CORE needle biopsy of orbital tumors.

BACKGROUND: In some orbital mass lesions, histology may be the only way to establish an accurate diagnosis. Core needle biopsy (CNB) is widely performed in oncology, and unlike fine needle aspiration biopsy (FNAB), it can provide sufficient tissue sample for histology and immunohistochemistry. The purpose of this study was to evaluate the usefulness and possible complications of CNB of orbital tumors. METHODS: Fifty orbital lesions from 46 patients (age: 2-84 years) were biopsied using 20-gauge and 18-gauge semi-automated needles; 11 procedures were performed under ultrasound guidance. All 50 samples received routine histopathological examination and immunohistochemical analysis. RESULTS: Specimens diagnostically sufficient for histological analysis were obtained in all biopsies. The histopathological diagnosis was established in 94% of specimens: 30 tumors were malignant: lymphoma (20); rhabdomyosarcoma (six); lacrimal gland carcinoma (one); breast carcinoma metastasis (two); melanoma (one); three were benign; ten inflammatory; and four were orbital fibrosis. Three biopsies were nondiagnostic: two (lymphoma, angiofibroma) showed undetermined identification and one was a false-negative (lacrimal gland adenocarcinoma was misdiagnosed as fibrous tissue). The diagnoses were confirmed by excisional or incisional biopsy in 26 patients. The concordance rate in patients with a surgically confirmed diagnosis was 88%. The sensitivity, specificity and accuracy for differentiating malignant from benign lesions were 94%, 100%, and 96%, respectively. Two patients suffered complications: mild retrobulbar hematoma with no decreased visual acuity. There was no damage to the globe or optic nerve, motility disorder, or infection. CONCLUSIONS: CNB of orbital mass lesions is a safe procedure that provides a sufficient amount of tissue for histology and immunohistochemistry, and may be useful for improving the quality of the pathological diagnosis of orbital tumors.

Stereotactic Gamma Knife® Radiosurgery of Intraocular Retinoblastoma: Six-Year Experience.

Background External beam radiotherapy for resistant retinoblastoma is now seen as a last resort to saving the eye because of the risk of severe side effects: secondary cancers and cosmetic problems of orbital bone growth retardation. To reduce such complications, treatment modalities have shifted towards new radiation therapy techniques. No information on single fraction Gamma Knife® radiosurgery (GKRS) for intraocular retinoblastoma exists. Materials and methods Eighteen children (19 eyes) with retinoblastoma were treated with GKRS. The mean age at the time of treatment was 35 months (from 12 to 114 months). Before GKRS, all routes of chemotherapy delivery were held in all cases. The eligibility criteria for GKRS were retinoblastomas not amenable either to systemic or local chemotherapy and local ophthalmological treatment, retinoblastomas too large for conventional local methods, and inability to perform intraarterial chemotherapy. Conventional external beam radiotherapy was excluded in the presented cases, given the possible complications mentioned above. In every case, eye removal was suggested to the child's parents, but they flatly refused. GKRS was proposed as the last chance to save the eye (in four cases, it was performed on the only eye). The median prescribed dose was 22 Gy (interquartile range [IQR]: 18-35 Gy), and the median prescribed isodose was 50% (IQR: 36-90%). Results Local control was achieved in 79% of cases (complete tumor regression in 69%, incomplete regression in 10%). Two eyes (10.5%) could not be preserved and had to be enucleated due to the tumor recurrence. Two eyes (10.5%) developed secondary complications (total vitreous hemorrhage, retinal detachment, and iris neovascularization), making adequate tumor control nearly impossible. Overall, 15 eyes (79%) were preserved, and four eyes (21%) were enucleated after GKRS with no signs of tumor recurrence and metastasis in the mean follow-up of 41 months. No acute radiation side effects occurred in any patient after GKRS. Ten children (10 eyes, 53%) were diagnosed with vitreous hemorrhage from mild to severe. Three eyes presented with optic neuropathy one year after GKRS, and four eyes developed retinopathy. Radiation-induced cataract occurred in two eyes. There were no cases of secondary glaucoma or keratopathy in our study. All patients and eyes treated by GKRS were stable within 41 months (from seven to 74 months). Conclusions Single fraction Gamma Knife® radiosurgery may be a reasonable salvage treatment for resistant and recurrent retinoblastoma as an alternative approach to enucleation in selected cases. GKRS should be considered in retinoblastoma management.

Which choroidal melanoma should be treated with primary transpupillary thermotherapy? Our experience from 78 patients.

PURPOSE: To report the outcomes of primary transpupillary thermotherapy (TTT) for choroidal melanomas. METHODS: A total of 78 patients were treated with TTT. Tumor thickness was up to 3.6 mm (mean 2.05 mm), follow-up 2.5-108 months (mean, 32.8 months). The main outcome measures were local control, enucleations, tumor recurrences, complications, and visual acuity. The predictors of treatment failures were analyzed. RESULTS: A total of 51 tumors regressed completely, and 20 incompletely. Out of 7 patients without response, 2 had enucleation, and 5 had Ru-106 irradiation. Recurrences after primary response were observed in 10 patients in 7-54 months (mean 23.4), 8 of them after incomplete regression. Six recurrences were successfully treated with additional TTT, and 4 with brachytherapy. There were no metastases or deaths. Logistic regression showed the main predictive factors for TTT failure to be tumor height (OR=6.85; 95% CI=1.2-38.1; p=0.02), basal diameter (OR=22.85; 95% CI=3.6-144.7; p=0.0003), amelanotic pigmentation (for primary failure, OR=9.18; 95% CI=1.7-49.2; p=0.008), high maximum systolic velocity (for primary failure, OR=13.6; 95% CI=2.3-81.7; p=0.003), subretinal fluid (OR=11.04; 95% CI=1.2-100.4; p=0.03), and incomplete regression (OR=30.62; 95% CI=6.0-156.4; p=0.00001). Receiver operating characteristic analysis showed the following cutoff levels: for tumor height 3.0 mm, for basal diameter 10.2 mm, for maximum systolic velocity 11.7 cm/s. Kaplan-Meier analysis of negative results was fulfilled in all treated patients and in patients without negative predictors. Mean visual acuity before TTT was 0.65, and after treatment 0.58. A total of 23% of patients had increased visual acuity, 42% had decreased acuity, and there was no change in 35%. CONCLUSIONS: TTT is an investigative treatment for choroidal melanomas. TTT needs careful selection of patients, based on consideration of predictive factors and functional perspectives.

Intraocular surgery with melphalan irrigation for vitreous hemorrhage in an only eye with retinoblastoma.

PURPOSE: To illustrate the successful outcome of pars plana vitrectomy (PPV) with melphalan irrigation for vitreous hemorrhage (VH) with suspected viable retinoblastoma. Despite the high risk of intraocular surgery, it was performed to preserve the only potentially seeing eye with treated retinoblastoma. METHODS: Vitreous hemorrhage occurred in the only eye of a 4-year-old boy after treatment for recurrent multifocal group C retinoblastoma with systemic chemotherapy (carboplatin, etoposide, and cyclophosphamide; and vincristine, cyclophosphamide, and doxorubicin), ruthenium brachytherapy with plaque repositioning, cryotherapy, and external radiotherapy. The VH developed 8 months after repeated brachytherapy with subsequent intravitreal melphalan chemotherapy. The patient's parents refused to remove the eye. The fellow eye was enucleated earlier because of VH and secondary glaucoma, without histologic signs of a viable tumor. Pars plana lensectomy, 25-G vitrectomy with melphalan irrigation (5 µg/mL), and silicone oil tamponade were performed. RESULTS: No ophthalmoscopic or morphologic signs of a viable tumor were detected. Four months later, the silicone oil was removed. Visual acuity was 20/200 with aphakic correction. Follow-up for 34 months revealed no signs of tumor recurrence or dissemination. CONCLUSIONS: Despite the high risk of intraocular surgery, the need to preserve the only potentially seeing eye with treated retinoblastoma may require PPV. Thus, in unclear cases of VH with suspected viable tumor, PPV with intraocular melphalan irrigation, with caution, may be a reasonable procedure.

The comparison of ruthenium brachytherapy and simultaneous transpupillary thermotherapy of choroidal melanoma with brachytherapy alone.

PURPOSE: To compare the outcomes of combined treatment of choroidal melanoma with ruthenium brachytherapy (BT) simultaneously with transpupillary thermotherapy (TTT) and treatment with BT alone. METHODS AND MATERIALS: Two matched groups of patients, one treated with BT and simultaneous TTT (Group BT+TTT, n=63), the other treated with BT alone (Group BT, n=70) were analyzed retrospectively. The main outcome measures were rate of tumor regression, recurrences, enucleations, metastases, recurrence-free and overall survival rate, and visual acuity, assessed by Kaplan-Meier analysis. RESULTS: Patients were matched according to mean age (p=0.22), mean tumor thickness (6.4 vs. 6.25mm, range 2.5-10.8mm, p=0.59), and mean length of followup (42 vs. 34.4 months, range 3-109, p=0.052). Tumor largest basal diameter (13.0 vs. 12.9mm), tumor location, and mean radiation dose (apical 135 vs. 136Gy and scleral 1294 vs. 1438Gy) were also similar in both groups (p>0.1). Treatment with BT+TTT resulted in higher rate of tumor regression (63% vs. 49%, respectively, p=0.036), lower 5-year tumor recurrence rate (96% vs. 83%, p<0.034), and higher eye-globe preservation (98% vs. 87%, p<0.024) and recurrence-free survival rates (89% vs. 67%, p<0.017) than treatment with BT alone. There was no difference in complications (p>0.5), metastasis-free (93% vs. 81%, p>0.22) and overall survival rates (91% vs. 81%, p>0.39), or in visual outcomes. CONCLUSION: Combined treatment of choroidal melanoma with ruthenium BT and simultaneous TTT seems to provide higher local control, eye-globe preservation, and recurrence-free survival rates than treatment with BT alone and results in similar rates of metastases and overall survival.