去分化软骨肉瘤11例临床病理分析

目的探讨去分化软骨肉瘤的临床、影像学及病理学特点。方法收集11例去分化软骨肉瘤患者的临床及影像资料,对组织进行病理分析。结果患者以男性多见(7/11),平均年龄57.1岁,肿瘤多位于长管状骨及骨盆。影像学显示广泛的骨质破坏,病灶内可有钙化或骨化,病理性骨折3例。眼观:病理软骨成分常位于骨内,去分化肉瘤成分主要位于骨外。镜下包括高分化软骨肉瘤和低分化间叶源性肉瘤两种成分。随访材料中6例死亡,平均存活14个月。结论去分化软骨肉瘤检:呈少见的的软骨肉瘤亚型,去分化类型多样,只有加深对其的认识,才能提高诊断的准确率。     

胰腺黏液性囊性肿瘤临床病理分析

目的；探讨胰腺黏液性囊性肿瘤（MCN）的临床病理学特点。方法：6例MCN均行B超或CT检查。除1例外，均作了手术切除治疗。对6例（MCN）作常规HE及免疫组织化学染色观察。结果：6例MCN中，女性4例，男性2例，平均年龄47岁，均位于胰腺体尾部。黏液性囊腺瘤（MCA）3例，囊壁内衬高柱状黏液上皮，上皮周围可见卵巢样间质组织；黏液性囊腺癌（MCC）3例，黏液上皮 有不典型增生，均有囊壁或胰腺组织的浸润，1例上皮周围可见卵巢样间质。CEA和CK 7在上皮中均阳性，c-erbB－2均阴性，间质SMA均阳性。结论：胰腺MCN是好发于中年女性的少见肿瘤，绝大多数发生于胰腺体尾部。MCA内衬高柱状上皮，上皮外来卵巢样间质包绕；MCC上皮细胞有不典型增生， 浸润性生长。MCN手术切除率高。     

Extra-salivary gland presentations of adenoid cystic carcinoma: a report of three cases

Adenoid cystic carcinoma (ACC) is a malignant neoplasm most commonly originating in salivary glands of the head and neck area. When ACC presents outside of these locations, the diagnosis may become more challenging. We describe three cases of ACC presenting in extra-salivary gland sites. Two cases were metastatic; in case 1 the initial presentation was widespread bony metastasis of unknown primary origin. The other metastatic case (case 2) was from a patient presenting with a pleural effusion and a history of previously treated metastatic pulmonary ACC. The pleural effusion cytology was unusual in that exfoliated ACC cells were present in the effusion itself, a rare occurrence. Case 3 was a primary bronchial ACC. In conclusion, ACC can present in various body sites and cytologists must consider this neoplasm when presented with a basaloid carcinoma of uncertain origin.     

组织细胞肉瘤的临床病理观察

目的 探讨组织细胞肉瘤(histiocytic sarcoma,HS)的病理形态学、免疫表型、鉴别诊断和预后.方法 分析6例HS患者临床病理资料,对标本行光镜观察、免疫组织化学EliVision法染色,并对患者进行6～36个月的随访.结果 6例HS中男女各3例,年龄12～81岁(平均54.6岁).发生部位为淋巴结(2例)、皮肤及软组织(4例).组织形态改变:瘤细胞弥漫性浸润,呈中至大的多角形上皮样细胞,胞质丰富,嗜酸性,核圆形或不规则,空泡状,有1至多个明显核仁,核分裂象多见.细胞境界较清.6例均见双核瘤细胞,2例多形性明显,可见多核瘤巨细胞,3例可见灶状泡沫状胞质的瘤细胞,1例可见灶状肉瘤样梭性细胞区,2例可见噬血细胞现象.6例均见到多少不一的炎细胞背景.均弥漫表达白细胞共同抗原、CD4、CD68、CD163,溶菌酶染色的5例中4例阳性.4例得到随访资料,3例确诊后6～11个月内死亡,1例局限于皮肤及皮下软组织者已存活3年.结论 明确HS诊断需结合形态改变与免疫表型.该病就诊时多处于进展期,对化疗反应差,但少数病变局限的病例不表现为侵袭性过程,预后较好.     

Vaginal adenoid cystic carcinoma: a case report

Adenoid cystic carcinoma generally arises from the salivary glands and is rarely found in the female genital tract. Infection with HPV is implicated in this cervical lesion. Differential diagnosis includes adenoid basal carcinoma, polymorphous low-grade adenocarcinoma and basaloid squamous cell carcinoma. Only one case of vaginal localisation was previously described. We report a case of adenoid cystic carcinoma in a 48-year-old woman with previous cervical HPV infection. Histological examination revealed nests of cells with peripheral palisading organisation and glandular lumina containing material produced by the tumor cells.     

胃原发性淋巴上皮瘤样癌6例临床病理学观察

目的探讨胃淋巴上皮瘤样癌(lymphoepithelioma-like gastric carcinoma,LLGC)临床病理学特征、免疫表型及与EB病毒(EBV)感染的关系。方法收集6例LLGC,对其临床特征、病理学形态和免疫表型进行观察,采用原位杂交法检测EBV编码小RNA(EBER)的表达。结果 6例均为男性,发病年龄47~68岁,平均55岁,6例均累及胃体。镜检:在丰富的淋巴细胞背景中见散在的未分化癌细胞条索或细胞巢,偶尔在近黏膜表面出现发育差的腺管结构,间质内纤维组织少,见淋巴滤泡形成。瘤细胞表达CKpan、CK19、CEA及EBER,不表达CK7、CK20、CK5/6及LMP1;间质淋巴细胞表达CD3或CD20,不表达EBER。6例患者随访6~57个月,均无瘤存活。结论 LLGC是一种罕见的具有独特临床病理学特征的胃癌亚型,预后较好,与EBV感染密切相关。     

Laryngeal adenoid cystic carcinoma: a rare case report

Laryngeal adenoid cystic carcinomas (LACC) are extremely rare and only account for less than 1% of all malignant laryngeal tumors. This tumor commonly occurs in the subglottic region of larynx, so dyspnea and hoarseness are its most common presenting symptoms. Adenoid cystic carcinoma is characterized by slow progression, late recurrence, and late distant metastasis. Total or partial laryngectomy is its treatment. Although it does not respond to radiotherapy completely, adjuvant radiotherapy or chemotherapy may be considered. In this study, we report a 41 year-old man, who had a rare recurrence of LACC, and we evaluate his clinical and pathologic characteristics.     

Dedifferentiated endometrioid adenocarcinoma of the uterus: a clinicopathologic study of a case

Dedifferentiation confers more aggressive malignant behaviour than would be otherwise shown by the original tumor if present alone. This phenomenon has been described in several tumors, both mesenchymal and epithelial. Dedifferentiated endometrioid carcinoma either ovarian or endometrial is the latest addition to this family of tumors. Only 2 papers have appeared in the literature so far on the topic of dedifferentiated endometrioid carcinoma, both from the same institution. We report herein a case of endometrial dedifferentiated endometrioid carcinoma in a 45-year old lady with ovarian metastasis from the undifferentiated component. The primary endometrial tumor showed an undifferentiated component in an otherwise low grade endometrioid carcinoma. The undifferentiated component of these tumors can be misdiagnosed as the solid component of FIGO grade 3 in a pure endometrioid carcinoma. The recognition of an undifferentiated component in an otherwise low grade endometrioid carcinoma is very important, since dedifferentiated endometrioid adenocarcinoma has a worse prognosis when compared with FIGO grade 3 endometrioid carcinoma.     

Metastatic adenoid cystic carcinoma of lung: a case report

A 48 year old male patient, operated five years back for sub mandibular swelling proved on histopathologic examination to be adenoid cystic carcinoma presented in the ENT department, I. G. medical hospital with pain on both sides of the chest. CT scan of the chest showed multiple lesions of variable sizes. CT guided FNAC as well as biopsy of the lung lesion was performed. Cytodiagnosis and histopathological examinations revealed features of cribiform type of adenoid cystic carcinoma. This case is reported here for its metastasis to the lungs and prolonged survival even with multiple metastasis.     

Early gallbladder carcinoma: a clinicopathologic study of 13 cases of intramucosal carcinoma

We report the clinicopathologic features of 13 cases of intramucosal carcinoma (IMC) of the gallbladder. All IMCs were incidental findings in cholecystectomy specimens for cholelithiasis. However, one of the patients had a carcinoma of the pancreas, and the gallbladder incidentally removed during the Whipple procedure showed an IMC. Another patient had a small cell carcinoma of the gallbladder, and one of the sections showed an IMC. Of the IMCs, 10 were well-differentiated adenocarcinomas, 1 was a moderately differentiated adenocarcinoma, 1 was an undifferentiated carcinoma, and 1 was a squamous cell carcinoma. Of the patients, 8 were disease-free from 3 to 11 years, and 2 patients died, one as a result of the pancreatic ductal carcinoma and the other with disseminated metastases of the small cell carcinoma. The follow-up of another patient was too short to be significant. Two patients were lost to follow-up. Our findings suggest that a simple cholecystectomy is a curative procedure for IMCs of the gallbladder.     

胰腺胶样癌四例临床病理学分析

目的 探讨胰腺胶样癌的临床病理学特征、诊断、鉴别诊断及分子生物学特点.方法 分析4例胰腺胶样癌的临床特点,对标本进行病理形态学观察、免疫组织化学EnVision法和K-ras基因突变检测.结果 4例胶样癌中3例发生在胰头部,患者均为男性;另1例在胰体尾部,为女性;平均发病年龄为56.5岁.其中2例首发症状为腹痛,1例为尿糖增高,1例为查体发现.3例大体形态为囊实性结节,囊内含黏液,1例大体呈实性.低倍镜下,纤维及胰腺组织中可见边界清楚的黏液结节,大的黏液湖中可见纤细的纤维结缔组织间隔成多个小黏液湖;肿瘤细胞漂浮在黏液湖中,呈小巢或条索状,或腺管状,也可看到印戒细胞漂浮其中.3例癌周可见肠型胰腺导管内乳头状黏液性肿瘤(IPMN),仅例1伴发胰胆管型IPMN.免疫组织化学染色3例MUC2细胞膜阳性,1例MUC1阳性.3例中例1和例3发现K-ras基因突变,突变位点均位于12密码子Gly12Asp(GGT＞GAT)和Gly12Arg( GGT＞ CGT).结论 胰腺胶样癌是少见的胰腺导管腺癌亚型,经常伴发于IPMN和胰腺黏液性囊性肿瘤,应与普通胰腺导管腺癌、印戒细胞癌及假性囊肿等病变相鉴别.免疫组织化学MUC2多阳性表达,MUC1多为阴性,K-ras基因突变率较低.     

Serous surface carcinoma of the peritoneum: a clinicopathologic study of 22 cases.

Serous surface carcinoma (SSC) of the peritoneum is defined as a primary tumor histologically indistinguishable from serous carcinoma of the ovary, diffusely involving the peritoneal surface but sparing or only superficially invading the ovaries. In this study of 22 cases of SSC, it was found that the main clinical manifestations of SSC were abdominal pain and enlargement. In most cases, SSC evenly involved the entire mesothelial surface but rarely was predominant in or even limited to the pelvis. It frequently invaded the submesothelium, but deep invasion into abdominal and pelvic organs or local metastasis was rare, and distant metastasis was not seen at presentation. Microscopically, SSC was a high-grade tumor frequently showing high mitotic rate, psammomas bodies, and necrosis. The tumor was usually contiguous with hyperplastic mesothelium on either ovarian surface or other locations. Tumor cells in all cases except one showed cytoplasmic or surface neutral or acidic mucin or both. Tumor cells stained positive for keratin (100% of cases), epithelial membrane antigen (100%), Leu-M1 (45%), B72.3 (85%), vimentin (35%), and carcinoembryonic antigen (25%). Electron microscopic studies of six cases showed epithelial differentiation in each. Seven patients (32%) were alive with no clinical disease at 3 to 31 months, one patient (4%) was alive with extensive local disease at 24 months, 11 patients (50%) died almost exclusively of local recurrence at 1 to 70 months, and three patients (14%) died of operative complications. It is concluded that SSC arises from peritoneal mesothelium but has epithelial phenotype. It can be morphologically differentiated from other conditions with similar laparotomy findings, such as malignant mesothelioma, benign papillary mesothelioma, cystic mesothelioma, and benign or borderline peritoneal serous tumors. The prognosis of SSC is poor, and most patients die of uncontrollable local disease.     

Adenoid cystic carcinoma. A clinicopathologic study with flow cytometric analysis

Forty-five patients with adenoid cystic carcinoma (ACC) of salivary glands were retrospectively studied to determine the prognostic use of DNA ploidy analysis compared with clinicopathologic features. Nuclear suspensions were prepared from 37 of these tumors by the Hedley technique on paraffin-embedded material. The DNA content was analyzed by flow cytometry after propidium iodide staining. Thirty-five tumors were diploid and 2 were tetraploid. Mean survival was 117 and 52 months for the diploid and tetraploid patients, respectively. The median S-phase fraction (Spf) of the 35 diploid tumors was 4.45%. Of the 17 diploid patients who died of disease, there were 11 tumors with high Spf (greater than 4.45%) and 6 tumors with a low Spf (P less than 0.05 chi-square test). The presence of more than 30% of a solid pattern in the tumor was strongly associated with poor median survival in Kaplan-Meier survival curve analysis (P less than 0.05 log rank test). Grade, stage, recurrence, and metastases were also found to be important prognostic factors. Because few tumors were nondiploid, these results suggest that S-phase fraction analysis may be a more useful prognostic indicator than ploidy classification.     

Laminin and fibronectin in adenoid cystic carcinoma.

The distribution of fibronectin and laminin was examined by immunohistochemistry in 11 adenoid cystic breast carcinomas, six adenoid cystic carcinomas of mouth and salivary gland, and six cribriform ductal breast carcinomas. Both proteins were present lining cystic lumina and around tumour islands in all the adenoid cystic breast carcinomas and in five of six salivary gland tumours. Abundant laminin and fibronectin were dispersed among adenoid cystic tumour cells arranged in sheets. One adenoid cystic carcinoma from buccal mucosa showed a transition from a cribriform tumour positive for both fibronectin and laminin to a cribriform tumour negative for fibronectin and laminin to undifferentiated carcinoma. Fibronectin and laminin seemed to disappear simultaneously from tumour cell surfaces. Another adenoid cystic carcinoma from buccal mucosa was negative for fibronectin and laminin from the time of initial biopsy. This was the only tumour that gave rise to disseminated metastases, resulting in the death of the patient within two years of surgery. In cribriform invasive ductal breast carcinomas the linings of cystic lumina were always negative for fibronectin and laminin. Varying quantities were present at the tumour boundaries. We suggest that staining for fibronectin and laminin may be a valuable aid to the diagnosis of adenoid cystic carcinomas and that the absence of these proteins may have important prognostic implications.     

Promoter methylation and protein expression of the E-cadherin gene in the clinicopathologic assessment of adenoid cystic carcinoma.

Adenoid cystic carcinoma, a relatively uncommon tumor of salivary glands, is characterized by a prolonged clinical course and a fatal outcome. The molecular events underlying their progression are unknown. In this study, we examined the methylation status of E-cadherin gene and its protein expression in 23 cases of adenoid cystic carcinoma and correlated the results with the clinicopathologic factors to determine its role in these tumors. We also analyzed the effect of 5-azacytidine on the re-expression in a methylated cell line of adenoid cystic carcinoma for this gene. In our study, E-cadherin immunoreactivity, although heterogeneous, showed a progressive reduction with high histological grade and in metastatic and recurrent lesions. Promoter methylation was detected in 16 of 23 cases (70%), but there was no correlation with the histological grade or patient prognosis. Microdissection of immuno-negative cells in heterogeneous tumors showed positive methlyation. In the cell line from salivary adenoid cystic carcinoma with methylated E-cadherin, 5-azacytidine restored the E-cadherin expression. Our results indicate that: (1) E-cadherin gene promoter is frequently methylated in adenoid cystic carcinoma, leading to reduced E-cadherin expression, (2) variable E-cadherin expression might result from the intratumoral heterogeneity, and (3) increased extent of methylated areas may be associated with progression and advancement of the disease.