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PubMed2005

Ocular adnexal lymphoma: a clinicopathologic study of a large cohort of patients with no evidence for an association with Chlamydia psittaci.

Non-Hodgkin lymphomas are among the most common primary tumors occurring in the ocular adnexa. Herein, we present a 14-year single-institution experience in 62 patients with primary ocular adnexal lymphomas (OALs). Association with Chlamydia psittaci infection is examined in 57 tumor specimens. Extranodal marginal zone lymphoma (EMZL) was the most frequent histologic subtype (89%). The majority of patients with EMZL (84%) presented with stage E-extranodal (IE), however only 16% had an advanced stage. All stage IE patients were treated with local radiotherapy, whereas patients with disseminated disease received systemic therapy with or without local irradiation. All but 1 patient with EMZL achieved complete remission (CR). During a median follow-up of 52 months (range, 3-153 months), the estimated 5-year overall survival (OS) and freedom from progression (FFP) were 96% and 79%, respectively. During the follow-up, 22% of patients relapsed, mainly in extranodal sites, and 4% transformed to diffuse large B-cell lymphoma. None of the patients exhibited local orbital failure in the radiation field. None of the OAL specimens harbored C psittaci DNA. Our study demonstrates that EMZLs, accounting for the majority of primary OALs, are characterized by an indolent natural history with frequent, continuous extranodal relapses. In South Florida, OALs are not associated with C psittaci infections.

Adult↗
PubMed2004

Expression of cell proliferation and apoptosis markers in papillomas and cancers of conjunctiva and eyelid.

Cell proliferation and programmed cell death are considered to be important events in carcinogenesis. The object of our study was to evaluate the expression of the Bcl-2 protein family (Bcl-2, Bak, Bax), p53, proliferating cell nuclear antigen (PCNA), and Ki-67 protein immunoexpression as well as the correlation between the examined markers and some clinicopathological features in papillomas and cancers of conjunctiva and eyelid. Forty-five squamous cell papillomas (SCP), 11 squamous cell cancers (SCC), and 27 basal cell cancers (BCC) were estimated. In the SCP group, p53 protein expression was observed in 30 cases (66.6%), Ki-67 in 14 (31.1%), PCNA in 44 (97.8%), Bcl-2 in 24 (53.3%), Bak in 28 (62.2%), Bax in 31 (68.9%), and Bcl-xl in 11 (100%). In the SCC group, p53 protein expression was evaluated in 8 cases (72.8%), Ki-67 in 2 (18.2%), PCNA in 8 (72.7%), Bcl-2 in 5 (45.4%), Bax and Bak both in 10 (90.9%), and Bcl-xl in 100%. In the BCC group, p53 protein expression was estimated in 23 cases (85.1%), Ki-67 in 13 (48.1%), PCNA in 26 (96.2%), Bcl-2 in 13 (48.1%), Bak in 21 (77.8%), Bax in 22 (81.5%), and Bcl-xl in 23 (85.2%). We observed a correlation between some clinicopathological features and the examined markers of apoptosis and cell proliferation, which seemed to be important events in cancer development.

Apoptosis↗
PubMed1987

Elevation of cerebrospinal fluid catecholamine metabolites in patients with intracranial tumors of neuroectodermal origin.

The concentrations of homovanillic acid (HVA), hydroxymethoxyphenylethyleneglycol (HMPG), and vanillylmandelic acid (VMA) were determined in lumbar cerebrospinal fluid (CSF) specimens. The study population consisted of the following groups: control subjects with malignancies of nonneuroectodermal origin (mostly leukemia in remission), neuroblastoma (extracranial and intracranial or cranial metastases), brain tumors (neuroectodermal and glial), and retinoblastoma. A significant increase in the CSF concentration of HVA was observed in patients with brain tumors (neuroectodermal), neuroblastoma (intracranial or cranial metastases), and retinoblastoma when compared with age-matched controls. In contrast, HMPG and VMA concentrations did not differ from controls except in patients with neuroblastoma (intracranial or cranial metastases) and brain tumors (neuroectodermal) who had significant elevations in HMPG and VMA, respectively. An inverse correlation was noted between the CSF concentration of HVA and clinical response to therapy. Nonresponding patients exhibited increases in HVA when compared with pretreatment values. These data suggest that the quantitative determination of catecholamine metabolites in lumbar CSF is an effective method for diagnosing intracranial tumors of neuroectodermal origin and assessing their response to therapy.

Brain Neoplasms↗
PubMed2004

Salvage therapy for primary CNS lymphoma with a combination of rituximab and temozolomide.

The authors evaluated the efficacy of a combination of rituximab and temozolomide for recurrent or refractory primary CNS lymphoma (PCNSL). Fifteen patients with a median age of 69 years had a 53% objective response rate with acceptable toxicity. Median overall survival is 14 months and median progression free survival of responding patients is 7.7 months. This combination merits further study and provides a reasonable therapeutic alternative for older patients with progressive PCNSL.

Aged↗
PubMed1981

Von Hippel-Lindau Disease with multiple manifestations: diagnosis and management.

The association of a recurrent cerebellar hemangioblastoma, retinal angioma, pheochromocytoma, renal carcinoma, and multiple renal and pancreatic cysts in one patient is reported, and the pertinent diagnostic studies are discussed briefly. Awareness of the possibility of late manifestations of various components of this syndrome in patients treated successfully for cerebellar hemangioblastoma will lead to their early identification and successful surgical treatment. Genetic counseling for patients affected by this syndrome and their families is recommended.

Adult↗
PubMed1981

Retinoblastoma as a possible primary intracranial tumor.

A 10-month-old infant with a small, unilateral, peripheral retinal lesion also had a large, well-encapsulated, suprasellar retinoblastoma. The primary vs. the metastatic nature of the intracranial tumor is considered. It is postulated that primary intracranial tumors may arise within areas of the central nervous system embryologically related to the retina in patients with the genetic form of retinoblastoma.

Brain Neoplasms↗
PubMedDate not supplied

[Exenteration: a retrospective study].

PURPOSE: To review all cases of orbit exenteration performed at the Orbit Sector, Ophthalmology Department - Federal University of São Paulo, from 1998 to 2003. METHODS: We reviewed conditions leading to orbital exenteration in 21 patients at the Orbit Sector of Unifesp-EPM from August 1998 to May 2003. Data regarding sex, age, race, primary lesion site, visual acuity at the moment of diagnosis, previous surgeries related to the exenteration, type of performed surgery, histopathologic diagnosis, postoperative complications and use of adjuvant treatment were collected. RESULTS: 21 patient charts were retrospectively analyzed. Ages ranged from 5 to 91 years (mean of 58.5 years). Of these, 12 were male and 9 were female, most of them Caucasian. All lesions that led to exenteration were malignant neoplasias; however, none were metastatic. Lesions originated from eyelids in twelve patients, from bulbar conjunctiva in six and from the orbit in three. Cases were also classified as squamous cell carcinoma (eleven cases), basal cell carcinoma (four cases), sebaceous gland carcinoma (two cases), rhabdomyosarcoma (two cases), mucoepidermoid carcinoma (one case) and adnexal microcistic carcinoma (one case). Visual acuity at the moment of diagnosis ranged from 20/40 to no light perception. Only six patients had been submitted to previous surgeries related to the exenteration. After surgery, three patients suffered graft necrosis, one presented ethmoidal sinus fistula to the orbit and one presented orbital socket shrinkage. Six patients needed postoperative radiotherapy and two had been previously submitted to chemotherapy. CONCLUSION: Most patients analyzed in our study presented lesions that are usually small in the beginning; however, they can disseminate to the orbit in the absence of adequate treatment.

Adolescent↗
PubMed2004

The past decade of experience with isolated hepatic perfusion.

Metastatic or primary unresectable cancers confined to the liver are the sole or life-limiting component of disease for many patients with colorectal cancer, ocular melanoma, neuroendocrine tumors, or primary colangio- or hepatocellular carcinomas. Regional treatment strategies including infusional chemotherapy and local ablative therapy are under investigation, but have limitations with respect to the clinical conditions under which they can be employed. Isolated hepatic perfusion (IHP) was first clinically applied over 40 years ago, but because of its technical complexity, the attendant potential morbidity, and the lack of documented efficacy, it has not enjoyed consistent or widespread evaluation. In light of the antitumor activity with isolated limb perfusion with tumor necrosis factor (TNF) and melphalan in patients with unresectable extremity sarcoma or in transit melanoma, this regimen has been administered via IHP at several centers worldwide for patients with unresectable liver cancers. IHP with TNF and melphalan can result in significant regression of advanced refractory cancers from multiple histologies confined to the liver. Patient selection is important to ensure good results with minimal morbidity and mortality. Work to define the appropriate clinical groups is ongoing at many clinical centers.

Antineoplastic Agents, Alkylating↗
PubMed1983

Effect of oncogenic nitrosourea on intraocular tumor induction by adenovirus in rats.

Interference of oncogenic N-nitrosourea in intraocular tumor induction by human adenovirus type 12 in rats was examined. Transplacental administration of methylnitrosourea to rat embryo reduced significantly the latent period of the intraocular adenovirus tumor in each animal whereas in groups preadministered with ethylnitrosourea the decrease in the latent period showed marked individual variation. Preadministration of N-nitrosourea caused little change in the morphology and incidence of adenovirus tumors. The histological picture of adenovirus induced intraocular tumors which developed in each group of rats was that of retinoblastoma-like tumor identical to the tumor induced by single virus injection.

Adenoviridae↗
PubMed1990

Multiple intracranial metastases following malignant evolution in recurrent pleomorphic adenoma of the lacrimal gland--case report.

A 54-year-old female presented with multiple intracranial metastases following malignant transformation in the third recurrence of pleomorphic adenoma of the left lacrimal gland, 25 years after the first surgical treatment. The preoperative computed tomography and magnetic resonance imaging demonstrated direct invasion of the orbital tumor into subdural and epidural spaces in the ipsilateral frontotemporal region and also an intracerebral metastasis in the ipsilateral parietal lobe. Histological examination of the surgical specimen revealed features of poorly differentiated adenocarcinoma, suggesting carcinomatous changes. The relevant literature is reviewed.

Adenocarcinoma↗
PubMed1991

The prevalence and location of metastases from ocular melanoma: imaging study in 110 patients.

Ocular melanoma is characterized by an unpredictable clinical course, during which fulminant metastatic disease may occur after a prolonged disease-free interval. The purpose of this study was to determine the pattern of metastatic involvement in this disease. The clinical and radiologic findings in 110 patients with metastatic ocular melanoma were reviewed. The 54 men and 56 women were 24-79 years old (mean, 50 years) when the primary tumor was first diagnosed. Metastases were present in three patients at the time of first diagnosis and occurred in 107 patients 2 months to 36 years later (mean, 52 months). One hundred five patients died between 1 and 38 months after the onset of metastatic disease. Hepatic metastases developed in 101 patients (92%), and in 60 (55%) of these, the liver was the only organ involved initially. Pulmonary parenchymal metastases developed in 34 patients (31%), but in only four of them were metastases confined to the lungs. Twenty-five patients (23%) had bone involvement, mostly affecting the spine. Nineteen patients (17%) had skin or subcutaneous metastases, but in only two of them was this the initial finding. Nodal involvement was shown in 15 patients (14%), almost always associated with extensive hepatic metastases. Brain and adrenal metastases were seen in five and three patients, respectively. Hepatic involvement occurs in almost all patients who develop metastatic ocular melanoma, and the liver is the most common initial site of metastatic involvement. Metastases may develop after a long disease-free interval.

Adult↗
PubMed1992

Retinoblastoma: messenger RNA for interphotoreceptor retinoid binding protein.

Surgically excised retinoblastomas from 14 patients (age range nine months to two years) were assessed by immunocytochemistry for the expression of photoreceptor-specific proteins and neuronal and glial cell markers. Adjacent tissues were examined for messenger RNA expression of interphotoreceptor retinoid-binding protein (IRBP) using Northern blots. For immunocytochemical stains (ABC method), monoclonal and polyclonal antibodies included S-Ag, rhodopsin, neuron specific enolase (NSE), glial fibrillary acidic protein (GFAP), IRBP, neural adhesion molecule (N-CAM), and rod and cone specific transducin (TR alpha and TC alpha). Histopathology revealed mostly poorly differentiated tumors with necrosis and lack of Flexner-Wintersteiner rosettes. Immunocytochemical staining showed focal IRBP expression in one of the tumors and S-antigen in two cases. Immunoreactivity with rhodopsin was negative. N-CAM, a neural adhesive protein which appears to be involved in the regulation of adhesive interaction during neuronal differentiation, was positive except in two cases. All tumors showed immunoreactivity with NSE, whereas GFAP staining was limited to the perivascular glial tissue confirming the essential neuronal nature of retinoblastoma cells. TC alpha was detected in all tumors and TR alpha in one case. Messenger RNA for IRBP was detected in tumors in which IRBP immunoreactivity could not be detected.

Antibodies, Monoclonal↗
PubMed1994

Glial-, neuronal- and photoreceptor-specific cell markers in rosettes of retinoblastoma and retinal dysplasia.

Previous studies have shown that a rosette formation represents an attempt to form embryonic retinal tissue, primarily rods and cones. To test the theories as to the origin and characteristics of retinoblastoma cells, we compared the characteristics of tumor rosettes with those of dysplastic rosettes seen in retinal dysplasia using the glial, neuronal and photoreceptor markers. Forty-four retinoblastoma and one retinal dysplasia specimens were analyzed by indirect immunohistochemistry, using specific antibodies against glial fibrillary acidic protein, S-100 protein, myelin basic protein, neuron-specific enolase, neurofilament, retinal S-antigen and retinal pigment epithelial antigen. In human retinoblastoma, all the glial, neuronal, retinal pigment epithelial, and photoreceptor cell markers, except for the neurofilament, were present in parts of rosette-forming tumor cells. However, their localization was different for each antigen and it was not clear whether each tumor cell possesses several antigens. These immuno-positive tumor cells were cytologically indistinguishable from other rosette-forming cells at the light microscopic level. In retinal dysplasia, neuron specific enolase and retinal S-antigen were diffusely expressed in the dysplastic rosettes, however, other antigen were not seen in those rosettes. The staining pattern by immunocytochemistry is totally different in tumor rosettes from dysplastic ones. We found varying localizations of different immunoreactivities within tumor rosettes. These results led us to suggest that tumor cells in the rosettes of retinoblastoma may have the ability to differentiate into neural and glial cells. To prove the theory that retinoblastoma cells may have originated from a primitive neuroectodermal cell capable of multipotentiality, further investigation is needed.

Biomarkers, Tumor↗