Intravascular papillary endothelial hyperplasia with presumed bilateral orbital varices.
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Aged↗
Biomedical subjects
C L Shields
Publications and source records attributed to C L Shields.
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↗
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↗
The outcome of chemoreduction treatment in patients with Reese-Ellsworth group V retinoblastoma.
OBJECTIVE: To determine the outcome of chemoreduction treatment in patients with Reese-Ellsworth group V retinoblastoma. METHODS: Prospective analysis of 27 eyes in 22 patients with group V retinoblastoma treated with either 2- or 6-cycle chemoreduction and focal treatment methods (argon laser photocoagulation, transpupillary thermotherapy, cryotherapy, and plaque radiotherapy). The need for external beam irradiation and the eventual globe salvage rate were assessed. Median follow-up was 28 months. RESULTS: There were 16 eyes in the 2-cycle chemoreduction treatment group and 11 eyes in the 6-cycle chemoreduction treatment group. No significant difference was noted between the 2 groups with respect to baseline patient and eye findings. After chemoreduction treatment, external beam irradiation was necessary in 12 (75%) of 16 eyes in the 2-cycle chemoreduction treatment group and in 4 (36%) of 11 eyes in the 6-cycle chemoreduction treatment group. There was no statistical difference between the 2- and 6-cycle chemoreduction treatment groups with respect to necessity for external beam irradiation (logistic regression analysis). All 4 eyes in the 2-cycle chemoreduction treatment group and 3 of 12 eyes in the 2-cycle chemoreduction treatment and irradiation group were eventually enucleated, the globe salvage rates being 0% and 75%, respectively. Two of 7 eyes in the 6-cycle chemoreduction treatment group and 1 of 4 eyes in the 6-cycle chemoreduction treatment and irradiation group were enucleated, the globe salvage rates being 71% and 75%, respectively. Except for the 2-cycle chemoreduction treatment group, in which the globe salvage rate was significantly lower (P = .03), there was no difference among the other 3 groups (2-cycle chemoreduction treatment and irradiation, 6-cycle chemoreduction treatment, and 6-cycle chemoreduction treatment and irradiation) with respect to globe salvage (logistic regression analysis). CONCLUSIONS: Local tumor control of group V retinoblastoma is possible with 6-cycle chemoreduction and focal therapy when external beam irradiation is not used. A larger sample size is necessary to determine how often external beam irradiation can be avoided.
Antineoplastic Combined Chemotherapy Protocols↗
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↗
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↗
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↗
Plaque radiation therapy for recurrent conjunctival pyogenic granuloma.
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Brachytherapy↗
Pleomorphic adenoma of the lacrimal gland in a teenager.
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Adenoma, Pleomorphic↗
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↗
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↗
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↗
Surgical removal of solitary hepatic metastasis from choroidal melanoma.
PURPOSE: To report surgical management of solitary hepatic metastasis that occurred 25 years after an enucleation for uveal melanoma. METHODS: A 16-year-old girl, diagnosed with choroidal melanoma and treated with enucleation, was found 25 years later to have liver metastasis affecting the entire left lobe of the liver. RESULTS: She underwent partial hepatectomy with removal of the metastatic melanoma. Four years later, she was found to have a smaller circumscribed nodular melanoma in the remaining right lobe of the liver, also treated with resection. She has remained disease-free for 2 years, with no evidence of systemic disease. CONCLUSION: Surgical excision may be a useful treatment option in selected cases with solitary, circumscribed liver metastasis from uveal melanoma.
Adolescent↗
Primary cysts of the iris pigment epithelium. Clinical features and natural course in 234 patients.
OBJECTIVE: To describe the clinical characteristics, natural course, and complications of a large group of patients with primary iris pigment epithelium (IPE) cysts. DESIGN: Observational case series. PARTICIPANTS: Two hundred thirty-four patients with primary IPE cysts participated. RESULTS: Primary IPE cysts were classified as central in 6 patients (3%), midzonal in 50 patients (21%), peripheral in 170 patients (73%), and dislodged in 8 patients (3%). Central (pupillary) IPE cysts were found only in males, peripheral IPE cysts were found most often in females (69%), and no gender predilection was detected for midzonal and dislodged IPE cysts. Central and peripheral IPE cysts occurred in young patients (mean age, 20 and 33 years, respectively), whereas midzonal and dislodged IPE cysts were seen in slightly older patients (mean age, 52 and 45 years, respectively). Central IPE cysts were visible when the pupil was not dilated and appeared most often as a round or collapsed brown lesion arising from the pupillary margin, most commonly superonasally. Midzonal IPE cysts were brown and fusiform, best visualized after pupillary dilation. Peripheral IPE cysts produced a characteristic bulging in the iris stroma near the iris root, but they were directly visible in only 78% of cases. After wide dilation and patient and slit-lamp positioning, they appeared as a round clear lesion behind the iris, most often in the inferotemporal quadrant. Finally, dislodged IPE cysts appeared as a brown oval lesion, free floating in the anterior chamber (12%) or in the vitreous (12%), or fixed in the anterior chamber angle (75%). One hundred twenty-four patients (53%) were followed for a mean of 35 months (range, 3 months-19 years). In these patients, complications associated with IPE cysts included lens subluxation in one case (1%), iritis in one case (1%), focal cataract in two cases (2%), glaucoma in two cases (2%), and corneal touch in five cases (4%). CONCLUSION: Primary IPE cysts have characteristic clinical features that serve to differentiate them from intraocular malignancies. Most cysts have a benign clinical course, and treatment is rarely necessary.
Adolescent↗
Periorbital cellular blue nevus leading to orbitopalpebral and intracranial melanoma.
OBJECTIVE: To report the rare occurrence of orbitopalpebral and intracranial melanoma in patients with periorbital cellular blue nevus. DESIGN: A case series. INTERVENTION: The clinicopathologic records of two patients with periorbital cellular blue nevus were reviewed. RESULTS: In case 1, a 32-year-old woman with congenital blue discoloration of her eyelids had gradual increase in nodularity of the eyelid tissue develop over the prior 20 years. The lesion enlarged to the point that the eyelids were almost completely closed from the massive tumor. Results from a biopsy disclosed orbitopalpebral melanoma arising from cellular blue nevus. Because of the diffuse infiltrative pattern of the lesion, she was treated by surgical debulking of the mass. She had a separate focus of ipsilateral intracranial melanoma develop at 40 years of age and eventually died of meningeal melanoma. In case 2, a 29-year-old man with blue discoloration of his left lower eyelid since 10 years of age had an anterior orbital mass develop. He underwent excisional biopsy, the results of which showed an orbitopalpebral melanoma arising from the periorbital cellular blue nevus. Orbital melanoma recurrence and ipsilateral lymph node metastasis were found 9 years later and treated with resection and chemotherapy. He was free of recurrence or metastasis 2 years later. CONCLUSIONS: Patients with periorbital cellular blue nevus may present in the preteen years with a bluish eyelid discoloration. They are at risk of having orbitopalpebral and intracranial malignant melanoma develop.
Adult↗
Surgical repair of rhegmatogenous retinal detachment after treatment for retinoblastoma.
OBJECTIVE: This study aimed to evaluate the causes of rhegmatogenous retinal detachment in children with retinoblastoma and to assess the surgical approach and results of retinal reattachment surgery. DESIGN: The study design was a retrospective review of all children in whom a rhegmatogenous retinal detachment developed after treatment for retinoblastoma over a 20-year period. PARTICIPANTS: Nine children, 5 to 33 months of age, participated. MAIN OUTCOME MEASURES: Assessment of the treatment methods for retinoblastoma, apparent cause of the rhegmatogenous retinal detachment, interval from the most recent retinoblastoma treatment until repair of the rhegmatogenous retinal detachment, method of surgical repair, and long-term anatomic and visual results were measured. RESULTS: Rhegmatogenous retinal detachment was diagnosed in nine eyes of nine children with treated retinoblastoma. Prior treatment of retinoblastoma included cryotherapy (n = 5), laser photocoagulation (n = 1), external beam radiation therapy (n = 7), and systemic chemotherapy (n = 4). An atrophic retinal hole was present at the most recent site of cryotherapy in three eyes and of laser photocoagulation in one eye. Extreme retinal thinning with a possible retinal break was noted in two eyes at a cryotherapy site. A dialysis was noted in one eye, and a retinal break could not be detected in two eyes. Treatment of retinoblastoma tumors was performed between 1 week and 15 years before retinal reattachment surgery. The retinal detachment was repaired externally with a scleral buckling procedure in seven eyes, with subretinal fluid drainage in five eyes, and without drainage in two eyes. Cytologic evaluation of subretinal fluid was performed in three eyes and showed no tumor cells. The retinal detachment was repaired by pars plana vitrectomy in one eye, and the vitrectomy specimen showed no tumor cells. In one child, the retinal detachment was not repaired. The retina remained attached after surgery in five eyes with follow-up ranging from 5 to 115 months. In three eyes, retinal redetachment associated with recurrent viable intraocular retinoblastoma was noted after surgery, necessitating enucleation. In no case was orbital or metastatic retinoblastoma detected at follow-up. CONCLUSIONS: Rhegmatogenous retinal detachment in children with treated retinoblastoma generally originates from an atrophic retinal hole at the site of prior cryotherapy or laser photocoagulation. If there appears to be complete regression of the intraocular tumor after treatment, surgical repair of the rhegmatogenous retinal detachment may be approached cautiously.
Antineoplastic Agents↗
Lifetime prevalence of uveal melanoma in white patients with oculo(dermal) melanocytosis.
OBJECTIVE: In the white population, an association between oculo(dermal) melanocytosis (ODM) and uveal melanoma is well recognized. However, the lifetime prevalence of uveal melanoma in the ODM population is not known. This study was designed to determine the lifetime prevalence of uveal melanoma among patients with ocular melanocytosis. DESIGN: Fifty-six white patients manifesting ODM with uveal melanoma formed the basis of the study. MAIN OUTCOME MEASURES: Published prevalence rates of ODM and uveal melanoma were used for calculations using Bayes' theorem. RESULTS: The lifetime prevalence of uveal melanoma in white patients with ODM is estimated to be 2.6 x 10(-3). The median age at diagnosis of uveal melanoma in the ODM population was similar to a randomly selected population (60.5 years and 62.5 years, respectively). In the vast majority of patients (90%) with ODM-associated uveal melanoma, the uveal melanoma was diagnosed between the ages of 31 years and 80 years. CONCLUSIONS: One of about 400 patients with ODM followed for life is estimated to develop uveal melanoma. Excessive melanocytes in the uveal tract in ODM may provide the biologic basis for susceptibility to the development of uveal melanoma. Patients with ODM should be monitored ophthalmoscopically, especially during the susceptible period, for the development of uveal melanoma. The authors suggest that a national registry of ODM patients be created and prospective data collected to better assess the risk of developing uveal melanoma.
Adolescent↗
Melanocytomalytic glaucoma in eyes with necrotic iris melanocytoma.
OBJECTIVE: Iris melanocytoma, although histologically benign, may undergo spontaneous necrosis with the resultant pigment dispersion causing secondary open-angle glaucoma. The authors describe the clinical findings in three patients with this syndrome and review the current literature. DESIGN: The study design was a small case series. PARTICIPANTS: Three patients with secondary glaucoma caused by a necrotic iris melanocytoma participated. INTERVENTION: Clinical records and pathologic preparations were reviewed. The diagnosis of iris melanocytoma was confirmed by histopathologic examination in all three cases. RESULTS: All patients had intrinsically pigmented localized iris tumors and pigmentation of the angle. Hyperchromic heterochromia iridum was noticeable in all patients. In two instances, a central crater in the lesion signified tumor necrosis. Two patients were treated successfully by surgical resection of the iris tumor and the glaucoma resolved. One patient was treated with enucleation. In all patients, histopathologic analysis disclosed extensively necrotic tumor comprised of maximally pigmented nevus cells with bland nuclei and infiltration of the trabecular meshwork by melanophages. CONCLUSION: Secondary glaucoma may result from obstruction of the trabecular meshwork with necrotic iris melanocytoma. Resection of the tumor may relieve the glaucoma.
Adult↗