Orbital Malignancies Research Articles

Chemotherapy for Eye Cancer

Chemotherapy has been used to treat a multitude of eye cancers. We attempted to review the role of chemotherapy in the treatment of ocular, adnexal, and orbital malignancies by conducting an extensive search of the medical literature. Unfortunately, the published reports typically contain few patients with limited follow-up, precluding definitive recommendations. For most eye cancers, multicenter trials will offer the potential to gather the numbers of patients required to determine the clinical utility of chemotherapy.

MANAGEMENT OF ORBITAL LYMPHOID LESIONS

Within the realm of systemic lymphoid tumors, those involving the orbit constitute just over 2% of the total number; however, within ocular oncology, lymphoid lesions represent one of the most common primary orbital malignancies. 10 Traditionally, these tumors were characterized histopathologically primarily by morphologic criteria. The advent of immunophenotyping and gene rearrangement studies has greatly expanded our understanding of these tumors and aided in our ability to predict those patients at risk for dissemination of their disease and those patients in whom the disease process is likely to be indolent. This added knowledge has enabled clinicians to design improved treatment plans based on both clinical and pathologic data.

Orbital malignancy in Papua New Guinea: a 21 year review.

Information about orbital malignancies in Papua New Guinea (PNG) is limited to reports published 20 years ago. The present retrospective study was performed to establish the pattern of malignant orbital lesions occurring in PNG and to see whether the pattern is any different from that reported in the earlier series and in other countries. Papua New Guinea is special in that it has a large and relatively homogenous population unaffected,to a large extent, by contact with the'outside world'. The records of 176 proven cases of orbital malignancy treated between 1975 and 1996 were studied with respect to type of tumour, age, gender; mode of presentation and, where possible, treatment and its outcome. Orbital tumours were found to be uncommon relative to other malignancies. The most common orbital tumour encountered in the present study was orbital extension of retinoblastoma (40.3%). These tumours were all unilateral and were associated with a very high mortality. No bilateral or familial cases were seen. Squamous cell carcinomas were the second most common tumours (25.6%) and were seen in young adults (average age 36.6 years). Basal cell carcinomas (9.1%) occurred later (average age 54 years) and were more common in the lighter-skinned Southern Highlanders and expatriates. The other malignancies seen included lacrimal gland tumours (3.4%), malignant melanomas (4.5%), lymphomas (3.4%), rhabdomyosarcomas (1.7%), meibomian cell carcinomas (1.7%) and soft tissue sarcomas. Most patients presented late and the mortality rate was high. This could also reflect the relative paucity of ophthalmic and oncological services in the country as well as psychosocial attitudes to disease. Many patients live far from medical services and may be treated by traditional healers before coming to a hospital.

RHABDOMYOSARCOMA OF THE ORBIT: Evaluation with MR Imaging and CT

Rhabdomyosarcoma is the most common primary orbital malignancy of childhood. It can present insidiously, mimicking other (benign) processes clinically and radiographically. CT and MR imaging are crucial in the diagnostic evaluation, treatment planning, and follow-up monitoring of the disease. Such imaging, especially when contrast is used, can accurately detect and state the extent of tumor involvement.

16 Peroperative eye-preserving interstitial PDR brachy-therapy (BT) for orbital tumors and malignancies involving the base of skull

16 Peroperative eye-preserving interstitial PDR brachy-therapy (BT) for orbital tumors and malignancies involving the base of skull

Malignant orbital and orbitomaxillary tumors: surgical considerations

Malignant tumors of the orbit and the orbitomaxillary region constitute 4-8% of head and neck malignancies. The lesions can be primary orbital malignant tumors or can invade the orbit from the eyelids, the eyeball or the paranasal sinuses, especially the maxillary sinus and ethmoids. The commonest primary malignant tumors of the orbit are lymphomas, rhabdomyosarcomas and lacrimal gland epithelial tumors. These tumors are excluded from the present study since their management differs from that of metastatic tumors. Our experience is based on a series of 48 patients operated during the last five years. There was an equal distribution according to both age and sex, with two-thirds of the patients being over 60 years of age. Histologically, the basal cell carcinomas (B.C.C.) prevailed with 19 cases, followed by squamous cell carcinomas (Sq.C.C.) in 12, adenocarcinomas (AdenoCa) in 10 and orbital melanomas (O.M.) in 7. In 13 cases the maxilla was also affected. Surgical treatment varied from exenteration to wide cran iofacial resections; immediate reconstruction followed in all cases. Only 10 patients were treated with surgery alone. In the remaining 38 patients radiotherapy was given, alone or in combination with chemotherapy. The surgical techniques included exente ration with split-thickness skin grafting in 19 cases, exenteration with coverage with median forehead flap in 16, and maxillectomy with forehead and temporalis flaps in 7 cases. The tumors recurred in 12 patients, all of them in the first postoperative year. Ten patients died from intracranial involvement or distant metastases. In six of these patients the lesions were Sq.C.C., in two AdenoCa and in two O.M. It is concluded that the surgical treatment of orbital and orbitomaxillary malignancies, although subject to broad guidelines that determine the operability of the tumors, should be individualized according to the extent of the lesion and the experience of the surgeon.

Malignant tumors of the orbit: Analysis of the Florida cancer registry

The purpose of the study is to determine the relative frequency and incidence of primary malignant tumors of the orbit occurring in Florida. Cases of primary orbital malignancy were identified in the Florida Cancer Data System registry from 1981 through 1993. Analysis was restricted to persons who reside within the state. Three hundred fourteen primary orbital malignancies were reported; 55% of malignant orbital tumors were lymphoma. The average annual incidence for all malignancies was 2 cases per million. There was a steady increase in the annual incidence of orbital malignancy during the 13 years of the study. Lymphoma showed the greatest rise in annual incidence. The average annual incidence of lymphoma increased 166% the last 6 years of the study compared to the first 6 years. A large increase in orbital lymphoma was documented in the Florida cancer registry from 1981 through 1993. The increase in incidence cannot be explained by improved compliance in reporting or by the small increase in incidence of systemic lymphoma during the survey period. Additional studies are needed to determine whether the observed increase in incidence in orbital lymphoma is genuine or spurious.

The Effect of External Eye Irradiation on Choroidal Circulation

The effect of external beam radiation therapy (teletherapy) on the choroidal circulation is poorly known. Eyes irradiated with teletherapy represent a good model to study, without confounding factors, the pathophysiologic and clinical aspects of radiation-induced chorioretinal damage. This study used fluorescein and indocyanine green choroidal angiography to investigate the late effects of external eye irradiation on the choroidal circulation. Fluorescein angiography and indocyanine green choroidal videoangiography were performed on patients with radiation retinopathy because of external eye irradiation for orbital and paranasal sinus malignancies. Patients were divided into two groups according to the treatment field (anterior unilateral or bilateral). Indocyanine green angiograms showed areas of choriocapillaris hypoperfusion in all eyes-unilateral or bilateral irradiation-affected by radiation retinopathy. Late indocyanine green choroidal staining was found in five eyes (28%) of the patients who received unilateral anterior irradiation. In the same group, nine eyes (52%) had signs of choroidal precapillary occlusion and four eyes (23%) had rubeosis iridis without retinal neovascularization. One case of subfoveal choroidal neovascularization was documented in the bilateral irradiation group. Radiation side effects are not limited to the retinal vessels but also involve choroidal circulation. The damage to the choroid is primarily vascular, and its clinical aspects depend on the treatment fields. Anterior irradiation may be a critical factor for the appearance of unusual rubeosis iridis and neovascular glaucoma.

A Review of 340 Orbital Tumors in Children During a 60-Year Period

We reviewed cases of histopathologically verified orbital tumors in children at our institution over 60 years to determine the distribution of various pathologic processes and trends over time. We studied the medical records and pathology specimens from 340 patients aged 18 years or younger who underwent biopsy for orbital mass from 1932 through 1991. The most common tumors were cysts (79 of 340, 23.2%), vascular lesions (60 of 340, 17.6%), optic nerve and meningeal neoplasms (56 of 340, 16.5%), inflammatory masses (29 of 340, 8.5%), osseous and fibrocystic lesions (27 of 340, 7.9%), and rhabdomyosarcomas (24 of 340, 7.1%). The overall frequency of malignancies was 18.2% (62 of 340): 11.5% (39) were primary tumors and 6.8% (23) were secondary and metastatic. The frequency of orbital malignancies was 25.4% (36 of 142) in the first 30 years (1932 through 1961) and 13.1% (26 of 198) in the second 30 years (1962 through 1991). The percentage of primary orbital malignancies was almost identical during the first and second periods (11.9% [17 of 142] and 11.1% [22 of 198], respectively). However, secondary and metastatic orbital neoplasms occurred in 13.4% (19 of 142) of the patients during the first 30 years and in only 2.0% (four of 198) during the second 30 years. The frequency of primary orbital malignancies in biopsy material remained the same over 60 years. However, the overall incidence of orbital biopsy specimens containing a malignancy decreased as a result of a reduction in the number of secondary and metastatic neoplasms that underwent biopsy.

Orbital Exenteration With Eyelid Sparing: Indications, Technique, and Results

Of 22 orbital exenterations performed from January 1980 to December 1989, 16 were performed by an eyelid-sparing technique, and six were allowed to heal by spontaneous granulation. Reviewing these procedures, we conclude that the eyelid-sparing method is indicated mainly for advanced primary orbital malignancies and for certain secondary orbital malignancies such as orbital extension of uveal melanoma, conjunctival melanoma, and conjunctival squamous cell carcinoma. It is not usually applicable to orbital invasion of malignant eyelid tumors such as basal cell carcinoma and sebaceous gland carcinoma. Advantages of the eyelid-sparing method are more rapid healing and earlier fitting of a prosthesis.

Craniofacial resection of extensive benign lesions of the anterior skull base.

The craniofacial resection operation was developed for the treatment of advanced nasal, paranasal and orbital malignancies. It has been refined in recent years, giving increased cure rates and better palliation when cure is not possible. When used to treat extensive benign lesions involving the anterior skull base, this procedure allows more complete and safer resection with better access for repair and avoidance of major complications of brain damage, cerebrospinal fluid leak and haemorrhage. Presented here is a technique for craniofacial resection. The study demonstrates its effectiveness and low morbidity in treating 10 patients with extensive benign disease.

Space-occupying Orbital Masses in Children: A Review of 250 Consecutive Biopsies

A review was done of 250 consecutive biopsies for orbital spaceoccupying lesions in children. Benign cystic lesions were by far the most common, accounting for 52% of the lesions. Inflammatory lesions, usually biopsied to exclude the diagnosis of rhabdomyosarcoma, accounted for 16%. Among the 250 biopsies there were 14 primary malignant tumors (6%), ten of which were rhabdomyosarcoma. Although rhabdomyosarcoma is not the most common space-occupying orbital lesion in children, clinicians should be familiar with its clinical features and proceed with immediate biopsy followed by appropriate irradiation and chemotherapy if the diagnosis is established. However, biopsy should only be undertaken if there is reasonable suspicion that the lesion could be rhabdomyosarcoma or other childhood orbital malignancy.

Afferent limb of the human jaw reflex: electrophysiologic and anatomic study.

Jaw reflexes were recorded before and after neurosurgery of the trigeminal sensory root or the gasserian ganglion in 11 patients with idiopathic trigeminal neuralgia and in 2 patients with orbital malignancies. The reflex was also studied in three patients with unilateral midbrain lesions verified by postmortem findings. Jaw reflex abnormalities appeared in all cases, although there were no clinical or EMG signs of masseter dysfunction. Proprioceptive afferent fibers of the jaw reflex therefore seem to be mediated by the sensory and not the motor root. The afferent limb of the jaw reflex seems to run centrally through the midbrain, probably through the mesencephalic nucleus of the trigeminal nerve.

Orbital malignancies in infancy and childhood in Denmark. Recommendations for diagnosis and therapy.

Based on two clinical series concerning primary orbital tumours in Denmark (Eldrup‐Jørgensen & Fledelius 1975; Fledelius 1976) recommendations are given for diagnosis and therapy of orbital malignancies in infancy and childhood. Centralization is strongly advocated.

Pattern of orbital exenteration at a tertiary eye care centre in North India

To analyze cases undergoing orbital exenteration in terms of demographic and socioeconomic profile of patients, indications for surgery, histopathological diagnosis and to assess the magnitude of eyelid malignancies as indication of orbital exenteration. A case record analysis of 25 patients who underwent orbital ex enteration at a tertiary care centre in India between October 2002 and October 2007 was undertaken. The case records were reviewed to obtain demographic data, presenting symptoms, duration of symptoms, laterality, best-corrected visual acuity (BCVA), and the clinical and histopathological diagnosis. The age group of patients undergoing exenteration ranged from 1 year to 78 years (Mean 46.92 +/- 10 years). Proptosis and/or a palpable mass were the main presenting complaints in 80 % of cases. The duration of symptoms was more than 5 years in 80 % cases. Primary orbital malignancies were the commonest indication for exenteration and were diagnosed in 11 cases (44 %), followed by lid malignancies in 8 cases (32 %), retinoblastoma in 4 cases (16 %) and conjunctival malignancies in 2 cases (8 %). Sebaceous gland carcinoma was the commonest lid malignancy followed by basal cell carcinoma and squamous cell carcinoma. Lid malignancies contribute the majority of the patients undergoing orbital exenteration in this part of the world, leading to an extreme sacrifice of eyes with good visual acuity. Increase in awareness, early diagnosis and prompt referral may help to overcome this grim scenario.