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Biomedical subjects

R C Eagle

Publications and source records attributed to R C Eagle.

At least 73 records · Page 4Linked to original sources
PubMed1999

Extraocular extension of unrecognized choroidal melanoma simulating a primary optic nerve tumor: report of two cases.

BACKGROUND: Orbital extraocular extension of choroidal melanoma is well known and is usually detected in eyes with medium and large tumors, but it is very rare with small melanomas. It is particularly unusual for choroidal melanomas of any size to invade the optic nerve or its meninges. DESIGN: Two case reports. PARTICIPANTS: Two patients with small, relatively inconspicuous juxtapapillary pigmented choroidal lesions were referred with the diagnosis of primary optic nerve tumor. Both demonstrated a large nodular tumor in the meninges of the optic nerve, immediately posterior to the globe. METHODS: Retrospective review of clinical records and histopathology. RESULTS: In both cases, orbital magnetic resonance imaging confirmed the presence of a hyperintense enhancing nodular mass near the anterior portion of the optic nerve, prompting optic nerve biopsy in one case. Subsequent fundus examination disclosed a small juxtapapillary pigmented choroidal lesion measuring 1.0 mm or less in thickness. These observations suggested that the optic nerve tumor might be nodular extraocular extension of a small choroidal melanoma. Modified enucleation was performed in both cases, and histopathologic examination revealed a nodule of malignant melanoma within the meninges that compressed the optic nerve and extended extraocularly from a small, relatively inapparent juxtapapillary choroidal melanoma. In both cases, the extraocular component was large and symptomatic, whereas the intraocular component was inconspicuous. CONCLUSIONS: Small juxtapapillary choroidal melanomas can exhibit prominent extension into the orbit. All patients with orbital tumors should have careful ophthalmoscopy.

Aged↗
PubMed1999

Invasive giant cell astrocytoma of the retina in a patient with tuberous sclerosis.

OBJECTIVE: To report an unusual case of giant cell astrocytoma of the retina. DESIGN: Case report. INTERVENTION: A 10-month-old girl with tuberous sclerosis was found to have bilateral astrocytic hamartomas, the right eye being prominently involved by elevated and pedunculated lesions. At 7 years of age, she had posterior subcapsular cataract, retinal detachment, and subretinal exudation develop in the right eye. At 12 years of age, her blind, painful right eye had to be enucleated because of neovascular glaucoma and a spontaneous scleral perforation. RESULTS: Histopathologic examination showed that the entire vitreous cavity was filled with a mixture of tumor, granulation tissue, and necrotic debris. Part of the tumor was composed of spindle-shaped glial cells. The remainder was composed of large gemistocytic cells that contained large atypical nuclei and copious amounts of cytoplasm, which was intensely eosinophilic in some areas. The tumor contained foci of necrosis and rare mitotic figures. It had infiltrated the parenchyma of the retrolaminar nerve and extended to the surgical margin. Areas of unequivocal choroidal invasion were also identified. The tumor cells were intensely immunoreactive for neuron-specific enolase and S-100 protein. In contrast, glial fibrillary acidic protein was only minimally positive. CONCLUSIONS: The histologic and immunohistochemical features of this retinal tumor resemble those of subependymal giant cell astrocytoma, a characteristic lesion in tuberous sclerosis. Although this unusual giant cell astrocytoma of the retina had atypical histopathologic features and local aggressive behavior, the systemic prognosis was excellent.

Astrocytoma↗
PubMed1999

The 1998 Pan American Lecture. Intraocular invasion of conjunctival squamous cell carcinoma in five patients.

PURPOSE: To report five patients with intraocular invasion of conjunctival squamous cell carcinoma and to make recommendations regarding clinical recognition and treatment of this condition. METHODS: The authors reviewed the clinical records and pathology slides on five patients who had intraocular invasion of conjunctival squamous cell carcinoma, and they describe the presenting features and histopathology in these cases. RESULTS: Intraocular invasion of conjunctival squamous cell carcinoma occurred in older patients who had one or more recurrences of a previously excised conjunctival epithelial tumor located near the comeoscleral limbus. The intraocular recurrence often was heralded by the onset of low-grade inflammation and secondary glaucoma, simulating a granulomatous iridocyclitis. A white mass generally was observed in the anterior chamber angle. Histopathologic examination revealed an ingrowth of malignant epithelial cells through the limbus with diffuse involvement of the anterior segment of the eye. The reported patients were managed by modified enucleation (standard enucleation with excision of affected conjunctival tissue). Metastatic disease did not develop in any of the patients. CONCLUSIONS: The onset of signs of uveitis and glaucoma and a white mass in the anterior chamber angle in a patient with prior excision of a conjunctival squamous cell neoplasm tumor should raise suspicion of intraocular recurrence of conjunctival squamous cell carcinoma. Most affected patients require enucleation or subtotal orbital exenteration. The prognosis is good.

Aged↗
PubMed1999

Use of microdissection and molecular genetics in the pathologic diagnosis of retinoblastoma.

BACKGROUND/PURPOSE: Retinoblastoma results from mutations or loss of both alleles of the retinoblastoma gene. Although retinoblastoma is usually recognized clinically, some forms of the disease can elude diagnosis. The purpose of this study was to determine whether the use of molecular genetics to detect a loss of heterozygosity (LOH) in the retinoblastoma gene could assist the ocular pathologist in the diagnosis of this malignancy. METHODS: Deoxyribonucleic acid (DNA) was obtained from tumor cells microdissected from three ocular specimens from two patients with diffuse retinoblastoma. Polymerase chain reaction was used to detect two microsatellite markers (D13S153 and D13S118) of the retinoblastoma gene. Loss of heterozygosity was identified when one of the two polymorphic alleles was present in the DNA from normal tissue but absent or reduced in the DNA obtained from tumor cells. RESULTS: Loss of heterozygosity was identified in all three specimens from the two patients with diffuse retinoblastoma. In one patient, the diagnosis of retinoblastoma was based on identification of LOH from tumor cells obtained from vitrectomy. CONCLUSIONS: This study demonstrates that identification of LOH in retinoblastoma cells not only can contribute to our understanding of the molecular genetics of this tumor, but also can help the ocular pathologist in the diagnosis of atypical forms of the disease.

Adolescent↗
PubMed1998

Intraocular neoplastic cyst from mucoepidermoid carcinoma of the conjunctiva.

An 89-year-old woman with recurrent conjunctival mucoepidermoid carcinoma developed intraocular inflammation and an elevated fundus lesion that simulated choroidal detachment in her affected right eye. Intraocular invasion of squamous cell carcinoma was suspected and the eye was enucleated. Pathologic examination of the enucleated eye showed intraocular invasion by conjunctival mucoepidermoid carcinoma that formed a suprauveal cyst lined with malignant epithelial cells. The patient developed an orbital recurrence 1 year later and underwent orbital exenteration. She died 2 years later from an unrelated cause. Conjunctival mucoepidermoid carcinoma can exhibit intraocular invasion and produce an intraocular neoplastic cyst.

Aged↗
PubMed1998

Solitary intraosseous infantile myofibroma of the orbital roof.

BACKGROUND: There are several rare tumors that can cause proptosis in an infant, including infantile myofibroma. METHODS: A 3-month-old infant developed a painless, bone-destructive superomedial orbital mass, raising concern for orbital malignant neoplasms. Computed tomography disclosed a bone-destructive mass of the sphenoid wing. On magnetic resonance imaging, the intraosseous mass was well-circumscribed, surrounded by cortical bone, and showed prominent enhancement. RESULTS: Superomedial orbitotomy and biopsy revealed a lesion composed of spindled to stellate cells, without mitotic activity, set in a fibromyxoid stroma. Immunohistochemical stains were positive for vimentin and actin. Ultrastructurally, there were actinlike thin filaments, mitochondria, and rough endoplasmic reticulum confirming a myofibroblastic proliferation and supporting the diagnosis of congenital infantile myofibroma. CONCLUSION: Infantile myofibroma is a benign tumor that occurs rarely in the ocular region but can cause prominent bone destruction, misleading the clinician to suspect a malignant neoplasm.

Female↗
PubMed1998

Adenoid cystic carcinoma of the lacrimal gland simulating a dermoid cyst in a 9-year-old boy.

Adenoid cystic carcinoma of the lacrimal gland is a malignant neoplasm that is generally found in adults and is usually managed by orbital exenteration and supplemental external beam irradiation or chemotherapy. A recent report has suggested that the tumor may have a less malignant course in children. We describe a case of adenoid cystic carcinoma of the lacrimal gland that simulated a dermoid cyst clinically and radiographically in a 9-year-old boy. The patient was treated with local surgical resection of the mass, followed by orbital plaque brachytherapy. Based on a review of the literature and our recent experience, the advisability of a more conservative approach to this tumor in selected cases is discussed. Although no prognostic conclusions can be drawn on the basis of a single case report with short follow-up, the relatively earlier detection of this tumor made possible by modern orbital imaging studies may allow total removal at an earlier stage and prevent orbital exenteration in a patient with normal vision. Recent developments suggest that there may be a basis for reassessing the advisability of a radical approach to the management of adenoid cystic carcinoma of the lacrimal gland in selected cases.

Brachytherapy↗
PubMed1998

Malignant rhabdoid tumor of the orbit.

A 36-month-old girl had a 3-week history of proptosis of the right eye. Computed tomography showed an ill-defined homogeneous mass filling the intraconal space. Histopathologic examination and immunohistochemistry findings of an incisional biopsy specimen were consistent with malignant undifferentiated tumor with rhabdoid features. Despite chemotherapy (a combination of vincristine sulfate and dactinomycin) and radiotherapy, massive orbital recurrence occurred 6 months later and orbital exenteration was performed. The recurrent tumor was composed entirely of pleomorphic epithelial cells with prominent nucleoli and many filamentous cytoplasmic inclusions. Immunohistochemical staining showed positive immunoreactivity for vimentin, cytokeratin, and epithelial membrane antigen, and negative immunoreactivity for muscle-specific antigen, melanoma, neural, and histiocytic markers. Electron microscopy excluded myogenic differentiation and showed that the filamentous cytoplasmic inclusions were composed of whorls of intermediate filaments. Aggressive chemotherapy with a combination of vincristine, doxorubicin, cyclophosphamide, ifosfamide, and etoposide phosphate was continued after exenteration. At 17 months' follow-up, orbital debulking surgery with externalization of the maxillary sinus was performed because of massive tumor recurrence in the right orbit and growth into the maxillary sinus. The child died 23 months after initial diagnosis from tumor invasion into the central nervous system. Extrarenal rhabdoid tumor is a rare orbital mass that carries a poor prognosis.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1998

Presumed adenocarcinoma of the retinal pigment epithelium in a blind eye with a staphyloma.

The retinal pigment epithelium (RPE) can undergo reactive hyperplasia and metaplasia following a variety of ocular insults. However, true neoplasms of the RPE are rare. We report a case of a papillary adenocarcinoma of the RPE arising in the blind staphylomatous right eye of a 79-year-old woman with a long history of bilateral posterior staphylomas who was seen with increasing pain and exophthalmos of the right eye. Findings from ultrasonography and computed tomography demonstrated linear calcification consistent with osseous metaplasia of the RPE. Progression of the exophthalmos and worsening exposure keratitis led to enucleation of the eye. Gross pathology showed a 79-mm-long globe. Histopathologic findings revealed a largely amelanotic papillary adenocarcinoma arising from the RPE. Positive immunoreactivity for cytokeratin supported the epithelial origin of the tumor. Adenocarcinoma of the RPE is rare but may develop in a blind eye.

Adenocarcinoma, Papillary↗
PubMed1998

Choroidal melanoma in a 14-year-old patient with ocular melanocytosis.

A 14-year-old male adolescent with ocular melanocytosis and secondary glaucoma in the left eye had a 2-year history of a progressively enlarging fundus lesion. Ocular examination revealed diffuse hyperpigmentation of the episclera and a smooth velvety thickening and hyperpigmentation of the left iris. Ophthalmoscopy disclosed diffuse choroidal pigmentation and a pigmented mass that occupied the macular area and surrounded the optic nerve. Ultrasonography showed an acoustically hollow lesion with scleral bowing and choroidal excavation. Based on clinical and ultrasonographic findings, the diagnosis was choroidal melanoma in a young patient with ocular melanocytosis. The eye was enucleated. Histopathologic examination revealed ocular melanocytosis with diffuse uveal melanocytosis and amelanotic malignant melanoma of the choroid. The choroidal melanoma apparently arose from a preexisting choroidal nevus. Even young patients with ocular melanocytosis should have regular follow-up examinations for early detection of uveal melanoma.

Adolescent↗
PubMed1998

Parry-Romberg syndrome associated with intracranial vascular malformations.

We describe a 23-year-old woman with iridocyclitis, enophthalmos, facial hemiatrophy, and transient numbness of her contralateral upper and lower extremities. The patient was found to have white matter densities in the right hemisphere in magnetic resonance T2-weighted images and vascular malformations involving right vertebral, right carotid, and right anterior cerebral arteries. Histopathologic evaluation of a biopsy specimen of anterior orbital fat and lacrimal gland revealed fibrosis and chronic inflammation. These findings were consistent with the diagnosis of progressive facial hemiatrophy (Parry-Romberg syndrome) in association with iridocyclitis and intracranial vascular malformations.

Adipose Tissue↗
PubMed1998

Coexistence of 3 tumors of neural crest origin: neurofibroma, meningioma, and uveal malignant melanoma.

OBJECTIVE: To describe the clinical findings in a patient who developed a neurofibroma, meningioma, and choroidal melanoma. METHODS: Clinical and histopathological findings of the case are reviewed and presented. RESULTS: The patient had a right superolateral periorbital neurofibroma, a right sphenoid wing meningioma, and a left choroidal juxtapapillary malignant melanoma. All 3 tumors are derived from neural crest cells. CONCLUSIONS: To our knowledge, this is the first report of a patient with this combination of 3 neural crest-derived tumors. This case is most appropriately classified as a complex neurocristopathy, a disorder involving the aberrant and pathological proliferation of multiple tissues derived from neural crest cells.

Choroid Neoplasms↗
PubMed1998

Cutaneous angiosarcoma with eyelid involvement.

PURPOSE: To report a clinicopathologic correlation of angiosarcoma affecting the eyelid skin. METHODS: An 82-year-old man developed multiple bruise-like maculopapular lesions, subcutaneous nodules, and diffuse edema over his scalp, face, and eyelids. Biopsy disclosed angiosarcoma, and the patient was treated with wide-field external beam radiotherapy (5,000 cGy). RESULTS: He responded to radiotherapy with partial regression of the tumor. Twelve months later, he developed extensive lymphedema secondary to the residual tumor. He was also found to have parotid gland and bone metastases, treated with radiotherapy. The patient died of widespread bone metastases 3 months later. CONCLUSIONS: Angiosarcoma is a rare skin tumor that has a poor prognosis despite treatment. It should be considered in the differential diagnosis of eyelid tumors and edema because it may affect only the central portion of the face.

Aged↗
PubMed1998

Trichoadenoma of the eyelid.

PURPOSE: To report the unique occurrence of a trichoadenoma of the eyelid. METHODS: An 80-year-old woman underwent surgical resection of a lesion of the right lower eyelid that was suspected to be a basal cell carcinoma. The lesion was studied with light microscopy. RESULTS: Histopathology showed a lesion in the dermis that contained keratin cysts surrounded by a proliferation of eosinophilic epidermoid cells. The findings were characteristic of a trichoadenoma. CONCLUSION: Trichoadenoma, a rare, benign, cutaneous tumor that usually occurs on the face, can develop on the eyelid and simulate a basal cell carcinoma.

Adenoma↗