition.     

Epithelial-myoepithelial carcinoma harboring p53 mutation

A case of epithelial-myoepithelial carcinoma of the parotid gland harboring p53 mutation is reported. The tumor removed from a 67-year-old Japanese female was composed of an organoid biphasic population of cells: inner dark epithelial cells were surrounded by clear myoepithelial cells. The cells were immunopositive for EMA and smooth muscle actin, respectively. Some of the epithelial cells formed solid nests. Immunostaining for proliferating cell nuclear antigen (PCNA) resulted in a higher percentage of labeled cells in the solid epithelial region than in the region with the more general biphasic pattern. Genetic analysis, including polymerase chain reaction-single strand conformational polymorphism (PCR-SSCP) and nucleotide sequencing, revealed a mutation in codon 207 (aspartic acid to glycine) of the p53 tumor-suppressor gene. To our knowledge, this is the first report of a mutation in the p53 gene in an epithelial-myoepithelial carcinoma of the salivary gland.     

膀胱癌肉瘤3例临床病理分析

目的探讨膀胱癌肉瘤的临床病理特征和组织发生。方法回顾性分析3例膀胱癌肉瘤患者的临床资料,进行病理组织学及免疫组化观察并进行文献复习。结果3例患者中男2例,女1例。发病年龄50～75岁,均以肉眼血尿入院,其中1例有尿痛。膀胱镜检查见膀胱顶部或侧壁可见菜花样或息肉状肿物,浸润性生长。2例行膀胱部分切除术,1例行经尿道膀胱肿瘤电切术。病理检查瘤组织均由明确的癌和肉瘤成分构成。分别构成于腺癌和梭形细胞肉瘤、移行上皮癌和横纹肌肉瘤、移行上皮癌和软骨肉瘤。结论膀胱癌肉瘤是一种少见的高度恶性的膀胱肿瘤,预后较差。诊断主要靠病理组织学检查及免疫组化染色。     

腹膜浆液性乳头状腺癌4例并临床病理分析

目的探讨腹膜浆液性乳头状腺癌(peritoneal papillary serous carcinoma,PPSC)的临床病理学特征、免疫表型、鉴别诊断及预后。方法对4例PPSC行免疫组化SP法染色及HPV检测,并复习相关文献。结果 4例PPSC均为女性,年龄23~62岁,平均51岁。临床表现多为无特异性的腹部不适,CA125升高,影像学表现为腹腔肿块。镜下肿瘤细胞形成形态不一、大小不等的乳头状结构;肿瘤细胞为低柱状,核质比大,核圆形,核仁大,异型性明显,核分裂象易见。腺体及间质内可见砂砾体。免疫表型:肿瘤细胞CA125、WT1、CK7、p53、CEA、p16均呈阳性,部分肿瘤细胞表达CK5/6、ER、PR;不表达GCDFP-15、CR、D2-40、CK20、Villin、CDX2、TTF1;HPV检测阴性。结论 PPSC是一种少见的原发于腹膜的恶性肿瘤,临床误诊率高,依据其临床和病理组织学特点,结合免疫组化染色可以明确诊断。     

Metaplastic carcinoma of the breast: a clinicopathological review

BACKGROUND: Mammary metaplastic carcinoma encompasses epithelial-only carcinoma (high-grade adenosquamous carcinoma or pure squamous cell carcinoma), biphasic epithelial and sarcomatoid carcinoma and monophasic spindle cell carcinoma. AIM: To evaluate the clinicopathological features of a large series of 34 metaplastic carcinomas. METHODS: 10 epithelial-only, 14 biphasic and 10 monophasic metaplastic carcinomas were assessed for nuclear grade, hormone receptor status, HER2/neu (cerbB2) oncogene expression, Ki-67 and p53, lymph node status and recurrence on follow-up. RESULTS: Intermediate to high nuclear grade were assessed in most (33/34) tumours. Oestrogen and progesterone receptors were negative in 8 of 10 epithelial-only, all 14 biphasic, and 9 of 10 monophasic tumours, cerbB2 was negative in 7 of 10 epithelial-only, all 14 biphasic and 8 of 10 monophasic tumours. Ki-67 was found to be positive in 6 of 10 epithelial-only, 6 of 14 biphasic, and 7 of 10 monophasic tumours, whereas p53 was positive in 6 of 10 epithelial-only, 7 of 14 biphasic, and 8 of 10 monophasic tumours. Lymph node metastases were seen in 7 of 7 epithelial-only, 7 of 11 biphasic, and 3 of 7 monophasic tumours. Recurrences were seen in 4 of 7 epithelial-only, 8 of 9 biphasic, and 4 of 9 monophasic tumours. CONCLUSIONS: All three subtypes of metaplastic carcinoma are known to behave aggressively, and should be differentiated from the low-grade fibromatosis-like metaplastic carcinoma, which does not metastasize. Oncological treatment options may be limited by the frequently negative status of hormonal receptor and cerbB2.     

Epithelial-myoepithelial carcinoma arising in the nasal cavity: a case report and review of literature.

Epithelial-myoepithelial carcinoma is an uncommon, low-grade, malignant epithelial neoplasm composed of variable proportions of ductular cells and large, clear staining, myoepithelial cells arranged around the periphery of the ducts. About 120 cases have been reported in the world literature, most of which were located in salivary glands, except for a few cases occurring in unusual locations such as breast, lacrimal gland, nose, paranasal sinus, trachea, bronchus, and lung. We here reported the second case of epithelial-myoepithelial carcinoma of the nasal cavity with extension to the nasopharynx. The patient was a 61 year old Chinese female with two month's history of progressive nasal obstruction. Histopathologically, the tumor showed typical myoepithelial and ductal cells biphasic differentiation, duct-like structure and infiltrating growth pattern. Some ductal cells showed the characteristics of oxyphilic cell, which had never been reported before. Recurrence and metastasis rates of epithelial-myoepithelial carcinoma varied from 35% to 50% and 8.1% to 25% respectively in different reports. The present case had neither recurrence nor metastasis twenty months after operation. When epithelial-myoepithelial carcinoma is mainly composed of spindle myoepithelial cells, the differential diagnosis should include myoepithelioma, neurofibroma, leiomyoma and hemangiopericytoma.     

乳腺腺肌上皮瘤临床病理观察及文献复习

目的探讨乳腺腺肌上皮瘤（adenomyoepithelioma,AME）的形态学及免疫组化表型特征。方法对2例乳腺AME进行临床病理学分析和免疫组织化学研究。结果肿瘤由增生的腺上皮和肌上皮组成。腺上皮CK、EMA、ER、PR（＋）;肌上皮CK（-）,S-100蛋白、SMA、actin（＋）、EMA局部弱（＋）,GFAP、desmin（-）。结论乳腺AME是一种具有独特病理学特征的良性肿瘤,由腺上皮和肌上皮共同增生而成。熟悉该肿瘤的组织学和免疫表型特点对鉴别诊断有重要意义。     

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.     

Myoepithelial carcinoma of the orbit: a clinicopathological and histopathological study

Tran TL, Broholm H, Daugaard S, Fugleholm K, Poulsgaard L, Prause JU, Kennedy SM, Heegaard S. Myoepithelial carcinoma of the orbit: a clinicopathological and histopathological study. APMIS 2010; 118: 324–30. Two cases of invasive myoepithelial carcinoma arising from the paranasal sinuses and invading the orbit are presented. Patient 1, a 53‐year‐old man, had a 3‐month history of proptosis, pain and epiphora of the right eye. The second patient, a 24‐year‐old man, had for a week been complaining of protrusion of his left eye and of orbital pain. Computed tomography scan and magnetic resonance imaging revealed tumour masses in the frontal, ethmoidal and maxillary sinuses with invasion of the orbit and the frontal lobe. Biopsies from both cases showed spindle and epithelioid tumour cells. Mitotic figures were frequent. Immunohistochemical staining showed positive reaction for bcl‐2, calponin, cytokeratins, CD99, S100, muscle‐specific antigen, smooth muscle antigen and vimentin. The Ki‐67 index was between 30–50% and 5–25%, respectively. Ultrastructurally, intermediate filaments, perinuclear tonofilaments and desmosomes were present. Based on these findings, a diagnosis of myoepithelial carcinoma of mixed cell type in both cases was evident. Both patients died shortly after the diagnosis was made even though both underwent radical surgery. Myoepithelial carcinoma of the paranasal sinuses is very rare and only six cases have been reported previously. We present the first two cases of myoepithelial carcinoma in the paranasal sinuses with invasion of the orbit. This is also the first report of myoepithelial carcinoma arising in the ethmoidal sinus.     

结肠浸润性微乳头状癌临床病理分析

目的探讨结肠浸润性微乳头状癌的临床病理特征。方法应用光镜观察、免疫组织化学染色等方法对3例结肠浸润性微乳头状癌进行分析,并复习文献。结果 3例中,男性2例,女性1例,年龄48～69岁,平均56岁,2例发生于升结肠,1例发生于乙状结肠,病变长度范围5～10cm,光镜下,3例均可见局灶性高-中分化腺管状腺癌,但主要由浸润性微乳头状癌成分组成,微乳头状成分位于肿瘤边缘及浸润部。免疫组化染色EMA显示特征性"外翻性"结构。随访:例1肿瘤切除后3个月肺转移,7个月后死亡;例2同时伴有腹壁转移结节;例3切除后6个月后锁骨上淋巴结转移。结论结肠浸润性微乳头状癌临床预后较差,具有高度侵袭性和结肠外侵犯,形态学上应与淋巴管内癌栓、人为脱水因素及其它转移性肿瘤相鉴别。