The multidisciplinary management of psammomatoid ossifying fibroma of the orbit.

OBJECTIVE: To discuss the multidisciplinary management of psammomatoid ossifying fibroma (POF) of the orbit and to clarify the clinicopathologic terminology. DESIGN: The authors present a cohort of cases of POF involving the frontal and ethmoid sinuses and the orbit and discuss the nomenclature and literature. PARTICIPANTS: Three patients with POF and their treatment are discussed. INTERVENTION: Patients were worked up and treated by a multidisciplinary team using imaging studies and histopathologic analysis. Reconstruction, if necessary, was carried out at the time of excision or in a second-stage procedure. MAIN OUTCOME MEASURES: In each case, the lesion was completely excised and has not recurred. RESULTS: The diagnosis of POF was made in each case, and the patient underwent successful resection of the tumor. CONCLUSION: The authors' experience suggests that a multidisciplinary approach, including a radiologist, pathologist, neurosurgeon, otolaryngologist, craniofacial surgeon, and orbital specialist, may be useful in the evaluation and management of these lesions.

The role of the integrin family of adhesion molecules in the development of tumors metastatic to the orbit.

Tumors metastatic to the orbit frequently originate from certain primary tumors such as breast, lung, prostate, and melanoma. The site-specific nature of orbital metastases, as well as that of other metastatic lesions, cannot be the result of random seeding. We present evidence from a review of the literature demonstrating that tumor cells express adhesion molecules of the integrin family, and that these receptors play a pivotal role in the development of a metastatic colony. We investigated orbital metastatic lesions from prostate carcinoma, malignant melanoma, and lobular breast carcinoma to determine the level of integrin expression by immunohistochemistry. Several integrin subunits (alpha2, alpha4, beta3) were found to have increased expression in the metastasis when compared to normal prostate tissue and normal melanocytes. The increased expression of these integrins may be responsible for the tendency of these tumors to metastasize to the orbit, as well as for the tendency of prostate tumors to metastasize to bone. The results from the staining of the breast metastasis were inconclusive.

Orbital and eyelid manifestations of neurofibromatosis: a clinical study and literature review.

Neurofibromatosis (NF) is an autosomal dominant abnormality that may effect multiple organ systems. Eyelids, orbits, and adjacent tissues may be involved with varying frequency and severity. Plexiform neurofibromas, causing mechanical ptosis and proptosis, optic nerve gliomas, and sphenoid wing dysplasia are some of the more common orbital and eyelid findings in NF. A series of 10 patients followed for up to 18 years is reported to describe features associated with NF, their clinical evolution, and their management.

Ocular adnexal lymphoma. A clinicopathologic study with identification of lymphomas of mucosa-associated lymphoid tissue type.

PURPOSE: Extranodal marginal zone B-cell lymphoma (low-grade B-cell lymphoma of mucosa-associated lymphoid tissue [MALT] type) is a distinctive type of lymphoma that usually arises in association with mucosa or other epithelial structures and has an indolent clinical course. The frequency and clinical features of MALT lymphomas in the ocular adnexa have not been well studied. METHODS: The authors examined the clinicopathologic features of ocular adnexal lymphoma, identified a subset of cases with MALT characteristics, and determined patient outcome. RESULTS: The 42 patients, 16 men and 26 women age 35-89 years (mean, 64) were followed an average of 4.8 years. Thirty-two patients had ocular adnexal involvement at presentation (primary ocular adnexal lymphoma) and 10 had a history of lymphoma that relapsed in the orbit (secondary ocular adnexal lymphoma). In the primary group, 23 patients had lymphoma confined to the ocular adnexa, 3 had a single lesion that invaded adjacent structures, and 6 had distant spread at the time of presentation. Twenty-five patients achieved a complete remission. Nine patients, including 6 patients whose disease was localized initially, had progression or relapse of disease in distant sites. At last follow-up, 21 patients were free of disease, 9 were alive with disease and 2 had died of lymphoma. In the secondary group, at last follow-up, 1 patient had died of other causes, free of lymphoma, 3 patients were alive with disease and 5 had died of lymphoma (outcome not known in 1 case). Using the recently described revised European-American lymphoma classification, we found 16 MALT lymphomas, 8 diffuse large B cell, 12 follicular center, 3 mantle cell, 1 B-small lymphocytic lymphoma, and 2 unclassifiable low-grade lymphomas. The most common type of primary lymphoma was MALT type (15 of 30 classifiable cases), and the most common secondary lymphoma was follicular center (6 of 10). No increased frequency of conjunctival or lacrimal gland involvement by MALT lymphomas was found. All 33 lymphomas with immunophenotyping were of B lineage. CONCLUSIONS: Ocular adnexal lymphomas are B-cell tumors that develop in older adults, predominantly among women. Primary orbital lymphomas have a favorable prognosis; a high proportion of them have MALT characteristics.

Merkel cell carcinoma of the eyelid and periocular tissues.

Five patients had eyelid and periocular Merkel cell carcinoma. The tumor was located on the left lower eyelid in two patients, the left upper eyelid in one patient, the right upper eyelid in one patient, and was metastatic to the right outer canthus in one patient. The mean duration of symptoms was approximately four months. The diagnosis of Merkel cell carcinoma was not suspected clinically in any of the four primary eyelid cases, but was only established on histopathologic examination of biopsy specimens. Light microscopy disclosed carcinoma with small primitive cells in all five tumor biopsy specimens. Immunohistochemical studies showed neuron-specific enolase and keratin and transmission electron microscopy demonstrated neurosecretory granules typical for Merkel cell carcinoma. All five patients in this study were treated with wide surgical excision of the eyelid tumors with intraoperative frozen-section monitoring of the margins of resection. The left lower eyelid Merkel cell carcinoma spread to the preauricular lymph node in one patient. This patient subsequently died of metastatic Merkel cell carcinoma. One patient with metastatic right outer canthus Merkel cell carcinoma received radiotherapy (6,550 cGy). Eyelid Merkel cell carcinoma has the potential for recurrence and metastatic spread. We recommend lifetime follow-up for patients treated for eyelid Merkel cell carcinoma.

Venous engorged tissue flaps treated with medicinal leeches.

We evaluated the ability of leeches to improve the tolerance of pedicle flaps to periods of venous congestion. Axial pedicle flaps were created on 14 rabbit ears; the venous circulation of each flap was compromised with a suture ligature. Medicinal leeches were applied to seven flaps; the other seven flaps were untreated. Tissue viability was assessed by both clinical examination and fluorometric analysis of fluorescein dye delivery. Leech-treated flaps showed poorer reperfusion than untreated flaps. No statistically significant difference was found in comparing the two groups.

A spectrum of bilateral squamous conjunctival tumors associated with human papillomavirus type 16.

Three patients with bilateral tumors presenting as multiple keratinizing and verrucous lesions of the bulbar and tarsal conjunctiva were determined by DNA amplification and hybridization studies to harbor human papillomavirus type 16 (HPV-16). Results of biopsy in two patients showed infiltrating squamous cell carcinoma in one eye and dysplasia or carcinoma in situ in the fellow eye. In the third patient, focal, inflamed, hypertrophic, papillary lesions with pseudoglandular invaginations of the surface epithelium were found in the tarsal conjunctivae of both eyes. These are the first documented cases of bilateral conjunctival tumors associated with human papillomavirus.

Sebaceous and meibomian carcinomas of the eyelid. Recognition, diagnosis, and management.

Sebaceous carcinoma of the eyelid is frequently misdiagnosed clinically and histopathologically. The tumor may present as a lid mass, recurrent chalazion, or diffuse unilateral blepharoconjunctivitis. Fifty percent of cases are misdiagnosed, often by an inexperienced general pathologist interpreting the initial biopsy. Frozen-section monitoring of surgical margins has been reported unreliable in 25% of cases. Mohs surgery is also unreliable in cases where there is pagetoid intraepithelial spread or skip lesions. This tumor may spread regionally into the lacrimal secretory and excretory systems, to regional lymph nodes, and rarely disseminate hematogenously. Guidelines for management are discussed.

Orbital augmentation by hydroxylapatite-based composites. A rabbit study and comparative analysis.

Hydroxylapatite, a synthetic bone mineral, was implanted alone or as a composite with either calcium sulfate (plaster of Paris) or Avitene (microfibrillar collagen hemostatic agent) into the orbital bony defects and orbital soft tissues of 23 rabbits. Plain Avitene was also placed into these bony and soft tissue sites and bone defects with no additives served as controls. Additionally, each implant material was placed into subcutaneous tissues to determine its relative inflammatory potential. New bone formation was noted in bony defects as early as 10 days. Bone ingrowth was greatest when hydroxylapatite was in direct apposition to bone. Material implanted away from bone showed predominantly connective tissue ingrowth. Minimal inflammatory reaction was noted early on in the hydroxylapatite and hydroxylapatite/calcium sulfate samples and had resolved by 2 months when placed in both soft and bony tissues. Samples of Avitene and the hydroxylapatite/Avitene composite demonstrated an intense inflammatory response, and in several cases large granulomas were seen after 2 months.

Orbital invasion by malignant eyelid tumors.

An important factor in the preoperative evaluation of patients with invasive periorbital carcinomas is the presence or absence of orbital bone involvement. A retrospective study was performed to evaluate the reliability of computed tomography (CT) in detecting orbital bone invasion. Eleven patients with extensive facial-orbital basal cell or squamous cell carcinomas were evaluated preoperatively by CT and the results were compared with the clinical findings and postoperative histopathologic examination confirmed bone and/or periosteal invasion, CT was positive in three (43%). Based on these findings, we strongly recommend that CT not be relied upon in excluding orbital bone involvement. Bony invasion may be histologically present, even though destruction is not seen on CT scans. Therefore, a normal CT scan should not deter one from removing clinically abnormal bone or periosteum and submitting it for histologic examination.

Sebaceous carcinoma. Diuretic use, lacrimal system spread, and surgical margins.

Twenty cases of adnexal sebaceous carcinoma managed by contemporary methods, including monitoring of surgical margins, were reviewed. Eight of 20 patients were taking diuretic medications. The initial clinical diagnosis was incorrect in all cases, and 50% of cases were misdiagnosed by the pathologist interpreting the initial biopsy. The canaliculus was identified in 7 cases, and was involved by tumor in 3 cases. Tumor was identified in the lacrimal sac and inferior turbinate in an additional case. Thus, tumor spread into the lacrimal excretory system was documented by histologic findings in 4 cases.

Eosinophilic granuloma of the orbit with spontaneous healing.

A 13-year-old boy presented with a 2-month history of swelling of the right upper eyelid and displacement of the right eye. Computed tomography (CT) showed a tumor in the right orbit and a large osteolytic defect of the orbital frontal bone. At surgery a yellowish-tan mass was found replacing the orbital roof and abutting the dura. Incisional biopsy established the diagnosis of eosinophilic granuloma. In the absence of further therapy, the mass resolved, and the bony defect completely reossified within 14 months. During more than 2 years of follow-up, there has been no evidence of local recurrence or systemic disease. This case demonstrates that eosinophilic granuloma may resolve spontaneously with healing of bony defects, thus eliminating the need for adjunctive resection or radiation therapy in selected patients.

Interferon-alpha therapy of recurrent conjunctival papillomas.

Five patients with multiple, recurrent conjunctival papillomas underwent surgical excision of their tumor and then received interferon alpha-N1, 5 X 10(6) units/m2 (5 Mu/m2), intramuscularly daily for one month. A similar dose was given two to three times per week for the next six months and tapered off or discontinued thereafter. The follow-up period varied from one to four years. Two patients have had no recurrence of tumor. The other three patients have had recurrences of lesser severity upon tapering or discontinuing the interferon, and repeat surgical or laser excision of these lesions has been performed. The presence of koilocytosis and human papillomavirus type 11a DNA sequences was noted in all specimens large enough to examine, whereas papillomavirus structural antigens were detected in only two of five specimens. A regimen of interferon therapy appears to be tumor suppressive, but not curative.

Subconjunctival orbital fat prolapse.

Subconjunctival orbital fat prolapse is a benign entity in which fat herniates through Tenon's capsule. It is often confused with other lesions such as lacrimal gland tumors, lymphoid tumors and lipodermoids. The clinical and computed tomographic findings in a series of 15 patients are presented, and the features that are helpful in the differential diagnosis are discussed.

The dural shunt syndrome. Pathophysiology and clinical course.

Dural shunts are vascular communications in which blood flows through small meningeal branches of the carotid arteries to enter the venous circulation near the cavernous sinus. The features of 10 patients with dural shunts are described. Most of these patients were postmenopausal women with dilated episcleral vessels, unilateral elevation of intraocular pressure, and exophthalmos. Pain or discomfort was common and ptosis or limited eye movement was usually present. These findings were characteristically more mild than those associated with carotid-cavernous sinus fistulas. CT scans usually showed enlargement of the extraocular muscles and the superior ophthalmic vein. Arteriography visualized partial or complete occlusion of intracranial venous drainage posterior to the cavernous sinus in all patients. Vascular dynamics of dural shunts are reviewed, and it is suggested that many of these communications may be congenital and that the onset of clinical abnormalities may be associated with the occurrence of intracranial venous thrombosis. Most patients eventually had resolution of their clinical abnormalities without treatment, and in some cases this improvement occurred soon after carotid arteriography.

Detection of human papillomavirus DNA sequences in conjunctival papilloma.

Two histologically proven conjunctival papillomas (one from a 33-month-old boy and the other from a 28-year-old woman) were examined for the presence of papillomavirus by DNA molecular hybridization. The first case, a recurrent tumor known to be positive for papillomavirus structural antigen, demonstrated human papillomavirus DNA sequences that cross-hybridized to a human papillomavirus type 11 DNA probe. The second case, an initial tumor, which was negative for papillomavirus structural antigen, demonstrated no viral DNA sequences by hybridization.

Choroidal folds and refractive errors associated with orbital tumors. An analysis.

Twenty patients with unilateral exophthalmos secondary to orbital tumors underwent fundus photography; 16 underwent fluorescein angiography. Choroidal folds were found in 15 eyes. The location of each tumor with respect to the globe was determined by computed tomography. In addition, most patients underwent B-scan ultrasonography and surgical exploratory procedures. Choroidal folds were more common in patients with greater amounts of exophthalmos and with anteriorly located tumors. Moreover, the pattern of choroidal folds generally reflected the location of the tumor within the orbit. Comparison with the uninvolved fellow eyes showed that preoperative refractive errors were usually shifted toward hyperopia with intraconal tumors, whereas extraconal tumors were typically associated with higher astigmatic errors on the involved side.

Computed tomography in the management of orbital trauma.

Computed tomography (CT scanning) is a valuable aid in the management of orbital trauma because it provides a method of visualizing tissues and objects of many different densities. CT scanning can be used to study orbital fractures in order to suggest the prognosis for improvement of ocular malpositions after surgery. The extent of fractures and the etiology of many soft tissue abnormalities can be determined by this technique. Foreign bodies can be accurately localized so that the approach to their removal can be planned.