Parameningeal rhabdomyosarcoma (including the orbit): results of orbital irradiation.

Twenty-three patients with parameningeal (including orbital rhabdomyosarcoma (RMS)) were treated at Memorial Sloan-Kettering Cancer Center (MSKCC) between July 1971 and January 1983. Twenty were children with a mean age of 6 and 3 were adults. In 6 patients, the primary tumor was from the orbit, whereas the remaining 17 had other parameningeal primary sites. The tumors were in a very progressive local stage, with extensive destruction of the facial bones in 19 patients. Eight patients were treated with T2 chemotherapy protocol and 15 received T6. Seven patients received 5,000 to 7,200 rad delivered to the primary tumor in 11-16 weeks, 15 patients received between 4,500 to 5,000 rad in 4-7 weeks, and 1 patient received 3,000 rad in 3 weeks for residual microscopic disease following surgery. Two patients were treated with radiation to the whole brain; no patients received radiation of the whole central nervous axis (CNA). Fifteen of the 23 patients (65%) are alive and well with a medical follow-up time of 5 years. Two patients died of therapeutic complications and six died of tumor spread. In five patients, involvement of the central nervous system (CNS) was the cause of death. The prognosis of orbital RMS with parameningeal involvement is no better than in other tumors of parameningeal sites. In those patients who had impaired vision because of optic nerve damage prior to treatment, the vision did not improve following treatment. There was no impaired vision seen due to radiation damage of eye structures except in the lens.

Scanning electron microscopy of corneal wound healing in the rabbit.

Corneal lesions 7.5 mm. in diameter were made with an ocular trephine in rabbits. The time periods studied were 0, 30 min., and 1, 2, 4, 8, 16, and 24 hr. At the end of the time period, the cornea was flooded with 4% glutaraldehyde, buffered with cacodylate, pH 7.4, and kept moist until removed. It was then fixed for 24 hr. Half of the sample was dehydrated in graded alcohols, critical-point-dried, coated with gold palladium alloy, and viewed in an AMR-1000 scanning electron microscope at an accelerating voltage of 20 kv. From 0 to 4 hr. cell trauma, debris, and retraction are seen at the margin of the lesion. From 8 to 24 hr. a significant number of polymorphonuclear leukocytes are present over the total surface but in especially large numbers at the wound margin. At 16 to 24 hr. evidence of cell movement is present. Cells show ruffling membranes a decreased number of microvilli, and a few filopodia along the advancing edge.

The ultrastructural defect in conjunctiva from a case of recessive dystrophic epidermolysis bullosa.

The predominant feature in the several forms of epidermolysis bullosa is the formation of cutaneous bullous lesions arising after minimal mechanical trauma. Ocular involvement has been noted as a complication. To our knowledge to date, only four investigators have correlated clinical eye disease with light microscopic findings. Ultrastructure of the ocular lesions has not been described previously. We present four cases of recessive dystrophic epidermolysis bullosa emphasizing their associated ocular complications. Diagnosis was confirmed by skin biopsy specimen and in one patient by demonstrating light and electron microscopic findings in eyelid skin. This tissue exhibited ultrastructural recessive cutaneous lesions; namely, bullous separation occurring below the basal lamina and absence of anchoring fibrils in both bullous and nonbullous areas. By electron microscopy, the conjunctiva in this patient exhibited an absence of clear anchoring fibrils that were numerous in control tissue. This defect may increase the susceptibility of the conjunctiva to minor mechanical trauma, resulting in the bullous and cicatricial changes seen clinically.

Radiography of the eye and orbit: a historical overview.

The history of radiography and orbital imaging begins in 1895 with Wilhelm Roentgen's discovery of x-rays. Over the next three quarters of a century, radiographic pioneers like Dr. William Sweet, who developed the Sweet method, and Dr. George E. Pfahler, who made the first successful pictures of a brain tumor, helped to bring radiography into the 20th century. With each new radiologic innovation producing a forward surge followed by a period of refinement, new methods were invented and utilized to their diagnostic limits. But perhaps none of the radiologic innovations of this century--the Coolidge tube, the Potter-Bucky diaphragm, tomography and angiography-will have more impact than computed tomography and magnetic resonance imaging.

Capillary hemangioma (infantile periocular hemangioma).

Capillary hemangiomas are the most common orbital tumors in children. They typically arise early in life, grow rapidly during a proliferative phase and then slowly regress in an involutional phase. The tumors may present as small isolated lesions of minimal clinical significance or as large disfiguring masses that can cause visual impairment and systemic symptomatology. Capillary hemangiomas are managed effectively by establishing a secure diagnosis, outlining the extent of the tumor, and understanding the natural history of the lesion, as well as its response to therapy. The ophthalmic and systemic manifestations of capillary hemangiomas are discussed in detail, as are the histopathology, radiologic findings, differential diagnosis, and therapeutic alternatives.

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.

A pigmented adenoma of the ciliary epithelium examined by magnetic resonance imaging.

PURPOSE: We studied a case of a pigmented adenoma of the ciliary epithelium. METHODS: We used magnetic resonance imaging in the clinical diagnosis of this tumor. RESULTS: The tumor was successfully treated by local excision. CONCLUSIONS: Magnetic resonance imaging is most useful in evaluating a ciliary body mass for local extension. It cannot clinically distinguish a pigmented adenoma of the ciliary epithelium from a uveal melanoma.

Renal cell carcinoma with involvement of iris and conjunctiva.

PURPOSE: To report a case of metastatic renal cell carcinoma with involvement of the iris and bulbar conjunctiva. METHODS: Case report. A 70-year-old man without known systemic disease developed an iris mass in his left eye. Iridocyclectomy was performed to remove the iris mass. RESULTS: Histopathologically, the iris mass was consistent with metastatic renal cell carcinoma. Further evaluation disclosed a mass of the left kidney. The patient underwent a left nephrectomy and was found to have renal cell carcinoma, with focal penetration into the renal capsule. One month after the nephrectomy, he developed a highly vascular nodule of the left bulbar conjunctiva. An excisional biopsy was performed, and histopathology disclosed an additional focus of renal cell carcinoma. CONCLUSIONS: Iris and conjunctival involvement may be a clinical manifestation of renal cell carcinoma. Renal cell carcinoma should be considered in the differential diagnosis of a fleshy, vascular iris and a conjunctival nodule.

Orbital hemangiopericytoma: clinical and morphologic features.

PURPOSE: To report the clinical and histopathologic features of orbital hemangiopericytoma. METHOD: We review the clinical and histopathologic features in seven patients. RESULTS: Ultrasonography, computed tomography, and magnetic resonance imaging defined the location and extent of the tumor in each patient but did not disclose pathognomonic features for the specific diagnosis of hemangiopericytoma. The predominating histopathologic feature of each tumor was a mixed pattern of ovoid cells and sinusoidal space formations. Five patients showed mild to severe cellular atypia; three had obvious pleomorphism and increased number of abnormal mitotic figures. Tumor cells disclosed cytoplasmic reactivity for vimentin but in five cases were negative for other immunologic markers. Six patients received surgical treatment with an attempt for total removal of the tumor; one had biopsy and radiation therapy. In two patients, radiation therapy was given in addition to tumor removal with orbital exenterations. Three patients died with recurrent and metastatic disease, and four patients are alive without tumor for a follow-up period ranging from 3 to 9 years. CONCLUSIONS: Orbital hemangiopericytoma may behave as a malignant tumor, leading to local recurrence or metastasis, or both. Clinical and histopathologic findings should be considered jointly to evaluate the clinical course; histopathologic findings alone are not sufficient to predict the biologic behavior of this tumor.

Wrapping hydroxyapatite orbital implants with posterior auricular muscle complex grafts.

PURPOSE: To report the use of posterior auricular muscle complex grafts as a wrapping material for hydroxyapatite orbital implants in enucleation surgery. METHOD: In a retrospective multicenter study, autogenous posterior auricular muscle complex grafts were used to cover hydroxyapatite orbital implants in 83 patients with a mean age of 38.6 years (range, 1 to 85 years), of whom 63 had primary unilateral enucleation and 20 had secondary orbital implants following enucleation. The mean follow-up period after posterior auricular muscle complex grafts was 36 months (range, 14 to 60 months). RESULTS: Of the 83 patients, two (2.4%) developed limited orbital implant exposure, which was treated with a second posterior auricular muscle complex "patch graft." No patient developed postoperative orbital infection or implant extrusion. All patients were fitted with an acceptable prosthesis and had satisfactory cosmetic and functional results. No patient developed vascular compromise or a wound defect associated with the posterior auricular donor site. CONCLUSION: Autogenous posterior auricular muscle complex grafts should be considered as an appropriate wrapping material for hydroxyapatite orbital implants for primary enucleation and for secondary orbital implants after enucleation.

Treatment of total retinal detachment in morning glory syndrome.

A 14-year-old girl had headaches and sudden loss of vision as a result of total retinal detachment in association with the morning glory optic nerve anomaly. Metrizamide cisternography with contrast dye introduced into the subarachnoid space disclosed migration of metrizamide with radiographic enhancement in the subretinal space. The demonstration of an abnormal communication between the subarachnoid and subretinal spaces suggested that the subretinal fluid is of cranial origin. The patient underwent surgical removal of a window of dura from the optic nerve sheath. This resulted in retinal reattachment and improvement of visual acuity to 20/200. The retina was still attached after a 15-month follow-up period.

Reversal of optic canal stenosis in osteopetrosis after bone marrow transplant.

PURPOSE: To describe a patient with infantile osteopetrosis and optic atrophy secondary to optic canal stenosis who demonstrated optic canal enlargement after bone marrow transplant. METHODS: Case report. A 3-month-old infant with infantile "malignant" osteopetrosis underwent ophthalmic examination, including visual evoked potentials, electroretinogram, and computed tomography (CT). Bone marrow transplant was performed at 8 months of age. RESULTS: Examination revealed visual loss and optic atrophy, left eye greater than right eye, secondary to optic canal stenosis. Flash visual evoked potentials revealed a normal waveform in both eyes with increased latency in the left eye. Electroretinogram was normal in both eyes. CT after bone marrow transplant showed enlargement of the optic canals. Vision remains stable 43 months after bone marrow transplant. CONCLUSIONS: Bone marrow transplant in infantile osteopetrosis may be followed by reversal of optic canal stenosis and preservation of vision.

Ultrasonic hyperthermia and radiation in the management of intraocular malignant melanoma.

Hyperthermia and radiation were used in combination to treat four patients with choroidal malignant melanoma. This technique uses ultrasonically induced hyperthermia synergistically with radiation to destroy tumor cells. The lower levels of radiation used should avoid the late vascular and inflammatory complications seen in conventional radiation therapy. Tumors were scanned by a computerized diagnostic ultrasound system before treatment and assigned an acoustic tissue type on the basis of a statistical comparison of their ultrasound backscatter spectrum with spectra of tumors of known pathologic status. During the follow-up period, the longest of which was 15 months, all tumors demonstrated regression patterns consistent with choroidal tumors of the same acoustic tissue types treated with conventional radiation therapy.

MR imaging of malignant uveal melanoma: role of pulse sequence and contrast agent.

To determine the most sensitive pulse sequence and to clarify the role of each pulse sequence in the MR diagnosis of uveal malignant melanoma, noncontrast T1- and T2-weighted, and postcontrast T1-weighted, spin-echo images were compared blindly and independently by two experienced observers. Thirty uveal malignant melanomas, preselected by ophthalmoscopy and sonography for size greater than 2 mm, were examined with a 1.5-T superconducting MR unit with an orbital surface coil. Fifteen tumor studies were done after the patient was injected with gadopentetate dimeglumine. Postcontrast T1-weighted images were the most sensitive in detecting melanomas, demonstrating tumors 2 mm in height accurately on axial planes and 1.6 mm in height on combined orthogonal planes. The contrast-to-noise ratio between melanoma and vitreous fluid was greatest on postcontrast T1-weighted images (average, 72.1), followed by noncontrast T1-weighted images (average, 32.9), and then by T2-weighted images (average, -21.2). Postcontrast T1-weighted images also proved useful in differentiating melanomas from subretinal fluid collections when combined with noncontrast images. We conclude that postcontrast T1-weighted images are most helpful in detecting small uveal melanomas and in differentiating melanomas from subretinal fluid collections.

Lacrimal gland and fossa lesions: role of computed tomography.

The lacrimal gland region can be involved in a wide spectrum of orbital pathology, including tumors and inflammatory and lymphoid lesions. This article focuses special attention on benign mixed tumors because of the excellent prognosis for the patient when it is completely excised at first surgery. The appearance of these lesions on CT is detailed in the illustrations of this article, and the findings of experts in the field are presented as well.

Orbital metastasis: role of magnetic resonance imaging and computed tomography.

Imaging of ocular and oral metastasis with CT and MRI is discussed. The relative advantages and disadvantages of the two techniques are presented. MRI seems to be particularly useful in discriminating intraocular tumor from retinal detachment. Both techniques depict orbital lesions well.

Anophthalmic socket and orbital implants. Role of CT and MR imaging.

The role of CT and MR imaging examination of orbital implants and the anophthalmic socket is expanding constantly. As the imaging techniques gain refinement and resolution, the list of potential clinical application grows. Frequent clinical-radiologic queries regarding the anophthalmic socket include neuro-radiologic findings with congenital anophthalmia or bilateral microphthalmia, the vascularization or position of an orbital implant following enucleation, or orbital recurrence of tumor following enucleation. Common clinical-radiologic questions regarding orbital implants relate to the associated findings of trauma, infection, bleeding, or recurrence of tumor in relationship to the existing orbital implant.

Peripheral nerve tumors of the orbit.

Neurofibromas and schwannomas are orbital neoplasms with similar clinical and radiologic features. Solitary or circumscribed neurofibromas and schwannomas demonstrate slow progressive growth with ocular displacement and are amenable to surgical resection. Plexiform and diffuse neurofibromas are diffusely infiltrative in the orbit and periocular region, they are difficult to resect, and they have a high rate of recurrence. Malignant peripheral nerve tumors are rare, but they have a very high rate of recurrence and a low 5-year survival rate because of extension through the superior orbital fissure to the midbrain region. This article describes the clinical and radiologic features of peripheral nerve sheath tumors.

Cobalt plaque versus enucleation for uveal melanoma: comparison of survival rates.

Two hundred and twenty-three patients treated by cobalt plaque for uveal melanoma were compared with 416 patients treated by enucleation for uveal melanoma in terms of patient survival. The median follow-up time for the patients treated by cobalt plaque was 4.3 years. Kaplan-Meier survival curves were calculated up to five years following treatment based on time to tumour-related deaths. Cox's proportional hazards multivariate analysis was performed to determine which variables were related to melanoma-related deaths while controlling for age, size, and location of the tumours. Statistically significant predictive factors were location of tumour and largest tumour dimension. There was not a statistically significant difference in survival between patients treated by cobalt plaque and those treated by enucleation.

Identification of intraocular tumors by specular microscopy.

Five patients with confirmed diagnoses of intraocular tumors and anterior chamber extension were examined with a wide-field specular microscope. Uniquely shaped dark areas seen on the endothelial surface could be readily distinguished from inflammatory cells by their size and shape. These structures were often associated with a surrounding bright reflection similar to that produced by pigment. The cellular morphology provided by specular microscopy offers the potential for differentiating neoplastic from inflammatory processes in the anterior chamber.