Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0268596 (EMA)
 2,520 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Chondroid syringoma belongs to the group of so-called mixed tumors, like pleomorphic adenomas of the lacrimal and salivary glands. The histogenesis of this tumour is still disputed, in particular with respect to its stromal component. The distribution of cytokeratins (CKs), CEA, EMA, vimentin, S-100 protein, desmin and actin [alpha-smooth muscle actin (alpha-SMA)] was investigated by immunohistological examination of paraffin sections from a chondroid syringoma of the apocrine type. The neoplastic formations have been classified into tubuloalveolar structures, solid nests/aggregations and stromal cells of varying morphology. The inner-most cells of tubuloalveolar structures were characterized by marked expression of CKs (KL1 and MNF116), CEA and EMA, while in the outer ones there was moderate expression of vimentin, S-100 was expressed to a lesser extent and KL1, weakly but there was marked and consistent expression of MNF116. Whereas the solid nests expressed vimentin, S-100 protein, MNF116 markedly and KL1 weakly, the stromal cells were consistently positive for vimentin, S-100 protein and, focally, CKs and alpha-SMA. Anti-alpha-SMA specifically detects myoepithelial cells. In addition, the partly overlapping immunoreactivity of the intermediate filaments, membrane proteins and proteins in the different structures may indicate a common clonal origin of all neoplastic cells in chondroid syringoma.
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PMID:[Chondroid syringoma. Immunohistologic indications of myoepithelial differentiation]. 805 Sep 3

Myofibroblastic differentiation occurs in 10-20% of all dermatofibromas, affecting < 25% of cells. We report on a series of 36 dermatofibromas collected from > 1,500 fibrohistiocytic lesions (2%), with more prominent (> 25%) myofibroblastic differentiation characterized by markedly slender and elongated spindle cells positive for smooth muscle markers. While most of the lesions did not otherwise differ from ordinary dermatofibromas, three cases (0.2%) from the neck-shoulder region of male adults showed extensive myofibroblastic features (> 90%). Clinically, these three lesions measured approximately 1 cm and had a firm consistency, with the differential diagnosis of some fibrohistiocytic tissue response. Histologically, densely packed cells and prominent, partially nodular, stromal sclerosis with focal palisading of nuclei indicate some overlap with other rare variants of fibrohistiocytic tissue response, such as cellular benign and palisading cutaneous fibrous histiocytoma. Yet, these features together with focal whorled nesting of more epithelioid cells (one case) also caused considerable diagnostic problems to exclude other myofibroblastic as well as (malignant) spindle cell lesions such as (palisaded) myofibroblastoma, dermatofibrosarcoma protuberans, and neurothekeoma. Immunohistochemically, all lesions were markedly (> 90%) labeled for smooth muscle markers (HHF35, anti-SMA) and with NK1C3 (CD57), while a broad panel for other spindle cell tumors, such as pan-keratin, S100 protein, EMA, desmin, CD34, CD31, and KiM1p, were negative. Electron microscopy of two cases revealed prominent endoplasmic reticulum and Golgi complex, numerous intermediate filaments, attachment plaques, and focal basal lamina formation. No recurrence was seen during a follow-up of 9 (two cases) and two years, respectively.
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PMID:Prominent myofibroblastic differentiation. A pitfall in the diagnosis of dermatofibroma. 912 98

We report on eight cases of a distinct variant of cutaneous schwannoma characterized by prominent Verocay body formation (75-100% of the tumor bulk) that may cause considerable diagnostic difficulties. Like ordinary cutaneous schwannomas, these lesions preferred the head and neck region of young adults without sexual predilection and were clinically interpreted as cyst, basal cell carcinoma, or nevus. Histological examination revealed well-circumscribed nodules. Three of them consisted exclusively of nodular or ribbon-like Verocay bodies. A variable admixture of Antoni A or B type of differentiation (< 25%) was seen in five other cases. The following patterns were seen: fascicular spindle-shaped, onion-like epithelioid, myxoid-hypocellular, and degenerated ("ancient") with prominent fibrosis/hyalinosis and occasional bizarre giant cells. Immunohistochemically, the lesions were positive for S-100 protein (and vimentin) but negative for a broad panel of neurogenic and intermediate filament markers. The capsule showed focal labeling for EMA and--when it was markedly thickened--also for SMA. Labeling with E9, an anti-metallothionein marker indicative of cell activity, was negative, underscoring the slow growth potential of these lesions. No recurrence was seen in the six patients with follow-up information. The differential diagnosis includes other lesions with prominent palisading. (Amianthoid) myofibroblastoma and palisading leiomyoma are consistently positive for SMA and desmin, respectively. Palisading cutaneous fibrous histiocytoma and myofibroblastic dermatofibroma are variably positive for Factor XIIIa, SMA, and E9 and/or NK1C3 (CD57). Palisaded encapsulated neuromas are primarilly differentiated by the presence of nerve fibers with myelin sheaths.
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PMID:Verocay body--prominent cutaneous schwannoma. 1002 39

A 6-month-old girl was admitted to another hospital because of consciousness disturbance, preceded by 2 weeks of decreased activity and vomiting. She was referred to our hospital after ventricular drainage had been instituted for hydrocephalus and the tumor in the pineal region. The patient was noted to have conjugate upward gaze palsy and papilledema. CT scan and MRI revealed a large tumor in the pineal region with tumoral hemorrhage and a small mass in the right frontal lobe. At surgery, the pineal region tumor was removed subtotally. Histological examination showed the tumor to be composed of sheets of large polyhedra or round cells with an eccentric round nuclei, prominent nucleoli, and cytoplasmic inclusions. Immunohistochemical studies were positive for GFAP, vimentin, S-100, CK, EMA, and SMA, but negative for AFP, HCG, PLAP, and CEA. Following surgery, she received three 5-day cycles of chemotherapy, consisting of intravenous administration of cisplatin 20 mg/m2/day and etoposide 60mg/m2/day. After these therapies, MRI showed a decrease in the area of high intensity in the pineal region, but almost no change in the right frontal mass lesion. Follow-up radiological examination showed that the tumor had grown rapidly one month after chemotherapy and the patient died 5 months after her first hospitalization. Malignant rhabdoid tumor of the CNS is rare and remarkably malignant. This tumor should be treated using multidisciplinary management with surgery, intensive chemotherapy, and radiotherapy depending on the patient's age.
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PMID:[A case of malignant rhabdoid tumor in the pineal region in early infancy]. 930 Apr 49

Rhabdoid tumor is a well-accepted clincopathologic entity among childhood renal neoplasms; similar tumors have been described in extrarenal locations. We present the clinicopathologic profile and the immunohistochemical features of a series of soft tissue rhabdoid tumors. Twenty-eight cases coded as extrarenal rhabdoid tumor (ERRT), RT, possible ERRT, and "large cell sarcoma" were retrieved from the Armed Forces Institute of Pathology soft tissue registry. The tumors were reclassified according to strict criteria by light microscopy, clinical information, immunohistochemistry, and, in some cases, electron microscopy. Soft tissue rhabdoid tumor (STRT) was defined as (1) a tumor composed of noncohesive single cells, clusters, or sheets of large tumor cells with abundant glassy eosinophilic cytoplasm, an eccentric vesicular nucleus, and an extremely large nucleolus; (2) positivity for vimentin and/or cytokeratin or other epithelial markers by immunostaining; and (3) exclusion of other tumor types with rhabdoid inclusions (melanoma, other sarcomas, carcinoma). Eighteen cases met our criteria for soft tissue rhabdoid tumors. The median patient age was 13 years (range, 6 months to 56 years). Ninety-four percent of STRT cases were positive for vimentin and 59% for pan-cytokeratin. Sixty-three percent and 60% were positive for CAM 5.2 and EMA, respectively. Seventy-nine percent stained for at least one epithelial marker; 76% stained for both vimentin and epithelial markers simultaneously. Forty-two percent stained for MSA, and 14% for CEA and SMA. CD99, synaptophysin, CD57 (Leu-7), NSE, and focal S100 protein were identified in 75%, 66%, 56%, 54%, and 31% of the STRT cases, respectively. All STRT cases examined were negative for HMB-45, chromogranin, BER-EP4, desmin, myoglobin, CD34, and GFAP. Follow-up examination in 61% of the STRT patients revealed that 64% of patients died of disease within a median follow-up interval of 19 months (range, 4 months to 5 years); 82% had metastases to lung, lymph nodes, or liver; 22% had local recurrences before metastasis; and 18% were alive without known disease status (median, 5.5 years). Soft tissue rhabdoid tumor is a highly aggressive sarcoma, predominantly of childhood. Besides having nearly consistent coexpression of vimentin and epithelial markers, STRTs show positivity for multiple neural/neuroectodermal markers that overlap with those of primitive neuroectodermal tumor.
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PMID:Extrarenal rhabdoid tumors of soft tissue: a clinicopathologic and immunohistochemical study of 18 cases. 993 May 72

Malignant rhabdoid tumor (MRT), described for the first time in 1978 in the kidney, has rarely been reported in other organs including the brain and has involved adults in only 3 cases. We described a case of MRT in a 32-year-old woman who presented with severe headache, nausea and sudden onset of visual disturbance. MRI showed a well-enhanced mass at the suprasellar region. Subtotal removal of the tumor was performed. However, tumor regrowth occurred after the operation (doubling time, 8.36 days) and spinal dissemination was detected. Therefore, chemotherapy and radiotherapy were administered focusing on the suprasellar lesion and the spinal cord. Pathologically, light micrographs showed rhabdoid cells with large, round, single or double nuclei with one prominent nucleolus and eosinophilic cytoplasmic inclusions. Electron micrographs were made of typical rhabdoid cells displaying bundles of intermediate filaments within the perikaryon. In immunohistochemical studies, EMA, vimentin, cytokeratin and SMA were positive. Pathological findings were consistent with those of MRT. Optimal treatment for this tumor has not been established. Our case may be useful in defining treatment for MRT.
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PMID:[Suprasellar primary malignant rhabdoid tumor in an adult: a case report]. 1076 34

A 74-year-old woman complained of a small nodule in the outer lower quadrant of her left breast. On physical examination, a 0.9 x 0.8 cm, round-shaped and firmly elastic nodule was palpated. Excisional biopsy was performed. Histologically, the tumor was separated into, with a bicellular pattern, containing both numerous glandular structures and numerous spindle-shaped cells. Immunohistochemical staining for EMA and cytokeratin showed strongly positive immunoreactivity for epithelial cells. Staining for &aipha;-SMA showed strongly positive immunoreactivity for myoepithelial components. Staining for keratin and S-100 protein showed weakly positive immunoreactivity for myoepithelial cells. Microscopically, the tumor was diagnosed as adenomyoepithelioma of the breast. Immunohistochemical examination is needed to distinguish epithelial cell proliferation from myoepithelial cell proliferation. Immunohistochemical examination using antibodies against EMA, alpha-SMA, Keratin, cytokeratin and S-100 protein, is indispensable.
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PMID:Adenomyoepithelioma of the Breast: A Case Reprot and a Review of Literature. 1109 56

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

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

Allergic sensitisation of the airways occurs in the mucosa of the shock organ, or in the lymphatic stations draining these structures. The lymphatic structure closest to the nasal mucosa in humans is the adenoid and tonsils. Research done in respect to Waldeyer's ring on people dealing at the same time with allergy, is seldom the subject of dissertation in polish as well as world wide medical literature. In the enclosed dissertation, writers present the results of histopathological and immunohistochemical analysis of hyperplastic tonsils and adenoid, taken from children with coexisting atopy. The authors describe an immunohistochemical analyses of the hyperplastic human palatine tonsil. They present the expression of antigen CD3, CD20, CD45, CD68, EMA, SMA, Vimentin, Desmin, S-100, von Willebrand Factor, p35, Bcl-2, melanin, cytokeratin, estrogen receptor, progesteron receptor, kolagen IV and NSE in every region of pallatine tonsil. It is worthwhile to remark, that many of these cases, the presence of eosinophilia and subepithelial oedema was observed. Our immunohistochemical findings support the hypothesis that allergic sensitization takes place in the adenoid and tonsils. Furthermore, this study confirms that dendritic cells ad macrophages are involved and important in allergic disease.
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PMID:[Histopathological and immunological analysis of hyperplastic palatine tonsils and adenoids in children with coexisting atopic dermatitis]. 1459 70


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