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BackgroundThe travel distance from home to a treatment centre, which may impact the stage at diagnosis, has not been investigated for retinoblastoma, the most common childhood eye cancer. We aimed to investigate the travel burden and its impact on clinical presentation in a large sample of patients with retinoblastoma from Africa and Europe.MethodsA cross-sectional analysis including 518 treatment-nai¨ve patients with retinoblastoma residing in 40 European countries and 1024 treatment-nai¨ve patients with retinoblastoma residing in 43 African countries.ResultsCapture rate was 42.2% of expected patients from Africa and 108.8% from Europe. African patients were older (95% CI −12.4 to −5.4, p<0.001), had fewer cases of familial retinoblastoma (95% CI 2.0 to 5.3, p<0.001) and presented with more advanced disease (95% CI 6.0 to 9.8, p<0.001); 43.4% and 15.4% of Africans had extraocular retinoblastoma and distant metastasis at the time of diagnosis, respectively, compared to 2.9% and 1.0% of the Europeans. To reach a retinoblastoma centre, European patients travelled 421.8 km compared to Africans who travelled 185.7 km (p<0.001). On regression analysis, lower-national income level, African residence and older age (p<0.001), but not travel distance (p=0.19), were risk factors for advanced disease.ConclusionsFewer than half the expected number of patients with retinoblastoma presented to African referral centres in 2017, suggesting poor awareness or other barriers to access. Despite the relatively shorter distance travelled by African patients, they presented with later-stage disease. Health education about retinoblastoma is needed for carers and health workers in Africa in order to increase capture rate and promote early referral.

To assess the efficacy of chemotherapy (chemoreduction) plus local treatments as an alternative to external beam and enucleation for intraocular retinoblastoma. A prospective study was performed on 21 patients with retinoblastoma treated in our institution from September 1997 to December 2000 to study the ocular outcome of those 33 eyes. There were 9 unilateral and 12 bilateral retinoblastoma cases. There were 12 eyes with Reese-Ellsworth group I–IV and 21 eyes with group V. Among 33 eyes, nine eyes (27%) were initially managed by enucleation. The remaining 24 eyes (73%) were initially treated with chemoreduction (maximum of six cycles of carboplatin, vincristine, etoposide) or chemothermotherapy. Among those 24 eyes, 20 were successfully treated with local treatments (thermotherapy plus cryotherapy in 16 eyes and thermotherapy plus cryotherapy plus ¹²⁵I plaque radiotherapy in 4 eyes), enucleation eventually underwent in two eyes and was proposed but refused in one child with bilateral group V retinoblastoma. With a median follow-up of 21 months, conservative management without external beam radiation was successful in all 12 eyes with group I–IV and in a total of 20/33 eyes (60%). Among the nine cases of unilateral retinoblastoma, eight were enucleated but among the 24 eyes with bilateral retinoblastoma, 19 (79%) were successfully treated with conservative therapy. It may be possible to eradicate viable tumor in all eyes with Reese-Ellsworth group I–IV retinoblastoma by chemoreduction followed by local treatments. Although 8 out of 21 eyes (38%) with group V retinoblastoma may be salvaged after chemoreduction and local therapies, enucleation remained the treatment of choice in those eyes with total retinal detachment and diffuse vitreous seeding. Med Pediatr Oncol 2002;38:411–415. © 2002 Wiley-Liss, Inc.

Cancer may affect the eye and orbit by a direct effect of metastatic neoplastic infiltration or compression or by circulating antibodies involving paraneoplastic retinal degeneration. Metastatic tumor to the uvea is the most common form of an intraocular malignant process. The choroid is the most common site for uveal metastasis; metastases to the ciliary body, iris, retina, optic disc, and vitreous are rare. Approximately one third of patients have no history of primary cancer at the time of ocular diagnosis. Breast and lung carcinomas for women and lung and gastrointestinal carcinomas for men most commonly metastasize to the eye and orbit. The short-term prognosis for vision is usually good but the systemic prognosis is poor. The visual paraneoplastic syndromes encompass several distinct clinical and pathologic entities including carcinoma-associated retinopathy, melanoma-associated retinopathy, and bilateral diffuse melanocytic uveal proliferation. The first affects photoreceptors, the second is thought to affect bipolar cell function, and the third targets the uveal tract. Identification of circulating antibodies against retinal proteins (recoverin, 23-kD retinal protein; 46-kD and 60-kD retinal proteins) serves to recognize the paraneoplastic nature of the patient's symptoms, which frequently develop before the cancer is diagnosed. No therapy exists to stop the inexorable progressive loss of vision. Metastasis to the eye and orbit and paraneoplastic disorders represent a very bad prognostic sign. Recognition of their visual symptoms and ocular findings should alert the ophthalmologist to the possibility of cancer and systemic evaluation should be pursued.

There have been many recent advances in the diagnosis and management of children with retinoblastoma. Regarding diagnosis, the judicial use of ancillary studies, particularly B-scan ultrasonography, has been instrumental in supplementing the diagnosis when opaque media preclude a clear view of the underlying neoplasm. Computed tomography and magnetic resonance imaging are important in the early detection of associated pinealoblastoma, which occurs as part of the recently described syndrome of “trilateral retinoblastoma.” With regard to management, the technique of enucleation continues to improve and the scleralized hydroxyapatite implant is now available to provide a better cosmetic appearance and better motility of the artificial eye. Methods have been described for harvesting fresh tumor tissue immediately after enucleation for special DNA studies. Recent reports have documented a decreasing frequency of enucleation and an increasing tendency to use eye-preserving methods of management. Plaque radiotherapy, which provides more localized irradiation to the specific tumor, is gradually supplanting external beam irradiation for localized retinoblastoma that does not exhibit extensive vitreous seeding. The indications and contraindications for cryotherapy and photocoagulation have been more clearly delineated. The role of chemotherapy is being evaluated in the treatment of primary retinoblastoma and for metastatic disease. New breakthroughs in genetic research are being applied to clinical genetic counseling. The prognosis for life and for preservation of vision has improved greatly in recent years.

Circumscribed conjunctival melanoma usually arises in the bulbar conjunctiva and less often in the forniceal or palpebral conjunctiva. After simple superficial removal, employed by many ophthalmologists, these tumors have an increased tendency toward local recurrence and distant metastasis. A surgical procedure designed to remove the tumors completely and minimize the chances of recurrence would be desirable. The authors employed a surgical approach to conjunctival melanoma excision, which they believe insures more complete tumor removal and decreases the chances of recurrence and metastasis. The surgical management of melanoma in the limbal region of the bulbar conjunctiva consists of localized alcohol epitheliectomy, removal of the mass by a partial lamellar scleroconjunctivectomy, and supplemental double freeze-thaw cryotherapy to the adjacent remaining conjunctiva by a specific technique. For tumors located in the forniceal or palpebral conjunctiva, wide surgical resection with alcohol treatment to the scleral base and cryotherapy to the surrounding conjunctiva is performed. A “no touch” technique is employed and direct manipulation of the tumor is strictly avoided in an effort to prevent tumor cell seeding into a new area. The technique currently employed has evolved from experience with circumscribed conjunctival melanoma excision during a 20-year period. About 80 patients had circumscribed conjunctival melanoma unassociated with appreciable primary acquired melanosis. Although it is not the purpose of this article on surgical technique to provided a detailed statistical analysis of the results, the authors currently believe that this technique should be employed in all cases of circumscribed lesions in which conjunctival melanoma is a diagnostic consideration. Incisional biopsy and frozen sections are generally not advisable. Preliminary observations suggest that this method decreases the chances of local recurrence.

A 29-year-old woman presented with a 1-year history of blurred vision in her left eye while chewing. Examination revealed a rubbery mass with ill-defined borders in the superotemporal brow region. Imaging studies demonstrated a cystic lesion extending from the temporal fossa into the orbit through a bony defect in the lateral orbital wall, assuming a dumbbell configuration. Histopathology of the excised specimen confirmed the clinical diagnosis of a dermoid cyst of cutaneous origin. We believe that the patient's visual disturbance was caused by transient displacement of orbital contents by the dermoid cyst as it was compressed into the orbit during mastication. Dermoid cysts are frequently associated with bony defects, which may require preoperative imaging and may produce unexpected symptoms.

A 73-year-old white man presented with painless proptosis of the right eye 18 years after retinal detachment surgery on the same eye. Computed tomography (CT) showed a mass in the area of the lateral rectus muscle and magnetic resonance imaging (MRI) defined the multiloculated cystic mass to be located within the lateral rectus muscle. At the time of surgery, a large, thin-walled cystic lesion was found within the lateral rectus muscle. Histopathologically, the cystic mass was of nonkeratinizing stratified squamous conjunctival epithelium. Massive orbital cysts after retinal detachment surgery are rare. The authors review the literature and comment on the differential diagnosis and presumed pathogenesis of this lesion.

Magnetic resonance imaging MRI is becoming a very useful diagnostic tool for the ophthalmologist. However, there is still some confusion among ophthalmologists and other clinicians regarding ordering and interpreting imaging studies. The role of MRI in the evaluation of vitreoretinal lesions is reviewed in this article by emphasizing the MR features of retinal tumors and simulating retinal or vitreal lesions. A special description of MR requirements for imaging the intraocular structures is provided as well as a review of the MR features of solid and fluid materials used in retinal surgery.

Patients with primary ocular tumors are seen infrequently in the medical profession, and most of these patients are referred to specialty centers which has resulted in a good study population. In the past, ocular tumors were treated with enucleation, but the current emphasis is now on organ preservation with sparing of all or partial visual acuity. In the management of these tumors, plaque brachytherapy and particle beam therapy have been used more frequently as an alternative to enucleation. A multi-institutional study, the Collaborative Ocular Melanoma Study (COMS), is currently underway, organized by the National Eye Institute. The COMS isotope of choice is Iodine-125 (I-125). Recurrence after plaque therapy is approximately 15%, although it may be as high as 37% at 15 years for metastatic disease. In one study, nondiffuse iris melanoma has been controlled in 93% of patients by custom plaques utilizing I-125. Plaque brachytherapy also utilizes I-125 for the treatment of retinoblastoma tumors either as primary therapy or following external beam radiation. Currently, through the utilization of plaque radiation therapy, enucleation may be avoided in the majority of patients, and many patients may retrieve some visual acuity. We will review plaque brachytherapy techniques, diagnosis, staging, and some of the pertinent literature of the two most frequently encountered primary ocular tumors: choroidal melanoma, sometimes referred to as uveal melanoma, with an incidence of approximately 1,500 new cases per year in the adult population; and retinoblastoma, the most common intraocular primary malignancy found in childhood, with a frequency of approximately 15,000 new cases per year. Semin. Surg. Oncol. 13:167–176, 1997. © 1997 Wiley-Liss, Inc.

A 35-year-old woman developed painful proptosis of the left eye over a period of 3 weeks. Orbital computed tomography and magnetic resonance imaging revealed a well-circumscribed superior orbital tumor with variable density. The mass was removed entirely by way of a superior orbitotomy. Histopathologically, it was found to be a neurofibroma. The patient had no clinical findings of neurofibromatosis. The presence of extensive myxomatous degeneration and the dense collection of collagen bundles in the tumor are correlated with the unusual computed tomography and magnetic resonance imaging features.

We report two cases of choroidal melanoma with massive orbital extension that were managed by modified enucleation by way of a lateral orbitotomy. In both cases, the orbital component of the tumor was removed intact along with the globe. This approach allows placement of an orbital implant and avoids the long healing process associated with orbital exenteration.

Metastatic cancer to the iris is rare, and the best approaches to clinical recognition and treatment of this neoplasm are not widely known. We reviewed a series of patients with metastatic cancer to the iris and elucidated the clinical variations, diagnostic approaches, and treatment of this neoplasm.

A 10-year-old girl developed a lump in the lateral aspect of the right eyebrow over a 3-week period. Computed tomography and magnetic resonance imaging revealed a soft-tissue mass in the superolateral aspect of the right orbit associated with zygomaticofrontal bone erosion and hyperostosis. An incisional biopsy specimen was studied using light microscopy, immunohistochemical staining, and electron microscopy, resulting in a diagnosis of peripheral primitive neuroectodermal tumor. The results of an extensive evaluation for systemic involvement were negative. The patient was subsequently treated with chemotherapy and radiation therapy. Primary primitive neuroectodermal tumor of the orbit is rare. The differential diagnosis and the diagnostic features of this entity are discussed herein.

To report an unusual case of orbital involvement with non-African Burkitt lymphoma in a child.

To report the singular case of a patient who developed three noncontiguous uveal melanomas over a 30-year period.

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A 39-year-old woman presented with a vermiform superior conjunctival mass that progressively enlarged during 4 years of follow-up. Although magnetic resonance imaging suggested that the lesion was located in the superior forniceal conjunctiva, surgical exploration disclosed a vascular anomaly (varix) that extended deep into the orbit. The clinical features and management of orbital varices are discussed.

A 64-year-old man with a history of maxillary antrum melanoma had abrupt-onset night blindness and photopsia.