PURPOSE: To evaluate the clinical results of proton beam radiation therapy (PBRT) for treatment of retinoblastoma. METHODS: Children with retinoblastoma who were treated with chemotherapy and focal treatment such as brachytherapy and thermotherapy but showed no response or developed recurrences later received PBRT. The PBRT strategy was designed to concentrate the radiation energy to the retinoblastoma and spare the surrounding healthy tissue or organs. RESULTS: There were three patients who received PBRT. The first patient received PBRT because of an initial lack of tumor regression with chemotherapy and brachytherapy. This patient showed regression after PBRT. The second patient who developed recurrence of retinoblastoma as diffuse infiltrating subretinal seeding was taken PBRT. After complete regression, there was recurrence of tumor and the eye was enucleated. The third patient had unilateral extensively advanced retinoblastoma. Initial chemotherapy failed and tumor recurred. The tumor responded to PBRT and regressed significantly. However, the eye developed sudden multiple recurrences, so we had to perform enucleation. CONCLUSIONS: PBRT for retinoblastoma was effective in cases of showing no response to other treatment modalities. However, it should be carefully applied when there was recurrence of diffuse infiltrating subretinal seeding or extensively advanced retinoblastoma initially.
Child, Preschool ; Combined Modality Therapy ; Drug Resistance ; Female ; Humans ; Infant ; Male ; Retinal Neoplasms/drug therapy/*radiotherapy/surgery ; Retinoblastoma/drug therapy/*radiotherapy/surgery ; Retrospective Studies ; Treatment Outcome

Antineoplastic Agents, Alkylating ; administration & dosage ; Child ; Humans ; Intravitreal Injections ; Male ; Melphalan ; administration & dosage ; Neoplasm Seeding ; Retinal Neoplasms ; drug therapy ; pathology ; Retinoblastoma ; drug therapy ; pathology ; Vitreous Body ; pathology

PURPOSE: To report a case of choroidal metastasis caused by lung cancer in a young female who had no history. CASE SUMMARY: A 31-year-old female presented with decreased vision for 1 week. Fundus examination revealed an orange colored choroidal tumor and serous retinal detachment at superotemporal area of the optic disc on the left eye. On chest X-ray, atypical pneumonia or hematogenous metastasis was shown. Additionally, mammography, chest-abdomen computed tomography, lumbar magnetic resonance imaging, and transbronchial lung biopsy were performed and the patient was finally diagnosed with adenocarcinoma. The patient started systemic chemotherapy and visual acuity improved after 1 month. Tumor size and subretinal fluid also decreased. The tumor disappeared 2 months later and there was no recurrence. CONCLUSIONS: There are only few cases in which choroidal metastasis was observed in a young female patient with no history who had decreased visual acuity and was later diagnosed with lung cancer. Authors report this case because a satisfactory result was obtained from chemotherapy alone.
Adenocarcinoma ; Adult ; Biopsy ; Choroid* ; Citrus sinensis ; Drug Therapy ; Female ; Humans ; Lung Neoplasms* ; Lung* ; Magnetic Resonance Imaging ; Mammography ; Neoplasm Metastasis* ; Pneumonia ; Recurrence ; Retinal Detachment ; Subretinal Fluid ; Thorax ; Visual Acuity

The most frequent primary site of metastatic tumors to the choroid is the breast in women and the bronchus in men. Invasive ductal carcinoma is the most common malignant tumor in breast, and it appears bilateral involvement, distant or adjacent lymph node metastasis. Metastatic tumors are more likely to occur multifocally in both eyes and have a definite predilection for the posterior segment and are most commonly located in the macular area. The patient with a metastatic tumor to the choroid may be asymptomatic or may experience painless blurred vision if the small mass or exudative retinal detachment involve the macula. In general, although the prognosis of the metastatic choroidal tumor for vision is usually good, the prognosis for life is poor. We experienced a case of choroidal metastasis with rib metastasis which had been treated with radical mastectomy and chemotherapy 2 years ago, because of multicentric and multifocal invasive ductal carcinoma of left breast. The patient had improvement of visual acuity and diminution of tumor size and extent of retinal detachment by radiotherapy.
Breast Neoplasms ; Breast* ; Bronchi ; Carcinoma, Ductal* ; Choroid* ; Drug Therapy ; Female ; Humans ; Lymph Nodes ; Male ; Mastectomy, Radical ; Neoplasm Metastasis* ; Prognosis ; Radiotherapy ; Retinal Detachment ; Ribs ; Visual Acuity

To describe the clinical and optical coherence tomography (OCT) features of a macular hole (MH) or its precursor lesion in patients treated with systemic antiestrogen agents. We reviewed the medical history of the patient, ophthalmic examination, and both fundus and OCT findings. Three female patients receiving antiestrogen therapy sought treatment for visual disturbance. All of the patients showed foveal cystic changes with outer retinal defect upon OCT. Visual improvement was achieved through surgery for the treatment of MH in two patients. Antiestrogen therapy may result in MH or its precursor lesion, in addition to perifoveal refractile deposits. OCT examination would be helpful for early detection in such cases.
Adult ; Breast Neoplasms/drug therapy/surgery ; Estrogen Antagonists/*adverse effects ; Female ; Humans ; Middle Aged ; Retinal Perforations/*chemically induced/diagnosis/surgery ; Tamoxifen/*adverse effects ; Vitrectomy

Adaptor Proteins, Signal Transducing ; analysis ; Apoptosis ; drug effects ; Female ; Humans ; Inhibitor of Apoptosis Proteins ; analysis ; Male ; Neoplasm Proteins ; analysis ; PTEN Phosphohydrolase ; analysis ; Retinal Neoplasms ; drug therapy ; Retinoblastoma ; drug therapy ; Topoisomerase I Inhibitors ; pharmacology ; Topotecan ; pharmacology

Intraocular (IO) retinoblastoma (RB) has traditionally been treated with enucleation (ENU) or external beam radiotherapy (EBRT). Recently, clinical trials are in progress to cure RB without ENU or EBRT in order to salvage the globe and to avoid unacceptable side effects of EBRT. We performed a pilot study to treat patients with advanced Reese-Ellsworth (RE) stage IO RB with initial chemotherapy (CRx) followed by local therapy (LT) and adjuvant CRx. Ten eyes (8 RE group V, 2 RE group IV) from 9 patients were enrolled from March 2001 to November 2001. All tumors responded to CRx. In 5 of 10 eyes, the RB was enough to be treated with LT after chemoreduction. One patient who underwent LT is waiting for ENU due to post-cryotherapy complication. For a median follow-up of 13 months (8-16 mo), 4 eyes that received LT and adjuvant CRx were relapse-free. A patient with bilateral RB who failed to be a candidate for LT was rescued with high-dose CRx and hematopoietic stem cell transplantation. Consequently, by treating patients according to our strategy, we were able to salvage 6 out of 10 eyes without ENU or EBRT. These results suggest that chemoreduction followed by LT and adjuvant CRx might offer the opportunity to salvage the globe and vision even in patients with advanced stage IO RB.
Antineoplastic Combined Chemotherapy Protocols/administration & dosage/therapeutic use ; *Chemotherapy, Adjuvant ; Child, Preschool ; Cyclophosphamide/therapeutic use ; Epirubicin/therapeutic use ; Female ; Humans ; Infant ; Male ; Pilot Projects ; Retinal Neoplasms/*drug therapy ; Retinoblastoma/*drug therapy ; Time Factors ; Vincristine/therapeutic use

No abstract available.
Angiogenesis Inhibitors/administration & dosage/adverse effects ; Bevacizumab/administration & dosage/*adverse effects ; Follow-Up Studies ; Hemangioma, Capillary/diagnosis/*drug therapy ; Humans ; Intravitreal Injections ; Male ; Photochemotherapy/*adverse effects ; Retina/*pathology ; Retinal Detachment/*chemically induced/diagnosis ; Retinal Neoplasms/diagnosis/*drug therapy ; Time Factors ; Young Adult

Familial adenomatous polyposis (FAP) is a hereditary disease characterized by the appearance of numerous polyps in the large bowel with a high potential for malignant transformation unless untreated. A variety of extracolonic manifestations were reported such as osteoma, epidermoid cyst, desmoid tumor, gastroduodenal polyps, small bowel tumor, congenital hypertrophy of the retinal pigment epithelium, hepatobiliary tumor, thyroid tumor, and tumor of the central nervous system. However, the ovarian involvement of FAP as an extracolonic manifestation was very rare and there have been only few reports. We experienced a rare case of ovarian cystadenofibroma in a patient with FAP as an extracolonic manifestation. We also found colon cancer with multiple hepatic metastasis initially manifested as intestinal obstruction in the same patient. Surgical treatment and subsequent chemotherapy for colon cancer and intraoperative radiofrequency ablation of hepatic metastasis were performed.
Adenomatous Polyposis Coli* ; Catheter Ablation ; Central Nervous System ; Colonic Neoplasms ; Cystadenofibroma* ; Drug Therapy ; Epidermal Cyst ; Fibromatosis, Aggressive ; Genetic Diseases, Inborn ; Humans ; Hypertrophy ; Intestinal Obstruction ; Neoplasm Metastasis ; Osteoma ; Polyps ; Retinal Pigment Epithelium ; Thyroid Gland

BACKGROUNDRetinocytoma (RB) is a very common intraocular malignant tumor during infancy. Chemotherapy has gradually been used as the first-line treatment for intraocular RB in recent years. In this study, Livin and PTEN expressions were observed in the RB tissue, along with the growth-inhibiting and apoptosis-induced effects of topotecan (TPT) on RB HXO-Rb44 cell strain. This study aimed to investigate the antigrowth effects of TPT on RB cell strain HXO-Rb44.

METHODSMax-Vision(TM) rapid immunohistochemistry was adopted to detect Livin and PTEN expressions in the normal retina and in RB, and their relationship with RB clinicopathologic features was analyzed. Human RB cell strain HXO-Rb44 was cultivated and passaged. MTT method was used to measure the survival rates of HXO-Rb44 cell strains under various TPT concentrations. IC50 values were calculated. Flow cytometry was used to detect the effects of various TPT concentrations on HXO-Rb44 cell apoptosis. Western blotting was used to detect the differences of Livin and PTEN protein expressions during cell apoptosis.

RESULTSThe positive expressions of Livin and PTEN in the RB group were obviously different from those in the normal control group. In RB tissue, Livin expression was relevant to PTEN expression. TPT could significantly induce the occurrence of cell apoptosis and had a dependent relationship with drug concentration. Livin and PTEN expression levels varied with the extension of the effect time of TPT based on Western blotting analysis.

CONCLUSIONSLivin and PTEN have high and low expression levels in the RB tissue, respectively. Both of them have key roles in RB occurrence and development. TPT could induce human RB cell strain HXO-Rb44 cell apoptosis, and its mechanism is associated with the inhibition of Livin and PTEN expressions.

Adaptor Proteins, Signal Transducing ; analysis ; Apoptosis ; drug effects ; Cell Line, Tumor ; Child ; Child, Preschool ; Dose-Response Relationship, Drug ; Female ; Humans ; Infant ; Inhibitor of Apoptosis Proteins ; analysis ; Male ; Neoplasm Proteins ; analysis ; PTEN Phosphohydrolase ; analysis ; Retinal Neoplasms ; drug therapy ; pathology ; Retinoblastoma ; drug therapy ; pathology ; Topoisomerase I Inhibitors ; pharmacology ; Topotecan ; pharmacology