1/184. Middle ear adenocarcinoma with intracranial extension. Case report.Middle ear adenocarcinoma is a very rare, locally invasive neoplasm assumed to arise from the middle ear mucosa. Although endolymphatic sac tumor (aggressive papillary middle ear tumor) and jugulotympanic paraganglioma may show brain invasion, intracranial extension of histologically confirmed middle ear adenocarcinoma has not been previously reported. The authors describe a 53-year-old man who suffered from otalgia and tinnitus for more than 10 years and from neurological deficits for 1 year due to a large temporal bone tumor that invaded the temporal lobe. A combined neurosurgical and otolaryngological resection was performed. Pathological analysis revealed a low-grade adenocarcinoma of a mixed epithelial-neuroendocrine phenotype, which showed a close histological similarity to, and topographical relationship with, middle ear epithelium. The authors conclude that middle ear adenocarcinoma belongs to the spectrum of extracranial tumors that have possible local extension to the brain.- - - - - - - - - - ranking = 1keywords = mucosa (Clic here for more details about this article) |
2/184. Primary intracerebral Hodgkin's disease: report of a case with Epstein-Barr virus association and review of the literature.A case of primary intracerebral Hodgkin's disease (HD) without dural attachment in a 54-year-old immunocompetent patient is described. The infiltrate was located superficially in the occipital lobe and corresponded to the histologic type of nodular sclerosis. A typical immunohistochemical profile (membrane and cytoplasmic staining with dotlike Golgi enhancement of CD30, moderate cytoplasmic staining of CD15 in the Golgi area, membrane staining of CD20 of <10% of blastic cells, CD45RB negative) and in addition Epstein-Barr virus (EBV) latent membrane protein was detectable in reed-sternberg cells. Staging revealed no other organ sites of involvement. After combined surgery, postoperative radiotherapy, and chemotherapy, there are no signs of recurrence or systemic disease on follow-up for >1 year. To the authors' best knowledge, an association of EBV with primary central nervous system HD has not been demonstrated before.- - - - - - - - - - ranking = 13.796581727127keywords = membrane (Clic here for more details about this article) |
3/184. Establishment of two glioma cell lines from two surgical specimens obtained at different times from the same individual.We established two glioma cell lines from two surgical specimens obtained at different times from the same patient. One (No. 9R), which was derived from the recurrent tumor (glioblastoma, grade IV), proliferated more rapidly in vitro than the other (No. 9) from the primary tumor (slightly anaplastic astrocytoma, grade II-III). No. 9R showed heterotransplantability in nude mice, whereas No. 9 did not. These findings indicate that No. 9R has a more aggressive or malignant nature than No. 9. Both cell lines showed homozygous deletion of the representative tumor suppressor p16 and p15 genes, but no p53 gene alteration. However, examination of the overall mRNA expression profile using a commercially available cDNA-spotted membrane revealed much higher expression levels of several mRNAs, at least, in No. 9R than in No. 9, although the relationship between these mRNAs and the growth potentials remained unknown. These two cell lines, derived from the same individual, with different proliferating potentials may be useful for studies on the molecular bases of glioma malignancy and progression.- - - - - - - - - - ranking = 4.5988605757091keywords = membrane (Clic here for more details about this article) |
4/184. Unique desmoplastic cerebral tumor in a patient with complex partial seizures.Primary brain tumors with prominent desmoplasia include the gliofibroma, desmoplastic infantile ganglioglioma (DIG), pleomorphic xanthoastrocytoma (PXA), and desmoplastic cerebral astrocytoma. In the present report, we describe unusual pathological appearances in two successive resections of a left temporal tumor in a patient with complex partial seizures. Both tumors showed focal astroglial and very prominent neuronal differentiation. In the first resection at age 11 years, the tumor showed only focal desmoplastic areas and prominent neuronal differentiation with bizarre atypical giant cells. In the second resection at age 14 years, the morphology was dramatically different. Now the tumor showed marked desmoplasia with tumor cells coexpressing neuronal and glial markers. Electron microscopy showed prominent neuronal differentiation in both resections and presence of basal lamina around tumor cells. Our case represents a unique example of an extraordinary degree of neuronal differentiation in a desmoplastic cerebral tumor. While cellular pleomorphism in a desmoplastic tumor traditionally suggests the diagnosis of PXA, we wish to underscore that many cells exhibiting marked cytologic atypia may in fact be of neuronal rather than of glial origin as in our case.- - - - - - - - - - ranking = 12.100611153195keywords = lamina (Clic here for more details about this article) |
5/184. Dysembryoplastic neuroepithelial tumor (DNT): an ultrastructural study of six cases.We report six cases od DNT with a detailed ultrastructural characteristics. The patient age ranged from 7 to 16 years (mean 12), the location was temporal in three cases and frontal, temporooccipital and parietooccipital in each of one remaining cases. The predominant clinical feature in each case was history of episodes of intractable seizures. Histopathologically, the neoplasms were multinodular, each nodule was well-circumscribed and was composed of glioneuronal elements embedded in the variable amount of myxoid matrix. The oligodendroglial-like cells (OLC) predominated in the nodules with some accompanying mature neurons. The nodules were frequently surrounded by small calcifications which could be found also within the tumors. OLCs were immunoreactive for S-100 protein and neurons had the expression of synaptophysin and neurofilament proteins. Ultrastructurally, each tumor consisted of three major elements: neoplastic cells (OLC), elongated processes forming neuropil-like structure and expanded "mucoid" extracellular space: the latter gave an impression of cellular elements floating within it. Neoplastic cells had round, oval or elongated nuclei, no discernible nucleoli and a relatively narrow rim of the cytoplasm. Some nuclei were irregular and invaginated and pseudoinclusions were observed; a part of cytoplasm sequestered within pseudoinclusions often appeared degenerated with large blabs and electron-lucent vesicles, some of these contained in turn semicircular profiles of unknown significance. The second element consisted of innumerable cellular processes. Some of these were elongated and formed stacks connected by symmetrical symmetric or asymmetric adhesive plaque junctions. The others had shorter "neck" containing microtubules, these extended into bullous extensions. Dense-cored vesicles were occasionally observed, in both cytoplasm of neoplastic cells and within processes. In one cell, cross-sectioned annulate lamellae were found. In cytoplasm of a few cells, unusual inclusions reminiscent ribosome-lamellae complexes were observed. These were cylindrical resembling "laboratory tubes" with a cone-like endings. At higher power, walls of the "tubes" resolved into layered structures composed of several laminae; between laminae, ribosome-like structures were visible.- - - - - - - - - - ranking = 24.20122230639keywords = lamina (Clic here for more details about this article) |
6/184. ganglioglioma with a tanycytic ependymoma as the glial component.We studied a cystic ganglioglioma (GG) located in the right frontal lobe of the brain. Interestingly, the fibrillary spindle glial cells were often arranged in a fascicular pattern, and the generally uniform, round-to-oval delicate nuclei appeared to resemble those of ependymoma; and the neoplastic neurons often contained neurofibrillary tangles (NFTs). The glial component was positive for glial fibrillary acidic protein and occasionally contained granular or microvesicular structures positive for epithelial membrane antigen. Ultrastructural investigation revealed that the glial cells were ependymal in nature; intracytoplasmic lumina and intercellular microrosettes lined with cilia and microvilli, as well as long zonulae adherentes, were evident. In addition, chromogranin a-positive granular staining, neurosecretory-granule-like structures, and parallel arrays of microtubules were sometimes associated with the blood vessels. We considered the present case to be an unusual example of GG with an ependymoma, more precisely a tanycytic ependymoma, as the glial component; to our knowledge, the existence of ependymoma as the main glial component of this particular tumor has not been described before. The occurrence of NFTs, which has been reported in several cases of GG, was an additional, unusual feature.- - - - - - - - - - ranking = 4.5988605757091keywords = membrane (Clic here for more details about this article) |
7/184. An intrasylvian "fibroma" in a child with cystic fibrosis: case report.OBJECTIVE AND IMPORTANCE: Intracranial fibrous tumors are uncommon during childhood. An unusual case of benign intrasylvian "fibroma" that has remained clinically and radiographically stable more than 3 years after a subtotal resection is described. CLINICAL PRESENTATION: A 9-year-old girl with cystic fibrosis presented with new-onset focal seizures referable to a large calcified left sylvian fissure mass. INTERVENTION: An open biopsy with subtotal resection of the lesion revealed a benign process characterized by exuberant fibrocollagenous tissue intermeshed with chronic inflammatory cells and foreign body giant cells, encompassing islands of gliotic brain tissue. Immunohistochemical analysis showed staining for epithelial membrane antigen and reticulin within some of the spindle cells, although the majority were nonreactive. The majority of tumor cells exhibited staining for laminin; CD34 staining was absent. Ultrastructural studies were also suggestive of a fibroblastic rather than a meningothelial origin of the lesion, with elongated cells separated by abundant extracellular collagen. Although dense adherence of the mass to the pial surface and the middle cerebral artery vessels precluded a complete resection, the patient remains seizure-free without anticonvulsant therapy more than 3 years postoperatively with no evidence of growth of the lesion. CONCLUSION: The lesion in this patient bears morphological similarity to a rare group of tumors referred to as "intracerebral fibromas," although a variety of other rare mesenchymal neoplasms were also considered within the differential diagnosis. However, the absence of any definite neoplastic features, the finding of chronic inflammatory changes, and the lack of growth of the residual tumor during an extended follow-up interval indicate that the mass may represent either an extremely indolent neoplasm or a nonneoplastic process. The differential diagnosis of intracranial fibrous tumors is contrasted with that of the reported case.- - - - - - - - - - ranking = 4.5988605757091keywords = membrane (Clic here for more details about this article) |
8/184. radiation-induced blood-brain barrier damage in astrocytoma: relation to elevated gelatinase B and urokinase.Successful management of brain tumors prolongs life, raising the risk of delayed injury secondary to the treatment. radiation therapy, a mainstay of brain tumor treatment, can damage the cerebral blood vessels. Acutely a breakdown of the blood-brain barrier (BBB) may be seen, but fibrosis complicates radiation injury in the chronic phase. matrix metalloproteinases (MMPs) and plasminogen activators are two matrix-degrading proteolytic enzymes, which are induced by radiation. They disrupt the basal lamina around cerebral capillaries and open the BBB. We report a patient with an astrocytoma managed by partial resection and external beam irradiation to maximal tolerable doses. The patient later developed malignant brain edema shortly after stereotactic radiosurgery. Tissue obtained during surgical debulking to control the edema showed very high levels of gelatinase B (92 kDa type IV collagenase) and urokinase-type plasminogen activator (uPA). Tumor cells were absent from the biopsy and subsequent autopsy specimens, but necrosis with fibrosis of the blood vessels was seen. If abnormal matrix enzyme function participates in the expression of radiation injury, then inhibitors to such enzymes may provide one strategy for controlling cerebrovascular damage after therapeutic brain radiation.- - - - - - - - - - ranking = 12.100611153195keywords = lamina (Clic here for more details about this article) |
9/184. Evidence for a recessive inheritance of Turcot's syndrome caused by compound heterozygous mutations within the PMS2 gene.Turcot's syndrome is a genetic disease characterized by the concurrence of primary brain tumors and colon cancers and/or multiple colorectal adenomas. We report a Turcot family with no parental consanguinity, in which two affected sisters, with no history of tumors in their parents, died of a brain tumor and of a colorectal tumor, respectively, at a very early age. The proband had a severe microsatellite instability (MIN) phenotype in both tumor and normal colon mucosa, and mutations in the TGFbeta-RII and APC genes in the colorectal tumor. We identified two germline mutations within the PMS2 gene: a G deletion (1221delG) in exon 11 and a four-base-pair deletion (2361delCTTC) in exon 14, both of which were inherited from the patient's unaffected parents. These results represent the first evidence that two germline frameshift mutations in PMS2, an MMR gene which is only rarely involved in HNPCC, are not pathogenic per se, but become so when occurring together in a compound heterozygote. The compound heterozygosity for two mutations in the PMS2 gene has implications for the role of protein PMS2 in the mismatch repair mechanism, as well as for the presymptomatic molecular diagnosis of at-risk family members. Furthermore, our data support and enlarge the notion that high dna instability in normal tissues might trigger the development of cancer in this syndrome.- - - - - - - - - - ranking = 1keywords = mucosa (Clic here for more details about this article) |
10/184. Hepatocellular carcinoma metastatic to the oral mucosa: report of a case with multiple gingival localizations.BACKGROUND: Metastases to the oral mucosa are rare, representing less than 1% of the tumors at this site. Most of these metastatic neoplasms originate in the lungs, kidneys, and liver. methods: The clinicopathologic features of an occult hepatocellular carcinoma, metastatic to the oral mucosa, are reported. The patient, a 70-year-old male, complained of 3 distinct polypoid, reddish lesions of the antero-inferior alveolar crest and both the right and left postero-superior attached gingiva, without bone involvement. The lesions were excised, with the clinical diagnosis of multiple vascular tumors, formalin-fixed, paraffin-embedded, cut and stained with hematoxylin and eosin. Consecutive sections were immunostained for alpha-1-antichymotrypsin, CEA, cytokeratins, EMA, hepatocyte antigen, PSA, S-100 protein, and thyroglobulin, using the alkaline phosphatase/anti-alkaline phosphatase technique. RESULTS: The morphologic features of the lesions were consistent with the diagnosis of carcinoma with trabecular and glandular patterns and bile secretion; furthermore, immunohistochemical reactivity for alpha-1-antichymotrypsin, cytokeratins, CEA, EMA, and hepatocyte antigen was demonstrated and the hepatic origin of the tumor was postulated. ultrasonography demonstrated a liver mass, which was biopsied and treated by chemoembolization. While no further complications occurred in the oral mucosa, the patient died 8 months after the diagnosis for widespread diffusion of the tumor to the lungs and brain. CONCLUSIONS: This case emphasizes the need to include metastatic tumors in the differential diagnosis of atypical neoplasms of the oral mucosa and to evaluate the opportunity of surgical treatment in order to preserve the functions of the mouth, even if the prognosis of the primary tumors remains unfavorable.- - - - - - - - - - ranking = 8keywords = mucosa (Clic here for more details about this article) |
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