Retinoblastoma is caused by mutational inactivation of both alleles of the RB1 gene, which maps to chromosome 13 q14 and encodes retinoblastoma protein that acts as a tumor suppressor. Histopathological high-risk feat...Retinoblastoma is caused by mutational inactivation of both alleles of the RB1 gene, which maps to chromosome 13 q14 and encodes retinoblastoma protein that acts as a tumor suppressor. Histopathological high-risk features of retinoblastoma are predictive of metastasis or local recurrence. The focus of this update is to emphasize the recent advances in pathology, various molecular key pathways and genome wide approaches for newer potential therapeutic future targets associated with retinoblastoma tumor biology. This review article highlights the new biomarkers expressed by the retinoblastoma tumor for the better survival of patients.展开更多
Intraocular lymphoma(IOL) is a rare lymphocytic malignancy which contains two main distinct forms. Primary intraocular lymphoma(PIOL) is mainly a subtype of primary central nervous system lymphoma(PCNSL). Alternativel...Intraocular lymphoma(IOL) is a rare lymphocytic malignancy which contains two main distinct forms. Primary intraocular lymphoma(PIOL) is mainly a subtype of primary central nervous system lymphoma(PCNSL). Alternatively,IOL can originate from outside the central nervous system(CNS) by metastasizing to the eye. These tumors are known as secondary intraocular lymphoma(SIOL). The IOL can arise in the retina,uvea,vitreous,Bruch's membrane and optic nerve. There are predominantly of B-cell origin; however there are also rare T-cell variants. Diagnosis remains challenging for ophthalmologists and pathologists,due to its ability to masquerade as noninfectious or infectious uveitis,white dot syndromes,or occasionally as other metastatic cancers. Laboratory tests include flow cytometry,immunocytochemistry,interleukin detection(IL-10: IL-6,ratio >1),and polymerase chain reaction(PCR) amplification. Methotrexate-based systemic chemotherapy with external beam radiotherapy and intravitreal chemotherapy with methotrexate are useful for controlling the disease,but the prognosis remains poor. Therefore,it is important to make an early diagnose and treatment. This review is focused on the clinical manifestations,diagnosis,treatment and prognosis of the IOL.展开更多
AIM:To study eyes with extraocular dissemination(EORB),with the following aims:first to establish the mean lag period and to understand various reasons for delayed presentation,second to study their imaging profiles a...AIM:To study eyes with extraocular dissemination(EORB),with the following aims:first to establish the mean lag period and to understand various reasons for delayed presentation,second to study their imaging profiles and third to analyze histopathological features of eyes enucleated after neoadjuvant chemotherapy.·METHODS:Prospective study of clinical and imaging features of EORBs(stage Ⅲ and Ⅳ International Retinoblastoma Staging System) presenting to a tertiary eye care centre.Histopathological features of eyes enucleated after receiving neoadjuvant chemotherapy were analyzed.A pictorial illustration of the varied imaging profile of EORB was also presented.·RESULTS:Over a period of one year,97 eyes were diagnosed with retinoblastoma;32 children(36 eyes)(37.1%) had EORB.Mean age 3.6±1.9 years,71.9% males,71.9% unilateral,3.1% with positive family history and40.6% with metastasis.On imaging,there was extrascleral involvement in 22.2%,involvement of orbital part of optic nerve in 33.3%,involvement of central nervous system in 27.8% and orbital wall involvement in2.9% eyes.On histopathological analysis of eyesenucleated after neoadjuvant chemotherapy,25.0% had no residual viable tumour tissue and rest all tumours were poorly differentiated.·CONCLUSION:There are very few human malignancies where definitive treatment is started without any confirmed histopathological diagnosis and imaging plays an important role in diagnosis and appropriate staging of the disease.Chemotherapy has a variable effect on EORB,75.0% of eyes with EORB had residual viable tumour tissue when enucleated after receiving neoadjuvant chemotherapy.展开更多
AIM: To investigate C-myc, Ki-67, pan-cytokeratin, and vimentin immunohistochemical features of carcinoma ex pleomorphic adenoma(Ca-ex-PA) and pleomorphic adenoma(PA) in the lacrimal gland in order to find some clues ...AIM: To investigate C-myc, Ki-67, pan-cytokeratin, and vimentin immunohistochemical features of carcinoma ex pleomorphic adenoma(Ca-ex-PA) and pleomorphic adenoma(PA) in the lacrimal gland in order to find some clues in the differential diagnosis between them.METHODS: We reviewed microscopic slides and clinical records of 64 cases of PA and 15 cases of Ca-ex-PA in the lacrimal gland. Immunohistochemical antibodies for C-myc, Ki-67, pan-cytokeratin, and vimentin were employed.RESULTS: Median age of PA was 43.2 y(from 21 to 75). The 35 patients(54.7%) were male and 29 patients(45.3%) were female. For the PAs, the average positivity of C-myc was 4.6%;the average proliferation index of Ki-67 was 3.2%;pan-cytokeratin was positive in ductal cells, and vimentin was positive in myoepithelial cells. Median age of Ca-ex-PA was 54.3 y(from 26 to 76). There were 7 male patients(46.7%) and 8 female patients(53.3%). Among 15 Ca-ex-PAs, there were 6 myoepithelial carcinomas, 4 adenocarcinomas, 3 epithelial-myoepithelial carcinomas, and 2 squamous cell carcinomas. For the Ca-ex-PAs, the average positivity of C-myc was 36.4%;the average proliferation index of Ki-67 was 29.2%;pan-cytokeratin was positive in all cases, and vimentin was positive in myoepithelial carcinomas.CONCLUSION: PA has a lower positivity of C-myc and Ki-67, while Ca-ex-PA had a higher positivity of these two biomarkers. These four biomarkers as a set could provide valuable clues in the differential diagnosis between Ca-exPA and PA. Our results indicate that the activation of C-myc could play an important role in the pathogenesis of Ca-exPA and PA.展开更多
AIM: To elucidate the mechanism of multidrug resistance in retinoblastoma, and to acquire more insights into in vivo drug resistance.METHODS: Three anticancer drug resistant Y79 human RB cells were generated against v...AIM: To elucidate the mechanism of multidrug resistance in retinoblastoma, and to acquire more insights into in vivo drug resistance.METHODS: Three anticancer drug resistant Y79 human RB cells were generated against vincristine, etoposide or carboplatin, which are used for conventional chemotherapy in RB. Primary cultures from enucleated eyes after chemotherapy(PCNC) were also prepared. Their chemosensitivity to chemotherapeutic agents(vincristine, etoposide and carboplatin) were measured using MTT assay. Western blot analysis was performed to evaluate the expression of p53, Bcl-2 and various multidrug resistant proteins in retinoblastoma cells.RESULTS: Following exposure to chemotherapeutic drugs, PCNC showed less sensitivity to drugs. No significant changes observed in the p53 expression, whereas Bcl-2 expression was found to be increased in the drug resistant cells as well as in PCNC. Increased expression of P-glycoprotein(P-gp) was observed in drug resistant Y79 cells; however there was no significant change in the expression of P-gp found between primary cultures of primarily enucleated eyes and PCNC. Multidrug resistance protein 1(Mrp-1) expression was found to be elevated in the drug resistant Y79 cells as well as in PCNC. No significant change in the expression of lung resistance associatedprotein(Lrp) was observed in the drug resistant Y79 cells as well as in PCNC.CONCLUSION: Our results suggest that multidrug resistant proteins are intrinsically present in retinoblastoma which causes treatment failure in managing retinoblastoma with chemotherapy.展开更多
文摘Retinoblastoma is caused by mutational inactivation of both alleles of the RB1 gene, which maps to chromosome 13 q14 and encodes retinoblastoma protein that acts as a tumor suppressor. Histopathological high-risk features of retinoblastoma are predictive of metastasis or local recurrence. The focus of this update is to emphasize the recent advances in pathology, various molecular key pathways and genome wide approaches for newer potential therapeutic future targets associated with retinoblastoma tumor biology. This review article highlights the new biomarkers expressed by the retinoblastoma tumor for the better survival of patients.
基金Supported by the National Natural Science Foundation of China(No.30371515)
文摘Intraocular lymphoma(IOL) is a rare lymphocytic malignancy which contains two main distinct forms. Primary intraocular lymphoma(PIOL) is mainly a subtype of primary central nervous system lymphoma(PCNSL). Alternatively,IOL can originate from outside the central nervous system(CNS) by metastasizing to the eye. These tumors are known as secondary intraocular lymphoma(SIOL). The IOL can arise in the retina,uvea,vitreous,Bruch's membrane and optic nerve. There are predominantly of B-cell origin; however there are also rare T-cell variants. Diagnosis remains challenging for ophthalmologists and pathologists,due to its ability to masquerade as noninfectious or infectious uveitis,white dot syndromes,or occasionally as other metastatic cancers. Laboratory tests include flow cytometry,immunocytochemistry,interleukin detection(IL-10: IL-6,ratio >1),and polymerase chain reaction(PCR) amplification. Methotrexate-based systemic chemotherapy with external beam radiotherapy and intravitreal chemotherapy with methotrexate are useful for controlling the disease,but the prognosis remains poor. Therefore,it is important to make an early diagnose and treatment. This review is focused on the clinical manifestations,diagnosis,treatment and prognosis of the IOL.
文摘AIM:To study eyes with extraocular dissemination(EORB),with the following aims:first to establish the mean lag period and to understand various reasons for delayed presentation,second to study their imaging profiles and third to analyze histopathological features of eyes enucleated after neoadjuvant chemotherapy.·METHODS:Prospective study of clinical and imaging features of EORBs(stage Ⅲ and Ⅳ International Retinoblastoma Staging System) presenting to a tertiary eye care centre.Histopathological features of eyes enucleated after receiving neoadjuvant chemotherapy were analyzed.A pictorial illustration of the varied imaging profile of EORB was also presented.·RESULTS:Over a period of one year,97 eyes were diagnosed with retinoblastoma;32 children(36 eyes)(37.1%) had EORB.Mean age 3.6±1.9 years,71.9% males,71.9% unilateral,3.1% with positive family history and40.6% with metastasis.On imaging,there was extrascleral involvement in 22.2%,involvement of orbital part of optic nerve in 33.3%,involvement of central nervous system in 27.8% and orbital wall involvement in2.9% eyes.On histopathological analysis of eyesenucleated after neoadjuvant chemotherapy,25.0% had no residual viable tumour tissue and rest all tumours were poorly differentiated.·CONCLUSION:There are very few human malignancies where definitive treatment is started without any confirmed histopathological diagnosis and imaging plays an important role in diagnosis and appropriate staging of the disease.Chemotherapy has a variable effect on EORB,75.0% of eyes with EORB had residual viable tumour tissue when enucleated after receiving neoadjuvant chemotherapy.
基金Supported by the National Natural Science Foundation of China (No.30371515)
文摘AIM: To investigate C-myc, Ki-67, pan-cytokeratin, and vimentin immunohistochemical features of carcinoma ex pleomorphic adenoma(Ca-ex-PA) and pleomorphic adenoma(PA) in the lacrimal gland in order to find some clues in the differential diagnosis between them.METHODS: We reviewed microscopic slides and clinical records of 64 cases of PA and 15 cases of Ca-ex-PA in the lacrimal gland. Immunohistochemical antibodies for C-myc, Ki-67, pan-cytokeratin, and vimentin were employed.RESULTS: Median age of PA was 43.2 y(from 21 to 75). The 35 patients(54.7%) were male and 29 patients(45.3%) were female. For the PAs, the average positivity of C-myc was 4.6%;the average proliferation index of Ki-67 was 3.2%;pan-cytokeratin was positive in ductal cells, and vimentin was positive in myoepithelial cells. Median age of Ca-ex-PA was 54.3 y(from 26 to 76). There were 7 male patients(46.7%) and 8 female patients(53.3%). Among 15 Ca-ex-PAs, there were 6 myoepithelial carcinomas, 4 adenocarcinomas, 3 epithelial-myoepithelial carcinomas, and 2 squamous cell carcinomas. For the Ca-ex-PAs, the average positivity of C-myc was 36.4%;the average proliferation index of Ki-67 was 29.2%;pan-cytokeratin was positive in all cases, and vimentin was positive in myoepithelial carcinomas.CONCLUSION: PA has a lower positivity of C-myc and Ki-67, while Ca-ex-PA had a higher positivity of these two biomarkers. These four biomarkers as a set could provide valuable clues in the differential diagnosis between Ca-exPA and PA. Our results indicate that the activation of C-myc could play an important role in the pathogenesis of Ca-exPA and PA.
基金the support of Department of Biotechnology, Govt. of India
文摘AIM: To elucidate the mechanism of multidrug resistance in retinoblastoma, and to acquire more insights into in vivo drug resistance.METHODS: Three anticancer drug resistant Y79 human RB cells were generated against vincristine, etoposide or carboplatin, which are used for conventional chemotherapy in RB. Primary cultures from enucleated eyes after chemotherapy(PCNC) were also prepared. Their chemosensitivity to chemotherapeutic agents(vincristine, etoposide and carboplatin) were measured using MTT assay. Western blot analysis was performed to evaluate the expression of p53, Bcl-2 and various multidrug resistant proteins in retinoblastoma cells.RESULTS: Following exposure to chemotherapeutic drugs, PCNC showed less sensitivity to drugs. No significant changes observed in the p53 expression, whereas Bcl-2 expression was found to be increased in the drug resistant cells as well as in PCNC. Increased expression of P-glycoprotein(P-gp) was observed in drug resistant Y79 cells; however there was no significant change in the expression of P-gp found between primary cultures of primarily enucleated eyes and PCNC. Multidrug resistance protein 1(Mrp-1) expression was found to be elevated in the drug resistant Y79 cells as well as in PCNC. No significant change in the expression of lung resistance associatedprotein(Lrp) was observed in the drug resistant Y79 cells as well as in PCNC.CONCLUSION: Our results suggest that multidrug resistant proteins are intrinsically present in retinoblastoma which causes treatment failure in managing retinoblastoma with chemotherapy.
