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PubMed2006

Expressions of Rac1, Tiam1 and Cdc42 in retinoblastoma.

The Rho GTPases are the molecular regulators of the cell motility processes and are involved in cell cycle progression and gene transcription. We studied the expression of Rho-like GTPases molecules, particularly Rac, Tiam1 and cdc42, in retinoblastoma and correlated these with clinicopathological parameters of the tumors. Sixty-seven tumors were included which were divided in to two groups; group A: tumors with optic nerve/choroidal/orbital invasion (n=35) and group B: tumors with no invasion (n=32). Immunohistochemistry was done on paraffin sections for all the proteins and were confirmed by Western blot on fresh tumor samples. In group A tumors, Rac was positive in 10/35 (28%), cdc42 was positive in 12/35 (34%) and Tiam1 was positive in 30/35 (85%) tumors. In group 2 tumors, Rac was positive in 5/32 (15%), cdc42 was positive in 4/32 (12%) and Tiam1 was positive in 30/32 (93%) tumors. Two groups (both invasive and non-invasive tumors) showed decreased expression of Rac1 and cdc42 whereas Tiam1 was significantly expressed in invasive tumors compared to non-invasive tumors (P<0.0001). We observed a 70K cleavage product of Tiam1 along with an 110K product by blotting in RB samples. Caspase-3 was also demonstrated in RB samples, which showed Tiam1 cleavage products. This is the first study that showed the expression patterns of Rac, cdc42 and Tiam1 in retinoblastoma tumors. Thus, further studies are required to prove the involvement of caspase-3 in the cleavage of Tiam1 in vitro in RB cells and to trace out alternative pathways involved in tumor progression.

Adolescent↗
PubMed2005

Chemotherapy for retinoblastoma.

Retinoblastoma is the most common eye cancer in children. Pilot studies of chemotherapy for intraocular retinoblastoma have been reported by several groups, using different combinations, dosages, schedules, and durations of carboplatin, etoposide, or teniposide, with or without vincristine, and with or without cyclosporine to counteract multidrug resistance. All studies of chemotherapy for intraocular retinoblastoma have included consolidation by focal therapy, with or without radiation. Chemotherapy alone reduces tumor size but does not cure retinoblastoma. Focal therapy, consisting of photocoagulation, thermotherapy, cryotherapy, or brachytherapy, is necessary to consolidate chemotherapy response.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed2002

Retinoblastoma metastatic to the ovary in a patient with Waardenburg syndrome.

PURPOSE: To report a child with retinoblastoma and Waardenburg syndrome who developed ovarian metastases. DESIGN: Interventional case report. METHODS: Unilateral retinoblastoma was diagnosed in a 3-year-old girl with Waardenburg syndrome and leukocoria in the right eye. The patient had a Reese-Ellsworth Group Va tumor and underwent enucleation. Two years later, she developed metastatic disease involving the bone marrow, right humerus, both supraorbital bones, and both tibias. She was treated with chemotherapy, orbital irradiation, and bone marrow transplant but returned 7 months later with back pain and urinary retention. RESULTS: Exploratory laparotomy revealed a right ovarian mass, and the excised ovary showed metastatic retinoblastoma. The child underwent chemotherapy and remained asymptomatic for 9 months, when brain metastases were diagnosed. She died within 2 days of admission. CONCLUSION: We believe that this is the first description of a patient with retinoblastoma and Waardenburg syndrome and of an ovarian metastasis from retinoblastoma.

Bone Neoplasms↗
PubMedDate not supplied

Retinoblastoma in Taiwan: survival and clinical characteristics 1978-2000.

PURPOSE: Few estimates of the survival rates ofwas retinoblastoma have been reported from the Asia region. In this study, we aim to describe the survival and clinical characteristics of 96 retinoblastoma cases treated at Chang Gung Medical Center, Taipei, between 1978 and 2000. METHODS: We retrospectively analyzed the clinical records of 96 children (116 eyes) diagnosed with retinoblastoma and treated between 1978 and 2000. Information on sex, laterality, age at diagnosis, presenting signs, spread of tumor, treatment modality, survival rate, and family history were collected. RESULTS: Seventy-six (79.2%) cases were unilateral and 20 (20.8%) were bilateral. The mean age overall at the time of diagnosis was 24.7 months; in unilateral cases, 27.1 months; and in bilateral cases, 15.6 months. The most common presenting signs were leukocoria (75 cases, 78.1.0%), buphthalmos (34 cases, 35.4%), proptosis (16 cases, 16.7%), and strabismus (12 cases, 12.5%). Forty-two eyes had orbital extension, 27 patients had central nervous system invasion, 16 cases exhibited bone marrow involvement, and 3 cases had liver metastasis. Three (3.1%) patients had a family history of retinoblastoma. None of the cases developed a secondary neoplasm. The 3-year cumulative survival rate of the 96 patients was 64.41% (unilateral, 71.97%; bilateral 40.01%). CONCLUSIONS: The mortality was much higher than that in reports on Western and Japanese patients. Delayed diagnosis with frequent extraocular spread at the time of diagnosis caused the low survival rate. Fewer familial cases were encountered in our study than in other studies.

Aging↗
PubMed1998

Treatment of retinoblastoma vitreous base seeding.

OBJECTIVE: The purpose of the study is to describe results of a new treatment for retinoblastoma vitreous base seeding. DESIGN: The study design was a retrospective review of patients treated at an ocular oncology referral center. PARTICIPANTS: Five eyes of five patients with vitreous base seeding that occurred after previous external beam radiation therapy were treated between October 1987 and December 1994. INTERVENTION: A customized plaque made from iridium-192/platinum wire was placed to deliver 4000 cGy to the tumor apex along its entire length and systemic chemotherapy (consisting of carboplatin, vincristine, and etoposide) was given. MAIN OUTCOME MEASURES: Eye preservation and tumor control were measured. RESULTS: Vitreous base seeding was controlled completely and the eye preserved in four of the five treated eyes with an average follow-up of 26.2 months. CONCLUSIONS: The combination of a customized iridium-192 plaque and systemic chemotherapy is an effective means of treating vitreous base seeding of retinoblastoma.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed2000

Engraftment and growth of patient-derived retinoblastoma tumour in severe combined immunodeficiency mice.

The development of an in vivo model of retinoblastoma could be important for studying its biological behaviour and developing novel therapeutic strategies. We examined the ability of patient-derived retinoblastoma cells to grow and disseminate in severe combined immunodeficiency CB-17-SCID mice after subcutaneous (s.c.) inoculation without conditioning treatment. 24/30 (80%) of patient-derived tumours engrafted and grew as s.c. nodules in SCID mice. Whilst most xenografted tumours appeared to be localised, by PCR assay a positive DNA band of human minisatellite region (YNZ.22) was determined in the bone marrow of 19/25 (76%), in the spleen of 14/25 (56%) and in the liver of 16/25 (64%) mice, respectively, indicating dissemination to distant organs. Cytogenetic analysis demonstrated i(6p) in 5/12 (42%) and trisomy 1 or 1q abnormalities in 8/12 (67%) of the xenografted tumour samples studied, respectively, suggesting that retinoblastoma tumour cells maintain their cytogenetic abnormalities following adoptive growth in SCID mice. In this report we demonstrate the ability to propagate human primary retinoblastoma cells in SCID mice after s.c. inoculation and suggest the possibility of using the SCID mouse model to study the intrinsic biological behaviour of human retinoblastoma and to develop novel therapeutic strategies in the treatment of this disease.

Animals↗
PubMed2001

MNU induction of neoplasia in a platyfish model.

Interspecific hybrid crosses between members of the fish genus Xiphophorus have been used for over 70 years to study the genetic aspects of melanoma formation. In the well-established "Gordon-Kosswig" cross, the platyfish X. maculatus is outcrossed to the swordtail X. helleri, and the resulting backcross segregants spontaneously develop melanoma. We recently produced a distinct cross between X. maculatus and another platyfish species, X. couchianus. X. maculatus strain Jp 163 A is homozygous for several X-linked pigment pattern genes, including the Spotted dorsal (Sd), Dorsal red (Dr), and Anal fin spot (Af). Af is a sex-limited trait, coding exclusively for melanophores distributed on the modified anal fin or "gonopodium" in the adult male fish. Within F1 and BC1 hybrids (to X. couchianus), the Sd pigment pattern is phenotypically suppressed, whereas Dr and Af are enhanced. We exposed BC1 hybrids to the direct-acting carcinogen N-methyl-N-nitrosourea (MNU). Treatment led to the development of schwannomas, fibrosarcomas, and retinoblastomas. In addition, numerous MNU-treated males that inherited Af developed a pronounced melanotic phenotype, with melanin-containing cells oftentimes totally covering the gonopodium and extending further to grow within the ventral regions of the fish. Genetic linkage analysis of the BC1 hybrids revealed a significant (p < 0.01) association between CDKN2X genotype and the phenotypic degree of melanization. Such an association is consistent with a locus within linkage group V playing a role in the development of melanosis and delineates three genetic preconditions and a carcinogenic scheme resulting in melanosis of the ventral regions of hybrid fish. The overall study further alludes to the potential of using Xiphophorus fish to study carcinogenic mechanisms for tumors other than melanoma (schwannoma, fibrosarcoma, and retinoblastoma) and should enable extensive pathologic and molecular genetic studies of derived neoplastic abnormalities.

Alkylating Agents↗
PubMed2006

RB family members as predictive and prognostic factors in human cancer.

The retinoblastoma family members--pRb, pRb2/p130 and p107--are tumor suppressor genes involved in controlling four major cellular processes: growth arrest, apoptosis, differentiation and angiogenesis. Molecular genetic studies have identified abnormalities of these tumor suppressor genes in a large proportion of human cancers. These genetic alterations have emerged as significant factors in the pathogenesis and progression of many types of tumors and are therefore likely to provide relevant information to assess risk in cancer patients. There is a pressing clinical need to identify prognostic and predictive factors for patients with cancer, because there is an undeniable importance in being able to determine which patients will have a favorable outcome without further therapy (prognostic factor) and which will need some additional treatment (predictive factor). This review examines the predictive and/or prognostic role of each retinoblastoma family member in human cancer.

Female↗
PubMed2002

Epidemiological characteristics of retinoblastoma in children attending the Mexican Social Security Institute in Mexico City, 1990-94.

The object of this study is to present descriptive epidemiological characteristics of retinoblastoma (Rb) in children aged 0-14 years, seen at the Mexican Social Security Institute hospitals in Mexico City (MC) from 1990 to 1994. This is a retrospective, observational hospital survey. Clinical records of 52 Rb cases were reviewed; 39 were patients who did not reside in MC (non-residents), and 13 were MC residents. The study period was 1990-94. The male/female ratio (M/F) was 1.6. Average annual incidence (AAI) was estimated by age and sex (rates per 1000000). Annual average percentage change (AAPC) in incidence rates was estimated in children from 0 to 14 years. The AAI for MC residents was 3.2; the highest rate being for those <1 year olds (rate of 20.8); AAPC was 6.9% [95% CI -27.5, 57.4]; the highest incidence was for the south-eastern region of MC residents (rate of 5.9); 13 cases (25%) were diagnosed at stage III or IV, and 11 cases (21.2%) were bilateral. Incidence of Rb is similar to that in developed countries and shows no increasing trend. Patients from communities outside MC are more frequently diagnosed at stages III and IV.

Adolescent↗
PubMed2003

Questioning the need for routine bone marrow aspiration and lumbar puncture in patients with retinoblastoma.

PURPOSE: This study assesses the value of routinely investigating children with retinoblastoma with bone marrow aspiration and lumbar puncture, staging investigations not without risk and trauma to the patient, emotional stress on parents and financial cost to the community. METHODS: Medical files and specimens were obtained and examined for patients with retinoblastoma presenting to the Royal Alexandra Hospital for Children, Camperdown and the Children's Hospital at Westmead, Sydney, from 1975 to 2001. RESULTS: In total, 123 patients presented; 62 (50.4%) were boys and 61 (49.6%) were girls. Of these 123 patients, 74 (60.2%) had unilateral disease, 46 (37.4%) involving the left eye and 28 (22.8%) involving the right eye. There were 47 (38.2%) patients with bilateral disease, and two (1.6%) with trilateral disease. Mean age of presentation was 17.9 months (23.1 months for unilateral subjects; 10.3 months for bi-lateral subjects; 3.5 months for trilateral subjects). There were 13 (10.6%) with a positive family history. Of 74 unilateral subjects, 70 (94.6%) required enucleation and four (5.4%) were salvaged. Of 47 bilateral subjects, 13 (27.7%) did not require enucleation, 27 (57.4%) required unilateral enucleation and seven (14.9%) required bilateral enucleation. Both trilateral subjects died. Of all 123 subjects, 112 (91.1%) had bone marrow aspiration and lumbar puncture performed during initial assessment, and none showed evidence of malignancy. CONCLUSIONS: Given the small but significant risks associated with these procedures, the results of this study cannot support bone marrow aspiration and lumbar puncture as routine investigations in all patients presenting with retinoblastoma, suggesting a more limited usage of these investigations is warranted.

Biopsy, Needle↗
PubMed1999

B16LS9 melanoma cells spread to the liver from the murine ocular posterior compartment (PC).

PURPOSE: To create a murine ocular melanoma model that consistently metastasizes to the liver. METHODS: Twelve-week-old C57BL6 mice (n=10) were inoculated in the posterior compartment (PC) of one eye with 5x10(5) tissue culture B16LS9 melanoma cells. The inoculated eyes were enucleated at two weeks and the mice were sacrificed with necropsies performed at four weeks post-inoculation. RESULTS: Melanoma grew and was confined to the eyes of all 10 mice. The melanoma hematogenously spread to the lungs in 9 of 10 mice and the liver in 8 of 10 mice. There was a positive correlation between pulmonary and liver metastasis (r=0.94). CONCLUSIONS: B16LS9 melanoma cells consistently hematogenously spread to the liver when implanted into C57BL6 mice eyes. The value of this model is unknown at this time since it is not known if the intrahepatic melanoma is capable of growing and killing the host.

Animals↗
PubMed1999

Retinoblastoma with low risk for extraocular relapse.

OBJECTIVE: To define a subgroup of patients with retino-blastoma and low risk of extraocular relapse through histopathological and clinical variables. PATIENTS AND METHODS: Inclusion criteria consisted of stage I (intraocular disease), stage IIb1 (without concomitant choroid and/or scleral invasion), and nonenucleated patients (according to the Grabowski-Abramson classification). A total of 112 consecutive patients admitted to Hospital JP Garrahan from 1987 to 1997 were evaluable. Treatment included enucleation or local therapy and no chemotherapy. RESULTS: Forty-one patients had stage Ia (intraretinal), 8 stage Ib (prelaminar optic nerve invasion), 40 stage Ic (uveal invasion), and 12 stage IIb1 (postlaminar optic nerve invasion and cut end free of tumor). Eleven patients had neither eye enucleated. Median follow-up was 60 months. Only two events occurred: one patient had progressive disease in the contralateral globe and died of CNS metastasis and another had an orbital relapse that was successfully treated. Both had choroidal invasion. Five-year pEFS and pOS were 0.97 and 0.98, respectively. Neither length of the optic nerve stump, tumor size, anterior chamber invasion, degree of differentiation, nor degree of ocular coat invasion correlated with increased risk of metastasis. CONCLUSIONS: A subset of patients with retinoblastoma with low risk of relapse can be determined using histopathological evaluation of the invasion of ocular coats. Adjuvant chemotherapy is not warranted for patients with intraretinal extension and prelaminar optic nerve invasion. It is also probable that those patients with isolated choroidal invasion and those with postlaminar optic nerve extension with surgical margins clear of tumor do not need chemotherapy.

Antineoplastic Agents↗
PubMed2006

Retinoblastoma: expression of HLA-G.

PURPOSE: Human leukocyte antigen (HLA) mediates interactions of tumor cells with cytotoxic T lymphocytes (CTLs) and natural killer (NK) cells. Retinoblastoma (RB) is the most common intraocular malignant tumor in childhood and is characterized by direct spread to the optic nerve and orbit as well as hematogenous and lymphatic spread. Earlier, we observed that invasive RB showed reduced HLA, which could contribute to its escape from the immune system. Little is known about the role of the nonclassical HLA molecule, HLA-G, in RB and its role in tumor escape mechanisms in RB. METHODS: Forty archival paraffin-embedded RB tumors were analyzed for the expression of HLA-G by immunohistochemistry using a monoclonal antibody; fresh tumor samples were also subjected to Western blot analysis. There were 22 tumors with no invasion and 18 with invasion of the choroid/optic nerve. Immunoanalysis was performed based on the International Histocompatibility Working Group Project Description. RESULTS: HLA-G was negative in the non-neoplastic retina, reduced in 22/22 tumors with no invasion, and positive in 15/18 with invasion. The immunohistochemistry results were confirmed by Western blot analysis. The difference in expression between the two groups was significant ( p < 0.001). There was no correlation of HLA-G expression with differentiation of the tumors. CONCLUSION: Increased expression of HLA-G was observed in invasive RB. This preliminary observation deserves further investigation and may shed more light on the immune escape mechanisms of this tumor and thus enable novel therapeutic strategies.

Antibodies, Monoclonal↗
PubMed2006

Von Hippel-Lindau disease: molecular pathological basis, clinical criteria, genetic testing, clinical features of tumors and treatment.

von Hippel-Lindau (VHL) disease is an autosomal dominant disorder that is associated with various tumors and cysts in the central nervous system (CNS) and other visceral organs. Inactivation of the VHL tumor suppressor protein with loss of function of the VHL protein, and Elongin B, C complex results in a dysfunction of the ubquitination of hypoxia-inducible factor, which is an important step in the development of highly vascular tumors. The most frequent tumors are hemangioblastoma in the CNS and retina, pheochromocytoma in the adrenal gland, renal cell carcinoma and pancreatic neuroendocrine tumors. In this review, we summarize the recent literatures on the pathogenesis, clinical characteristics, diagnosis and treatment of VHL disease. Progress in molecular diagnosis and molecular targeting therapy is expected to improve the diagnosis and treatment of this disease. Medical, psychological and societal supports for patients with VHL disease and their families and supportive communication among the VHL families are also very important. They have proved to be of benefit for patients with this disease to overcome various social and psychological problems in the US and Europe. Since some drugs targeting the vascular endothelial growth factors or its receptor are undergoing clinical trials, a better prognosis of the tumor in VHL disease can be expected.

Adrenal Gland Neoplasms↗
PubMed1998

Diffuse anterior retinoblastoma.

PURPOSE: The authors describe the clinicopathologic features of an anterior variant of diffuse retinoblastoma. METHODS: The clinical history of a child with an anterior chamber infiltrate and cells in the anterior vitreous was reviewed. An anterior chamber aspirate was processed by a Millipore filter technique. The eye was enucleated and routinely processed for light and transmission electron microscopic examination. RESULTS: An 8-year-old girl was treated for anterior uveitis in her right eye that failed to resolve. Examination of an anterior chamber aspirate showed cells suggestive of retinoblastoma. The eye was enucleated, and the enucleation specimen showed retinoblastoma confined to the iris, ciliary body, and anterior vitreous with one microscopic focus of tumor in the peripheral retina. CONCLUSION: This variant of diffuse retinoblastoma involved only anterior ocular structures with minimal involvement of the retina.

Anterior Chamber↗
PubMed2004

Continuing challenges in the management of retinoblastoma with chemotherapy.

The management of retinoblastoma has gradually changed over the past 10 years. Over 95% of children with retinoblastoma in the United States are cured with modern techniques. The challenge remains, however, in maintaining the eye and vision. There is a trend away from enucleation and external beam radiotherapy toward focal conservative treatments involving primary chemoreduction in conjunction with thermotherapy and cryotherapy. This is related to earlier detection of the disease, recognition of more effective chemotherapeutic agents, more focused local treatment modalities, and, most importantly, knowledge of the long-term risks of external beam radiotherapy. Enucleation is still preferable for retinoblastoma that fills most of the eye, especially when the disease is unilateral or when there is concern for tumor invasion into the optic nerve, choroid, or orbit. The orbital integrated implant is placed after enucleation and provides acceptable prosthesis motility and appearance. External beam radiotherapy is still vital for treating advanced retinoblastoma, especially when there is diffuse vitreous or subretinal seeding after failure of other methods and preservation of vision is a priority. The most important recent advance in the management of retinoblastoma is the use of intravenous chemotherapy for tumor reduction, a technique of neoadjuvant chemotherapy termed "chemoreduction." This is followed by tumor consolidation with focal measures such as thermotherapy, cryotherapy, and plaque radiotherapy. This strategy provides reduced tumor volume and often permits consolidation with methods other than radiotherapy. It appears that vision can be preserved in some cases with these methods, avoiding some of the local complications like radiation cataract or macular edema that have been found with radiotherapy techniques. External beam radiotherapy and enucleation can now be avoided in most cases of Reese-Ellsworth groups I (minimal disease) through IV (moderate disease) retinoblastoma. The most advanced stage of retinoblastoma, Reese-Ellsworth group V, continues to provide the greatest difficulty for management, and external beam radiotherapy and enucleation are often employed in addition to chemoreduction to save the child's life. A collaborative prospective study in North America is currently under way to further study the benefits and risks of chemoreduction for minimal, moderate, and advanced retinoblastoma.

Antineoplastic Agents↗