Search PubMed⌕ Search

Biomedical subjects

V A White

Publications and source records attributed to V A White.

At least 37 records · Page 2Linked to original sources

Epithelioid sarcoma of the orbit.

BACKGROUND: Epithelioid sarcoma is a rare and aggressive malignancy commonly occurring in the tendon sheaths of the distal extremities of young adults. This article presents the clinical and pathologic details of two patients with epithelioid sarcoma in the orbit, the first to be reported as occurring in this location. METHODS: The clinical history, results of physical and radiologic examination, surgical procedures, pathologic specimens, and clinical course of each patient were reviewed and recorded. RESULTS: Biopsies were performed of the masses that were found in the superotemporal orbit of both patients. Results of pathologic examination in each patient caused considerable difficulty, and the correct diagnosis was reached only after extensive consultation. The first patient underwent wide soft tissue and bone exenteration and is alive without recurrence at 3 years. The second patient had multiple recurrences and died of recurrent and metastatic disease at 29 months. CONCLUSIONS: Epithelioid sarcoma must be added to the differential diagnosis of orbital sarcomas. The histology is nodular with a pseudogranulomatous appearance. Based on our limited experience, epithelioid sarcoma of the orbit may have a course similar to that which occurs elsewhere in the body, requiring aggressive surgical treatment at the outset.

Adolescent↗

Atelencephalic aprosencephaly.

Absence of the telencephalon and diencephalon characterizes the syndrome of aprosencephaly, while in atelencephaly, only the telencephalon is absent. Atelencephalic aprosencephaly is characterized by the presence of at least a rudimentary diencephalon. Embryologically, aprosencephaly is thought to occur after the optic vesicles form but before the cerebral vesicles appear. The syndrome is quite rare, with only 10 cases previously reported. We describe two fetuses with atelencephalic aprosencephaly. A 25-week estimated gestational age fetus was born to first-cousin parents and had a prenatal ultrasonographic diagnosis of anencephaly. The second, a 19-week estimated gestational age fetus, was thought to have semilobar holoprosencephaly by prenatal ultrasound. At autopsy, neuropathologic examination in both cases showed virtual absence of the cerebral hemispheres with an incomplete diencephalon. Microscopic examination in one case revealed disorganized neuropil with a proliferative vasculopathy. The optic globes were completely formed and attached to hypoplastic optic nerves, but retinal dysplasia was apparent histologically in both cases, and bilateral colobomata were present in one case. The findings in these cases demonstrate a spectrum of congenital variations that lie between the syndromes of atelencephaly and aprosencephaly, underscoring the complexity of the congenital anomalies.

Abnormalities, Multiple↗

Cytogenetic analysis of uveal melanoma. Consistent occurrence of monosomy 3 and trisomy 8q.

BACKGROUND: The genetic alterations associated with the pathogenesis of uveal melanoma have not been determined. To address this issue, the authors performed a prospective cytogenetic study of 35 uveal melanomas, including 23 primary untreated tumors and 12 tumors that were removed after local radiation therapy. METHODS: Representative tumor tissue was processed by established methods for histopathologic and cytogenetic studies. Tumor cells were disaggregated and established in short-term culture; metaphases were prepared by standard methods for karyotypic analysis. RESULTS: Successful analyses were achieved in 27 of the tumor specimens, including 20 of 23 tumors not exposed to radiation and 7 of 12 tumors exposed to radiation. All of the tumors had an abnormal karyotype. Recurrent chromosomal abnormalities detected in the tumors not exposed to radiation included monosomy 3 (13 of 20), trisomy 8 or 8q (11 of 20), loss of a sex chromosome (10 of 20), and loss of 6q (8 of 20). The tumors previously exposed to radiation were characterized by more complex changes, with monosomy 3 and trisomy 8q detected in three cases each. CONCLUSIONS: Uveal melanoma is characterized by monosomy 3 and trisomy 8q in most cases. These findings, which are supported by data from other investigators, provide compelling evidence that loss of gene sequences on chromosome 3 and duplication of gene sequences on chromosome 8 are implicated in the genetic alterations associated with uveal melanoma and offer a basis for additional molecular genetic investigations.

Adolescent↗

Idiopathic sclerosing inflammation of the orbit: immunohistologic analysis and comparison with retroperitoneal fibrosis.

Idiopathic sclerosing inflammation of the orbit is clinically characterized by an insidious, chronic and progressive fibrosing process damaging orbital structures through entrapment and mass effect. Histologically, desmoplasia and a sparse infiltrate of lymphocytes, histiocytes, plasma cells, and occasional neutrophils and eosinophils are seen. An immune pathogenesis is suspected but presently poorly understood. To characterize the inflammatory infiltrate and to compare orbital and other inflammatory fibrosing lesions, immunoperoxidase studies using the streptavidin method were performed on 16 formalin or Bouins' fixed, paraffin-embedded orbital biopsy specimens and six specimens of retroperitoneal fibrosis. Positive staining of orbital tissue occurred as follows: T-cells (UCHL-1) 94% of cases, B-cells (L26) 40%, tissue macrophages (KP-1) 56%, HLA Dr positive antigen presenting cells and activated T-cells (LN3) 44%, and immunoglobulins (kappa, 80%; lambda, 63%, IgG, 73%, IgA, 44% and IgM, 31%). Results were strikingly similar for retroperitoneal fibrosis. These findings imply a cell mediated pathogenesis in idiopathic sclerosing inflammation of the orbit that is similar to retroperitoneal fibrosis and suggest therapeutic potential for agents modifying this facet of the immune system.

Humans↗

Cellular blue nevus of the conjunctiva.

BACKGROUND: Cellular blue nevi of the conjunctiva are extremely rare, and their natural history and malignant potential have not been fully ascertained. METHODS: A report of an unusually well-documented case of growth of a cellular blue nevus is presented, along with a review of current knowledge of this lesion. RESULTS: A 71-year-old woman presented with a darkly pigmented raised lesion of the conjunctiva, which had slowly enlarged over 47 years. There were nonconfluent areas of involvement of the upper and lower lids. Results of biopsy showed the lesion to be a cellular blue nevus, with no evidence of malignancy. CONCLUSION: This well-documented case of slow growth and spread without malignant transformation adds to the knowledge of this rare lesion.

Aged↗

Histopathologic, immunophenotypic and genotypic analyses in ocular adnexal lymphoproliferative disorders.

This study reports on fourteen biopsies from patients presenting to our orbital and oncology service with ocular adnexal lymphoid proliferations between November 1988 to September 1991. The biopsies were studied using histologic, immunophenotypic and genotypic analyses. By histologic criteria, there were two reactive, five indeterminate and seven lymphomatous lesions. On immunophenotypic analysis, there were two monoclonal and 10 polyclonal lesions in the 12 specimens analysed. Genotypic analysis confirmed the histopathologic diagnoses for the reactive lesions by showing them to be germline. It also confirmed that both the histopathologic lymphomas and immunophenotypically monoclonal lesions were clonally rearranged. Genotypic analysis was able to separate the histologically indeterminate group into two subsets: clonally rearranged, of which there was one, and germline, of which there were four. In addition, it demonstrated that immunophenotypic polyclonality cannot always be equated with genotypic polyclonality as was the situation in four out of 10 lesions in our series. The significance of clonal arrangements in the histologically indeterminate and immunophenotypically polyclonal groups can only be determined by prospective study.

Adult↗

Effects of cigarette smoke on the immune response. II. Chronic exposure to cigarette smoke inhibits surface immunoglobulin-mediated responses in B cells.

We have previously reported that chronic exposure of rats to cigarette smoke inhibits the antibody-forming cell (AFC) response to both T-dependent and T-independent antigens and may reflect B cell dysfunction. In this communication we extend these studies to show that T cell functions are normal in chronically smoke-exposed rats (SM) as judged by their responses to mitogens and "nominal" or alloantigens. While B cells from SM respond significantly to the B cell mitogen lipopolysaccharide (LPS), they fail to proliferate in response to anti-IgM (anti-mu) or to produce significant AFC response to sheep red blood cells. On the basis of the number of rosettes formed with trinitrophenylated (TNP) horse red blood cells; the frequency of TNP-binding cells (TNP-ABC) in the spleens of SM is comparable to sham control rats. However, the proliferation of TNP-ABC to TNP-Brucella abortus is significantly decreased in SM. These differences in SM B cell responses, i.e., between LPS and anti-mu/antigen, may to be related to the ability of LPS to bypass a portion of the membrane signal transduction cascade. These results suggest that cigarette smoke affects an early step(s) in the antigen-dependent B cell signal transduction pathway.

Animals↗

Leiomyosarcoma of the conjunctiva.

The clinical, light microscopic, immunohistologic, and ultrastructural findings of a leiomyosarcoma of the conjunctiva are presented. This tumor was diagnosed after a 26-year history and is the first to be adequately documented as having arisen in the conjunctiva.

Aged↗

Relation between optic nerve axon number and axon diameter to scleral canal area.

To determine whether there are more axons present in eyes with larger optic discs, the authors studied 16 eyes from 16 individuals whose eyes were donated for corneal transplantation. For each nerve, the axon count, axon diameter, and scleral canal area was measured. Total axon count decreased with age. Mean axon diameter increased with age. There was no statistically significant relation detected between axon count and scleral canal area. Multiple regression revealed that when corrected for the effect of age, eyes with smaller scleral canal areas had larger number of axons.

Adult↗

Orbital lesions with granulomatous inflammation.

Orbital lesions characterized by granulomatous inflammation are a heterogeneous group of diseases of various causes with a common histopathological substrate involving aggregates of epithelioid cells. Forty-one patients (27 females and 14 males) with biopsy-proven granulomatous inflammation were seen at an orbital clinic between 1978 and 1989. The mean age at presentation was 40.2 (extremes 6 and 77) years. Two main clinical presentations were noted: painless, subacute or chronic mass effect, and tender, subacute inflammatory process. Six patients had secondary features that were infiltrative in character. The lesions were primarily located in the anterior superior orbit. In nearly half the patients the granulomatous reaction was confined to the orbit (predominantly ruptured dermoid and localized orbital sarcoid), and the remainder had either regional involvement (Wegener's granulomatosis or fibro-osseous process) or systemic involvement (sarcoidosis).

Adolescent↗

Monosomy 3 and isochromosome 8q in a uveal melanoma.

Chromosome analysis of a locally invasive uveal melanoma revealed monosomy 3 and i(8q). Sublines with multiple copies of the i(8q) were also present. Loss of heterozygosity for genes on chromosome 3 and duplication of genes on 8q may play an important role in progression of uveal melanoma.

Chromosome Aberrations↗

Large cell orbital lymphoma in a patient with acquired immune deficiency syndrome. Case report and review.

Despite the protean ocular manifestations of acquired immune deficiency syndrome (AIDS), orbital involvement is rare and only one previous case of orbital non-Hodgkin's lymphoma (NHL) has been documented. We report a second case of NHL (large cell) of the orbit in a 28-year-old patient with a 4-year history of AIDS and review the context in which this lesion presented.

Acquired Immunodeficiency Syndrome↗

Uveal malignant melanoma and optic nerve glioma in von Recklinghausen's neurofibromatosis.

A case of uveal malignant melanoma and contralateral optic nerve glioma is described in a 53-year-old Caucasian male with multiple uveal melanocytic hamartomas and neurofibromatosis. The eye was enucleated, and histologically the melanoma was found to consist of 70% epithelioid cells, with many bizarre, multinucleated forms. CT scan demonstrated a non-enhancing, fusiform enlargement of the contralateral optic nerve with enlargement of the optic canal and intracranial extension. This combination of tumours has not previously been reported in a patient with neurofibromatosis and serves to emphasise the common neuroectodermal origin of tumours in this autosomal dominant condition.

Cranial Nerve Neoplasms↗

Changing indications for penetrating keratoplasty in Vancouver, 1978-87.

Indications for penetrating keratoplasty (PK) were assessed by clinicopathological review of 659 corneal buttons submitted from 1978 to 1987 to the Ophthalmic Pathology Service in Vancouver. Leading indications for PK were bullous keratopathy (22.2%), keratoconus (17.1%), scarring with or without chronic inflammation (13.5%), graft failure (12.1%), scarring or active keratitis secondary to virus (9.0%) and Fuchs' dystrophy (8.3%). The principal factors responsible for graft failure were also judged by clinicopathological correlation. The authors compare their findings with those in other series.

Adolescent↗

Ductal carcinoma-in-situ of the breast: fine-needle aspiration cytology of 12 cases.

Aspiration specimens from 12 patients with histologically documented ductal carcinoma-in-situ (DCIS) of the breast (seven patients) or DCIS with minute foci of stromal invasion (five) were evaluated. Five patients presented with palpable masses, 1.5-4.0 cm, and four patients presented with localized thickening, associated with nipple erosion and discharge in two of them. One patient had nipple inversion, and one patient had bilateral nipple discharge. In one patient, no apparent abnormality of the breast was present. Mammography was either suspicious for or strongly suggestive of carcinoma in 10 patients and negative in two. Aspirates from all patients were composed of fragments of atypical ductal epithelium and numerous single epithelial cells. In nine cases, the smears were hypercellular and similar to aspirates of typical invasive ductal carcinoma. Calcifications were present in six cases. In four of these, associated tumor necrosis was evident. Cytologic features separating DCIS patients from those showing minimal stromal invasion or common types of invasive ductal carcinoma (IDC) were not identified. We conclude that fine-needle aspiration cytology of DCIS is identical to that of IDC. If preoperative radiotherapy or chemotherapy is considered in the management of invasive breast carcinoma, cutting-needle biopsy for confirmation of tumor invasion is necessary.

Adult↗

Pulmonary dirofilariasis: the great imitator of primary or metastatic lung tumor. A clinicopathologic analysis of seven cases and a review of the literature.

The clinical, radiologic, and pathologic features of seven patients with pulmonary dirofilariasis were studied. The findings were analyzed in conjunction with those of 76 cases previously reported from the United States. We found that, in most instances, the disease was acquired in states along the Atlantic and Gulf coasts and occurred predominantly in whites (94.7%) in their fifth or sixth decades of life, with a male to female ratio of 2:1. Symptoms, commonly chest pain, cough, or hemoptysis, were present in 37.6% of patients. Most patients (62.4%) were asymptomatic, and the disease was discovered incidentally on routine radiography or during the investigation of another problem. Peripheral eosinophilia was present in 20% of patients. The radiologic findings consisted of single (89.8%) or multiple (10.2%) pulmonary nodules that simulated primary or metastatic lung tumor. Dirofilariasis was not included in the clinical differential diagnosis in any of the patients. In one case, the diagnosis was accurately obtained by fine needle aspiration biopsy. All other patients required thoracotomy with excisional lung biopsy for diagnosis. Pathologically, the dirofilaria nodule consisted of a spherical subpleural infarct with a central thrombosed artery containing Dirofilaria immitis in various stages of disintegration.

Adult↗