PubMed Health⌕ Search

Biomedical subjects

V Edwards

Publications and source records attributed to V Edwards.

50 records · Page 3Linked to original sources

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.

Adolescent↗

Diffuse leiomyomatosis of the esophagus: disorder of cell-matrix interaction?

Diffuse leiomyomatosis (DL) is rare condition characterized by proliferation of smooth muscle in the upper gastrointestinal tract. Most cases are associated with X-linked Alport syndrome and have partial deletions in the genes encoding both the alpha5 and alpha6 chains of collagen type IV. We studied aspects of cell-matrix interaction of myocytes in an esophagogastrectomy specimen from a 12-year-old patient with DL. Myocytes had central areas of cytoplasmic rarefaction, which were actin positive and desmin poor, with the reverse pattern of staining at the cell periphery. Electron microscopy (EM) showed that the areas of rarefaction consisted of disorganized aggregates of filaments. The basement membranes ranged from thickened to thinned or absent. Immunohistochemical staining for the alpha1-alpha4 chains of collagen type IV, the alpha1, alpha2, beta2, and gamma1 chains of laminin, nidogen, type VI collagen, and fibronectin was normal. There was loss of the alpha5 and alpha6 chains of collagen type IV and the beta1 chain of laminin. Normal staining for alpha1, alpha2, alpha3, alpha4, alpha6, alpha8, and beta1 integrins was noted. Staining for alpha5 integrin varied from normal to reduced or negative in different cells. In DL, a primary abnormality of basement membrane may be associated with disorganization of the contractile apparatus and alterations of certain integrins. This may reflect a disturbance of cell-matrix interactions that play a role in cell differentiation and internal organization.

Barrett Esophagus↗

Vascular pathology in galactosialidosis.

This article immunohistochemically and ultrastructurally examines a brain with galactosialidosis, focusing on the structure of blood vessel endothelium. Lysosomes were observed in the expanded cytoplasm of the endothelial cells, which were vacuolated by light microscopy. Immunoreactivity for CD31, one of the vascular cell adhesion molecules, was minimal to faint in endothelial cells with vacuolations. The loss of CD31 immunoreactivity and breakdown of vascular cell adhesion molecules in vacuolated endothelial cells seem to promote the development of brain infarctions. Moreover, diffuse and various degrees of axonal damage, most likely caused by vascular disorder in cerebral or cerebellar white matter, was reported using the amyloid precursor protein (APP) immunohistochemical method.

Amyloid beta-Protein Precursor↗

Rhabdomyosarcoma metastasizing as a malignant ectomesenchymoma.

Rhabdomyosarcoma is a common childhood malignancy that may occasionally occur as a component of a mixed mesenchymal tumor, e.g., a triton tumor or malignant ectomesenchymoma. A case is reported of a 13-year-old boy who had resection of a paratesticular embryonal rhabdomyosarcoma with subsequent radiation and chemotherapy. Two years later, a retroperitoneal metastasis was resected. Histology showed a mixture of rhabdomyoblasts, ganglion cells, and a third population of cells with combined features of these two distinct cell types. Electron microscopy confirmed the presence of rhabdomyoblasts with characteristic bundles of myofilaments and Z-band material, and ganglion cells with prominent nuclei and nucleoli, rough endoplasmic reticulum, dense core granules, filaments, and tubules. Notably, the third cell population showed features of both rhabdomyoblasts and ganglion cells. Immunohistochemistry confirmed the mixed population of rhabdomyoblasts (positive for vimentin, desmin, negative for S-100, NSE), ganglion cells (positive for S-100 and NSE, negative for vimentin and desmin), and the third population expressing all test antigens. The features of this metastatic lesion are those of a malignant ectomesenchymoma with combined rhabdomyosarcoma and ganglioneuroma components. It is postulated that this lesion results from biphenotypic expression of tumor cells that previously expressed only rhabdomyoblastic differentiation. The role of prior chemotherapy and radiotherapy in this particular case is unclear.

Adolescent↗

Selected ultrastructural aspects of urothelial and prostatic tumors.

Ultrastructural techniques have been widely applied in the study of urothelial and prostatic tumors. In the common proliferative diseases affecting the lower urogenital tract, electron microscopy has provided us with a greater understanding of the pathobiology and morphology of these disease processes. Specific diagnostic application, however, has been limited. In the unusual tumors affecting urothelium and prostate such as neuroendocrine carcinoma, carcinosarcoma, and sarcomas, electron microscopy has provided some practical diagnostic information of value in patient management. This paper provides a survey of the fundamental and practical contributions of ultrastructural studies in the prostatic and urothelial areas.

Adenocarcinoma↗

Crystalline inclusions in a subependymal giant cell tumor in a patient with tuberous sclerosis.

Unusual crystalline cytoplasmic inclusions were encountered in tumor cells in a subependymal giant cell tumor (SEGT) in a 16-year-old girl with tuberous sclerosis. By electron microscopy, the tumor cells demonstrated typical features previously described in SEGT, including abundant dense bodies, prominent Golgi complexes, abundant mitochondria, rough and smooth endoplasmic reticulum, scattered intermediate filaments and microtubules, glycogen, and rare synaptic contacts as well as primitive intercellular junctions. The dense bodies were bound by a single membrane and were round, ovoid, irregular, or cylindric in appearance with electron-dense homogenous content or fingerprint profiles. Of note was the presence of numerous cytoplasmic rhomboidal or rectangular crystalline inclusions akin to those seen in alveolar soft part sarcoma. These inclusions measured as much as 8 microns in length and had 7-nm periodicities, often with intersecting lamellae. Rarely, the membrane-bound dense bodies showed areas of similar periodicities, indicating that the crystalline inclusions are related to and might originate from the dense bodies. While crystalline inclusions have previously been described in one patient with SEGT (Bender and Yunis, Ultrastruct Pathol 1980; 1:287-299), the inclusions in the present case were a striking feature and add to the spectrum of the ultrastructural pathology of SEGT.

Adolescent↗

Filamentous aggregates in a meningioma.

The light microscopic and ultrastructural appearances of unusual filamentous aggregates in a right parietal meningioma in a 14-year-old boy are described. The tumor showed prominent meningothelial as well as fibroblastic components and was graded as an atypical meningioma. By light microscopy, eosinophilic, PAS-positive, granular, irregularly shaped Rosenthal fiber-like structures were widespread within the tumor, in both an intra- and an extracellular location. By immunohistochemical staining, similar location of positivity was obtained for vimentin, laminin, and collagen type IV. The inclusions were nonreactive for keratin, lysozyme, alpha-1-antitrypsin, and ubiquitin. Ultrastructurally, these aggregates were composed of an irregular tangle of filaments with electron dense condensations, sometimes with a lattice pattern. The intracellular aggregates were membrane-bound, and some were found within dilated rough endoplasmic reticulum, while extracellularly, they filled up spaces between adjacent tumor cells. Less prominently, flocculent osmiophilic nonfilamentous material was also seen within the inclusions. These observations suggest that these novel inclusions in a meningioma are composed of intermediate filaments (vimentin) and extracellular matrix proteins, with active synthesis in the rough endoplasmic reticulum and subsequent extrusion from the tumor cells into the extracellular spaces.

Adolescent↗

Unique intracerebral tumor with divergent differentiation in a patient presenting as NF2: report of a case with features of astrocytoma, ependymoma, and PNET.

Patients with neurofibromatosis 2 (NF2) are predisposed to a variety of neoplastic and dysplastic lesions, including schwannomas, neurofibromas, meningiomas, astrocytomas, and ependymomas, as well as entities such as meningioangiomatosis, schwannosis, and hamartomas. This study reports a unique intracerebral frontotemporal tumor in a 6-year-old boy with presumed NF2, on the basis of bilateral cerebellopontine tumors consistent with acoustic neuromas. The intracerebral tumor revealed a variety of histological patterns, including foci of primitive neuroectodermal tumor (PNET), low-grade astrocytoma and ependymoma, as well as neuroepithelial rests with immature ganglion cells and hamartomatous areas. The MIB-1 labeling index ranged from 63% in the foci of PNET to 4-7% in other foci. The PNET component revealed immunopositivity for synaptophysin and neurofilament and showed cells with delicate intercellular junctions, profiles of rough endoplasmic reticulum, mitochondria, and dense core granules, and cell processes with microtubules and neurofilaments. The glial and ependymal components showed bundles of glial filaments and prominent cell junctions, cilia, and microvilli. The hamartomatous component also included aggregates of cells with hyaline eosinophilic cytoplasm. By EM these cells contained abundant amorphous flocculent material. This constellation of pathologic findings, especially the finding of PNET, is unique and not previously reported in the setting of NF2.

Astrocytoma↗

Cerebellar pathology in tuberous sclerosis.

Cerebellar involvement in tuberous sclerosis is rare and generally nonsymptomatic. The authors describe a cerebellar tuber in a 5-year-old boy with tuberous sclerosis. A CT scan at age 2 years showed the characteristic cortical, white matter, and subependymal lesions of tuberous sclerosis. At 5 years, when the patient was symptomatic with ataxia, the CT and MRI scans revealed additionally the presence of a right cerebellar enhancing lesion with edema. A total surgical resection was undertaken. The cerebellar lesion was very firm and demonstrated marked disorganization of neuronal architecture in the cerebellar folia, with bizzare ectopic neurons in the molecular and granule cell layers and white matter, along with calcification, gliosis, and Rosenthal fiber deposition. Balloon cells with glassy, pale, eosinophilic cytoplasm were also present. There was a marked loss of myelin in the white matter, with significant vacuolation and gliosis. Electron microscopy documented abundant lysosomal inclusions, prominent rough endoplasmic reticulum and Golgi complexes, microtubules, intermediate filaments, and synaptic contacts. While there is much speculation as to the precise nature of cerebellar pathology in tuberous sclerosis, this case demonstrates conclusively that the cerebellar lesions reflect anomolous neuronal development and migration akin to supratentorial lesions and can rarely be symptomatic.

Cerebellum↗

Astroblastoma: report of a case with ultrastructural, cell kinetic, and cytogenetic analysis.

Astroblastomas are rare tumors of cerebral hemispheres of young adults. We report an astroblastoma in a 15-year-old girl and present the first descriptions of cytogenetic abnormalities in this tumor. The tumor was relatively well demarcated from the brain and revealed prominent perivascular rosettes as well as intervascular clear cells that contained abundant glycogen. Cytogenetic analysis revealed an abnormal hypodiploid karyotype with 45 chromosomes and monosomies of chromosomes 10, 21, and 22 and two marker chromosomes in all cells examined. The tumor had a Ki-67 labelling index of 4.7% and assessment of ploidy by flow cytometry revealed 96% of cells in the G0G1 phase and 4% of cells in the G2M phase. Assessment of proliferation and ploidy indices in further cases may provide important prognostic data for this poorly understood entity. Further cytogenetic studies will also help to identify if there are consistent karyotypic abnormalities in these enigmatic tumors.

Adolescent↗

Changing breast self-examination behavior.

Breast self-examination instruction was presented to 130 women. Four instructional methods used were: modeling alone, modeling plus guided practice, modeling plus self-monitoring, and modeling plus peer support. Follow-up data were collected at three- and six-month intervals. Data analyses revealed that, although the study population as a whole made statistically significant changes in breast self-examination behavior, no statistically significant outcomes were related to the dependent variables of frequency and knowledge of self-examination and confidence in ability to detect breast abnormalities between subjects in the modeling alone method--which served as the control--and subjects in the other three instructional methods.

Adolescent↗

"Polyphenotypic" tumors in the central nervous system: problems in nosology and classification.

In recent years, there is increasing recognition of polyphenotypic high-grade malignancies in the non-central nervous system (CNS) tumor literature. Some of these tumors have been regarded as variants of primitive neuroectodermal tumor (PNET) or as extrarenal malignant rhabdoid tumors (MRTs). This report concerns two posterior fossa neoplasms, both of which displayed a "polyphenotypic" expression of neural, epithelial, myogenic, and glial markers, including synaptophysin, neurofilament, vimentin, glial fibrillary acidic protein, S-100, neuron-specific enolase, desmin, S antigen, MIC2, cytokeratin, epithelial membrane antigen, and carcinoembryonic antigen. One tumor showed complex intercellular junctions, cytoplasmic intermediate filaments, well-developed rough and smooth endoplasmic reticulum and Golgi apparatus, cilia, and neurosecretory granules. The other neoplasm showed pools of glycogen, desmosomes, and tonofilaments. The histological and ultrastructural appearances were inconsistent with glioma, PNET, meningioma, ependymoma, choroid plexus carcinoma, sarcoma, germ cell tumor, and other tumors in the World Health Organization classification. Although the polyphenotype raises the issue that these may represent variants of MRT or the atypical teratoid-rhabdoid tumor, the morphologic findings in the two cases were very dissimilar. Our two cases underscore the problems in nosology and classification of polyphenotypic tumors of the CNS. This is particularly significant, as therapeutic protocols for PNET, MRT, and non-CNS polyphenotypic tumors are different. We review the literature on polyphenotypic tumors and reiterate the difficulties in precise classification of these complex tumors.

Biomarkers↗