Glycogen-rich clear cell carcinoma arising from minor salivary glands of the uvula. A case report

Glycogen-rich clear cell carcinoma arising from minor salivary glands of the uvula in a 35-year-old woman is reported. This tumor was composed of nests and cords of clear cells containing cytoplasmic glycogen but no mucin. The epithelial nature of this lesion was obvious because of positive immunoreactivity for cytokeratin and epithelial membrane antigen, and the presence of immature lumina and intercellular spaces lined by many microvilli, associated with desmosomal junctions and basal lamina, as revealed by ultrastructural study. However, no myoepithelial cells could be detected. From these findings, it may be concluded that this tumor corresponds to glycogen-rich clear cell carcinoma (a variant of clear cell tumor), revealing glandular differentiation.     

Expression of carbonic anhydrase IX,PAX2 and PAX8 and their association with clinicopathologic characteristics in renal epithelial tumors北大核心CSCD

Objective: To study the expression of carbonic anhydrase IX (CAIX), PAX2 and PAX8 in different types of renal epithelial tumor and their association with clinicopathologic characteristics. Methods: Immunohistochemical study by EnVision method was performed in order to assess the expression of CAIX, PAX2 and PAX8 in 155 cases of renal cell carcinoma and 4 cases of metastatic clear cell renal cell carcinoma (CCRCC). Ninety-six cases of non-neoplastic renal parenchymal tissue adjacent to CCRCC, 8 cases of clear cell hepatocellular carcinoma and 2 cases of clear cell hidradenoma were used as controls. Results: CAIX was commonly expressed in CCRCC (94. 0%, 63/67), of which 77. 8% (49/63) showed strong positivity. CAIX was focally positive in papillary renal cell carcinoma, collecting duct carcinoma and urothelial carcinoma of renal pelvis. It was negative in chromophobe renal cell carcinoma, oncocytoma and adjacent non-neoplastic renal tissue. CAIX was also strongly expressed in the 4 cases of metastatic CCRCC. Focal expression of CAIX was demonstrated in the 8 cases of clear cell hepatocellular carcinoma and 2 cases of clear cell hidradenoma. The expression of CAIX in CCRCC did not correlate with tumor grading, clinical staging and presence of distal metastasis. On the other hand, PAX2 showed positive expression in different types of renal epithelial tumor, clear cell hepatocellular carcinoma and clear cell hidradenoma in various degrees. In contrast, PAX8 was commonly expressed in all types of renal epithelial tumor, with the exception of urothelial carcinoma of renal pelvis. PAX8 was not expressed in clear cell hepatocellular carcinoma and clear cell hidradenoma. Regarding diagnosis of CCRCC, CAIX demonstrated high sensitivity and specificity. PAX2 showed high specificity but low sensitivity. PAX8 was sensitive and specific in the diagnosis of renal epithelial tumor. Conclusions: CAIX is a useful immunohistochemical marker with high specificity and sensitivity in distinguishing CCRCC from other types of renal epithelial tumor and clear cell tumors of non-renal origin. PAX2 is a marker with high sensitivity and low specificity for diagnosis of renal epithelial tumors. PAX8 is typically expressed in renal epithelial tumors. The combined detection of CAIX, PAX2 and PAX8 is useful in the diagnosis and differential diagnosis of renal epithelial tumors.     

多囊性肾透明细胞癌的病理学诊断

目的：探讨多囊性肾透明细胞癌的临床病理特点。方法：对1例多囊性肾透明细胞癌进行了免疫组化染色,并进行文献复习。结果：本例右肾肿物18年。大体见肿物由多发不等的囊腔组成。镜下囊内壁主要由单层立方或柱状上皮被覆,部分为多层并有乳头形成。瘤细胞胞质透亮,无明显异型性。癌细胞免疫表型cytokeratin、CEA和vimentin呈阳性表达。本例诊断为多囊性肾透明细胞癌。结论：多囊性肾透明细胞癌是一种罕见的肾癌病理类型。临床上主要采用根治切除术。本瘤的生物学行为属于低度恶性肿瘤。     

多囊性肾细胞癌1例报道及文献复习

目的：阐明多囊性肾细胞癌的病理形态学特征及鉴别诊断要点。方法：报道1例多囊性肾细胞癌,结合文献对本病的临床病理特征进行探讨。结果：肿瘤由厚的假纤维被膜环绕,瘤体全部由大小不等的囊腔及无膨胀性实性结节的薄的间隔构成,囊腔被覆透明细胞,异形性小,间隔内含聚集的透明细胞上皮巢,结论：多囊性肾细胞癌是一种罕见的有低度恶性倾向的肿瘤,术前影像学,细胞学检查及术后的肉眼特征均与囊性肾瘤,囊性部分分化性肾母细胞瘤相似,最重要的是本瘤缺乏肉眼所见的膨胀性实性结节,显微镜下的关键特征是间隔内可见聚集的透明细胞巢。     

Cystic Squamous Cell Carcinoma of the Thyroid A Possible New Subgroup of Intrathyroidal Epithelial Thymoma

In the thyroid gland, both squamous cell carcinoma and intrathyroidal epithelial thymoma are rare tumors with squamous features, but their prognoses differ; the former is usually fatal, and the latter is favorable. We describe a 38-yr-old female patient with a cystic tumor diagnosed cytologically as squamous cell carcinoma, who was treated with subtotal thyroidectomy. The tumor was located intrathyroidally in the middle third of the left lobe, and no invasion to the surrounding tissues was found. Histologic examination disclosed a cyst lined by epithelial cells with squamous cell differentiation. The tumor cells had ill-defined cell borders and large nuclei with prominent nucleoli. Invasion of the tumor capsule, a few mitoses, and infiltration of lymphocytes in the tumor periphery were noted, but neither necrosis nor metastasis to lymph nodes was observed. The patient has been free from recurrence for 3 yr after thyroid lobectomy. We propose that this type of cystic intrathyroidal squamous cell carcinoma may represent a cystic variant of intrathyroidal epithelial thymoma.     

Histopathological and immunohistochemical findings in a case of glycogen-rich clear cell carcinoma of the breast

Glycogen-rich clear cell carcinoma (GRCC) of the breast is a rare variant of primary breast carcinoma that was first described by Hull et al. in 1981, and is characterized by carcinoma cells containing an optically clear cytoplasm and intracytoplasmic glycogen. The present case involved a 33-year-old female. She had noticed a lump in the inner quadrant of the left breast. The tumor obtained by enucleation biopsy had an irregular shape. The tumor cells exhibited sharply defined borders, polygonal contours, a clear or finely granular cytoplasm, and moderate nuclear atypia. The tumor cells showed a positive reaction with periodic acid Schiff, eliminated by diastase digestion. The tumor was diagnosed as GRCC. There was no nodal metastasis. Immunohistochemically, the tumor cells were positive for cytokeratin, epithelial membrane antigen, HER2, and p53, but negative for estrogen receptor (ER) and progesterone receptor (PR). Although the biological behavior of GRCC is difficult to predict in view of the very limited number of case reports, the prognosis of GRCC may be associated with not only histopathological subtype but also other clinicopathological factors, such as size, status of invasion, status of nodal metastasis, nuclear grade, ER, PR, HER-2, p53 and so on. To clarify the pathogenesis of mammary GRCC, the systematic study of additional well-documented cases with long-term follow up will be necessary.     

CA125-producing adenocarcinoma of the seminal vesicle

A case of primary seminal vesicle carcinoma is reported. The tumor was a CA125-producing adenocarcinoma consisting of fine papillary-tubular, intricate branching or anastomosing glandular structures and was composed of small cuboidal, but occasionally hobnailed, cells with mostly clear, but occasionally granular, cytoplasm. Some tumor cells showed evidence of secretion of seromucinous materials into the interpapillary and cystic space. lmmunohistochemically, almost half of the tumor cells expressed a positive reaction with anti-CAl25, a common serological marker for ovarian epithelial carcinomas; however, no tumor cells expressed any other serological tumor markers such as carcinoem-bryonic antigen, α-fetoprotein, human chorionic gonadotropin, prostatic specific acid phosphatase, or prostatic specific antigen. The patient showed a high level of serological CA125, which fluctuated parallel with the growth, removal and recurrence of the tumor. The morphological and immunohistochemical findings suggested a close relationship between the present tumor and clear cell carcinoma of the ovary, which is thought to be of a Müllerian-Wolfian duct origin.     

Malignant epithelial odontogenic tumors

Malignant epithelial odontogenic tumors are very rare. They may arise from the epithelial components of the odontogenic apparatus. The rests of Malassez, the reduced enamel epithelium surrounding the crown of an impacted tooth, the rests of Serres in the gingiva, and the linings of odontogenic cysts represent the precursor cells for malignant transformation. Because metastatic carcinoma is the most common malignancy of the jaws, the diagnosis of a primary intraosseous carcinoma must always be made to the exclusion of metastatic disease. Odontogenic carcinomas include malignant (metastasizing) ameloblastoma, ameloblastic carcinoma, primary intraosseous squamous cell carcinoma, clear cell odontogenic carcinoma, and malignant epithelial ghost cell tumor. There are specific histopathologic features that support the diagnosis of a primary carcinoma of odontogenic epithelium which are presented in this article. Immunohistochemical (IHC) staining is important for distinguishing clear cell odontogenic carcinoma from metastatic renal cell tumors, yet IHC stains are not particularly helpful for other lesions in this group-all of which exhibit low molecular weight cytokeratin positivity. Aggressive growth and nodal and distant metastases occur with all of these entities.     

Sarcomatoid renal carcinoma: the final common dedifferentiation pathway of renal epithelial malignancies.

Sarcomatoid renal carcinoma (SRC) is an aggressive neoplasm with an age and gender distribution similar to that of conventional (clear cell) renal cell carcinoma (RCC). Genetic and morphologic evidence indicates that the tumor results from de-differentiation of renal epithelial malignancy and associations with RCC, papillary renal carcinoma, chromophobe renal carcinoma and collecting duct carcinoma have been reported. The tumor is composed of sheets of malignant spindle cells that have immunohistochemical and ultrastructural features of both stromal and epithelial cells, and may contain myxoid areas containing osteoclast-like giant cells or pleomorphic sarcomatoid spindle cells resembling rhabdomyoblasts. Rare cases of osteogenic SRC have been described. The tumor shows marked proliferative activity in growth kinetic studies and is usually associated with a poor patient survival that is best predicted by staging.     

原代人宫颈癌细胞的培养方法

背景：在体外分离、培养宫颈癌上皮细胞, 从细胞水平及分子水平研究肿瘤病因及治疗的基础,是宫颈癌组织工程研究的最基本环节。 目的：探讨适用于组织工程宫颈癌研究的人宫颈癌原代细胞培养方法以及传代后细胞形态变化、增殖的特性。 方法：0.25%胰蛋白酶和2%Ⅰ型胶原酶联合消化法体外培养宫颈癌原代细胞,荧光倒置显微镜下观察肿瘤细胞形态、体外生长情况,免疫组化法检测体外培养宫颈癌细胞表面标记物CK17和肿瘤细胞增殖抗原Ki67表达。 结果与结论：采用胰蛋白酶与Ⅰ型胶原酶联合消化法体外分离培养人宫颈癌细胞,该法相对简单且重复性较好,所得到的原代细胞纯度较高。经体外传代培养的宫颈癌细胞仍可保持稳定的细胞表型。宫颈癌细胞表面标记物CK17阳性表达,证实细胞为上皮源性,肿瘤细胞增殖抗原Ki67表达阳性提示所培养细胞具有肿瘤细胞恶性增殖的特性。     

易混淆的膀胱活检小细胞性浸润性尿路上皮癌的诊断与鉴别诊断：附1例报告

目的：探讨膀胱活检中小细胞性浸润性尿路上皮癌的病理形态学特征以及与其它小细胞性病变的鉴别诊断。方法：对北京中医药大学东直门医院1例膀胱黏膜活检的病理及临床资料进行回顾性分析,并对相关国内外文献进行复习。结果：患者,男性,82岁,膀胱镜下见：在膀胱颈部10~12点可见黏膜突起如蕈状,蒂不明显。活检组织光镜下见：1)尿路上皮下小细胞肿瘤细胞呈弥漫性及巢状分布,间质增生;2)瘤细胞体积小,呈短梭形或淋巴细胞样,胞浆极少,局部瘤细胞胞浆宽广透明;3)核为圆形、椭圆形或梭形,核深染且结构不清,部分核不规则,可见双核、核重叠及多核瘤巨细胞,核仁不明显;4)未见核分裂象;5)局部组织可见挤压现象及局灶凝固性坏死;6)可见脉管浸润;7)局部异型增生的尿路上皮基底层处与固有膜内的小细胞性肿瘤成分有移行。免疫组织化学结果显示免疫表型CK少数细胞弱(+),其余标记均(?),病理诊断为：小细胞低分化癌。临床行经尿道膀胱肿瘤电切术,未送病理,数月后发现肠系膜多个转移瘤结节,瘤细胞形态与活检相似,局部瘤细胞核偏位,可见核仁及病理性核分裂象,免疫组化显示上皮性标记、p63及CD44V6均(+),神经内分泌标记(?),综合考虑最后病理诊断为：膀胱小细胞性浸润性尿路上皮癌伴肠壁转移。结论：膀胱小细胞性浸润性尿路上皮癌在膀胱镜活检标本中诊断难度较大,应重视其病理特点,鉴别诊断需结合临床和免疫组化特征综合评价,当二者不能提供有价值的帮助时,确诊还需以HE切片形态学特征为主,同时在报告中加以注明。膀胱镜活检标本病理诊断尿路上皮癌核分裂象应是有或无。     

Renal angiomyoadenomatous tumor: morphologic, immunohistochemical, and molecular genetic study of a distinct entity

We present a series of a distinct tumorous entity named renal angiomyoadenomatous tumor (RAT). Five cases were retrieved from the consultation files of the authors. Histologic and immunohistochemical features were evaluated. Sequencing analysis of coding region of the VHL gene was carried out in all cases. The tumors were composed of admixture of an epithelial clear cell component and prominent leiomyomatous stroma. Epithelial cells formed adenomatous tubular formations endowed with blister-like apical snouts. All tubular/glandular structures were lined by a fine capillary network. The epithelial component was positive for epithelial membrane antigen, CK7, CK20, AE1-AE3, CAM5.2, and vimentin in all cases. In all analyzed samples, no mutation of the VHL gene was found. RAT is a distinct morphologic entity, being different morphologically, immunohistochemically, and genetically from all renal tumors including conventional clear cell carcinoma and mixed epithelial and stromal tumor of kidney.     

Primary clear cell meningioma of the orbit mimicking a metastatic carcinoma: usefulness of immunohistochemistry and cytogenetic analysis

Clear cell meningiomas (CCM) are rare tumors of the nervous system that usually occur in young patients and display high recurrence rates and potentially aggressive behavior. In this report, we describe a primary CCM of the orbit in an 84-year-old man with a previous history of a clear cell carcinoma of the kidney. Histologically, the tumor demonstrated a sheet-like proliferation of clear polygonal cells. Differential diagnosis includes metastasis of clear cell carcinomas. Immunohistochemistry, by showing that tumor cells expressed vimentin, epithelial membrane antigen, and progesterone antigens, and cytogenetic analysis, by identifying a monosomy 22, confirmed the diagnosis of CCM.     

Clear cell carcinoma of minor salivary glands

Two cases of carcinoma of the minor salivary glands are presented in which most cells had clear cytoplasm. Both patients had clinical histories in excess of 10 years and, in the one case with adequate follow-up, no recurrence had occurred after a further 11 years. Both tumours were locally invasive. The clear cells contained small amounts of glycogen, but no intracytoplasmic mucin. Immunohistochemical and ultrastructural studies showed epithelial features, with no evidence of myoepithelial differentiation. These tumours were very similar to the small number of previously reported cases, which were all considered to be low-grade carcinomas. Amongst the differential diagnoses, the most important is metastatic clear cell carcinoma of the kidney and this can only be confidently excluded clinically or by the use of imaging techniques. In summary, we consider intraoral clear cell carcinoma to be a distinct tumour of low malignant potential.     

Clear cell adenocarcinoma of the uterus--ultrastructural and hormonal study

A clear of clear cell adenocarcinoma of the uterus combined with early invasive squamous cell carcinoma of the cervix was reported. The adenocarcinoma was found to be in histologic continuity with the squamous cell carcinoma as well as with the cervical glandular epithelium. Ultrastructurally, the epithelial cells of the adenocarcinoma showed junctional complexes, well developed microvilli, parallel stacks of granular endoplasmic reticulums and twisted rope-like nucleoli, all of which were in accord with the cytologic characteristics of endometrial carcinoma. Hormonal study of the tumor suggested the presence of estrogen receptor and a lack of endocrine productibility. These findings provide an additional support for the Müllerian duct origin of uterine clear cell adenocarcinoma.     

Altered pattern of major histocompatibility complex expression in renal carcinoma: tumor-specific expression of the nonclassical human leukocyte antigen-G molecule is restricted to clear cell carcinoma while up-regulation of other major histocompatibility complex antigens is primarily distributed in all subtypes of renal carcinoma

Renal epithelial cancers represent a heterogeneous group of neoplasms arising from the malignant transformation of presumed diverse cell lineages. We recently demonstrated that tumor-specific up-regulation of human leukocyte antigen (HLA)-G, a nonclassical HLA class Ib molecule that might be involved in immune evasion by tumor cells, frequently occurs in conventional (clear cell) renal carcinoma. We here examined whether HLA-G activation is a common process affecting all types of renal epithelial tumors. We analyzed a series of 38 paraffin-embedded tumors including clear cell, papillary, chromophobe, collecting duct carcinoma, and oncocytoma. Seven of 12 (58%) clear cell tumors were positive by immunohistochemistry, whereas all of the other subtypes of renal carcinoma were negative for HLA-G expression. Developing or adult normal renal tissue were devoid of HLA-G expression. We also observed that ectopic expression of HLA class II antigens occurs more frequently in clear cell renal carcinoma than in other subtypes of renal tumors. Moreover, in contrast to the common observation of a down-regulation of major histocompatibility complex class Ia antigens reported in various tumors, the concomitant study of the same biopsies for classical HLA class Ia antigen expression revealed a general increase of HLA class Ia expression, regardless of histological subtypes. These results provide evidence for the heterogeneity of major histocompatibility complex expression patterns in renal carcinoma and support the hypothesis that specific mechanisms underlying the malignant transformation into clear cell renal carcinoma up-regulate expression of HLA-G and to a lesser extent HLA class II molecule expression. Considering the immunotolerant role of HLA-G toward the immune response, these mechanisms may thus provide renal cell carcinoma tumor cells with additional means to escape immune surveillance.     

肾脏上皮样色素性透明细胞肿瘤病理形态观察

目的 探讨上皮样色素性透明细胞肾脏肿瘤形态学特点,加强对该肿瘤的认识,减少误诊.方法 回顾性分析2000余例肾脏肿瘤,符合上皮样色素性透明细胞肿瘤3例,通过HE、免疫组织化学EnVision法观察3例肿瘤形态学特点,对其中1例行超微结构观察,同时复习相关文献.结果 女性2例,男性1例,2例形态学表现为透明细胞癌样,1例见透明细胞和嗜酸性细胞构成乳头结构.免疫组织化学上皮标记及S-100蛋白均为阴性,HMB45均为阳性,2例Melan A阳性.电镜末见黑色素小体结构.结论 色素性透明细胞上皮样肿瘤是非常罕见的肾脏肿瘤,病理学特征兼有肾细胞癌、上皮样血管平滑肌脂肪瘤与黑色素瘤等肿瘤特征,免疫组织化学有利于鉴别诊断.其可能是上皮样血管平滑肌脂肪瘤的一种亚型.     

Clear cell variant of fibrolamellar carcinoma of the liver

Fibrolamellar carcinoma of the liver is a distinctive variant of hepatocellular carcinoma characterized histologically by trabeculae of oncocytic cells with intervening lamellae of collagen fibers. We describe a case with a prominent component of clear cells, a feature not previously recognized in this tumor type. The patient was a 59-year-old woman incidentally found to have a solitary liver tumor, measuring 5 cm. Pathologic examination revealed a circumscribed, firm, tan tumor with peculiar concentric streaks. Oncocytic cells and clear cells were arranged in trabeculae separated by lamellae of collagen or sinusoids. The clear cells possessed abundant finely reticulated clear cytoplasm, which was highlighted by trichrome stain and immunostaining with antimitochondria antibody. Ultrastructurally, the cytoplasm of the clear cells was packed with empty membrane-bound vesicles that occasionally contained short cristae. The features suggested that the clear cell change resulted from ballooning and rarefactive changes of mitochondria. Clear cell fibrolamellar carcinoma should not be confused with conventional clear cell hepatocellular carcinoma, since the former is associated with a more favorable prognosis.     

Angiosarcoma of the kidney with minute clear cell carcinomas: A case report

A case of renal angiosarcoma with minute clear cell carcinomas in a 61-year-old male is described. The tumor was clinically considered a renal cell carcinoma. The lesion, removed by nephrectomy, was an unencapsulated hemorrhagic mass measuring 8.0 cm in diameter. The tumor was histologically characterized by anastomosing vascular channels lined by cytologically atypical endothelial cells. The tumor cells reacted positively with CD31, factor-VIII related antigen, and CD34. A few minute clear cell carcinomas measuring less than 1 mm were observed in the periphery of the angiosarcoma. There was no transition between angiosarcoma and renal cell carcinoma. The patient died of a widespread disease 13 months after surgery. This tumor is considered a primary renal angiosarcoma with clear cell carcinomas and not a sarcomatoid carcinoma.