1/13. retinoblastoma with acute lymphoblastic leukemia, polyposis coli, and multiple hamartomas.It has long been recognized that compared with their age- and sex-matched controls, survivors of hereditary retinoblastoma have a considerably higher risk of the development of second malignancies (10% at 20 years and 15% at 30 years of follow-up), including osteosarcoma, leiomyosarcoma, melanoma, fibrosarcoma, and other rare spindle cell sarcomas. patients with the nongenetic variety of retinoblastoma do not particularly seem to have an increased incidence of other malignancies than the general population. However, it should be noted that a child with unilateral disease carries a 15% chance of having germline mutation. The cumulative mortality rate from second malignancies was 1.5% at 40 years after unilateral retinoblastoma diagnosis and 26% for bilateral cases in a large survey of 1458 patients. A child with unilateral retinoblastoma, cafe au lait spots, hairy nevus, and grouped pigmentation of retina in the fellow eye is described who furthermore developed acute leukemia and polyposis coli.- - - - - - - - - - ranking = 1keywords = leukemia (Clic here for more details about this article) |
2/13. osteosarcoma following retinoblastoma: age at onset and latency period.In order to assess the role of genetic predisposition in the induction of radiation-induced tumors, we performed statistical analysis on data from the literature and from our own Institute with regard to the age at onset and the latency period of osteosarcoma as the second primary tumor for retinoblastoma with or without subsequent radiotherapy. In retinoblastoma survivors who subsequently developed osteosarcoma, the age at onset of retinoblastoma was young (average of 12 months) in both unilateral and bilateral forms. This suggests that all or almost all of the patients were genetically predisposed by a mutation of one allele of the RB1 gene. For retinoblastoma patients, osteosarcomas occurred 1.2 years earlier inside than outside the radiation field. The latency period between radiotherapy and osteosarcoma onset was 1.3 years shorter inside than outside the radiation field. Interestingly, a bimodal distribution of latency periods was observed for osteosarcomas arising inside, but not outside the radiation field: 40% occurred after a short latency, while the latency of the remaining 60% was comparable to that of osteosarcoma occurring outside the radiation field. This suggests that different mechanisms may be involved in radiocarcinogenesis. A radiation-induced mutation of the second RB1 allele may be the cause of osteosarcomas occurring after a short delay, while other genes may be affected in those occurring after a longer delay.- - - - - - - - - - ranking = 8.8128220633911keywords = radiation-induced (Clic here for more details about this article) |
3/13. Masquerade syndrome: T-cell prolymphocytic leukemia presenting as panuveitis.PURPOSE: To report a case of T-cell prolymphocytic leukemia with panuveitis as the primary presenting feature. methods: Case report. RESULTS: A 46-year-old woman presented with pain and blurred vision in the right eye. She was found to have signs of panuveitis with a central exudative retinal detachment. Further investigations revealed that she was suffering from the rare T-cell prolymphocytic leukemia. Both systemic and ocular manifestations of the disease resolved after chemotherapy with Campath-IH antigen and as she went into complete remission. The exudative detachment settled, and visual acuity recovered to 20/20. CONCLUSION: This case illustrates that leukemias can present with primarily ocular findings, and the sudden appearance of a serous retinal detachment with inflammatory signs in an otherwise healthy person warrants a thorough systemic screening for an underlying malignancy.- - - - - - - - - - ranking = 1.4keywords = leukemia (Clic here for more details about this article) |
4/13. Human T-cell lymphotropic virus type-1 associated t-cell leukemia/lymphoma masquerading as necrotizing retinal vasculitis.OBJECTIVE: To report a case of adult T-cell leukemia/lymphoma (ATL) presenting as a bilateral retinal vasculitis and diagnosed by molecular detection of a rearrangement in the T-cell receptor (TCR) and the presence of the human T-cell lymphotropic virus type 1 (HTLV-1) pol gene in the malignant lymphoid cells. DESIGN: Case report. methods: Routine histologic and immunohistochemical analyses were performed on the retinal biopsy specimen before referral to the National eye Institute. Lymphoid cells associated with granulomatous inflammation infiltrating the retina and surrounding retinal blood vessels were microdissected from the paraffin sections of the retinal biopsy specimen. The polymerase chain reaction (PCR) was performed using primers for the TCR gene and HTLV-1 pol and gag genes. RESULTS: Microscopic examination showed a necrotizing granulomatous retinal vasculitis with a predominant T-cell infiltrate detected by immunohistochemistry. Molecular analysis demonstrated a clonal rearrangement of the TCR and the presence of the HTLV-1 pol gene in the microdissected lymphoid cells diagnostic of ATL. CONCLUSIONS: Necrotizing retinitis and retinal vasculitis are rare manifestations of ATL. Human T-cell lymphotropic virus type 1 infection should be considered in the differential diagnosis of patients from endemic areas who have retinal vasculitis at presentation. This case further demonstrates the usefulness of microdissection and PCR for the diagnosis of ocular disease, including HTLV-1 infection.- - - - - - - - - - ranking = 1keywords = leukemia (Clic here for more details about this article) |
5/13. Resolution of leukemic retinopathy following treatment with imatinib mesylate for chronic myelogenous leukemia.PURPOSE: To report a case of leukemic retinopathy before and after treatment with imatinib mesylate (formerly STI-571) for chronic myelogenous leukemia (CML). DESIGN: Interventional case report. methods: A 58-year-old man diagnosed with stable phase CML (SP-CML) presented with blurry vision. Fundoscopy revealed several flame-shaped hemorrhages in the macular region in both eyes. One month after this initial visit, imatinib therapy was initiated. RESULTS: : The patient noticed improvement in his visual as well as his medical symptoms; on repeat examination 6 months after the initial visit, the retinal hemorrhages had resolved and remained so after 18 months. CONCLUSIONS: Imatinib appears to be an effective treatment for SP-CML, and the improvement in visual and medical symptoms in our case report correlates with this.- - - - - - - - - - ranking = 1keywords = leukemia (Clic here for more details about this article) |
6/13. Peripheral retinal nonperfusion associated with chronic myeloid leukemia.PURPOSE: To report a case of peripheral retinal nonperfusion and chronic myeloid leukemia in a 23-year-old woman. DESIGN: Observational case report. methods: A complete ophthalmic and systemic evaluation was performed. RESULTS: Ophthalmic examination revealed peripheral retinal nonperfusion with retinal neovascularization in both eyes. fluorescein angiography of both eyes showed a marked midperipheral and peripheral avascular retina temporally with arteriovenous anastomosis and seafan neovascularizations. Blood work showed no abnormalities, although marked leucocytosis (up to 750 x 10(9)/l) and thrombocytosis (646 x 10(9)/l) were present in 1998 when the patient was diagnosed with leukemia. Following treatment, the patient has been in remission. CONCLUSIONS: Peripheral retinal nonperfusion with retinal neovascularization may occur as a complication of chronic myeloid leukemia. In contrast to other studies describing this association, our patient had a bilateral peripheral retinal nonperfusion with seafan neovascularizations without relapse of the myeloid leukemia and without any of the other retinal signs associated with chronic myeloid leukemia, such as tortuosity of veins, intraretinal or preretinal hemorrhages, and cotton-wool exudates.- - - - - - - - - - ranking = 1.8keywords = leukemia (Clic here for more details about this article) |
7/13. Reconstruction of a complex hemifacial deformity with multiple simultaneous free-flap transfers: case report.Severe disfigurement, facial paralysis, abnormal continuity of oral and nasal passages, and velopharyngeal incompetence resulted, following maxillectomy and cranial-base resection for a radiation-induced sarcoma. Oral lining, bone support, facial muscle, and outer skin were provided with a single-staged transfer of radial forearm, scapular bone, scapular skin, and serratus anterior muscle with anastamoses to the contralateral neck. This combination of flaps may be considered in complex facial deformities, and may obviate the need for staged prefabrication.- - - - - - - - - - ranking = 4.4064110316955keywords = radiation-induced (Clic here for more details about this article) |
8/13. Cataract surgery and intraocular lens implantation in a retinoblastoma case treated by external-beam radiation therapy.We report a case of extracapsular cataract extraction with posterior chamber intraocular lens implantation in a pediatric patient with a radiation-induced cataract that developed after combined external-beam radiation therapy and chemotherapy for retinoblastoma. A 12-year follow-up revealed effective tumor regression and sustained visual recovery.- - - - - - - - - - ranking = 4.4064110316955keywords = radiation-induced (Clic here for more details about this article) |
9/13. Visual recovery after radiation therapy for bilateral subfoveal acute myelogenous leukemia (AML).PURPOSE: Present a case of bilateral foveal acute myelogenous leukemic tumors that responded to radiation therapy. DESIGN: Case report. methods: A patient was diagnosed with bilateral subfoveal infiltration of known systemic acute myelogenous leukemia. He received a standard induction chemotherapy, followed by consolidation therapy for his systemic leukemia. However, despite a complete marrow response, the intraocular tumors did not regress. Therefore, he was given low dose (1950-cGy) ocular external beam radiation therapy. RESULTS: One course of systemic cytarabine chemotherapy failed to control the subfoveal tumors, leaving the patient at risk for permanent vision loss. In contrast, external beam radiation therapy improved his vision from 20/60 in his right eye to 20/20 and from 20/70 in his left eye to 20/25. CONCLUSION: Although systemic chemotherapy can be used to treat intraocular metastasis, external beam radiation may provide more prompt resolution of vision-threatening tumors.- - - - - - - - - - ranking = 1.2keywords = leukemia (Clic here for more details about this article) |
10/13. Visual loss as a first sign of adult-type chronic myeloid leukemia in a child.PURPOSE: To report the unusual case of retinal leukemic infiltration in a child with adult-type chronic myelocytic leukemia. DESIGN: Interventional case report. methods: Complete ophthalmologic examination, including visual acuity measurement and funduscopy. The diagnosis was established by blood cell counts and confirmed by a positive philadelphia-chromosome translocation (t [9,22]). Treatment consisted of hydroxyurea and stem-cell transplantation. RESULTS: After therapy, complete regression of the retinal lesion was observed with full recovery of visual acuity. CONCLUSION: This case underlines the importance of interdisciplinary cooperation between ophthalmologists and pediatric oncologists in the diagnosis and treatment of this disease.- - - - - - - - - - ranking = 1keywords = leukemia (Clic here for more details about this article) |
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