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Biomedical subjects

Arman Mashayekhi

Publications and source records attributed to Arman Mashayekhi.

At least 19 recordsLinked to original sources

The International Classification of Retinoblastoma predicts chemoreduction success.

PURPOSE: To evaluate the reliability of the International Classification of Retinoblastoma (ICRB) for predicting treatment success with chemoreduction (CRD). DESIGN: Noncomparative interventional case series. PARTICIPANTS: Two hundred forty-nine consecutive eyes. METHODS: All eyes were treated with CRD and were classified according to the ICRB: group A included those eyes with retinoblastoma </=3 mm; group B included those eyes with retinoblastoma >3 mm, macular location, or minor subretinal fluid; group C included those eyes with retinoblastoma with localized seeds; group D included those eyes with retinoblastoma with diffuse seeds; group E included those eyes with massive retinoblastoma necessitating enucleation. The CRD regimen included vincristine, etoposide, and carboplatin for 6 cycles plus local consolidation with thermotherapy or cryotherapy. MAIN OUTCOME MEASURE: Chemoreduction success, defined as avoidance of external beam radiotherapy or enucleation. RESULTS: Of the 249 eyes, 23 (9%) were in group A, 96 (39%) were in group B, 21 (8%) were in group C, and 109 (44%) were in group D. In this series, group E eyes were managed with enucleation. Treatment success was achieved in 100% of group A, 93% of group B, 90% of group C, and 47% of group D eyes. CONCLUSIONS: The ICRB can be of assistance in predicting CRD success for retinoblastoma. Additional treatment methods are necessary to salvage more group D eyes.

Antineoplastic Combined Chemotherapy Protocols↗

Iodine 125 plaque radiotherapy as salvage treatment for retinoblastoma recurrence after chemoreduction in 84 tumors.

PURPOSE: To assess tumor control and complications of iodine 125 (I125) plaque radiotherapy for retinoblastoma recurrence after chemoreduction (CRD). DESIGN: Interventional retrospective case series. PARTICIPANTS: Children with retinoblastoma treated on the Oncology Service, Wills Eye Hospital, initially examined from July 1994 to April 2005. METHODS: Eighty-four retinoblastomas with solid recurrence after CRD were treated with custom designed I125 plaque radiotherapy. Eyes with vitreous or subretinal seed recurrence were excluded. The CRD regimen included vincristine, etoposide, and carboplatin for 6 cycles plus local consolidation with thermotherapy or cryotherapy. The only alternatives to plaque radiotherapy in these cases would have been external beam radiotherapy (EBRT), additional chemotherapy, or enucleation. MAIN OUTCOME MEASURES: Tumor control and radiation complications. RESULTS: Tumor control was achieved in 95% of eyes by 5 years follow-up. Of the 59 recurrent tumors after CRD alone, 56 (95%) were controlled with plaque radiotherapy. Of the 25 recurrent tumors after CRD and EBRT, 100% were controlled with plaque radiotherapy. Of the 3 failures after plaque radiotherapy, the median time to failure (tumor recurrence) was 4 months and all recurrences were detected within 1 year. There were no identifiable factors predictive of plaque radiotherapy failure. Using Kaplan-Meier analysis, radiation complications at 5 years included proliferative retinopathy (19%), nonproliferative maculopathy (24%), papillopathy (16%), transient mild vitreous hemorrhage (54%), cataract (43%), iris neovascularization (NVI; 8%), and glaucoma (4%). Nonresolving vitreous hemorrhage and scleral necrosis did not occur. Radiation complications showed minimal change at 10 and 15 years of follow-up. CONCLUSIONS: Iodine 125 plaque radiotherapy offers 95% tumor control for selected retinoblastomas that fail CRD. Complications such as radiation retinopathy and mild vitreous hemorrhage should be anticipated, depending on the location, size, and extent of the tumor.

Adolescent↗

Fine-needle aspiration biopsy of iris tumors in 100 consecutive cases: technique and complications.

OBJECTIVE: To evaluate the technique and complications of fine-needle aspiration biopsy (FNAB) for iris tumors. DESIGN: Retrospective, nonrandomized, single-center case series. PARTICIPANTS: One hundred eyes of 100 patients with diagnostically challenging iris tumors evaluated with FNAB. MAIN OUTCOME MEASURES: Biopsy technique and complications. RESULTS: Of more than 1400 patients referred with an iris tumor over a 24-year period, 100 underwent FNAB for diagnostic purposes. The median patient age was 44 years. The median basal dimension of the lesion was 9.0 mm and median thickness was 2.5 mm. A limbal entry with transaqueous approach parallel to the iris was used in 100% of patients. Entry was from the superotemporal (52%) or inferotemporal (42%) direction. Needle gauge size was 22 (9%), 25 (55%), 27 (25%), or 30 (9%), depending on the friability and vascularity of the tumor. Adequate tumor sample was achieved in 99 eyes (99%). At the time of FNAB, the only immediate complication was partial hyphema (34%). Two weeks after FNAB, persistent hyphema was found in 6 eyes (6%), requiring surgical washout in 1 patient. There were no cases of recurrent hyphema, vitreous hemorrhage, prolonged hypotony, lens damage, endophthalmitis, or extraocular tumor seeding. At mean follow-up of 5 years, there was no incident of tumor recurrence on the iris, along the needle tract, or on the corneal or epibulbar surface. CONCLUSIONS: Using our technique, FNAB for diagnostically challenging iris tumors can achieve high yield with relatively few complications.

Adolescent↗

Macular retinoblastoma managed with chemoreduction: analysis of tumor control with or without adjuvant thermotherapy in 68 tumors.

OBJECTIVE: To evaluate the effectiveness of chemoreduction alone and chemoreduction with thermotherapy for macular retinoblastoma. DESIGN: Prospective, nonrandomized, single-center case series. SETTING: Ocular Oncology Service at Wills Eye Hospital of Thomas Jefferson University in conjunction with the Division of Oncology at the Children's Hospital of Philadelphia (Pa). PARTICIPANTS: There were 68 macular retinoblastomas in 62 eyes of 49 patients managed with chemoreduction from January 1995 through January 2003. Intervention All patients received 6 cycles of intravenous chemoreduction using vincristine, etoposide, and carboplatin. The patients were then treated according to 1 of 2 approaches: chemoreduction alone with no adjuvant focal therapy (group A) or chemoreduction combined with adjuvant foveal-sparing thermotherapy to each macular retinoblastoma (group B). Main Outcome Measure Tumor recurrence. RESULTS: Of the 68 tumors, 28 were in group A and 40 were in group B. A comparison of both groups revealed that the tumors were similar with regard to clinical features. The mean tumor basal dimension was 12.3 mm for group A and 12.1 mm for group B, and the mean tumor thickness was 6.8 mm for group A and 6.1 mm for group B. Tumors in group A occupied a mean of 71% of the macula, and those in group B occupied 74% of the macula. Following treatment, Kaplan-Meier estimates revealed that group A tumors showed recurrence in 25% by 1 year and 35% by 4 years whereas those in group B showed recurrence in 17% by 1 year and 17% by 4 years. All recurrences were treated with additional focal thermotherapy, cryotherapy, or plaque radiotherapy except for 1 that required external beam radiotherapy and 1 that required enucleation, both in group A. Univariate analysis revealed that predictors of tumor recurrence were intraretinal growth pattern (vs endophytic); small tumor basal dimension (less than 3 mm and occupying a smaller percentage of the macula); absence of subretinal fluid, subretinal seeds, and vitreous seeds; and chemoreduction response with less tumor calcification and tumor regression of type 0 (complete disappearance without a scar). By multivariate analysis, the most important factors predictive of tumor recurrence were smaller macular tumor size (judged by percentage of the macula occupied by the tumor), absence of subretinal or vitreous seeds, and unilateral disease. CONCLUSIONS: Treatment of macular retinoblastoma with chemoreduction plus adjuvant foveal-sparing thermotherapy provides tumor control of 83% by 4 years, and this is slightly more favorable than chemoreduction alone, which provides control of 65% by 4 years. Tumors most destined for recurrence are small tumors.

Antineoplastic Combined Chemotherapy Protocols↗

Iris melanocytoma: clinical features and natural course in 47 cases.

PURPOSE: To describe the clinical features, natural course, management and histopathologic features of iris melanocytoma. DESIGN: Single-center retrospective case series. METHODS PATIENT POPULATION: Forty-seven consecutive patients (47 eyes) with iris melanocytoma. INTERVENTION PROCEDURE: Data regarding patient and tumor features were analyzed for their impact on the main outcome measures using univariate and multivariate regression models. Kaplan-Meier estimates were used to analyze the main outcomes as a function of time. MAIN OUTCOME MEASURES: Increased intraocular pressure (IOP), tumor seeding, and tumor growth. RESULTS: Associated findings at initial presentation included iris stromal seeds in 20 patients (43%), and anterior chamber angle seeds in 12 (26%). Intrinsic vascularization and sector cataract were not seen in any eyes. The management at presentation included observation in 39 patients (83%), tumor removal by sector iridectomy/iridocyclectomy in 7 (15%), and enucleation for blind painful eye with secondary increased IOP in 1 (2%). The diagnosis was confirmed by histopathologic examination in 11 patients (23%). The mean follow-up was 58 months. Using Kaplan-Meier estimates, clinical evidence of growth was observed in 23% at 5 years, 48% at 10 years, and 74% at 15 years. New tumor seeds developed in 34% at 5 years, 63% at 10 years, and 75% at 15 years. Increased IOP was observed in 11% at 5 years, 11% at 10 years, and 55% at 15 years. CONCLUSIONS: Iris melanocytoma represented only 3% of all iris nevi. Related iris stromal and anterior chamber angle seeds were common, and secondary glaucoma occurred in 11% at 5 years. Growth was observed in 23% at 5 years but no malignant transformation was found.

Adolescent↗

Cavitary changes in retinoblastoma: relationship to chemoresistance.

OBJECTIVE: To describe the clinical features and outcome of treatment of retinoblastomas presenting with ophthalmoscopically visible cavities. DESIGN: Retrospective, noncomparative, interventional case series. PARTICIPANTS: Sixteen eyes of 15 patients harboring retinoblastomas with ophthalmoscopically visible cavities treated between February 1995 and July 2003. METHODS: Patient demographics, eye and tumor characteristics, and response to treatment were recorded. MAIN OUTCOME MEASURES: Decrease in tumor size and tumor recurrence. RESULTS: The mean patient age at diagnosis was 20 months. The mean number of retinoblastomas per eye was 2 (range, 1-6). Associated subretinal fluid was graded as absent in 5 eyes, minimal in 6 eyes, mild in 4 eyes, and severe in 1 eye. None of the tumors had vitreous seeds, and 5 (31%) had subretinal seeds. Ophthalmoscopically visible calcification occupied a mean of only 3% of the tumor (median, 0%; range, 0%-30%). Intratumoral cavities were characteristically visible in the superficial portion of the tumor and had a mean diameter of 2.0 mm (median, 1.5; range, 0.5-5.0). The mean number of cavities per tumor was 2 (median, 1; range, 1-5). Three tumors were treated with enucleation, 1 with observation, and 12 with chemoreduction. Two months after chemoreduction, the mean reduction in tumor base was 19% (median, 16%; range, 0%-42%), and the mean reduction in tumor thickness was 18% (median, 12%; range, 0%-59%). During a mean follow-up of 32 months (median, 23; range, 12-58), none of the cavitary retinoblastomas recurred or gave rise to vitreous or new subretinal seeds. CONCLUSION: Retinoblastomas containing ophthalmoscopically visible cavitary spaces do not show a substantial decrease in size after chemotherapy. Cavitary changes in retinoblastoma may be a sign that the tumor is well differentiated and may impart a better prognosis.

Antineoplastic Agents↗

Pseudomelanomas of the posterior uveal tract: the 2006 Taylor R. Smith Lecture.

PURPOSE: To determine the types and frequency of lesions that clinically simulate choroidal or ciliary body melanoma (posterior uveal melanoma; PUM). PATIENTS AND METHODS: A review was conducted on cases of patients referred to the ocular oncology service from October 1978 through September 2003 with the diagnosis of possible PUM but who were subsequently diagnosed by the authors to have a simulating lesion rather than PUM. The type and percent of pseudomelanomas were tabulated and compared with findings of a similar study from our service on data collected before 1978. RESULTS: There were approximately 12,000 patients referred because of a lesion believed to be a PUM during the 25 years included in the data collection. Of these patients, 1,739 (14%) were found to have a simulating condition. There were 54 different conditions that simulated melanoma. The most frequent condition was choroidal nevus, accounting for 851 cases (49%) of the pseudomelanomas. This was followed by peripheral exudative hemorrhagic chorioretinopathy (139 cases; 8%), congenital hypertrophy of the retinal pigment epithelium (108 cases; 6%), hemorrhagic detachment of the retina or pigment epithelium (86 cases; 5%), circumscribed choroidal hemangioma (79 cases; 5%) and age-related macular degeneration (76 cases; 4%). Compared with the 1980 report, the rate of pseudomelanomas diagnosed as choroidal nevus increased from 26% to 49%. CONCLUSION: A variety of lesions can simulate PUM. Suspicious choroidal nevus is still the lesion most difficult to differentiate from PUM. Most other pseudomelanomas account for a lower percent compared with findings from the prior study, suggesting that clinicians are now more familiar with the other pseudomelanomas and less likely to refer them to rule out PUM.

Adolescent↗

Expanding MIRAgel scleral buckle simulating an orbital tumor in four cases.

PURPOSE: To describe four patients with an enlarging orbital mass from a swollen MIRAgel scleral buckle that simulated an orbital neoplasm. METHODS: In a retrospective, single-center case series at the Ocular Oncology Service at Wills Eye Hospital of Thomas Jefferson University, 4 eyes of 4 patients were referred for evaluation and treatment of a suspected orbital tumor. RESULTS: The initial presenting features were orbital mass (case 1), strabismus (case 2), and conjunctival mass with orbital extension (cases 3 and 4). Each patient vaguely recalled previous uncomplicated retinal detachment surgery 12 to 20 years earlier. Confirmation of the buckling implant material was made with the retina surgeon in 3 cases. A nontender, forniceal conjunctival mass, deep to the Tenon fascia and appearing as a translucent firm elevation was seen in all 4 cases. Axial CT (case 1) revealed a circumscribed anterior temporal orbital mass, believed to be a large inclusion cyst, 4 times thicker than the nasal scleral buckle. Ocular ultrasonography depicted an echolucent mass in the episcleral region (cases 3 and 4) that was 2 times thicker than the nasal scleral buckle (case 3). Excision was attempted in case 1, but only piecemeal removal was achieved, leading to extensive postoperative inflammation and decreased vision. The other 3 cases were followed conservatively without excision because they were each recognized to be a swollen MIRAgel implant and not an orbital tumor. CONCLUSIONS: MIRAgel scleral buckle material can greatly enlarge over a period of 10 years and simulate an orbital tumor or orbital cyst. Patients often do not recall details of the retinal surgery. Caution is advised regarding excision of this material because it is friable and can lead to extensive postoperative inflammation.

Adult↗

Conjunctival nevi: clinical features and natural course in 410 consecutive patients.

OBJECTIVES: To describe the clinical features of a conjunctival nevus and to evaluate the lesion for changes in color and size over time. DESIGN: Retrospective, observational, noncomparative case series. PARTICIPANTS: Four hundred ten consecutive patients with conjunctival nevi. MAIN OUTCOME MEASURES: The 2 main outcome measures were changes in tumor color and size. RESULTS: Of the 410 patients, 365 (about 89%) were white, 23 (about 6%) were African American, 8 (2%) were Asian, 8 (2%) were Indian, and 6 (1%) were Hispanic. The iris color was brown in 55% (229/418), blue in 20% (85/418), green in 20% (83/418), and not indicated in 5% (21/418). The nevus was brown in 65%, tan in 19%, and completely nonpigmented in 16%. The anatomical location of the nevus was the bulbar conjunctiva (302 eyes, 72%), caruncle (61 eyes, 15%), plica semilunaris (44 eyes, 11%), fornix (6 eyes, 1%), tarsus (3 eyes, 1%), and cornea (2 eyes, <1%). The bulbar conjunctival lesions most commonly abutted the corneoscleral limbus. The nevus quadrant was temporal (190 eyes, 46%), nasal (184 eyes, 44%), superior (23 eyes, 6%), and inferior (21 eyes, 5%). Additional features included intralesional cysts (65%), feeder vessels (33%), and visible intrinsic vessels (38%). Cysts were clinically detected in 70% of histopathologically confirmed compound nevi, 58% of the subepithelial nevi, 40% of the junctional nevi, and 0% of the blue nevi. Of the 149 patients who returned for periodic observation for a mean of 11 years, the lesion color gradually became darker in 5% (7 patients), lighter in 8% (12 patients), and was stable in 87% (130 patients). The lesion size was larger in 7% (10 patients), appeared smaller in 1% (1 patient), and was stable in 92% (137 patients). There were 3 patients who developed malignant melanoma from a preexisting compound nevus (2 cases) or blue nevus (1 case) over a mean interval of 7 years. CONCLUSIONS: Conjunctival nevus is a benign tumor most often located at the nasal or temporal limbus and rarely in the fornix, tarsus, or cornea. Over time, a change in tumor color was detected in 13% (20/149) and a change in tumor size was detected in 8% (12/149).

Adolescent↗

Practical approach to management of retinoblastoma.

OBJECTIVE: To present a simplified approach to management of retinoblastoma using basic clinical features. DESIGN: In a prospective, nonrandomized, single-center clinical trial, 158 eyes of 103 patients with retinoblastoma were managed with 6 cycles of chemoreduction (vincristine sulfate, etoposide, and carboplatin). The eyes were classified according to the Reese-Ellsworth classification and were also grouped on the basis of clinical features as follows: group 1, tumor only; group 2, tumor plus subretinal fluid; group 3, tumor plus focal seeds (3a, focal subretinal seeds; 3b, focal vitreous seeds); group 4, tumor plus diffuse seeds (4a, diffuse subretinal seeds; 4b, diffuse vitreous seeds); and group 5, neovascular glaucoma or invasive retinoblastoma. MAIN OUTCOME MEASURE: Treatment success (avoidance of enucleation and external beam radiotherapy). RESULTS: According to the Reese-Ellsworth classification, chemoreduction was successful in 100% of group Ia, 100% of group Ib, 86% of group IIa, 100% of group IIb, 91% of group IIIa, 100% of group IIIb, 50% of group IVa, 77% of group IVb, 50% of group Va, and 27% of group Vb. There was erratic correlation of the Reese-Ellsworth classification with treatment success. In contrast, the simplified grouping system displayed a smooth, nonerratic correlation for treatment success, with 100% success for group 1, 91% for group 2, 59% for group 3, and 12% for group 4 (group 5 always managed by primary enucleation). When all 6 subcategory groups were analyzed, there was consistent correlation for treatment success of 100% for group 1, 91% for group 2, 68% for group 3a, 54% for group 3b, 17% for group 4a, and 11% for group 4b. CONCLUSION: This practical approach to retinoblastoma using basic clinical features is predictive of treatment success for eyes in which modern conservative therapy for retinoblastoma is used.

Antineoplastic Combined Chemotherapy Protocols↗

Chemoreduction for retinoblastoma. Analysis of tumor control and risks for recurrence in 457 tumors.

PURPOSE: To evaluate retinoblastoma control following chemoreduction. DESIGN: Interventional case series. METHODS: Prospective. SETTING: Single center trial. PATIENT POPULATION: 457 retinoblastomas in 193 eyes of 125 patients. Nonrandomized, noncomparative study. INTERVENTION: All patients received intravenous vincristine, etoposide, and carboplatin,. The tumors were managed with chemoreduction alone (group W) or chemoreduction combined with thermotherapy (group X), cryotherapy (group Y), or both thermotherapy and cryotherapy (group Z). MAIN OUTCOME MEASURE: Tumor recurrence in each treatment group. RESULTS: Of 457 retinoblastomas, 63 (14%) were in group W, 256 (56%) in group X, 127 (28%) in group Y, and 11 (2%) in group Z. The tumor was located in the macula in 33 (52%) of group W, 109 (43%) of group X, 3 (2%) of group Y, and 9 (1%) of group Z. The mean tumor thickness at initial examination was 7 mm for group W, 4 mm for group X, 2 mm for group Y, and 3 mm for group Z. Using Kaplan-Meier estimates, recurrence of the individual retinoblastoma at 7 years was found in 45% of group W and 18% for combined groups X, Y, and Z. Risk factors predictive of tumor recurrence by multivariate analysis included macular tumor location for all groups and additionally female gender for group W and increasing tumor thickness for groups X, Y, and Z. CONCLUSIONS: Chemoreduction alone or combined with cryotherapy or thermotherapy is effective for treatment of retinoblastoma, but tumor recurrence rate is highest for those located in the macula and those with greater thickness.

Antineoplastic Combined Chemotherapy Protocols↗

Melanocytoma of optic disc in 115 cases: the 2004 Samuel Johnson Memorial Lecture, part 1.

PURPOSE: To report clinical variations and the natural course of melanocytoma of the optic disc. PATIENTS AND METHODS: A retrospective study was conducted on a consecutive series of patients with melanocytoma of the optic disc who were evaluated at the Ocular Oncology Service at Wills Eye Hospital. Demographic information and variations in appearance and size of the tumor and local ocular complications were noted. Frequency of tumor growth and malignant transformation was recorded. Clinical factors, including patient data and tumor features, were analyzed for their impact on visual acuity loss of 2 or more Snellen lines and the tumor growth, using Cox proportional hazards regression models. Kaplan-Meier survival estimates of probability of visual acuity loss of 2 or more Snellen lines and probability of tumor growth were performed as a function of time from the initial examination. MAIN OUTCOME MEASURES: Visual acuity loss of 2 or more Snellen lines, tumor growth, and malignant transformation of the tumor. RESULTS: There were 115 patients (116 eyes) with melanocytoma of the optic disc. The mean age at diagnosis was 50 years; 38% of patients were male and 62% of patients were female; 65% of patients were white, 29% of patients were African American, and 6% of patients were Asian, Hispanic, Indian, or Arabic. The lesion was unilateral in 99% of patients. Visual symptoms that seemed to be related to the melanocytoma were present in 24% of patients, and an afferent pupillary defect was noted in 9% of patients. Associated ocular abnormalities included ocular melanocytosis (8%), racial melanosis (7%), optic nerve hypoplasia (2%), and retinitis pigmentosa (1%). The melanocytoma was dark brown to black in 100% of patients. The mean tumor diameter was 2 mm and the mean thickness was 1 mm. Associated findings included a choroidal component (54%), retinal component (30%), optic disc edema (25%), retinal edema (16%), localized subretinal fluid (14%), retinal exudation (12%), retinal hemorrhage (5%), vitreous seeds (4%), and retinal vein obstruction (3%). Fluorescein and indocyanine green angiography typically showed persistent hypofluorescence of the lesion. Using Kaplan-Meier survival curves, related visual loss occurred in 18% of patients by 10 years, and minor tumor enlargement occurred in 11% of patients by 5 years and in 32% of patients by 10 years. Malignant transformation was documented in 2 patients (2%). CONCLUSIONS: Although melanocytoma of the optic disc generally is considered to be a benign, stationary lesion, it can produce several local complications, can cause visual loss, can grow slowly, and, rarely, can undergo malignant transformation into melanoma. Patients with optic disc melanocytoma should undergo periodic ocular examination.

Adolescent↗

Clinical factors in the identification of small choroidal melanoma.

The detection and treatment of choroidal melanoma early in its natural course is critical to providing the patient with the best prognosis. Studies of tumour doubling time have indicated that metastasis from choroidal melanoma can occur quite early in the course of the disease, when the tumour is about 3.0 mm in basal dimension and 1.5 mm in thickness. Clinical studies have shown that, at 5 years, metastasis occurs in 16% of patients with small choroidal melanomas (less than 4 mm thick), compared with 32% of those with medium-sized (4-8 mm thick) choroidal melanomas and 53% of those with large (more than 8 mm thick) choroidal melanomas. The difficulty with early detection of choroidal melanoma relates to its clinical similarity to benign choroidal nevus. Factors that assist in differentiating small choroidal melanoma from choroidal nevus can be remembered using the mnemonic "TFSOM" (to find small ocular melanoma), where T = thickness greater than 2 mm, F = subretinal fluid, S = symptoms, O = orange pigment and M = margin touching optic disc. Choroidal melanocytic tumours that display none of these factors have a 3% risk of growth into melanoma at 5 years and most likely represent choroidal nevi. Tumours that display one factor have a 38% risk of growth, and those with two or more factors show growth in over 50% of cases. Most tumours with two or more risk factors probably represent small choroidal melanomas, and early treatment is generally indicated. Therefore, ophthalmologists should be aware of the clinical factors that identify small choroidal melanoma so that early treatment and better prognosis can be achieved for their patients.

Choroid Neoplasms↗

Chemoreduction for retinoblastoma: analysis of tumor control and risks for recurrence in 457 tumors.

PURPOSE: To evaluate individual tumor control following chemoreduction for retinoblastoma. METHODS: Prospective nonrandomized single-center case series of 457 retinoblastomas managed with six cycles of chemoreduction (vincristine, etoposide, and carboplatin). The tumors were then managed with chemoreduction alone (group A) or chemoreduction combined with thermotherapy (group B), cryotherapy (group C), or both thermotherapy and cryotherapy (group D). The main outcome measure was development of tumor recurrence. RESULTS: Of 457 retinoblastomas, 63 (14%) were in group A, 256 (56%) in group B, 127 (28%) in group C, and 11 (2%) in group D. The tumor was located in the macula in 33 (52%) of group A, 109 (43%) of group B, 3 (2%) of group C, and 1 (9%) of group D. Using Kaplan-Meier analysis, recurrence of the individual retinoblastoma at 7 years was found in 45% of group A and in 18% of combined groups B, C, and D. Treatment of the 93 tumor recurrences included thermotherapy, cryotherapy, or plaque radiotherapy in 62 cases (67%) and external beam radiotherapy or enucleation in 31 cases (33%). Risk factors predictive of tumor recurrence by multivariate analysis included macular tumor location for all groups and, additionally, female sex for group A and increasing tumor thickness for groups B, C, and D. CONCLUSIONS: Chemoreduction alone or combined with cryotherapy and/or thermotherapy is effective for treatment of retinoblastoma, but tumor recurrence is greatest for those located in the macula and those with greater thickness. Globe salvage is usually achieved despite tumor recurrence.

Adolescent↗

Solitary congenital hypertrophy of the retinal pigment epithelium: clinical features and frequency of enlargement in 330 patients.

OBJECTIVE: To describe the clinical features of solitary congenital hypertrophy of the retinal pigment epithelium (CHRPE) and to determine the frequency of enlargement of this lesion DESIGN: Retrospective, observational, noncomparative case series. PARTICIPANTS: Three hundred thirty consecutive patients with solitary CHRPE. MAIN OUTCOME MEASURES: The 3 main outcome measures included flat lesion enlargement, intralesional lacunae enlargement, and development of an elevated nodule within the lesion. The clinical features at the time of presentation were analyzed for their impact on the main outcomes using a series of Cox proportional hazards regressions. RESULTS: The most common referring diagnosis included choroidal nevus (26%), choroidal melanoma (15%), CHRPE (9%), and unspecified lesion (48%). The median age at diagnosis was 45 years (range, 1-80 years), and there were no patients with familial adenomatous polyposis or related colon cancer, although a history of cancer was noted in 8% of patients, most commonly breast cancer (3%). The lesion most frequently was located inferotemporally (31%) and at the equatorial region (45%). Rarely, it was located in the macula (1%) or peripapillary region (1%). The median largest basal diameter was 4.5 mm, and the lesion was flat in all cases except in 5 (1.5%), in which there was an intralesional lesion nodule. The lesion was pigmented in 88% of cases and nonpigmented in 12%. Lacunae were noted in 43% of the pigmented CHRPE, and the lacunae showed gradual enlargement in 32%. Factors related to lacunae enlargement included number and relative size of lacunae. Flat enlargement of the lesion was documented in 46% of patients with comparative photographic follow-up and in 83% of those followed up for more than 3 years. The median rate of enlargement was 10 micro m per month. The most important factor associated with flat lesion enlargement was relative size of the lacunae within CHRPE. There were no cases of CHRPE in which a nodule developed while the patient was being followed up. Of the 5 lesions that had a nodule, progressive enlargement of the nodule was found in 3. CONCLUSIONS: Congenital hypertrophy of the retinal pigment epithelium generally has been regarded as a benign, stable lesion, but subtle, flat enlargement was noted in most patients (83%) followed up for 3 or more years using meticulous photographic comparison. Flat enlargement of the lesion appeared to be related to percentage of the mass occupied by lacunae.

Adolescent↗