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Query: UMLS:C0268596 (EMA)
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The atypical teratoid / rhabdoid tumour is a rare type of tumours of central nervous system appearing usually under 2 years of age, bearing a rather bad prognosis and it may cause serious differential diagnostic problem. The tumour is characterized histologically by the presence of the rhabdoid cells, immunohistochemically by vimentin, SMA, EMA positivity, the frequent presence of cytokeratin, GFAP positivity, but germ cell markers: AFP, hCG negativity, cytogenetically by aberrations of chromosome 22. The case of a one and half month old female infant is presented, who died 8 months after the appearance of the first symptoms. The diagnostic possibilities and the unsolved problem of the therapy are discussed.
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PMID:[A case of central nervous system atypical teratoid / rhabdoid tumor] 1205 Jul 40

Numerous studies of keratin expression by the more common odontogenic cysts were done to determine whether patterns of cytokeratin staining could provide accurate diagnostic markers for the different varieties; to see whether comparative studies with oral mucosa and developing odontogenic epithelium could explain the pathogenesis of the cysts; and whether cytokeratin patterns could provide clues in elucidating the aggressive nature of the OKC. This review was a complex task with a range of at least 19 different cytokeratins being studied and also a broad range of antibodies in use for the same cytokeratin or group of cytokeratins. Moreover, there was not always standardisation of laboratory techniques in the selection and preparation of material. These difficulties were, in general, recognised by the different workers in the field, particularly when there was disagreement on results and caution was expressed about drawing conclusions from some positive findings. It would be fair to conclude that cytokeratin immunocytochemistry has not advanced to any meaningful extent, its use as a diagnostic marker for the OKC nor in eludidating its pathogenesis. With regard to OKC behaviour, it has been pointed out that there was strong reaction of OKC lining for keratin 16, a cytokeratin that has been associated with high proliferative activity. Yet other studies have also shown keratin 16 expression in dentigerous and radicular cysts. Differences in cytokeratin, EMA and CEA immunocytochemical reactivity between the parakeratinised and orthokeratinised varieties of cyst were demonstrated and the suggestion made that the orthokeratinised type has a considerably less aggressive behaviour, is a different entity and should bear a different name. Furthermore, Ki67 positive cells in the parakeratinised OKC linings were considerably more frequent than in the orthokeratinised linings.OKC, dentigerous and radicular cyst epithelium reacted positively for epithelial growth factor receptor (EGFr) but a trend indicating the most intense staining in the OKCs, followed by the dentigerous and then the radicular cyst linings led to the conclusion that the OKCs have an intrinsic growth potential not present in other odontogenic cysts.
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PMID:The aggressive nature of the odontogenic keratocyst: is it a benign cystic neoplasm? Part 3. Immunocytochemistry of cytokeratin and other epithelial cell markers. 1211 Mar 33

Melanoma may present a great variety of cell morphology and mimic virtually almost all other neoplasms, and metastatic lesions pose major diagnostic difficulties. A typical immunoreactive profile of melanoma is: S-100p- and HMB-45-positivity, but these markers are not always present. Other new antigens for melanoma are NKIC3 and Anti-Melan-A (MART-1). This tumor may also present expression of unspecific markers, as cytokeratin, CEA, EMA, desmin, NFP, GFAP, CD68 and actins. We present here unusual features of two metastatic melanomas, showing the anaplastic pattern, immunopositive for desmin.
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PMID:Rhabdomyosarcomatous (myoblastic?) phenotype of metastatic malignant melanoma. A case report. 1214 Aug 74

Polymorphous low-grade adenocarcinoma (PLGA) is a distinctive salivary gland neoplasm with a predilection for intraoral sites. Histological and immunohistochemical analyses were used to study a case involving the minor salivary glands of the buccal mucosa in a 68-year-old woman. Histologically, the tumor was characterized by unenca-psulated, progressing and focally-infiltrative borders and showed variable growth patterns including solid, tubular, microcystic and cribriform; papillary and papillary-cystic areas of more than focal extent were present. Cytologically the neoplasm was composed of uniform, round to cuboidal cells with bland, round to oval nuclei. Immunohistochemical analysis showed that the tumor cells were positive for cytokeratin, S-100 protein, vimentin, focally-positive for EMA and actin and negative for CEA. The significance of the papillary component and the possible relationship to other salivary gland tumors is discussed.
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PMID:Polymorphous low-grade adenocarcinoma of the salivary glands: clinicopathological and immunohistochemical study of a case. 1216 49

The aim of this study is to report the experience of a case of benign multicystic mesothelioma of the peritoneum presented with acute appendicitis symptomatology. A 28 years old man with right lower and upper abdominal pain was admitted into hospital. Because of the clinical picture, the symptomatology and the leukocytosis a diagnosis of acute appendicitis was made and the patient underwent appendicectomy according to Mc Burney. At laparotomy some cc of purulent fluid were sucked and a cystic mass that contained clear fluid was revealed. In consequence of the incidental diagnosis a following middle laparotomy was made with a careful surgical excision of the mass and of the appendix. Macroscopically the lesion was identified like a neoplastic mass 25 centimeters in diameter, with a multicystic and fibrous-adipose aspect, with cysts 5 centimeters in diameter. The cystic spaces were lined by a layer of eptelial cells which presented positive reaction for cytokeratin and EMA, whereas endothelium markers were absent. The ultrastructural, morphological and immunohistochemical findings were diagnostic of a benign multicystic mesothelioma.
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PMID:Benign multicystic mesothelioma of the peritoneum: a case report. 1223 71

A case of mesothelioma with a small cell component in a 53-year-old, non-smoker woman. The patient had a history of asbestos exposure, and presented with thoracic pain. A total body computed tomogram showed a left pleural effusion and a 7.5-cm pleural mass. Thoracoscopy revealed a diffuse nodular thickening of the left parietal pleura, and a biopsy was performed. The patient died of the disease 4 months after diagnosis. Microscopically, the pleural neoplasm was composed of three different components: 40% of the tumor showed the classic histology of a malignant epithelial mesothelioma, 40% was composed of small- to medium-sized cells with open nuclear chromatin, evident nucleoli and high mitotic activity, and 20% of the neoplasm was indistinguishable from a small cell carcinoma. Immunohistochemically, the first component was diffusely and strongly positive for cytokeratin AE1/AE3, cytokeratin CAM 5.2 and EMA, focally positive for BER-EP4, and negative for CD15, B 72.3, CEA, LCA, chromogranin, synaptophysin, TTF-1 and CD99. The cells of the second component were positive only for cytokeratin AE1/AE3 and cytokeratin CAM 5.2, and the elements of the third component were negative for all the antibodies tested. Pleural mesothelioma with a small cell component is rare. The most useful parameters to distinguish it from other small cell malignancies that may involve the pleura, particularly small cell carcinoma of pulmonary origin, are discussed.
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PMID:[Small-cell mesothelioma of the pleura: description of a case]. 1241 72

A rare case of papillary pneumocytoma of the lung is reported. The immunohistochemical positivities for EMA, cytokeratin and TTF-l strongly support the hypothesis that the neoplastic cells are originated from type 2 pneumocytes. The tumour also presented areas displaying stroma of non-specific mixoid appearance, simulating a benign tumour of the salivary gland type. We propose that these mixoid areas constitute structures of metaplastic origin.
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PMID:Papillary pneumocytoma of the lung simulating a pleomorphic adenoma. 1249 80

We report a case of a 65-year-old man with rare prostatic stromal sarcoma in which suprapubital radical prostatectomy was performed, but neither chemotherapy nor radiation therapy were administered before or after the operation. The well-circumscribed tumor, measuring 5 cm in diameter, showed a homogeneous white grayish cut surface with a hard consistency. Histopathologically, the tumor consisted mainly of medium-sized rounded cells with a sarcomatous and epithelioid appearance intermingled with collagen fiber. Hyalinized foci were also noted in the tumor. Immunohistochemistry revealed that the tumor cells were diffusely positive for vimentin and focally positive for progesterone receptor and CD34, but not for EMA, cytokeratin or estrogen receptor. No recurrence or distant metastasis of the tumor has occurred in 8 years of follow up. The tumor was diagnosed as prostatic stromal sarcoma (PSS) showing epithelioid differentiation and of a progesterone-dependent nature. Possible favorable nature of the PSS might be expected after complete resection.
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PMID:Prostatic stromal sarcoma: case report and review of the literature. 1278 17

Chondrosarcomas are malignant cartilage-forming tumors that represent the second most common malignant solid tumor of bone. These biologically poorly understood neoplasms vary considerably in clinical presentation and biologic behavior. Chemotherapy and radiation therapy are generally ineffective. Here we describe the establishment and characterization of a new human chondrosarcoma cell line named ch-2879, and we compare the cell line with its tumor of origin. The cell line was established from a recurrent grade 3 chondrosarcoma of the chest wall and characterized by growth kinetics and morphologic studies. Immunocytochemistry and RT-PCR were performed to examine the expression of cartilage-specific phenotypes. Genetic characterization was performed using cytogenetics, fluorescence in situ hybridization, flow cytometry, and molecular techniques for analysis of the genes implicated in cell cycle control, amplification of MDM2, CDK4, and Cyclin D1, and mutations in the p53 gene. ch-2879 cells were subcultured for more than 80 passages. They expressed vimentin, HNK-1, HBA-71, Ki-67, cyclin D1, Fli-1, S-100, p21, p27, and p53 and were negative for cytokeratin, EMA, p14, p16, MDM2, Rb, and c-erb-b2 antigens. Cytogenetically the recurrent tumor showed a hyperhaploid karyotype with clonal numerical and structural abnormalities. The sole structural abnormality was a chromosome derivative of a t(1;21) translocation. The cell line at passage 3 showed two populations: the hyperhaploid and an exactly duplicated, hypotriploid population. After the 18th passage, only the hypotriploid population was present. The cells expressed collagen 2. Molecular comparison of the primary and recurrent tumor evidenced an in vivo molecular change consisting of a deletion of 9p21 genes in the recurrence, probably caused by a selection process. Because of its gene expression profile, including expression of genes implicated in chondrogenesis in uncoated plastic dishes, this cell line may prove useful for cellular and molecular studies as well as studies of chondrosarcoma characterization and treatment.
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PMID:Establishment and characterization of a continuous human chondrosarcoma cell line, ch-2879: comparative histologic and genetic studies with its tumor of origin. 1280 23

Uterine tumors resembling ovarian sex-cord tumors (UTROSCTs) are unusual neoplasms with histologic features that resemble those within ovarian Sertoli and granulosa cell tumors. We report the case of a 24-year-old woman with a UTROSCT presenting as a cervical mass, which on initial evaluation was thought to represent cervical adenocarcinoma. The patient's cervical biopsy specimen contained epithelioid cells arranged in tubules and anastomosing cords, without significant cellular atypia or mitotic activity. Because this morphology elicited a broad differential diagnosis, immunohistochemical studies were performed. The tumor was found to be diffusely positive for cytokeratin cocktail, calretinin, and desmin, focally positive for CK7 and SMA, and negative for EMA, CEA, inhibin, CD10, CK20, chromogranin, and synaptophysin. Ultrastructural examination revealed occasional gland-like lumens with cells joined by desmosomes and a continuous basal lamina. UTROSCTs have features that may cause them to be confused with more common tumors, especially in limited biopsy samples, and should be included in the differential diagnosis when a gland-forming neoplasm with an unusual appearance is identified in a cervical or endometrial biopsy specimen.
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PMID:Uterine tumor resembling ovarian sex-cord tumor: report of a case mimicking cervical adenocarcinoma. 1281


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