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Quiescent nasal T/NK cell lymphoma manifested as primary central nervous system lymphoma.

A 57-year-old man was diagnosed as primary T/NK-cell central nervous system lymphoma (CNSL) with intraocular involvement. However, review of a surgical specimen taken three years before for chronic paranasal sinusitis revealed an overlooked nasal T/NK cell lymphoma (TNKL), which showed similar histomorphology and immunophenotype with the CNS disease. Another patient, a 43-year-old woman, was initially diagnosed as a rare primary leptomeningeal T-cell lymphoma with ocular manifestation. Three years later, an isolated nasal TNKL emerged. Immunohistochemical and cytogenetic studies confirmed the same nature of the CNSL and the nasal TNKL. The nasal TNKLs of both patients had a strong expression of CD3, CD56, and Epstein-Barr virus antigens, but features of angiodestruction and mucosal ulceration were absent. We propose that: 1. a locally silent "quiescent" form of nasal TNKL may exist; and 2. a thorough examination and even blind biopsy of the nasal cavity is indicated when primary T/NK-cell CNSL is diagnosed.

Adult↗

Retinoblastoma and intracranial malignancy.

The occurrence of independent brain tumors in two patients with retinoblastoma is described. One patient with well-differentiated biliteral retinoblastomas developed, over two years later, a pineal tumor but no other metastatic lesions. The pineal tumor was composed of small neuroblastic cells and a second population of larger cells with vesicular nuclei and more abundant cytoplasm. This feature is not characteristic of either primary or metastatic retinoblastoma. In the second patient the symptoms of a brain tumor led to the discovery of a small uniocular, well-differentiated retinoblastoma. The brain tumor was retrochiasmal, highly differentiated (showing areas of photoreceptor differentiation), and interpreted as an ectopic nonmetastatic retinoblastoma. The possible histogenetic origins of the brain tumors are discussed. Patients who develop symptoms of a brain tumor after a prolonged interval since the treatment of their ocular tumors should be suspected of harboring a second intracranial primary.

Brain Neoplasms↗

Multiple primary neoplasms and the nervous system.

Studies of multiple primary neoplasms and their relation to the nervous system should consider two important principles: 1) neoplasms of the nervous system have certain unusual features that distinguish them from tumors occurring elsewhere in the body; and 2) there is good evidence that the various histologic types of nervous system tumors should be regarded as separate diseases. The association of nervous system neoplasms and tumors of other sites may occur in patients with phacomatoses or particular genetic syndromes. In addition, certain nervous system neoplasms may be multicentric in origin. Retinoblastoma and osteosarcoma occur together in the same patient more often than expected by chance, as do meningioma and breast cancer. These relationships are important in that they serve to identify the high risk patient, may provide etiologic clues, may point to the presence of genetic syndromes, and may highlight sites in which subsequent tumors are most likely to develop.

Brain Neoplasms↗

Familial retinoblastoma and ataxia telangiectasia: human models for the study of DNA damage and repair.

A variety of genetic diseases are associated with a high incidence of malignant tumors as compared to the general population. Laboratory data are presented that suggest that the increased cancer incidence in ataxia telangiectasia and familial retinoblastoma patients may be related to deficiencies in the normal DNA repair processes in their cells. Finally, we offer a hypothesis regarding the types of possible repair defects involved in these diseases and the biological consequences of such defects.

Ataxia Telangiectasia↗

Current status of clinical particle radiotherapy at Lawrence Berkeley Laboratory.

Clinical experience with charged particle irradiation of human cancers has been underway at the University of California Lawrence Berkeley Laboratory. Over 150 patients have been irradiated with heavy charged particle beams including helium, carbon, neon, and argon ions. Pilot studies have included such tumor sites as glioma of the brain, carcinoma of the esophagus, carcinoma of the pancreas, carcinoma of the stomach, ocular melanoma, and carcinoma of the uterine cervix. Prospective studies are planned to investigate the improved dose localization potential (helium) and the enhanced biologic and physical dose potential (carbon, neon) in a controlled trial against the best available megavoltage irradiation techniques.

Argon↗

Malignant intraocular tumors.

The role of the radiation therapist in the management of malignant intraocular tumors is changing. With more active identification of malignant intraocular tumors, and a better recognization of the manner in which one can deal with problems of radiation sensitivity, radiation techniques of all sorts will be more actively employed in the treatment of these tumors. Special techniques must be selected for appropriate circumstances of management in order to diminish to an absolute minimum the impact upon the lens, the impact upon visual acuity and the impact upon the cornea. Cobalt-60 plaques are being used more commonly in the treatment of melanomas of the choroid, and the role for radiation therapy in the management of retinoblastoma is changing markably to where it may be used as the primary treatment program rather than enucleation. In metastatic disease involving the uveal tract, radiation therapy has assumed the most important role for management. Chemotherapy should be considered as an active adjuvant in the management of not only those individuals with retinoblastoma but also in those identified circumstances where metastases to the uveal tract are being treated. The role for chemotherapy or immunotherapy in malignant melanoma is unclear.

Aged↗

The gene responsible for the development of retinoblastoma and osteosarcoma.

The gene responsible for the formation of both retinoblastoma and osteosarcoma recently has been isolated. This represents the first human recessive cancer gene ever cloned. Structural deletions within one or both retinoblastoma gene alleles were commonly noted in the retinoblastomas and an osteosarcoma. Whether or not changes were observed at the DNA level there was either no expression or an abnormal expression of the gene in the tumor. The fact that we could detect changes in the fibroblasts of some patients with the hereditary form of retinoblastoma also indicates that the gene can be used for diagnostic purposes.

DNA, Neoplasm↗

SCE and UDS studies in a family with retinoblastoma: a case study.

The frequencies of both spontaneous and mitomycin C (MMC)-induced sister chromatid exchanges (SCE) were analyzed in mixed lymphocyte cultures from a kindred with retinoblastoma. Both the affected daughter and male proband, as well as two normal controls, were studied. No differences were observed in the spontaneous or in the MMC-induced SCE frequencies obtained from the peripheral lymphocytes of these patients compared to the normal controls. Unscheduled DNA synthesis (UDS) was observed in the lymphocytes treated with varying concentrations of both MMC and M-methyl-N-nitro-N-nitrosoguanidine (MNNG). Whereas no differences were observed in the rate of [3H]thymidine incorporation in the retinoblastoma-derived lymphocytes exposed to MMC when compared to the controls, differences were seen in the response to MNNG. Our data suggest that cells from these retinoblastoma patients respond like normal cells to damage induced by MMC, but that they are unable to repair damage induced by MNNG.

Crossing Over, Genetic↗

Radiotherapy for extranodal, marginal zone, B-cell lymphoma of mucosa-associated lymphoid tissue originating in the ocular adnexa: a multiinstitutional, retrospective review of 50 patients.

BACKGROUND: Due to the small number of patients and differences in the pathologic classification in most radiotherapy series, information regarding the adequacy of tumor control in patients with ocular-adnexal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) is limited. METHODS: A multiinstitutional, retrospective study was performed on 50 patients with Stage IE ocular-adnexal MALT lymphoma who were treated with radiotherapy between 1989 and 1999. The impact of patient characteristics and other variables on tumor control was analyzed. RESULTS: Responses to radiotherapy include a complete response (CR) in 26 patients, a partial response (PR) in 20 patients, and no change in 4 patients. Forty-nine of 50 patients obtained tumor control in the ocular adnexa at 24 months. Overall, 6 patients exhibited disease recurrence at 4-97 months. Three patients developed recurrence in the ocular adnexa. Two patients had isolated extranodal failure involving the oral floor and the submandibular gland, and one patient experienced failure in the neck lymph node. The initial tumor response had a marginal impact on the development of recurrence. None of the 26 patients who achieved a CR experienced ocular-adnexal recurrence. All three patients who experienced local treatment failure belonged to the initial PR group. In total, five of six patients who developed recurrent disease had obtained a PR after initial radiotherapy. Age, gender, tumor location, and dose of radiotherapy did not influence the development of recurrence. There was only one death due to lymphoma. The 5-year overall survival rate was 91% with a median follow-up of 46 months. CONCLUSIONS: Radiotherapy offers excellent local control with a prolonged clinical course for patients with MALT lymphoma in the ocular adnexa. The initial response to radiotherapy marginally influenced the probability of recurrence.

Adult↗

Retinoblastoma cell cycling.

Techniques for the measurement of bromodeoxyuridine (BrdUrd) positive cells generally include either microscopic evaluation of paraffin embedded sections or measurements on cell suspensions using a fluorescent activated cell sorter. The accuracy of these measurements and their correlations can be affected by a number of technical and intrinsic tumor factors. Extrinsic parameters including degree of necrosis and tumor growth fraction are less easily analyzed in BrdUrd stained material. Retinoblastoma tumor cell cycling was prospectively studied in 11 children using in vivo and one child using in vitro BrdUrd. BrdUrd measurements were made by staining cell suspensions or sections of paraffin embedded tumor and analyzing by microscopy. Approximately 14% of viable cells were in the synthesis-phase of the cell cycle. The correlation between BrdUrd in cell suspensions and BrdUrd in paraffin embedded sections did not reach significance (r = 0.48). DNA analysis of these tumors was also performed using flow cytometry. Nine tumors were found to have a normal diploid DNA content, one had a G1 peak below the diploid control, two had a G1 peak above the diploid control, and one had two G1 peaks (a diploid and a hyperdiploid peak). There was no correlation between abnormal DNA content and the percent of cells in synthesis.

Bromodeoxyuridine↗

Bronchial malignant melanoma.

We describe a case of malignant melanoma presenting initially as an endobronchial lesion located in the left main bronchus causing total atelectasis. This resolved with radiation therapy. Widespread metastases developed shortly thereafter. The differential diagnosis of primary and metastatic bronchial malignant melanoma is discussed. Other isolated case reports are reviewed.

Bronchial Neoplasms↗

Expression of fibroblast growth factors in ultraviolet radiation-induced corneal tumors and corneal tumor cell lines from Monodelphis domestica.

Chronic exposure of the gray, short-tailed opossum, Monodelphis domestica, to ultraviolet radiation (UVR) induces highly vascularized mesenchymal tumors of the cornea. Cell lines derived from these UVR-induced corneal tumors and the corneal tumors themselves were examined for the presence of mRNA coding for basic and acidic fibroblast growth factors (FGF), transforming growth factors-beta and -alpha (TGF-beta and TGF-alpha), epidermal growth factor (EGF), and tumor necrosis factor-alpha (TNF-alpha). Basic FGF was expressed in the cell lines derived from corneal tumors and in the corneal tumors. Expression of basic FGF was high in one corneal tumor. Transcripts for acidic FGF were detected only in the corneal tumor cell lines, not in primary tumors. TGF-beta expression was detected in the corneal tumors and tumor-derived cell lines. TGF-alpha, EGF, and TNF-alpha transcripts were not detectable in any opossum material; however, homologous gene sequences for TGF-alpha and EGF were detected on Southern blots of opossum genomic DNA. Southern blot analysis revealed no evidence of amplification or rearrangement of the genes for basic FGF or acidic FGF in the UVR-induced corneal tumor that expressed high levels of basic FGF. Opossum basic FGF, which stimulated the proliferation of fetal bovine heart endothelial cells, was purified by heparin affinity chromatography from a UVR-induced corneal tumor and a corneal tumor cell line. Immunoblotting of opossum basic FGF from a corneal tumor cell line using antiserum to bovine basic FGF showed two prominent immunoreactive bands of 17.5 and 18.5 kDa. Expression of basic FGF and acidic FGF may play a role in the development and progression of UVR-induced corneal tumors in M. domestica.

Animals↗