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)

Increasing numbers of solitary fibrous tumors (SFTs) in the meninges have been reported since this entity was first recognized. While most cases previously reported were considered to be benign, the malignant potential of extrathoracic SFTs has not been excluded. The authors report a rare case of a meningeal SFT with malignant behavior occurring in a Japanese female patient, initially resected when she was 44 years old and recurring in the same place four times during a 26-year follow-up period. A metastatic tumor to the right lung arose 25 years after the resection of the first meningeal tumor and focal invasion into the cerebellum was also observed with her last (5th) meningeal tumor. Immunohistochemical analysis showed all tumors to be diffusely positive for CD34 and negative for EMA, with a so-called "patternless" histological pattern, featuring thin collagen fibers between tumor cells. A focal "staghorn" vascular pattern was also observed. Ki67 (MIB-1) labeling indices and mitosis rates were 3.1+/-1.2% and less than 1/10 high power fields (HPF) in the first meningeal tumor and 16.1+/-6.4% and 6/10HPF in the last (5th) one, respectively. Thus, the present case suggests that meningeal SFTs possess malignant potential so that careful long-term follow up is required.
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PMID:Malignant solitary fibrous tumor of the meninges. 1501 90

We describe two elderly patients with follicular lymphoma (FL) involving the skin and superficial soft tissues, with a striking proliferation of follicular dendritic cells (FDC). In addition, one patient had bone marrow involvement by FL. Histopathologically, the most remarkable feature in both cases seen at low magnification was a striking pallor of the constituent cells, which were arranged in fascicles, whorls, and round islands. The majority of the cells had the typical cytologic features of FDCs. They were intimately intermingled with centroblasts and centrocytes. A large amount of the clear cytoplasm and the pale nuclei of FDCs, which predominated in the tumors, caused the striking overall pallor of the lesions. Small reactive lymphocytes were scattered between the fascicles. A vague follicular growth pattern was seen only focally. The mantle zones were markedly reduced or absent so that the follicles were seen lying unseparated. The close intermixture of the FDCs and the germinal center cells was responsible for the FDCs appearing to be decorated with B-associated marker, and the germinal center cells seemed to be stained to some degree with FDC-markers. The tumor bulk demonstrated a diffuse and strong reaction with CD10, CD20, CD21, CD35, and stained weakly with CD79a. Fascin and CD23 showed only a weak and focal staining pattern. Bcl-2 decorated large centroblasts and small reactive T-cells. The tumor bulk was negative for actin, EMA, cytokeratins, vimentin, desmin, and factor XIIIa. The proliferative index was rather low; MIB-1 mainly decorated large centroblasts. No monoclonal rearrangement of IgH genes was detected. Epstein-Barr virus was not identified. Electron microscopy revealed typical features of FDCs intermingled with germinal center cells. Such cases may represent a diagnostic pitfall, as FDC overgrowth can mask FL and give the neoplasm the appearance of FDC sarcoma/tumor. We believe that, in both cases, the FDC proliferation had a reactive character.
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PMID:Follicular lymphoma of the skin and superficial soft tissues associated with a prominent follicular dendritic cell proliferation: an unusual pattern which may represent a diagnostic pitfall. 1546 4

June 2004: Over the past year, this man in late-60s had complained about progressive weakness of concentration and memory disturbances, associated with word finding difficulties. MRI examination revealed an extra-axial, parasagittal tumor 3 cm in diameter located in the left frontoparietal region. Five years ago, a meningioma in the same region, with radiographic appearance comparable to the present tumor had been totally removed. The histological picture of the current tumor was dominated by sheets of large rounded pleomorphic tumor cells with abundant eosinophilic cytoplasm and eccentric nuclei (rhabdoid cells). Cytoplasmic inclusions were frequent; occasionally,multinucleatedtumorcellswereseen. Mitoticfigures were absent and the MIB was 3%. Meningothelial lobules were scarce, and regions with fibroblastic appearance were absent. There were no psammoma bodies, necrosis or brain invasion. Moderate immunoreactivity for EMA was found. Additionally, strong cytoplasmic immunoreaction for vimentin within the rhabdoid cells was observed. Review of the previous material showed small islets of rhabdoid cells. Rhabdoid meningioma is an uncommon meningioma variant. It has been suggested that rhabdoid meningiomas are highly aggressive tumors (WHO grade III)and that the rhabdoid phenotype represents a marker of malignant transformation in meningiomas. Histologically, rhabdoid meningiomas usually exhibit signs of anaplasia, a high mitotic activity, and a markedly increased MIB-1 labeling index. Extracranial metastases may occur in the course of the disease. However, not all rhabdoid tumors appear to have anaplastic features (as this case illustrates). Another interesting feature of rhabdoid meningiomas is that in a significant number of cases, the rhabdoid cells appear only at the time of recurrence. Alternatively, as seen in this case, the rhabdoid cells may be already present in the primary meningioma, but not as the predominating histological feature.
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PMID:June 2004: a male in his late 60s with recurrent extracerebral tumor. 1560 95

Leiomyomatosis peritonealis disseminata (LPD) is a rare disease presenting as multiple peritoneal nodules of smooth muscle cells, mimicking peritoneal carcinomatosis. This disease usually pursues a benign course. We report one case of LPD in a 32-year-old woman, G2P1, without gynecological history. At term, she had an elective caesarean section during which several firm peritoneal nodules, ranging from 0.2 to 0.4 mm, were found. Microscopic examination showed a smooth-muscle cell proliferation with no mitosis, no atypia and no necrosis. Immunohistochemical analysis showed diffuse and strong staining for progesterone receptors and partial expression of estrogen receptors. The cells coexpressed smooth muscle actin, desmin, h-caldesmon, calretinin, WT1, and CD117 (KIT). They were weakly positive for EMA but negative for CD34. Proliferation index was low with 5% of cells being positive for MIB-1. This case of LPD proved to have an unusual immunohistochemistry profile raising the question of its real origin.
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PMID:[Leiomyomatosis peritonealis disseminata: immunohistochemical profile and origin]. 1712 52

Tanycytic ependymomas are a subtype of ependymomas that were formally recognized as a new pathological entity in the latest World Health Organization (WHO) classification of 2000. They occur mostly in the spinal cord. Only a few reports have analyzed the proliferative potentials of these tumors; however, it has been reported that the MIB-1 labeling index of tanycytic ependymoma is lower than that of other subtypes of WHO grade II ependymomas. We report a rare case of cervicomedullary junction tanycytic ependymoma associated with marked cyst formation. A 62-year-old man had a history of progressive gait disturbance, diplopia, and swallowing disturbance over a one-month period prior to admission. Magnetic resonance imaging (MRI) showed a cystic mass with a mural nodule at the cervicomedullary junction with Gd-DTPA enhancement. Cyst-subarachnoid shunt was performed using a far lateral approach. After 6 years, however, the man was readmitted to the hospital because of reaccumulation of the cyst. Partial removal of a mural nodule and a cyst-subarachnoid shunt were performed simultaneously by a midline suboccipital approach. The pathological diagnosis was tanycytic ependymoma. Postoperatively, the patient recovered well and was discharged from the hospital without further treatment. Most of the tumor cells had small, round nuclei; pleomorphism was minimal. The cytoplasm was dilated. The tumor cells were positive for EMA and s-100, and negative for CD-34. GFAP was not determined due to difficulty caused by background glial processes. The MIB-1 labeling index was less than 1%. Ultrastructurally, the tumor cells had ependymal cell features, such as desmosomes and microvilli. Based on these findings, the pathological diagnosis was tanycytic ependymoma.
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PMID:A case of cervicomedullary junction tanycytic ependymoma associated with marked cyst formation. 1809 1

We report a rare case of tanycytic ependymoma arising from the cerebral hemisphere. A 59-year-old man was admitted to our hospital because of the incidental detection by MRI of a tumor lesion in the right temporooccipital paratrigonal region. The mass showed low-to iso-intensity on T1-weighted images and high intensity on T2/proton-weighted images. Partial resection was performed using a transsulcal approach to avoid compromising the visual field. Most of the tumor cells showed elongated spindle shapes arranged in dense fascicles. A few true ependymal rosettes and perivascular pseudorosettes were visible. The tumor cells were positive for GFAP, S-100, and vimentin, but negative for synaptophysin, EMA, and keratin. The MIB-1 labeling index was approximately 1%. Ultrastructurally, the tumor cells had ependymal cell features, such as microvilli and cilia. From these findings, a pathological diagnosis of tanycytic ependymoma was made.
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PMID:A case of tanycytic ependymoma arising from the cerebral hemisphere. 1809 25

Acral myxoinflammatory fibroblastic sarcoma (AMIFS) is a low-grade sarcoma that presents mostly in distal extremities of middle-aged patients. The clinicopathologic features, immunohistochemical profile and follow-up data of five cases (three men and two women; age 39-65 years) are presented. The tumors presented as a slow-growing, poorly circumscribed, subcutaneous masses in the hands (three), foot (one) and calf (one), with dermal involvement in two cases. They had myxoid and hyaline stroma with dense acute and chronic inflammation. Spindle cells, large bizarre ganglion-like cells and multivacuolated cells were seen. Variable reactivity in lesional cells were noted for vimentin, Alpha-1-antitrypsin (A1AT), factor XIIIa, CD68, CD95, CD117, Alpha-1-antichymotrypsin (A1ACT), CD34, AE1/3, S-100 protein, EBER, CD63 and CD15. MIB-1 showed 5-30% nuclear labeling. They were negative for cytokeratin AE1/3, smooth muscle actin, CD30, ALK-1, EMA, desmin, CMV, HMB-45 and Melan-A. Follow up ranged from 2 weeks to 95 months (mean 54). One patient was lost to follow up; three underwent excision and one patient had below the knee amputation. Two patients developed metastases (one died of disease), and two patients are alive without evidence of disease. AMIFS are rare tumors that may involve joints and tendons leading to clinical diagnosis of ganglion cyst or tenosynovitis.
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PMID:Acral myxoinflammatory fibroblastic sarcomas: are they all low-grade neoplasms? 1819 Apr 43

A 13-month-old Korean female presented with Cushing disease and diabetes insipidus. On MRI, a 3.5-cm, focally cystic, contrast-enhancing, sellar and suprasellar mass was noted. Aside from blood adrenocorticotropin (ACTH) and cortisol elevation, other pituitary hormone blood levels were normal or markedly reduced. The subtotally resected lesion consisted of synaptophysin-immunoreactive lobules of (a) large, polygonal, amphophilic, PAS-positive cells immunoreactive for ACTH, beta-endorphin, alpha melanocyte stimulating hormone (MSH), and keratin (CAM5.2) in some cells showing Crooke hyaline change, (b) less frequent acidophilic, growth hormone (GH) immunoreactive cells, and (c) rare luteinizing hormone (LH) and/or alpha subunit immunopositive cells. Also conspicuous were smaller cells resembling Rathke-type epithelium forming rosettes to sizable glands immunoreactive for EMA, keratin, S-100 protein, galectin-3 and rarely for synaptophysin and/or one of the above-noted adenohypophysial hormones. Transcription factors, including Neuro-D1 and Pit-1, were present in ACTH- and GH-producing cells, respectively, but only in occasional Rathke-type cells. The MIB-1 labeling index (LI) was 1.5% in secretory cells and 39% in Rathke-type epithelium. Ultrastructurally, the tissue resembled fetal pituitary of 10-12 weeks gestation and contained fully differentiated corticotrophs and somatotrophs, scant cells of glycoprotein-hormone producing type with small secretory granules, and glandular epithelial cells consistent with committed, but largely undifferentiated Rathke-type epithelium. We consider the tumor as a pituitary blastoma, a lesion composed of multiple cell types common to the development of the affected organ based upon (a) prominence of primitive Rathke-type epithelium, (b) disposition of secretory cells in lobules rather than acini, (c) the limited range of secretory cells represented, (d) the presence of their corresponding transcription factors, and (e) ultrastructural features indicating orderly development of the 10- to 12-week embryonic stage.
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PMID:Pituitary blastoma. 1855 Dec 99

A 71-year-old man presented with a slowly growing 2.0x2.0x1.0 cm scalp lesion that was surgically removed. Microscopic examination showed a well-circumscribed dermally located tumor composed of ductal elements lined by double to multiple cell layers of bland cuboidal inner cells and elongated spindled outer cells with areas showing cribriform and solid growth patterns. Some cells showed prominent cytoplasmic clearing. A few mitotic figures are noted ranging from 1-2 mitotic figure/10 hpf. There are also foci of squamous differentiation as well as occasional mature adipocytes. The background stroma was predominantly sclerotic with only small area of myxoid background (confirmed by Hale's colloidal iron). Immunohistochemical studies revealed positive immunoreactivity for EMA, CEA, CD117, HWMK, LWMK, CK7, Androgen receptor and S100 in the ductal (epithelial) cells and positive immunereactivity for calponin, SMA, CK 5/6 and p63 in the myoepithelial component. No immunoreactivity for Brst-2, ER, PR and CK20 was noted. MIB-1 showed mildly increased proliferrative index highlighting 5% of the nuclei. The overall morphology and immunohistochemical profile are that of a benign cutanoues mixed tumor (chondroid syringoma). Given the unusual striking celluarlity, we suggest to subclassify this as a hyper-cellular variant.
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PMID:Benign mixed tumor of the skin, hypercellular variant: a case report. 1961 93

We present a clinicopathologic and immunohistochemical study of 21 Chinese patients with oncocytoma of salivary gland origin, a rare benign tumour composed exclusively of large epithelial cells with eosinophilic granular cytoplasm (oncocytes). The median age was 60.1 years with a male predominance (67%). All the tumours occurred in the parotid except one in the palate. A painless mass was the most common feature, although intermittent pain was complained of in four cases. All the patients were treated by superficial parotidectomy, with no recurrence or metastasis. Histologically, most tumours displayed an encapsulated nodular growth pattern, but one case presented with an aggressive growing tendency. Typical oncocytes were observed in all cases, with one clear cell variant found. The oncocytes were arranged in solid sheets, trabecular or duct-like structures. Rarely, small foci of hemorrhage or lymphoid stroma were observed, but germinal centres were always absent. Phosphotungstic acid hematoxylin staining illustrated dark-blue cytoplasmic granules, demonstrated as mitochondria by electron microscopy. All the tumours showed immunoreactivity for CK5/6, CK8/18, CK10/13, CK19 and EMA, but were negative for SMA or S-100. MIB-1 antibody, used to identify the dividing cells by staining of the nucleus, was found to stain the cytoplasm of the oncocytes. In summary, clinical diagnosis for an oncocytoma is challenging for its similar features to other benign tumours. Histopathological diagnosis is reliable with histochemical and electron microscopic conformation of the oncocytes, but differential diagnosis is still challenging. MIB-1 immunostaining might be considered as a diagnostic aid.
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PMID:Oncocytoma of the salivary glands: a clinicopathologic and immunohistochemical study. 1979 83


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