A prospective comparison of fine-needle aspiration cytopathology and histopathology in the diagnosis of orbital mass lesions.

AIM: To assess the diagnostic value of the orbital fine needle aspiration biopsy (FNAB) with an in vitro technique, eliminating the sampling error. DESIGN: Prospective, non-randomised, interventional case series. METHODS: Sixty-eight patients were studied prospectively in institutional clinical practices. Immediately after excision of orbital mass lesions, the removed tissue was stabilised under the hand of the surgeon and biopsied with a 23- or 25-gauge needle. The samples were processed for cytopathological examination with Cytospin. The excised specimens were then submitted for routine histological examination. The cytopathological diagnoses were compared with the final histopathological diagnoses. RESULTS: Six out of 68 lesions were excluded and the remaining 62 cases were divided into four groups as primary malignant, primary benign, secondary malignant and inflammatory lesions, based on histopathological diagnoses. In 43 cases the cytopathological and histopathological diagnoses were the same, with a concordance rate of 69%. Among the malignant tumours, the cytopathological diagnoses correlated with the histopathological diagnoses in 14/14 and 17/27 cases of metastatic/secondary and primary orbital malignancies, respectively. Of 11 primary benign tumours, two cytopathological diagnoses correlated with histopathology. In inflammatory lesions, the cytopathological diagnoses were matched with the histopathological diagnoses in 10/10 biopsies. CONCLUSION: Even when the sampling error is eliminated with an "in vitro FNAB" technique, the concordance rates between histopathological and cytopathological diagnoses varied considerably among different types of orbital mass lesions. FNAB diagnoses were most reliable in metastatic and secondary malignancies and inflammatory lesions, and least reliable in benign orbital neoplasms and cysts.

Multiagent chemotherapy as neoadjuvant treatment for multifocal intraocular retinoblastoma.

PURPOSE: To evaluate the efficacy of multiagent chemotherapy in the neoadjuvant treatment of retinoblastoma. DESIGN: Noncomparative, prospective case series. PARTICIPANTS: Twenty consecutive patients with multifocal intraocular retinoblastoma (4 unilateral, 16 bilateral [36 eyes]). INTERVENTION: Eight cycles of chemotherapy with carboplatin and vincristine were administered at 3-week intervals over a 6-month period. Supplemental therapy was withheld until disease progression was documented. MAIN OUTCOME MEASURES: Disease progression (defined as tumor growth, vitreous or subretinal seed progression, and new tumor formation), delay of external beam radiotherapy, and ocular survival. RESULTS: Thirty-six eyes were treated. Eighteen eyes had Reese-Ellsworth group I-III tumors, and 16 eyes had Reese-Ellsworth group IV-V tumors at diagnosis. Two patients, who had unilateral disease at diagnosis, subsequently had tumors develop in the contralateral eye. Nineteen of 20 patients (95%) completed eight cycles of chemotherapy without disease progression. Three eyes of three different patients were successfully treated with chemotherapy alone. Thirty-three of 36 eyes (92%) progressed after completion of chemotherapy: 15 of the 18 eyes (83.3%) with Reese-Ellsworth group I-III and 16 of 16 eyes (100%) with Reese-Ellsworth group IV-V tumors. Seventeen eyes (52%) had growth of a tumor, whereas 14 eyes (42%) had progressive vitreous seeding, and 2 eyes (6%) had new tumors develop. Fifteen eyes (42%) required external beam radiotherapy. Twenty-nine of 36 (80.5%) eyes were salvaged. The median follow-up after chemotherapy was 19 months (range, 3-42 months). CONCLUSIONS: Multiagent chemotherapy alone does not ensure a cure for multifocal intraocular retinoblastoma. Supplemental focal therapy is needed to control disease progression.

Latanoprost-induced iris heterochromia and open-angle glaucoma: a clinicopathologic report.

PURPOSE: To report the histopathologic and immunohistochemical findings from the iridectomy specimen of a patient with acquired unilateral iris heterochromia due to latanoprost. PATIENT AND METHODS: A 45-year-old woman with open-angle glaucoma and unilateral iris heterochromia was evaluated for uncontrolled intraocular pressure increase. Subsequently, the patient underwent trabeculectomy with mitomycin C and an iridectomy specimen was obtained for analysis. RESULTS: The histopathologic analysis of the iridectomy specimen did not reveal any nuclear atypia, nuclear crowding, or mitotic figures. Immunohistochemical studies showed that the iris melanocytes were negative for HMB45 and S-100, and weakly positive for Melan A. CONCLUSION: Latanoprost-associated iris color change may exhibit a diffuse, uniform, dark velvet-brown appearance, thereby simulating diffuse iris melanoma. Histopathologic and immunohistochemical analysis confirmed the benign characteristics of the affected iris melanocytes.

Sentinel node biopsy for orbital and ocular adnexal tumors.

PURPOSE: To describe a technique for sentinel node mapping and biopsy in patients with orbital or adnexal tumors. METHODS: Five patients with orbital and adnexal tumors were studied. Two patients had malignant eyelid melanomas (one of the skin and one of the conjunctiva), one with orbital invasion. Two patients had sebaceous gland carcinoma, and one patient had a mucoepidermoid carcinoma of the conjunctiva; 500 microCi of Technetium-99m sulfur nanocolloid (Nycomed Amersham, Princeton, NJ) diluted to 1.0 mL was injected intradermally at the lateral canthus. The patients were positioned as they would be during surgery. Lymphoscintigraphy was performed by means of anterior, lateral, and oblique views. The tracer was followed to the first lymphatic basin, and the sentinel node was identified. Cutaneous markers were placed to denote the site. During surgery, lymphoscintigraphy scans and a hand-held gamma probe were used to locate the sentinel node. Once excised, the sentinel node was sent for histopathology. Frozen sectioning confirmed the presence of lymphoid tissue. Permanent sections with immunohistochemical markers were performed to examine for metastatic disease. RESULTS: The sentinel node biopsy technique was applied to 5 patients with orbital and adnexal tumors. All lymph nodes were free of tumor on histopathologic examination. CONCLUSIONS: Sentinel node mapping and biopsy are possible for orbital and adnexal tumors. The morbidity of elective lymph node dissection and adjuvant radiotherapy can be avoided. Our results are preliminary, and further work must be done to identify the lymphatic basins of the orbit and ocular adnexa.

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.

Intracranial paraganglioma (glomus tumor) with orbital extension.

PURPOSE: To describe the clinical features of an intracranial glomus tumor with orbital extension. METHODS: Case report. RESULTS: A 15-year-old girl was examined for a large, vascular intracranial mass extending into the orbit. Attempted orbital biopsy was aborted for fear of uncontrollable hemorrhage. Tumor embolization and partial resection was performed. Histology was consistent with paraganglioma (glomus tumor). CONCLUSION: The orbital surgeon should include glomus tumor in the differential diagnosis of vascular orbital lesions. Their vascularity and hemorrhagic potential should be considered when approaching these lesions surgically.

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.

Intraocular involvement in multifocal fibrosclerosis.

OBJECTIVE: To report the clinical and histopathologic findings of intraocular involvement in a patient with multifocal fibrosclerosis and the response of the patient's choroidal masses to external beam radiotherapy. DESIGN: Case report with clinicopathologic correlation of enucleated eyes. METHODS: The patient was studied by clinical observation, contact B- and A-scan ultrasonography, orbital magnetic resonance imaging, fluorescein angiography, indocyanine green angiography, choroidal biopsy, and gross and histopathologic examination of the enucleated eyes. Tissue obtained at an earlier laparotomy was also reviewed. External beam radiotherapy was used when high-dose corticosteroid and low-dose methotrexate therapy failed to decrease the size of the choroidal masses or improve the patient's vision. MAIN OUTCOME MEASURES: Changes in the clinical and ultrasonographic size of the choroidal masses, the clinical appearance of these masses, and the patient's visual acuity in response to external beam radiotherapy were monitored premortem. Histopathologic findings in the enucleated eyes were compared with the changes in previous abdominal and choroidal biopsy specimens and with tissue alterations reported in multifocal fibrosclerosis. RESULTS: Biopsy of the choroidal mass revealed a fibrosclerosing process similar to that found in the abdomen. The patient received external beam radiotherapy with disappearance of the masses. Fibrosclerosing changes similar to those seen in the abdomen were observed replacing the choroid in the enucleated eyes. CONCLUSIONS: Multifocal fibrosclerosis may involve the choroid with histopathologic changes similar to those that have been described in other locations in the body. External beam radiotherapy may be an effective treatment for intraocular involvement by multifocal fibrosclerosis.

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.

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.

Penetrating orbital injury with organic foreign bodies.

OBJECTIVE: The authors reviewed the clinical features, diagnostic workup, and management of patients of penetrating orbital injuries with retained organic foreign bodies. DESIGN: Retrospective, noncomparative case series. PARTICIPANTS: Nineteen patients (15 males, 4 females) with penetrating orbital injuries due to organic foreign bodies. RESULTS: The series included 15 (78.9%) males and 4 (21.1%) females who ranged in age from 6 months to 40 years (mean = 14.6 years); 12 (63.2%) patients were younger than 12 years of age. Twelve (63.2%) right and 7 (36.8%) left orbits were involved. Time between injury and presentation varied from a few hours to 9 months. Most common injury site was the superior orbit in 11 (57.9%) patients leading to abnormal extraocular motility (84.2%), proptosis (68.4%), and upper lid ptosis (47.4%). Associated pathologies also included acute cellulitis in 11, orbitocutaneous fistula in 5, and osteomyelitis in 2 patients. Preoperative computed tomography (CT) and magnetic resonance imaging (MRI) identified the foreign bodies in 42% and 57% of the patients, respectively. CONCLUSION: Preoperative identification of the foreign material in the orbit was found to be very helpful for patient management but was only possible in approximately 50% of our cases with the use of CT and MRI. The vision in our patients usually improved shortly after treatment; the long-term complications more often included extraocular muscle and eyelid motility problems and periorbital scarring.

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.

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.

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.

Exophthalmometry: a comparative study of the Naugle and Hertel instruments.

Exophthalmometry is frequently used as a tool in the evaluation of proptosis. The purpose of this study was to compare the clinical usefulness of the newly developed Naugle superior and inferior orbital rim-based exophthalmometer with that of the Hertel exophthalmometer, as well as to obtain normal values and analysis of possible race and sex differences. In the first part of this study, we measured 135 patients in a double-blind study in order to evaluate the repeatability of serial measurements using each instrument. Comfort was also evaluated in this series of patients. In the second part, a single observer measured 205 patients in order to obtain information for a multifaceted analysis. In the first part of the study, we found the Hertel measurement to be more variable in terms of coefficients of variation. We further found the Hertel base measurements to vary more between observers than those of the Naugle instrument. In the second part, we found that the mean values of globe position were not statistically different (mean Naugle, 17.23; mean Hertel, 18.61; p = 0.909). A significant difference was noted between black and white patients when using the Naugle instrument. A difference between white and black women was noted with the Hertel instrument. The results of this analysis strongly suggest that the values obtained from the Naugle exophthalmometer are more repeatable in serial measurements compared with the Hertel values. However, single measurements of globe position from all patients were statistically similar. We conclude that the Naugle instrument is comparable in accuracy to the Hertel instrument for measuring ocular position, as well as being more comfortable for the patient. The Naugle instrument has the advantage of measuring hyperophthalmos and hypoophthalmos with a vertical gradient scale.

Particle size and drug interactions of injectable corticosteroids used in ophthalmic practice.

PURPOSE: The aim of this study is to compare the particle sizes of commercially available corticosteroids for intralesional injection to manufacturers' specifications and to evaluate changes in size when mixed with other steroids and other commonly used solutions. METHODS: The particle sizes of dexamethasone sodium phosphate, methylprednisolone acetate, triamcinolone acetonide, and betamethasone sodium phosphate and beta-methasone acetate were measured with the Coulter counter. Each steroid was mixed with each of the others, lidocaine, and lidocaine with epinephrine, then measured immediately and after 1 hour to determine the effect on particle size. RESULTS: All steroids showed accurate manufacturer specifications. Dexamethasone showed an increase in particle size when mixed with lidocaine alone, immediately and after 1 hour. Triamcinolone showed an increase in particle size only after 1 hour after mixture with lidocaine alone. Triamcinolone, dexamethasone, and methylprednisolone particles increased in size when mixed with lidocaine and epinephrine. Dexamethasone and triamcinolone showed an additional increase in size when allowed to sit for 1 hour after mixture with lidocaine and epinephrine. Mixing steroids caused no increase in particle size. CONCLUSION: Because an increase in the number of particles greater than 20 microns in a solution also increases the likelihood of vascular occlusion, the authors urge care in the mixing of these drugs, especially with lidocaine and epinephrine. If mixture is necessary, the authors recommend that injection take place immediately subsequent.

Gadolinium enhancement: improved MRI detection of retinoblastoma extension into the optic nerve.

We performed T1-, T2-, proton density-weighted, and T1-weighted gadolinium-enhanced MRI on 24 patients with retinoblastoma, using a 1.5 T superconducting unit and head and orbital surface coil imaging. All patients underwent a complete ophthalmologic examination, including B-scan ultrasonography. CT was performed on 10 of 24 patients. Pathologic correlation was obtained in 18 patients who required enucleation. Contrast-enhanced T1-weighted MRI with fat suppression was the sequence most sensitive to optic nerve extension and provided the greatest differentiation between tumor and uninvolved extrascleral tissue. Retinoblastoma demonstrated contrast enhancement.

Rhabdomyosarcoma presumed metastatic to the orbit.

BACKGROUND: Orbital metastasis from rhabdomyosarcoma is a rare disorder with a poor prognosis for long-term survival. Only one other detailed account of this disorder has appeared in the ophthalmic literature. METHODS: The authors report the clinical features of four patients with presumed orbital metastasis from alveolar and embryonal rhabdomyosarcoma. RESULTS: The most common ophthalmic manifestations of orbital metastasis from rhabdomyosarcoma in these patients included proptosis, reduced visual acuity, orbital pain, and motility disorders. Computed tomography documented orbital masses in all of the patients. In those patients with a primary tumor of the maxillary sinus, there was no evidence of direct extension into the orbit. Despite combination chemotherapy and radiation, all four patients died of their illness within 6 months of orbital metastasis. CONCLUSIONS: Although uncommon, rhabdomyosarcoma should be considered in the differential diagnosis of metastatic tumors to the orbit in children and adults. Despite the poor prognosis, prompt diagnosis and palliative radiotherapy may improve the quality of life for these patients with terminal disease.