Cases reported "Eye Neoplasms"

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1/17. Reconstruction of the ocular surface by transplantation of a serum-free derived cultivated conjunctival epithelial equivalent.

    PURPOSE: The purpose of this study was to investigate the use of a serum-free derived cultivated conjunctival epithelial sheet for ocular surface transplantation and reconstruction. methods: Seven subjects with various ocular surface disorders were selected for the procedure: one patient had an extensive conjunctival nevus, three patients had pterygium, two patients had persistent leaking trabeculectomy blebs, and one patient had bilateral superior limbic keratoconjunctivitis. Conjunctival epithelial cells were harvested from the forniceal conjunctiva of patients 2 weeks before the definitive surgery. Cultivation of conjunctival epithelial cells on human amniotic membrane (HAM) was carried out under serum-free conditions. At the time of transplantation, the area of diseased conjunctiva was excised and the cultured conjunctival epithelium-HAM composite was transplanted onto the surgical defect. patients were followed up with serial slit-lamp examinations, fluorescein staining, and photographic documentation. RESULTS: A confluent stratified conjunctival epithelial sheet was formed on the HAM within 12 to 14 days. Transplanted grafts remained well-epithelialized after surgery. A successful outcome, defined as resolution of the disease, maintenance of conjunctival epithelialization, maintenance of graft integrity, and absence of significant complications, was obtained in all seven patients. A good functional and cosmetic result was achieved in all eyes. The mean follow-up period was 11.6 months (range, 6-18 months). CONCLUSIONS: transplantation of a serum-free derived autologous cultivated conjunctival epithelial sheet on HAM was successfully performed in seven patients with ocular surface disorders. This may provide a novel method for conjunctival replacement in conditions where the normal conjunctiva is damaged or deficient.
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2/17. Loss of 10p material in a child with human papillomavirus-positive disseminated bilateral retinoblastoma.

    retinoblastoma (RB) is a malignant childhood tumor that results from loss or inactivation of both alleles of the RB1 gene. Human papillomavirus (HPV) dna sequences have been found in RB tissue, suggesting a role of the viral infection with RB. We here describe a child with disseminated bilateral RB without familial history, who displayed a loss of material from 10p. fluorescence in situ hybridization studies showed a somatic loss of both alleles of the RB1 gene. Moreover, sequences for HPV-6a were detected on dna extracted from eye tumor tissue and from nonstimulated peripheral blood leukocyte cultures. The eye tumor tissue was also positive for HPV L1 viral proteins. Repeated loss of the short arm of chromosome 10 in HPV-transfected keratinocytes has been reported. Loss of heterozygosity in 10p14 approximately p15 is also frequent in cervical cancers. Therefore, it seems probable that the abnormalities on 10p detected in the present case are related to the HPV infection. Thus, HPV could be a cofactor in the progression of RB by promoting nonrandom additional mutations.
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3/17. anterior chamber paracentesis cytology (cytospin technique) for the diagnosis of intraocular lymphoma.

    AIM: To report on the diagnosis of intraocular lymphoma by aqueous cytology. methods: Four patients suspected of having intraocular lymphoma were evaluated by anterior chamber (AC) paracentesis with cytology (cytospin technique). All had a history of non-ocular lymphoma and presented with at least one plus anterior chamber cells despite intensive glucocorticoid therapy. A 25 gauge needle was inserted through clear cornea (bevel up), over the iris stroma, so as to drain the AC. The aqueous humour was sent for cytopathology (cytospin technique), culture, and sensitivity tests. RESULTS: All procedures were diagnostic. Three were lymphoma and the fourth was culture positive for propionibacterium endophthalmitis. No secondary glaucoma, hyphaema, cataract or infections were related to AC paracentesis. CONCLUSIONS: In this series, AC aspiration cytology enhanced by the cytospin technique was an effective, minimally invasive alternative to vitrectomy based biopsy. This technique should be considered to rule in the diagnosis of intraocular lymphoma in selected cases with cells in the anterior chamber.
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4/17. Pseudomeningocele as a complication of teratoma resection and aseptic meningitis following craniofacial reconstruction: a case report.

    This is a report of two extremely unusual complications of craniofacial surgery on a single patient. A 14-year-old female underwent resection of a recurrent teratoma four times within the first 9 years of life. This left her with two large cranial cavities in the left temple and posterior to the orbit communicating with the subarachnoid space constituting a pseudomeningocele and pulsatile mass in the left temple--an unusual complication. Following resection of the pseudoepithelial cavity linings, the spaces were filled with a split temporalis muscle. Two weeks following surgery, she developed signs and symptoms of meningitis with negative cultures from the drain sites and lumbar puncture. Six days following an unsuccessful attempt to treat her with wide spectrum antibiotics, she was treated with dexamethasone. As a result, there was a dramatic disappearance of signs and symptoms in less than 36 hours; she has not experienced any recurrence since. We feel that this aseptic meningitis was the result of cerebrospinal fluid exposure to muscle--a rare, yet, previously reported complication. The details of the case history and discussion of complications and the way in which they can be avoided, are subjects of this report.
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5/17. Increased number of chromosome aberrations in the peripheral blood culture of a retinoblastoma patient.

    Chromosome studies were performed on blood lymphocytes from an eight-year-old patient whose left eye had been enucleated earlier because of retinoblastoma. GTG-banded karyotypes showed both numerical and structural chromosome aberrations, and the number of the patient's lymphocytes with chromosome aberration increased. It was concluded that retinoblastoma survivors need continuous control because of the increased risk of second primary tumors.
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6/17. Translocation (X;13)(p11.21;q12.3) in a girl with incontinentia pigmenti and bilateral retinoblastoma.

    A de novo t(X;13)(p11.21;q12.3) translocation is described in an 19-month-old girl with incontinentia pigmenti (IP) and bilateral retinoblastoma. Based on previously reported two girls and this patient, each with a structural x chromosome abnormality and IP, it was assumed that the locus for IP is at Xp11.21. Q-banding analysis revealed that the translocated chromosomes were of paternal origin. The derivative x chromosome was late-replicating in 9% of cultured peripheral blood lymphocytes and in 1% of skin fibroblasts. The erythrocyte esterase D activity in the patient was normal. Several possibilities were considered for possible causative relationship between the X/13 translocation and the development of retinoblastoma. One possibility involved functional monosomy of 13q14 in a minority of retinoblasts due to the spreading of inactivation of the translocated x chromosome segment.
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7/17. Glial cell component in retinoblastoma.

    The right eye of a 4-month-old girl with a large, unilateral, sporadic retinoblastoma was enucleated. The tumor was unusual because it contained Flexner-Wintersteiner rosettes with extremely large lumina. Smaller rosettes and undifferentiated tumor cells were observed within the lumina. Also of importance were cells resembling glial cells which were intermixed with more typical cuboidal retinoblastoma cells. These cells had electron microscopic features typical of glial cells and stained positively for glial fibrillary acidic protein in immunohistochemical studies. Rosettes and glial cells continued to be observed in the tumor carried in tissue culture through two passages over a 7-month period. This tumor is presented because of its unusual rosette structures and because it confirms recent reports describing a glial cell component in retinoblastoma.
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8/17. Immunohistochemical evidence for preproenkephalin A synthesis in human retinoblastoma.

    Human retinoblastoma contains clusters of cells immunoreactive for methionine-enkephalin and methionine-enkephalin-arginine-phenylalanine. Some tumour cells also exhibited methionine-enkephalin-arginine-glycine-leucine-like immunoreactivity. The results are in agreement with those obtained with similar testing of neuroblastoma cell cultures. It is concluded that some human retinoblastoma cells are capable of synthesizing preproenkephalin A or parts of this molecule.
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9/17. Histopathological and tissue culture studies of a melanizing cell line derived from a retinoblastoma.

    Histological examination of the enucleated eye of a 7-mth-old child revealed a retinoblastoma with areas of rosette formation as well as focal areas of melanin pigmentation. biopsy derived cells readily established a continuous cell line in liquid culture. The cells which have now been cultured continuously for over 3 yr, were shown to be malignant by being non-contact inhibitable, by readily forming colonies in semi-solid nutrient agar medium and by producing tumours in nude mice. When grown to the point of overcrowding in liquid culture the cells became heavily melanized. Electron microscopy of the melanized cultured cells showed the melanin to be contained in melanosomes. These findings suggest that retinoblastomas may be derived from bipotential primitive retinal cells which retain the capacity for both nuclear cell and pigment cell differentiation. Alternatively, separate malignant transformations may have occurred in each of 2 different progenitor cell types committed to a separate differentiation pathway. The clinical behaviour of this tumour has not differed from that expected of non-pigmented retinoblastomas.
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10/17. Location of the retinoblastoma susceptibility gene(s) and the human esterase D locus.

    retinoblastoma occurs with increased frequency in children born with a deletion of the long arm of chromosome 13. Recent reviews have noted that the region 13q14 is consistently deleted in documented cases. prometaphase and late prophase banding allowed Yunis and Ramsay to determine that a deletion in one patient included the sub-bands q14 . 12, q14 . 13, and q14 . 2, and a portion of q14 . 11 and q14 . 3. We report the results of similar cytogenetic techniques applied in the case of a 26 month old Caucasian female with unilateral retinoblastoma, moderate developmental delay, and subtle dysmorphology. prometaphase banding of cultured skin fibroblasts revealed the karyotype: mos46,XX/46,XX,del(13)(q13 . 1q14 . 11). Only the sub-band q14 . 11 is deleted in both our patient and that of Yunis and Ramsay. The results are consistent with the localisation of the retinoblastoma susceptibility gene(s) in the sub-band 13q14 . 11. Electrophoretic analysis and activity assays of red blood cell esterase D are consistent with hemizygous expression of that marker in our proband. Comparison with published esterase D analyses in families with retinoblastoma permits the assignment of the esterase D locus to that same sub-band, 13q14 . 11.
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