Management and prognosis of Merkel cell carcinoma of the eyelid.

OBJECTIVE: To evaluate the clinical presentation, treatment, and long-term follow-up of eyelid Merkel cell carcinoma. DESIGN: Retrospective noncomparative interventional case series. PARTICIPANTS: Fourteen patients with primary eyelid Merkel cell carcinoma. METHODS: Cases of Merkel cell carcinoma for which long-term follow-up was available were solicited from members of the American Society of Ophthalmic Plastic and Reconstructive Surgery through an on-line e-mail/news group. MAIN OUTCOME MEASURES: Follow-up period, treatment history, presence and type of recurrence, and mortality. RESULTS: Average follow-up was 33.4 months. Of the 14 cases identified, only 2 patients (14%) received prophylactic therapy beyond wide surgical excision. Three patients (21%) had recurrences, none of whom initially received prophylactic therapy (i.e., radiation therapy, lymph node dissection, and/or chemotherapy) beyond wide surgical excision. One patient (7%) died of metastatic Merkel cell carcinoma. CONCLUSIONS: Merkel cell carcinoma is a rare skin malignancy that occasionally affects the eyelid, with the potential for regional and distant metastasis. Consideration should be given to the use of prophylactic adjunctive therapies beyond wide surgical excision while simultaneously considering the morbidity of these therapies.

Mucoepidermoid carcinoma of an accessory lacrimal gland with orbital invasion.

PURPOSE: To report a case of mucoepidermoid carcinoma of an accessory lacrimal gland with orbital invasion. METHODS: The clinical history and pathologic findings of a patient with a left upper eyelid lesion were reviewed. RESULTS: The patient was evaluated and found to have an epithelial tumor arising in an accessory lacrimal gland. Special stains showed mucin production by individual tumor cells. The tumor was classified as mucoepidermoid carcinoma. CONCLUSIONS: Mucoepidermoid carcinoma may arise in accessory lacrimal glands and invade the orbit.

The association between cigarette smoking and basal cell carcinoma of the eyelids in women.

PURPOSE: To determine if there is an association between basal cell carcinoma of the eyelid and cigarette smoking. METHODS: A cross-sectional, retrospective, case-controlled study was done comparing the prevalence of cigarette smoking in 112 patients with biopsy-proven basal cell carcinoma of the eyelid to age and sex-matched controls with other eyelid disorders. Exposure-odds ratios were computed for each group. RESULTS: Patients with basal cell carcinoma of the eyelid were no more likely to be smokers than controls (odds ratio [OR] 1.78, not statistically significant at the 95% confidence interval [CI]). When divided by sex however, there was an association between cigarette smoking and basal cell carcinoma in women (OR 2.87, statistically significant at the 95% CI) but not in men (OR 1.30, not statistically significant at the 95% CI). CONCLUSION: Basal cell carcinoma of the eyelid is associated with cigarette smoking in women but not in men.

Sotradecol (sodium tetradecyl sulfate) injection of orbital lymphangioma.

PURPOSE: To describe the results of intralesional injection of the sclerosing agent sodium tetradecyl sulfate in patients with lymphangioma. METHODS: Three patients (one child and two adults) were treated. RESULTS: Two patients had improvement in the size of the lymphangioma, although the result was short-lived in one instance. Minimal change was noted in the third patient. Two patients had transient edema and ecchymosis, and one patient had a mild allergic reaction to the injected solution. CONCLUSIONS: Sodium tetradecyl sulfate may be a useful therapeutic option for some patients with eyelid or orbital lymphangioma, particularly if a previous operation has not been performed.

Kimura disease of the orbit and ocular adnexa.

Kimura disease (KD) is a distinct clinicopathologic entity that has been the subject of considerable confusion and debate. Although common in Asia, KD rarely occurs in non-Asian patients. Kimura disease shares both clinical and histopathologic features with angiolymphoid hyperplasia with eosinophilia (ALHE). Because of this overlap and the rarity of KD in Europe and the United States, KD and ALHE have been used synonymously in the Western medical literature, as they were thought to represent variations of the same disease. Some pathologic reports have called for distinguishing KD and ALHE as two separate entities, based on their histologic features. Kimura disease occurs most commonly in the head and neck region and has been described in the orbit, eyelids, and lacrimal gland more frequently than ALHE. Because both diseases can cause proptosis, lid swelling, ocular dysmotility, or a palpable mass, they should be considered in the differential diagnosis of orbital lesions occurring in adults. We report two cases of KD involving the orbit and ocular adnexa, and review additional cases reported in the literature. The ophthalmic literature does not clearly reflect the current understanding that KD and ALHE are best considered two separate clinicopathologic entities.

Metastatic esophageal carcinoma to the orbit.

PURPOSE: To report a case of esophageal adenocarcinoma and areas of gastric differentiation in the esophagus (Barrett esophagus) metastatic to the orbit. METHODS: A 47-year-old man with a history of esophageal carcinoma developed turgescence around his left eye. He underwent a biopsy and histologic examination of a left orbital mass. RESULTS: Histopathology of the orbital tumor was consistent with metastatic adenocarcinoma from the esophagus. CONCLUSIONS: This metastatic adenocarcinoma to the orbit likely arose in Barrett esophagus.

Osseous metaplasia in proliferative vitreoretinopathy.

PURPOSE: To report the clinicopathologic features of intraocular osseous production in association with proliferative vitreoretinopathy. METHOD: The clinical and histopathologic features of two patients with proliferative vitreoretinopathy and intraocular bone formation are reviewed. RESULTS: Preretinal osseous tissue incorporated in the proliferative vitreoretinopathy was surgically removed in one patient, and osseous tissue was present in the proliferative vitreoretinopathy in the enucleated eye of the other patient. CONCLUSIONS: Bone formation, presumably from metaplastic retinal pigment epithelium, may be present in proliferative vitreoretinopathy tissue. The intraocular bone is present internal rather than external to the neurosensory retina.

Myoepithelioma of the lacrimal gland.

A right orbital tumor was excised from a 76-year-old woman. Pathological examination showed that the tumor was composed of spindle to cuboidal cells arranged in a solid to trabecular pattern. Immunohistochemical stains were positive for S-100 protein, muscle-specific actin, cytokeratins MAK6 and AE1,3, and glial fibrillary acid protein and negative for CD34 in tumor cells. Ultrastructural features of tumor cells included microvillous processes, intercellular junctions, and intracytoplasmic filaments with electron densities. To our knowledge, this is the first non-spindle cell myoepithelioma noted to arise in the lacrimal gland. This tumor likely has a similar biological behavior to pleomorphic adenoma (benign mixed tumor).

[Reparative giant cell granuloma with orbital involvement]. / Reparatives Riesenzellgranulom mit Orbitabeteiligung.

A 54-year-old woman was evaluated for a left orbital mass that had been present for one year. Examination showed mild superolateral displacement of the normal left eye and a firm medical orbital mass. A complete resection of the lesion was accomplished via a combined neurosurgical, otolaryngologic and ophthalmologic superior orbital approach. Histologic features of the mass were consistent with giant-cell (reparative) granuloma. However, it has to be pointed out that fibroosseous lesions with giant-cells display similar histologic features and require clinicopathologic correlation including radiographic studies for final diagnosis.

[Granular cell tumor of the eyelid]. / Granularzelltumor des Augenlides.

A 54-year-old woman was evaluated for a right lower eyelid lesion that had been present for four months. Examination showed a firm, yellow to brown mass with associated loss of eyelashes. A complete resection of the lesion was performed and the lid was reconstructed with the Hughes procedure. Histologic features of the excised mass were consistent with a granular cell tumor. Immunohistochemical stains were positive for S-100 protein, neuron specific enolase, laminin, and various myelin proteins in the tumor cells. Ultrastructural examination displayed that tumor cells were distended by autophagic granules and some cells contained angulated bodies (Bangle bodies). Granular cell tumors probably origin from Schwann cells and rarely involve the eyelids.

Invasive keratoacanthoma of the eyelid and ocular adnexa.

PURPOSE: To report three patients with superficially invasive crateriform squamous proliferations of periocular tissue. METHODS: The authors identified three patients with superficially invasive periocular tumors that had clinical features of keratoacanthoma. Clinical histories, radiographs, and surgical pathologic specimens were reviewed. RESULTS: All three tumors arose over several weeks, had a crateriform configuration, and exhibited superficial invasion of underlying tissues, including perineural invasion and infiltration into skeletal muscle. All three tumors were classified as invasive keratoacanthoma. One tumor exhibited late perineural extension into the cavernous sinus and convincing histologic features consistent with squamous cell carcinoma. CONCLUSION: The clinical importance of recognizing invasive keratoacanthoma is that although the tumor has the potential for spontaneous involution, locally aggressive behavior with deep perineural invasion is possible. This tumor is considered to represent a variant of squamous cell carcinoma. The authors recommend complete surgical excision of crateriform squamous proliferations with frozen section control of margins of resection.

Bone formation in hydroxyapatite orbital implants.

PURPOSE: We examined the histopathologic features of two hydroxyapatite orbital implants that were removed from two patients. METHODS: Two surgically removed hydroxyapatite orbital implants were decalcified and processed for routine light microscopic examination. RESULTS: Both implants demonstrated fibrovascular ingrowth and foci of bone formation. CONCLUSION: Bone may form in hydroxyapatite orbital implants.

Inflammatory pseudotumor of the eyelid.

We present three patients whom we believe developed idiopathic inflammatory pseudotumors confined to the eyelid tissues with no evidence of orbital involvement. All three patients were misdiagnosed initially as having lesions of an infectious etiology. All three patients responded completely and rapidly to oral steroid therapy after the correct diagnosis was confirmed by biopsy. We believe that pseudotumor of the eyelid is a distinct subtype of idiopathic inflammatory pseudotumor.

Pneumococcal endophthalmitis associated with nasolacrimal obstruction.

Pneumococcal endophthalmitis can be a devastating postoperative infection after cataract extraction or penetrating keratoplasty. Streptococcus pneumoniae was isolated as the causative agent in three of the 124 patients (2%) who were treated for endophthalmitis at our institution between 1984 and 1990. Two of the three patients lost light perception in the affected eye. All three patients had previously unrecognized or untreated chronic nasolacrimal obstruction of varying causes. We studied the role of pneumococcal lacrimal conjunctivitis in the pathogenesis of the postoperative pneumococcal endophthalmitis in these patients.

Downgaze ptosis.

In involutional blepharoptosis, an abnormally low lid position in primary gaze is accompanied by increased lid excursion in downgaze. Described here are four patients with little or no ptosis in primary position but significant and symptomatic obstruction of the visual axis in downgaze only. All responded well to minimal aponeurotic advancement with sufficient elevation of the lids in downgaze to allow reading but with little or no discernible change of the lids in primary position. This "downgaze ptosis," not previously described, may be an early stage of typical involutional ptosis.

Glomus cell tumor of the eyelid.

BACKGROUND/METHODS: Glomus cell tumor, a vascular neoplasm, has been rarely reported to occur in the eyelid. The authors examined the clinicopathologic features of two patients with glomus cell tumor of the eyelid. RESULTS: The tumors occurred as enlarging violaceous to bluish masses. One patient had a solitary eyelid tumor that contained sheets of individual glomus cells. The other patient had eyelid and wrist tumors and multiple family members with similar lesions. CONCLUSION: This report provides clinicopathologic evidence that glomus cell tumor can occur in the eyelid in two different settings; in one setting, the tumor is solitary and sporadic, and in the second setting, it is multiple and familial.