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At least 397 records · Page 22Linked to original sources
PubMed2003

Treatment of overt extraocular retinoblastoma.

BACKGROUND: Overt extraocular retinoblastoma is common in developing countries and little information about its treatment is available. The aim of this study is to report our experience in the treatment of these cases using a uniform approach. PROCEDURE: Patients with overt extraocular retinoblastoma including orbital extension, preauricular lymph node invasion and/or metastatic disease on diagnosis or after extraocular relapse admitted to the Hospital JP Garrahan from August 1987 to December 2000 were retrospectively reviewed. Patients were treated according to two different protocols (1987-1993 and 1994-2000). Treatment included: neoadjuvant combination chemotherapy followed by limited surgery in case of orbital extension (enucleation or resection of residual orbital mass) and adjuvant chemotherapy and radiotherapy. Chemotherapy included cyclophosphamide, vincristine, etoposide, doxorubicin (in protocol 87), idarubicin (in protocol 94), cisplatin (in protocol 87), and carboplatin (in protocol 94). RESULTS: Forty-one patients were included. Fifteen of them had orbital or preauricular disease and had a 5-year event-free survival (pEFS) of 84%. Twenty-six had distant metastatic disease and non survived 5-years. One patient died of toxicity and one died in complete remission. One patient had a secondary leukemia. The remaining adverse events included CNS and/or systemic relapse. CONCLUSIONS: This treatment strategy was highly efficacious for patients with orbital and/or lymph node extension. Orbital exenteration is not necessary for these patients. Those patients with distant metastatic or CNS disease were not curable with this approach.

Adolescent↗
PubMed2008

SRPK1: a cisplatin sensitive protein expressed in retinoblastoma.

Chemotherapy is an essential modality in the treatment of retinoblastoma (RB). Mammalian serine/arginine-rich protein-specific kinase 1 (SRPK1) is a cisplatin-sensitivity-related protein and its downregulation is known to be associated with decreased response to cisplatin and carboplatin. We investigated the expression of SRPK1 in 63 archival RB and correlated its expression with pathologic staging and exposure to chemotherapy. The majority of the RB (62/63) were advanced stage (Groups D and E) with intermediate to high risk of treatment failure according to the new international classification for intraocular RB and SRPK1 was reduced in 32/62 (51%) tumors. SRPK1 protein expression was reduced in (100%) 8/8 RB that had recurred in the orbit or had metastasized. SRPK1 protein expression is reduced in RB with advanced stage of presentation and this may add to drug resistance mechanisms in RB.

Adolescent↗
PubMed2005

Pars plana lensectomy and intraocular lens implantation in pediatric radiation-induced cataracts in retinoblastoma.

OBJECTIVE: To investigate visual outcomes, surgical complications, and tumor recurrence among children with retinoblastoma (RB) undergoing pars plana lensectomy, vitrectomy, and simultaneous intraocular lens insertion for radiation-related cataract secondary to external beam radiotherapy (EBRT). DESIGN: Retrospective, noncomparative, consecutive case series. METHODS: The medical records for all patients treated with pars plana lensectomy, vitrectomy, and posterior chamber intraocular lens implantation for radiation-induced cataract after EBRT for RB at a single institution between January 1, 1990, and December 31, 2000, were reviewed. PARTICIPANTS: The study included 16 eyes of 12 children with Reese-Ellsworth stage V RB. MAIN OUTCOME MEASURES: Visual acuity, surgical and postoperative complications, postoperative refraction, and tumor recurrence. RESULTS: The median age at diagnosis of RB was 6 months (range, 1-49 months). All patients received EBRT as primary treatment and experienced radiation-induced cataracts. The median interval from RB diagnosis to cataract surgery was 42 months (range, 28-95 months). Preoperative mean visual acuity was 20/400, with 12 of 16 eyes (75%) having macular tumor involvement. All patients underwent pars plana lensectomy, vitrectomy, and posterior chamber intraocular lens insertion. Vitreous samples were evaluated by cytopathologic examination, and no viable tumor cells were identified in any of the vitreous specimens. Postoperative complications included transient cystoid macular edema in 5 eyes (31%) and iridocyclitis in 3 eyes (19%). The mean follow-up after cataract surgery was 66 months (range, 30-94 months). Final visual acuity was 20/40 or better in 11 of 16 eyes (69%). No late intraocular recurrence, orbital tumors, or metastatic disease was noted in this study. CONCLUSIONS: Pars plana lensectomy, vitrectomy, and simultaneous intraocular lens implantation is an effective means of managing EBRT-induced cataracts in eyes with previously treated RB. There was no evidence of active tumor in eyes undergoing surgery at least 28 months after the diagnosis and commencement of therapy for RB, and no late intraocular, orbital, or metastatic tumors were detected. Visual acuity was limited by the presence of primary macular tumor pathologic features in a subset of patients, but final vision better than 20/400 may be achieved in these eyes.

Cataract↗
PubMed2003

Radiation-induced chorioretinal degeneration: a clinicopathological report of three cases.

BACKGROUND: With the shift in radiotherapy toward the posterior segment and with the use of lead screens to shield the anterior segment, posterior segment lesions have become more readily recognized. The purpose of this study is to highlight the effects of ionizing radiation on the choroid and to demonstrate how this can result in visual loss, particularly if the macula is involved. METHODS: Histopathological study of three enucleated eyes of three patients who had received ionizing radiation: a 27-year-old woman who had received radiation as a child for a hemangioma of the left side of the face, a 16-year-old girl who had received radiation at age 11 years for a malignant mesenchymoma of the right maxilla, and a 4-year-old girl who had received radiation at age 1 year for a retinoblastoma of the right eye. RESULTS: Histopathological examination of the three globes showed extensive chorioretinal degeneration, among other ocular findings. In all cases the fellow eye did not show similar chorioretinal lesions. As all three patients were relatively young, the degree of chorioretinal degeneration was considered to be secondary to radiation treatment. INTERPRETATION: Vascular damage from ionizing radiation is not limited to the retina. It can also affect the choroid in the form of chorioretinal degeneration. Since most of the intraocular circulation arises from the uveal vessels, chorioretinal degenerative lesions may be extensive and may even involve the macula.

Adult↗
PubMed1998

Management of massive orbital involvement of intraocular retinoblastoma.

OBJECTIVE: This study aimed to evaluate various combinations of therapeutic approaches in an attempt to improve the poor outcome of advanced cases of orbital involvement of intraocular retinoblastoma. DESIGN: The study design was a retrospective, uncontrolled descriptive case series of consecutive patients with massive orbital retinoblastoma treated and observed at a referral center. PARTICIPANTS: Sixteen patients with unilateral orbital involvement of intraocular retinoblastoma were studied. Eight patients had orbital involvement at initial presentation and eight children presented with orbital tumor after enucleation for intraocular retinoblastoma. Patients having microscopic evidence of tumor invasion at surgical section of the optic nerve and subsequently receiving other forms of treatment were not included in the study. INTERVENTION: Six patients had orbital exenteration followed by external beam radiation therapy (4600-6000 cGy in fractionated doses) and chemotherapy. Five patients received chemotherapy only. Three patients were treated by exenteration and chemotherapy, whereas two patients underwent radiation therapy and chemotherapy. MAIN OUTCOME MEASURES: Tumor regression monitored by clinical observations and imaging studies and patient survival was studied. RESULTS: The median follow-up was 22 months (range, 4-168 months). All forms of treatment caused satisfactory local and systemic tumor regression. Two children achieved a survival of more than 10 years. However, two patients in the exenteration followed by chemotherapy and radiation therapy group and one who received chemotherapy only had central nervous system metastases develop and died. Only one patient was lost to follow-up. CONCLUSION: Highly individualized aggressive treatment by combining radical surgery, chemotherapy, and external beam radiation therapy allowed most of the authors' patients to have longer survival and disease-free intervals than expected.

Chemotherapy, Adjuvant↗
PubMed2004

Diagnosis of intraocular lymphoma.

PURPOSE: To review current techniques for the diagnosis of intraocular lymphoma. METHODS: Literature review. RESULTS: There are a limited number of small case series and case reports reporting the diagnosis of intraocular lymphoma. Limitations in the ability to diagnose lymphoma through cytologic examination of the vitreous has led to investigations into immunohistochemistry either manually or by automated cytofluorography, intraocular cytokine determinations, and polymerase chain reaction of the vitreous or tissue lymphoid cells to detect gene rearrangements in the IgH, bcl-2, or T-cell receptor gamma gene. Data regarding diagnostic efficiency of the various testing strategies is limited by small patient numbers. Careful handling of the vitreous specimen and adequate laboratory and pathological support is critical. CONCLUSIONS: Despite a proliferation of new techniques for the diagnosis of intraocular lymphoma, none is clearly superior. Use of multiple testing strategies may improve diagnostic yield.

Diagnostic Techniques, Ophthalmological↗
PubMed1998

Identification and characterization of families with aggregation of lung cancer.

BACKGROUND: To clarify genetic factors involved in the susceptibility to lung cancer, it is essential to identify families with lung cancer clustering and to characterize the mode of clustering. Since somatic mutations of the p53, RB and p16 genes occur frequently in lung cancer and the replication error phenotype is seen in a subset of lung cancer, it is possible that germ-line mutations of the p53, RB, p16 and mismatch repair genes influence the susceptibility to lung cancer. METHODS: In this work, cases with familial clustering of lung cancer were selected from 1068 families with primary lung cancer cases in analogy with the criteria for hereditary non-polyposis colorectal cancer (HNPCC). Cases with Li-Fraumeni syndrome, familial retinoblastoma, familial melanoma and HNPCC were also searched among these 1068 families. RESULTS: There were only four families (0.4%) in which more than three relatives were affected by lung cancer. Two successive generations were affected in 36 families (3.4%). Patients with lung cancer before the age of 50 were present in 165 families (15.5%). However, no family conformed to all three criteria. There was only one family with Li-Fraumeni syndrome and no family with familial retinoblastoma, familial melanoma and HNPCC. CONCLUSION: Familial aggregation of lung cancer is rare and germ-line mutations of the p53, RB, p16 and mismatch repair genes may not contribute greatly to susceptibility to lung cancer.

Colorectal Neoplasms, Hereditary Nonpolyposis↗
PubMed2000

CD31 immunoreactivity in small round cell tumors.

CD31 has been shown to be a sensitive and specific marker for endothelial differentiation among epithelioid and spindled-pleomorphic human neoplasms. However, the role of this marker in the evaluation of small round cell tumors has not been evaluated. Formalin-fixed, paraffin-embedded tissue sections from 276 small round cell tumors, including 85 Ewing's sarcoma/primitive neuroectodermal tumors (ES/PNET), 52 rhabdomyosarcomas, 10 extraabdominal polyphenotypic small cell tumors, six desmoplastic small cell tumors, 11 neuroblastomas, 23 Wilms' tumors, 20 retinoblastomas, 13 esthesioneuroblastomas, and 56 small cell malignant lymphomas were stained with CD31 (JC/70A, 1:40), using a modified avidinbiotin-peroxidase complex technique, after citrate buffer microwave epitope retrieval. Among nonlymphoid small round cell tumors, four of 85 ES/PNET were at least focally reactive. No other lesion in this group was positive. In contrast, the majority of well-differentiated (11 of 17), intermediately differentiated (two of three), and lymphoblastic lymphomas (three of three) were positive. Small cleaved lymphomas (three of 13 follicular, one of 13 diffuse) were less often reactive, whereas small noncleaved lesions were negative. Although reactivity for CD31 in ES/PNET is uncommon, the presence of platelet/endothelial cell adhesion molecule in a small cell neoplasm should not in isolation be taken as evidence of hematopoietic origin. These results further define the utility of CD31 in the evaluation of human neoplasms.

Biomarkers, Tumor↗
PubMed2005

[Histopathologic findings in retinoblastoma].

OBJECTIVE: To study histopathological findings of enucleated eyes with retinoblastoma. METHODS: Twenty-eight cases of retinoblastoma treated by enucleation at the Federal University of São Paulo from December 2000 to October 2002 were histopathologically reviewed. Clinical data included age, gender, race, unilateral or bilateral involvement and previous treatment. The histopathological review evaluated the presence of iris and/or angle neovascularization, tumor differentiation and optic nerve and choroidal invasion according to Khelfaoui's classification. RESULTS: Of 27 patients, 13 (48.5%) were boys and 14 (59.3%) were girls, 16 were white, 6 were black and 5 were asiatic, age ranging from 2 to 96 months (mean, 22.7 months). 13 cases were bilateral and 14 cases were unilateral. All tumors were histologically characterized by a proliferation of small cells with high nuclear-to-cytoplasmic ratios and 20 (71.4%) of them were well differentiated. Choroidal involvement was observed in 18 (64.2%) cases (degree II, III) and optic nerve invasion in 8 (28.5%) cases (degree III, IV, V). CONCLUSION: Neovascularization, necrosis and calcification were the most commonly observed feature. The invasion into the optic nerve and choroid, which are the two most important predictors of patient outcome were found in 28.5% and 64.2% of the cases, respectively.

Adolescent↗
PubMed2006

Extensively necrotic retinoblastoma is associated with high-risk prognostic factors.

CONTEXT: Retinoblastoma is the most common malignant intraocular tumor in children. It has been shown that adjuvant therapy following enucleation in patients with high-risk histopathologic features significantly decreases the mortality. We describe the association of extensive necrosis of tumor and intraocular structures with 2 of the major risk factors: optic nerve invasion and choroidal invasion. This may alert the pathologist who makes the observation of extensive necrosis to carefully search for histologic features associated with adverse outcome. OBJECTIVE: To determine whether extensively necrotic retinoblastoma is associated with high-risk histologic prognostic factors for metastatic disease and patient survival. DESIGN: Retrospective case series. Forty-three eyes of 43 patients with retinoblastoma who underwent enucleation between 1990 and 2001 were evaluated. Medical records, histopathology specimens, pathology reports, and clinical photographs were reviewed. Tumors were designated as exhibiting extensive necrosis if more than 95% of tumor cells and intraocular tissues were necrotic. The main outcome measure was the association of extensive tumor necrosis with 3 high-risk histopathologic features: extraocular extension, optic nerve invasion, or choroidal invasion. Metastatic disease, patient survival, and associations with pathologic findings were also analyzed. RESULTS: Optic nerve head invasion (P < .001), post-lamina-cribrosal invasion (P < .001), and choroidal invasion by tumor (P = .004) were observed more frequently in eyes with extensive necrosis compared with eyes without extensive necrosis. Two of the 11 patients with extensively necrotic intraocular retinoblastoma died from metastatic disease (P = .06). None of the 32 patients without extensive necrosis developed metastatic disease or died. CONCLUSIONS: Extensive ocular tissue and tumor necrosis is associated with histologic high-risk prognostic factors for tumor metastasis and mortality.

Brain Neoplasms↗