1/52. Ocular-central nervous system lymphoma mimicking posterior scleritis with exudative retinal detachment.OBJECTIVE: We describe an unusual ocular presentation of ocular-central nervous system lymphoma in a young patient. DESIGN: Interventional case report and literature review. methods: A previously well 24-year-old white woman presented with left eye pain and reduced vision. Episcleral injection, globe tenderness, an afferent pupil defect, and exudative retinal detachment were present. Computed tomographic scan of the head and orbits demonstrated scleral thickening, retinal detachment, and no other abnormality. A provisional diagnosis of posterior scleritis with exudative retinal detachment was made. Investigation for underlying connective tissue diseases was negative. There was an initial prompt response to corticosteroid therapy. The patient's symptoms and signs then recurred, and a left third cranial nerve palsy developed. Systemic investigations including lumbar puncture ultimately led to the diagnosis of primary T-cell central nervous system (CNS) lymphoma. serologic tests for human immunodeficiency virus were negative. MAIN OUTCOME MEASURES AND RESULTS: The patient underwent orbital and cranial irradiation and intrathecal and systemic chemotherapy. Despite an initial response to treatment, she returned with a recurrence of the lymphoma in the anterior segment of the left eye. Her systemic disease progressed rapidly, and she died shortly thereafter. CONCLUSIONS: This patient's young age and initial presentation mimicking posterior scleritis with unilateral exudative retinal detachment, without evidence of vitreous involvement, are highly unusual for ocular involvement in primary CNS lymphoma. A review of the literature highlights the atypical nature of this presentation.- - - - - - - - - - ranking = 1keywords = nervous system (Clic here for more details about this article) |
2/52. Bilateral testicular lymphoma treated with chemotherapy and radiation without orchiectomy: complete response relapsed at 52 months in the vitreous humor.We report the first detailed case of testicular lymphoma managed with chemotherapy and radiation without orchiectomy. A 60-year-old man with Stage II extralymphatic bilateral testicular lymphoma refused orchiectomy, but underwent cyclophosphamide, doxorubicin, vincristine, and prednisone chemotherapy and radiation. He remained disease free for 52 months, when a solitary central nervous system relapse to the vitreous humor was diagnosed. The optimal therapy for testicular lymphoma is unclear but often includes orchiectomy with adjuvant chemotherapy and radiation. Stage I testicular lymphoma can be cured by surgery alone; however, the relapse rates for all stages of testicular lymphoma are high despite systemic therapy. For Stage II disease and higher, chemotherapy/radiation is recommended; orchiectomy may not be mandatory.- - - - - - - - - - ranking = 0.16666666666667keywords = nervous system (Clic here for more details about this article) |
3/52. Epstein-Barr virus-associated lymphoma in a child undergoing an autologous stem cell rescue.Epstein-Barr virus-associated lymphoproliferative disease (EBV-LPD) is a serious disorder seen in various states of immunodeficiency, often with a fatal outcome. In this article, a patient with EBV-lymphoma after autologous stem cell rescue for treatment of a nonhematologic solid tumor is described. The child, a 4-year-old boy, had unilateral retinoblastoma with metastatic spread to the central nervous system. He had previously received both local tumor bed and craniospinal radiation therapy together with intensive myeloablative alkylator chemotherapy before autologous stem cell rescue. Histologically confirmed lymphoma with evidence of active EBV proliferation developed within cervical lymph nodes 3 weeks after his first autologous stem cell rescue. A complete clinical remission of the lymphadenopathy was obtained after infusions of rituximab (an anti-CD20 monoclonal antibody), acyclovir, and high-titer anticytomegalovirus immunoglobulin. The patient died approximately 6 months later of persistent and progressive retinoblastoma without any clinical evidence of lymphoma. It is concluded that EBV-LPD should be included in the differential diagnosis in patients in whom lymphadenopathy develops after autologous stem cell rescue.- - - - - - - - - - ranking = 0.16666666666667keywords = nervous system (Clic here for more details about this article) |
4/52. Metastatic cutaneous melanoma to the vitreous cavity masquerading as intermediate uveitis.PURPOSE: To report a patient with metastatic cutaneous melanoma (MCM) presenting as intermediate uveitis. methods: We examined a 49-year-old man with malignant cutaneous melanoma and central nervous system (CNS) metastasis who was initially treated for bilateral intermediate uveitis. Biomicroscopic examination disclosed a normal anterior segment and vitreous organization with red blood cells (RBC) and some non-pigmented cells in both eyes. Funduscopy disclosed a retinal metastasis in the right eye, but vitreous organization precluded visualization of the retina in the left eye. RESULTS: The first diagnostic vitrectomy was negative for malignant cells in the left eye. However, progressive worsening of the condition persuaded us to repeat vitrectomy in the left eye and to do it in the right eye too. Vitreous samples were positive for malignant melanoma cells in both eyes. CONCLUSIONS: Metastatic cutaneous melanoma to the vitreous cavity is exceedingly rare and may masquerade as intermediate uveitis. It should be considered in the differential diagnosis of unusual uveitis and masquerade syndromes.- - - - - - - - - - ranking = 0.16666666666667keywords = nervous system (Clic here for more details about this article) |
5/52. Intrathecal chemotherapy for recurrent central nervous system intraocular lymphoma.PURPOSE: To report the use of intrathecal chemotherapy for two patients with recurrent central nervous system (CNS) intraocular lymphoma. DESIGN: Two interventional case reports. PARTICIPANTS: The clinical course of two patients with documented recurrent CNS intraocular lymphoma were retrospectively reviewed. INTERVENTION: Both patients had previously undergone external beam radiation therapy to the orbit and brain, as well as systemic methotrexate (MTX) and intrathecal cytarabine (Ara-C), for primary CNS intraocular lymphoma. Both patients developed recurrent CNS intraocular lymphoma and treatment involved intrathecal MTX and Ara-C delivered by means of an Ommaya reservoir. MAIN OUTCOME MEASURE: survival and resolution of intraocular lymphoma. RESULTS: Complete remission of the CNS intraocular lymphoma was seen in both patients. Both patients maintain reading visual acuity in at least one eye. Both patients remain disease free with lymphoma in remission for greater than 5 years after initial diagnosis. CONCLUSIONS: Intrathecal chemotherapy for recurrent CNS intraocular lymphoma seems to be effective in preserving vision and possibly increasing survival.- - - - - - - - - - ranking = 0.83333333333333keywords = nervous system (Clic here for more details about this article) |
6/52. Encephalocraniocutaneous lipomatosis: a case with unilateral odontomas and review of the literature.We report a 7-year-old boy with the typical features of encephalocraniocutaneous lipomatosis (ECCL) including unilateral skin, eye, bone, and asymptomatic central nervous system involvement. The presenting sign were numerous ipsilateral odontomas which have been described in only two previous patients so far. We reviewed all 32 cases reported to date and focus on the dermatological hallmark of the condition, namely a fatty tissue naevus of the scalp for which the term naevus psiloliparus was recently coined. Sporadic occurrence, patchy type of organ involvement and roughly balanced sex ratio in ECCL are compatible with the concept of a lethal autosomal mutation only surviving in a mosaic state. CONCLUSION: In children presenting with a congenital smooth hairless lesion on the scalp, the diagnosis of naevus psiloliparus should be considered and other signs of encephalocraniocutaneous lipomatosis should be looked for.- - - - - - - - - - ranking = 0.16666666666667keywords = nervous system (Clic here for more details about this article) |
7/52. acquired immunodeficiency syndrome-related primary intraocular lymphoma.A 37-year-old man with acquired immunodeficiency syndrome and cytomegalovirus retinitis developed primary intraocular and central nervous system lymphoma. Intraocular involvement was documented before death with vitrectomy. autopsy demonstrated the presence of cytomegalovirus retinitis in the right eye and lymphoma in both eyes and the brain. We believe this is the first report of autopsy-confirmed primary intraocular lymphoma in a patient with the acquired immunodeficiency syndrome.- - - - - - - - - - ranking = 0.16666666666667keywords = nervous system (Clic here for more details about this article) |
8/52. retinoblastoma, microphthalmia, coloboma, and neuroepithelioma of the pineal body.A one-month-old infant boy was examined early in life because his mother had bilateral retinoblastoma and his father had bilateral microphthalmia. The ophthalmologist found his right eye was normal size with a coloboma of the iris, choroid, and retina. The left eye was microphthalmic with a coloboma of the uveal tract and retina. A vascularized fluffy white mass in the posterior pole was diagnosed clinically as a retinoblastoma. The tumor regressed with radiation. When the patient was four years of age, a large tumor was found in the region of the pineal recess, causing hydrocephalus and seizures. A biopsy showed an undifferentiated malignant neuroepithelial neoplasm. The patient died within three months of diffuse central nervous system tumor. The unusual findings of a retinoblastoma in a microphthalmic eye with bilateral colobomas and a neuroepithelial neoplasm of the pineal gland are discussed.- - - - - - - - - - ranking = 0.16666666666667keywords = nervous system (Clic here for more details about this article) |
9/52. Applications and limitations of vitreoretinal biopsy techniques in intraocular large cell lymphoma.The cases of 4 patients with clinical signs of intraocular large cell lymphoma are described. Initial cytopathologic examination of vitrectomy specimens failed to establish the malignant character of the vitreous infiltrates. Three of the four patients eventually developed solid central nervous system tumors, intracranial biopsy samples of which revealed large cell lymphoma, 13 months to 42 months after initial examination. In one patient, transscleral retinochoroidal biopsy confirmed the diagnosis at the same time as negative vitreous cytologic examination. Results of cytopathologic examination alone of vitreous biopsy specimens may not be sufficient to make a diagnosis in certain cases of large cell lymphoma that are subsequently documented by CNS biopsy. Careful attention should be paid to the handling, processing, and interpretation of vitrectomy specimens from patients suspected of having intraocular large cell lymphoma. Consideration should be given to immunocytologic staining and interpretation by centers that are highly experienced in vitreous cytopathology.- - - - - - - - - - ranking = 0.16666666666667keywords = nervous system (Clic here for more details about this article) |
10/52. Encouraging result of tamoxifen in a retinoblastoma patient with central nervous system metastasis.Extraocular retinoblastoma occurs more frequently in developing countries as a delayed diagnosis and prognosis of patients with conventional therapy is very poor. Metastatic retinoblastoma, especially in the central nervous system (CNS), is a highly lethal disease. tamoxifen has been used in some previous studies with variety of responses to therapy in patients with unresectable recurrent brain tumors. A 7-year-old girl with recurrent metastatic retinoblastoma received 60 mg/m2 tamoxifen in addition to chemotherapy and CNS radiotherapy. She was in remission until she has died in a traffic accident at week 114. The authors think that tamoxifen can be added to treatment protocols of metastatic retinoblastoma to provide longer and at least higher quality of life for these patients.- - - - - - - - - - ranking = 0.16666666666667keywords = nervous system (Clic here for more details about this article) |
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