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Biomedical subjects

C L Shields

Publications and source records attributed to C L Shields.

At least 37 records · Page 2Linked to original sources
PubMed2001

Management of conjunctival and corneal melanoma with surgical excision, amniotic membrane allograft, and topical chemotherapy.

PURPOSE: To illustrate a novel method of management for extensive conjunctival and corneal melanoma. METHODS: Interventional case report. A 40-year-old Caucasian woman presented with a large, diffuse conjunctival melanoma involving 6 clock hours of the limbus. The remaining bulbar conjunctiva and the entire corneal epithelium were affected by diffuse, flat melanosis. RESULTS: The conjunctival melanoma was completely resected microsurgically in one piece without disrupting the tumor. The conjunctival melanosis was treated with double freeze-thaw cryotherapy. The extensive conjunctival defect, involving one-half of the bulbar conjunctiva, was reconstructed with an amniotic membrane allograft. The corneal melanosis was subsequently treated with topical mitomycin C eyedrops. At 8 months follow-up, the conjunctiva and the cornea were completely healed with resolution of all pigment and 20/20 visual acuity. CONCLUSION: Preliminary evidence suggests that combined therapeutic approaches, consisting of extensive tumor removal, cryotherapy, amniotic membrane allograft, and topical mitomyin C, can be effective in the management of diffuse conjunctival and corneal melanoma arising from primary acquired melanosis.

Adult↗
PubMed2001

Choroidal melanoma metastatic to the contralateral choroid.

PURPOSE: To report metastasis of choroidal melanoma to the contralateral choroid. METHODS: Interventional case report. In a 49-year-old Caucasian female, a primary choroidal melanoma, left eye, was diagnosed and treated with Ruthenium-106 plaque radiotherapy. The choroidal melanoma showed excellent regression with flattening of the mass and adjacent chorioretinal atrophy. RESULTS: Fifteen years after brachytherapy for choroidal melanoma, left eye, the patient developed a metastatic melanoma to the contralateral choroid as the first sign of metastasis. No history of oculo(dermal) melanocytosis or cutaneous melanoma existed. Systemic evaluation disclosed multiple metastases confined to the liver. CONCLUSION: Metastasis to the contralateral choroid can be the first sign of metastasis from choroidal melanoma.

Brachytherapy↗
PubMed2001

Iris melanoma: risk factors for metastasis in 169 consecutive patients.

OBJECTIVE: To identify risk factors that predict distant metastases of iris malignant melanoma. DESIGN: Retrospective case series. PARTICIPANTS: The participants included 169 consecutive patients with microscopically confirmed iris malignant melanoma managed on the Oncology Service at Wills Eye Hospital between 1974 and 1999. MAIN OUTCOME MEASURES: The main outcome measure was the development of distant tumor metastasis. Cox proportional regression models were used to calculate the risk of eventual metastatic spread. RESULTS: Of 1054 patients referred with suspicious iris melanocytic tumors (rule out malignant melanoma) over a 25-year period, 169 patients (16%) had microscopically proven iris melanoma, and the remainder (84%) had clinically diagnosed iris nevus. Of the patients with iris melanoma, the mean age at the time of diagnosis was 43 years (median, 45 years; range, 1-90 years). All patients were Caucasian. The mean tumor base was 6 mm (median, 5 mm; range, 1-17 mm), and mean tumor thickness was 2 mm (median, 2 mm; range, 1-4 mm). The mean number of clock hours of tumor involvement in the iris was four, tumor seeding on the iris was four, and tumor seeding into the anterior chamber angle was four. Extraocular extension was present in 10 eyes (6%). The tumor management consisted of local resection (iridectomy, iridocyclectomy, or iridocyclogoniectomy) in 102 patients (60%), enucleation in 51 (30%), plaque radiotherapy in 9 (5%), and observation in 7 patients (4%). Metastasis developed in nine patients (5%). Using Kaplan-Meier life table analysis, metastasis was found in 3% of patients at 5 years, 5% at 10 years, and 10% at 20 years. The clinical factors at initial evaluation predictive of eventual metastasis from iris melanoma included increasing age at diagnosis (P = 0.03), elevated intraocular pressure (P = 0.03), posterior tumor margin at angle or iris root (versus midzone) (P = 0.02), extraocular extension (P: = 0.02), and prior surgical treatment of the tumor elsewhere before referral (versus observation) (P = 0.006). The method of management (resection, radiotherapy, or enucleation) did not have an impact on metastasis. CONCLUSIONS: Microscopically confirmed iris melanoma demonstrates distant metastasis in 5% of patients at 10 years follow-up. Metastases are more likely to develop in those patients who are older and show tumor features of iris root/angle location with elevated intraocular pressure and extraocular extension.

Adolescent↗
PubMed2001

Conjunctival lymphoid tumors: clinical analysis of 117 cases and relationship to systemic lymphoma.

OBJECTIVE: To investigate the clinical features of conjunctival lymphoid tumors and factors predictive of systemic lymphoma. DESIGN: Retrospective, observational case series. SETTING: Clinical practice of ocular oncology. PARTICIPANTS: The participants included 117 consecutive patients with lymphoid tumors of the conjunctiva treated at the Oncology Service of Wills Eye Hospital between 1974 and 1999. MAIN OUTCOME MEASURE: The main outcome measure was the development of systemic lymphoma. Cox proportional regression models were used to calculate the risk of eventual systemic lymphoma. Kaplan-Meier survival estimates were used to analyze the development of systemic lymphoma as a function of time. RESULTS: There were 55 males (47%) and 62 females (53%); 110 patients (94%) were white and 7 patients (6%) were African AMERICAN: The mean age at ocular presentation was 61 years, and the conjunctival lymphoid infiltrate was unilateral in 72 patients (62%) and bilateral in 45 patients (38%). In 8 patients (7%), initial unilateral conjunctival disease evolved into bilateral involvement over a mean of 32 months. Additional sites of ocular involvement were found in 27 patients (23%) and included lymphoid tumor in the eyelid in 3 cases, orbit in 18, choroid in 5, and vitreous in 1. Systemic lymphoma was known to exist before ocular diagnosis in 16 patients; (14%; for a mean of 51 months) and was found subsequent to ocular diagnosis in 20 patients (17; at a mean of 21 months). Therefore, of 117 patients with conjunctival lymphoid infiltration, 36 (31%) had or eventually developed systemic lymphoma, and 81 (69%) did not manifest systemic lymphoma during the mean follow-up of 38 months. Of the 64 patients with unilateral conjunctival involvement, 11 (17%) manifested systemic lymphoma, and of the 53 patients with bilateral involvement, 25 (47%) manifested systemic lymphoma. By univariate analysis, the clinical factors at date first seen predictive of the presence or development of systemic lymphoma included location of the tumor at an extralimbal site (fornix or midbulbar conjunctiva; P = 0.02) and increasing number of conjunctival tumors (P = 0.02). Using Kaplan-Meier life table analysis of those 101 patients who had conjunctival lymphoid tumor(s) and no evident systemic lymphoma at presentation, systemic lymphoma was eventually discovered in 7% of patients at 1 year, 12% at 2 years, 15% at 5 years, and 28% at 10 years. Overall, only one patient (<1%) died of systemic lymphoma, at 28 months after the diagnosis of the ocular disease. CONCLUSIONS: Lymphoid tumors of the conjunctiva are associated with systemic lymphoma in 31% of patients. Systemic lymphoma is found more often in those patients with forniceal or midbulbar conjunctival involvement and in those with multiple conjunctival tumors. Long-term systemic follow-up is advised, because related systemic lymphoma can manifest many years later.

Adult↗
PubMed2001

Basal cell carcinoma of the eyelid associated with Gorlin-Goltz syndrome.

PURPOSE: To describe the ophthalmic and systemic features in a series of patients initially seen with eyelid basal cell carcinoma associated with Gorlin-Goltz syndrome. DESIGN: Retrospective noncomparative case series. PARTICIPANTS: Of 105 consecutive patients with eyelid basal cell carcinoma managed at an Ocular Oncology Center between January 1973 and December 1999, four patients with Gorlin-Goltz syndrome were identified. METHODS: The ophthalmic and systemic features, management, and outcome of patients with eyelid basal cell carcinoma associated with Gorlin-Goltz syndrome were analyzed. The published literature on Gorlin-Goltz syndrome, specifically related to genetics, systemic features, ophthalmic associations, and prophylactic management strategies, was reviewed. MAIN OUTCOME MEASURES: Response of the eyelid basal cell carcinoma to treatment and the final systemic condition were the main outcome measures. RESULTS: All four patients had a family history of Gorlin-Goltz syndrome. The systemic manifestations included multiple basal cell carcinomas in all the patients, frontal bossing or increased occipitofrontal circumference in three patients, palmar pits in two patients, odontogenic keratocyst in one patient, ectopic calcification in one patient, and bifid rib in one patient. The mean age at the detection of the first basal cell carcinoma was 30 years (range, 16-38 years). All four patients had multiple basal cell carcinomas on the face and elsewhere. The eyelid basal cell carcinoma was advanced with orbital infiltration in three patients, one of whom opted for palliative radiotherapy, whereas the other two underwent orbital exenteration. The fourth patient, who had localized recurrent basal cell carcinoma in the upper eyelid, was treated with excision and eyelid reconstruction. At the final follow-up (mean, 41 months), eyelid basal cell carcinoma was cured in three patients and stable in one patient. No patient had life-threatening sequelae of Gorlin-Goltz syndrome. CONCLUSIONS: Gorlin-Goltz syndrome is a rare autosomal dominant cancer predisposition syndrome that may be associated with eyelid basal cell carcinoma. The associated systemic findings may be a clue to the diagnosis of this condition. It is important to recognize Gorlin-Goltz syndrome when a patient has multiple basal cell carcinomas or when a young patient with eyelid basal cell carcinoma is seen by an ophthalmologist, because lifelong monitoring is essential for patient management.

Age of Onset↗
PubMed2001

Outcome of patients with periocular sebaceous gland carcinoma with and without conjunctival intraepithelial invasion.

PURPOSE: To evaluate the clinical findings, histopathologic features, methods of management, and outcomes in 25 patients with eyelid sebaceous gland carcinoma with and without conjunctival intraepithelial (pagetoid) invasion. PARTICIPANTS: Twenty-five patients with sebaceous gland carcinoma of the periocular region. DESIGN: Retrospective, observational case series. METHODS: The patients were divided into two groups: group A included those with sebaceous gland carcinoma with conjunctival intraepithelial (pagetoid) invasion, and group B included those with sebaceous gland carcinoma without conjunctival intraepithelial invasion. The demographic, clinical, and histopathologic features of sebaceous gland carcinoma were reviewed separately for each group. Ocular management results and systemic prognosis were reviewed. RESULTS: Of the 25 patients, 18 (72%) were females and 7 (28%) were males. The mean age at diagnosis was 65 years (range, 17-90 years). There were 11 patients (44%) in group A, and 14 (56%) in group B. The most common symptom in group A was ocular irritation (45%), and in group B the most common symptom was eyelid lump (86%). The most common sign in group A was diffuse eyelid thickening (72%), and in group B the most common sign was a distinct eyelid mass (43%). Six patients (55%) in group A, compared with one patient (7%) in group B, had both upper and lower eyelid involvement. The most frequent location of tumor epicenter was the outer third of the eyelid in both groups: 37% in group A and 57% in group B. Histopathologic origin of tumor was multicentric in four patients (36%) of group A and six patients (50%) of group B. Before referral, local recurrence had been found in 4 of 12 patients (33%) who underwent surgical treatment elsewhere. After referral, local recurrence was detected in 1 patient (9%) in group A and in 2 patients (14%) in group B. Orbital exenteration was necessary in 4 patients (36%) in group A and in one patient (7%) in group B. Tumor-related metastases occurred in two patients (18%) in group A and in two patients (14%) in group B. CONCLUSIONS: Sebaceous gland carcinoma with conjunctival intraepithelial (pagetoid) invasion has different clinical features compared with that without pagetoid invasion. Conjunctival intraepithelial (pagetoid) invasion carries a higher risk for orbital exenteration, but the risk of tumor-related metastases is similar in both groups.

Adolescent↗
PubMed2001

Retinal capillary hemangioma: a comparison of sporadic cases and cases associated with von Hippel-Lindau disease.

OBJECTIVE: To report observations regarding the onset of tumors in patients with retinal capillary hemangiomas (RCH) and comparing sporadic cases with those associated with von Hippel-Lindau (VHL) disease. DESIGN: Retrospective consecutive case series. PARTICIPANTS: Sixty-eight patients with RCH managed at a tertiary referral center. MAIN OUTCOME MEASURES: The data were analyzed for age at diagnosis of RCH, two-hit mutation model, onset of new tumors, and random (Poisson) distribution. RESULTS: Among 68 patients, we identified 174 RCHs in 86 eyes. Thirty-one patients had VHL disease, and 37 patients were sporadic. The median age at diagnosis in cases with VHL disease was 17.6 years (range, 2.8-46.7 years), and in those without VHL disease it was 36.1 years (range, 7.0-74 years). The age distribution of RCH followed a two-hit mutation model. In eyes with VHL disease, the total number of RCH were distributed randomly as calculated by the Poisson distribution (mean number of tumors = 4). Overall onset of 33 new tumors was documented in 11 eyes (13%) over a median interval of 16 months (range, 3-235 months). All new tumors developed before the age of 47 years. The retinal location of new tumors was similar to the previous tumors with most new tumors located in the superotemporal quadrant (42%) and in the midperipheral retina (58%). Of the 50 patients initially manifesting solitary RCH, only 4 had new tumors develop over a median follow-up duration of 6.4 years (range, 0-33 years). All four cases had VHL disease. CONCLUSIONS: The median age at diagnosis of RCH in patients with VHL disease was almost 18 years less than those without VHL disease. The age distribution of RCH followed the two-hit mutation model. In VHL disease, the number of RCH is determined randomly. Most of the new RCH develop by the age of 47 years. In the absence of VHL disease, it is unlikely for patients with solitary RCH to develop new tumors.

Adolescent↗
PubMed2001

Plaque radiotherapy for retinoblastoma: long-term tumor control and treatment complications in 208 tumors.

OBJECTIVE: To evaluate the clinical factors predictive for tumor recurrence and treatment complications in a large series of children who underwent plaque radiotherapy for retinoblastoma. DESIGN: Retrospective, noncomparative case series. PARTICIPANTS: The participants included 141 children with retinoblastoma who were managed on the Oncology Service at Wills Eye Hospital with plaque radiotherapy between July 1976 and June 1999. MAIN OUTCOME MEASURES: Tumor recurrence and treatment complications. RESULTS: There were 208 tumors managed with plaque radiotherapy. The mean patient age at plaque treatment was 19 months. Prior treatment to the retinoblastoma of concern was delivered to 148 tumors (71%) and included various combinations of treatments such as intravenous chemoreduction, external beam radiotherapy, laser photocoagulation, thermotherapy, and cryotherapy. For 72 retinoblastomas (35%), more than one therapeutic method had failed to achieve tumor control before the use of plaque radiotherapy. Of the 208 retinoblastomas managed with plaque radiotherapy, Kaplan-Meier estimates of tumor control were 83% at 1 year and 79% at 5 years. Of the 60 tumors treated only with plaque radiotherapy (primary treatment), recurrence at 1 year was 12%. Of the 148 tumors treated after failure of other methods (secondary treatment), specific Kaplan-Meier estimates of tumor recurrence at 1 year was detected in 8% of tumors previously treated with chemoreduction, 25% of tumors previously treated with external beam radiotherapy, 34% tumors previously treated with both chemoreduction and external beam radiotherapy, and 8% of tumors previously treated with laser photocoagulation, thermotherapy, or cryotherapy (methods other than chemoreduction and external beam radiotherapy). Using multivariable analysis, the risks for tumor recurrence included the presence of tumor seeds in the vitreous, presence of subretinal tumor seeds, and increasing patient age. Using Kaplan-Meier estimates, radiation complications at 5 years of follow-up included nonproliferative retinopathy in 27%, proliferative retinopathy in 15%, maculopathy in 25%, papillopathy in 26%, cataract in 31%, glaucoma in 11%, and scleral necrosis in 0%. CONCLUSIONS: Plaque radiotherapy for retinoblastoma provides tumor control in 79% of cases at 5 years of follow-up. It is particularly useful for those tumors that fail treatment with chemoreduction, laser photocoagulation, thermotherapy, and cryotherapy. Tumors in young patients without vitreous or subretinal seeding show the best long-term control.

Brachytherapy↗
PubMed2001

Circumscribed choroidal hemangioma: clinical manifestations and factors predictive of visual outcome in 200 consecutive cases.

PURPOSE: To review the clinical features and management of circumscribed choroidal hemangioma and determine factors predictive of poor visual outcome. DESIGN: Retrospective consecutive noncomparative interventional case series. PARTICIPANTS: Two hundred consecutive patients with circumscribed choroidal hemangioma. MAIN OUTCOME MEASURES: The main outcome measures were analyzed in 155 patients with follow-up of at least 3 months and included complete resolution of subretinal fluid, worsening of visual acuity (more than 2 Snellen lines), and poor final visual acuity (20/200 or worse). RESULTS: The patients were seen at a mean age of 45 years with symptoms of decreased visual acuity (81%), visual field defect (7%), metamorphopsia (3%), floaters (2%), progressive hypermetropia (1%), photopsia (1%), pain (1%), and no symptoms (6%). The referring diagnoses were choroidal hemangioma (29%), choroidal melanoma (29%), choroidal metastasis (9%), retinal detachment (6%), central serous chorioretinopathy (5%), and others. The tumor had a median base of 6.0 mm and median thickness of 3.0 mm. Secondary retinal detachment in the foveal region was present in 81% of the patients. Initial treatment included observation (51%), laser photocoagulation (44%), plaque radiotherapy (4%), external beam radiotherapy (1%), surgical repair of retinal detachment (1%), and enucleation for painful neovascular glaucoma (1%). Kaplan-Meier estimates revealed complete resolution of subretinal fluid in 60% patients at 5 years and 76% patients at 10 years follow-up. By multivariable analysis, clinical factors predictive of complete resolution of subretinal fluid included shorter duration of symptoms (P = 0.03) and inferior quadrant location of tumor (P = 0.001). At initial presentation, 82 of 155 (53%) patients had poor visual acuity (20/200 or worse), and 73 of 155 (47%) patients had good to moderate visual acuity (20/100 or better). Of those 82 patients with poor initial vision, poor final vision was found in 54% at 5 years and 80% at 10 years. Of the 73 patients with good to moderate initial vision, poor final vision was found in 12% at 5 years and 43% at 10 years. By multivariable analysis, clinical factors predictive of poor final visual acuity included poor initial visual acuity (P < 0.001), failure of previous laser photocoagulation before referral (P = 0.01), and tumor management with observation after referral (P = 0.02). Worsening of visual acuity (by more than 2 Snellen lines) was observed in 8% at 5 years and 28% at 10 years of those 82 patients who were initially seen with poor vision. Worsening of visual acuity was found in 10% at 5 years and 30% at 10 years of those 73 patients who initially were seen with good to moderate vision. CONCLUSIONS: Circumscribed choroidal hemangioma is a rare intraocular tumor. In 38% of cases, this tumor is initially misinterpreted before referral as choroidal melanoma or metastasis. Visual acuity is poor in more than 60% of patients at 10 years, despite successful control of associated subretinal fluid in 76% patients.

Adolescent↗
PubMed2001

Clinical spectrum of primary ophthalmic rhabdomyosarcoma.

PURPOSE: To review the clinical presentation, histopathologic analysis, management, and ocular and systemic outcome of rhabdomyosarcoma affecting the ocular region. DESIGN: Retrospective, noncomparative, consecutive, interventional case series. PARTICIPANTS: Thirty-three consecutive patients with primary ophthalmic involvement of rhabdomyosarcoma from a single tertiary care center specializing in ocular oncology. MAIN OUTCOME MEASURES: Final visual acuity, treatment complications, local recurrence, and distant metastasis. RESULTS: The mean age at presentation was 10 years (median, 7 years; range, 1 month-68 years). At presentation, 8 patients (24%) were older than age 10 years and 4 patients (12%) were older than 20 years. The rhabdomyosarcoma was primarily located in the orbit in 25 cases (76%), conjunctiva in 4 cases (12%), eyelid in 1 case (3%), and uveal tract in 3 cases (9%). Symptoms or signs related to the tumor were present for a mean of 5 weeks and included proptosis in 10 patients (30%), eyelid swelling in 7 patients (21%), and blepharoptosis in 6 patients (18%). The initial diagnosis before referral to us included rhabdomyosarcoma in 8 cases (24%), conjunctivitis in 5 cases (15%), orbital or preseptal cellulitis in 5 cases (15%), idiopathic orbital inflammatory pseudotumor in 4 cases (12%), and others. Using the Intergroup Rhabdomyosarcoma Study Group staging and treatment protocols, the tumor was classified as group I in 4 cases (12%), group II in 12 cases (36%), group III in 16 cases (48%), and group IV in 1 case (3%). Treatment included surgical debulking and various regimens of chemotherapy and radiotherapy for the periocular tumors and enucleation for the three intraocular tumors. Local tumor recurrence was detected in 6 patients (18%). Orbital exenteration was necessary for tumor recurrence in 2 cases (6%). Long-term visual outcome of the 28 patients who maintained their globe was 20/20 to 20/40 in 11 patients (39%), 20/50 to 20/100 in 5 patients (18%), and 20/200 to no light perception in 12 patients (43%). Regional lymph node metastasis was detected in 2 patients (6%), one at initial visit and one after therapy. Distant metastasis occurred in 2 patients (6%), one detected at initial visit and one after therapy. With mean follow-up of 8.3 years, tumor-related death occurred in 1 patient (3%). CONCLUSIONS: Rhabdomyosarcoma can present in the orbit, eyelid, conjunctiva, and uveal tract. After treatment, local tumor recurrence occurs in 18%, metastasis in 6%, and death in 3%.

Adolescent↗
PubMed2001

Orbital malignant fibrous histiocytoma following irradiation for retinoblastoma.

PURPOSE: An unusual case is reported of orbital malignant fibrous histiocytoma that developed after irradiation for retinoblastoma. METHODS: Case report. RESULTS: A 5-month-old girl underwent enucleation of the left eye, external beam irradiation of the right eye, and systemic chemotherapy for bilateral sporadic retinoblastoma. At age 17 years, a malignant fibrous histiocytoma developed in the medial aspect of the orbit and nasal cavity. After attempted excisional biopsy, she underwent craniofacial resection, orbital exenteration, maxillectomy, and tracheostomy. The patient was alive 12 months postoperatively without local or metastatic disease. CONCLUSIONS: Although orbital fibrous histiocytoma occurs usually as a primary tumor of adulthood, it can also develop as a secondary tumor after irradiation for retinoblastoma.

Biopsy↗
PubMed2001

Orbital nodular fasciitis simulating a dermoid cyst in an 8-month-old child. Case report and review of the literature.

PURPOSE: To report a clinicopathologic correlation of orbital nodular fasciitis that simulated a dermoid cyst in an infant and to review the literature on orbital nodular fasciitis. METHOD: Case report; literature review. RESULTS: A progressively enlarging subcutaneous mass superotemporal to the right eye that clinically resembled a dermoid cyst developed in an 8-month-old boy. Magnetic resonance imaging disclosed an enhancing, circumscribed, solid, soft-tissue mass in the anterior part of the orbit superotemporally. The tumor was removed intact by a superolateral orbitotomy. Histopathologic and immunohistochemical studies revealed findings consistent with nodular fasciitis, a pseudoneoplastic lesion marked by a proliferation of myofibroblasts. CONCLUSIONS: Although nodular fasciitis in the ocular region usually occurs in adults and older children, it should also be considered in the differential diagnosis of orbital or adnexal masses in infants.

Dermoid Cyst↗
PubMed2001

Metastatic renal cell carcinoma to the palpebral lobe of the lacrimal gland.

PURPOSE: To describe a clinicopathologic correlation of a metastatic renal cell carcinoma to lacrimal gland. METHODS: Case report. RESULTS: A 59-year-old man with a history of renal cell carcinoma had a hemorrhagic mass involving the palpebral lobe of the right lacrimal gland. Fundus examination disclosed two lesions with typical features of choroidal metastasis. The lacrimal gland mass was excised, and histopathologic examination revealed metastatic renal cell carcinoma. The patient was treated for systemic metastasis but required no further ocular treatment. CONCLUSIONS: Renal cell carcinoma can metastasize to the lacrimal gland, where it may appear as a hemorrhagic mass.

Carcinoma, Renal Cell↗
PubMed2001

Cancer metastatic to the orbit: the 2000 Robert M. Curts Lecture.

PURPOSE: To report the demographics and clinical features of a large series of patients with orbital metastasis. METHODS: Retrospective chart review on 100 consecutive patients and a literature review on orbital metastasis. RESULTS: Of 100 patients, the primary tumor site was breast in 53 (53%), prostate gland in 12 (12%), lung in 8 (8%), skin (melanoma) in 6 (6%), kidney in 5 (5%), gastrointestinal tract in 5 (5%), choroid (melanoma) in 2 (2%), parotid gland in 1 (1%), and adrenal gland (neuroblastoma) in 1 (1%). Of patients in whom a detailed history was available, there was no history of cancer at the time of presentation in 19%. In 10%, the primary tumor remained undetected despite systemic evaluation. There were 36 male patients and 64 female patients whose mean age at diagnosis was 62 years (median 60 years, range 5 to 91 years). Both the right and left orbits were affected equally, and 4 cases (4%) were bilateral. The most frequent clinical findings were limited ocular motility (54%), proptosis (50%), and palpable mass (43%). The diagnoses were established by history, systemic survey, imaging studies, and biopsy. Treatment included chemotherapy, hormone therapy, irradiation, surgical excision, or observation, depending on clinical circumstances. Among patients with sufficient follow-up, 95% died of metastasis, with overall mean survival of 15 months (median 15 months; range 3 to 96 months) after orbital diagnosis. CONCLUSIONS: The most common primary cancers that metastasize to the orbit are breast, prostate gland, and lung cancer. In 19%, there is no history of cancer when the patient presents with ophthalmic symptoms and in 10% the primary site remains obscure despite systemic evaluation. The systemic prognosis is generally poor.

Adolescent↗
PubMed2001

Experience with eyelid-sparing orbital exenteration: the 2000 Tullos O. Coston Lecture.

PURPOSE: To provide data on types of lesions that require orbital exenteration and discuss our experience with exenteration, emphasizing the eyelid-sparing technique. METHODS: The records were reviewed on all patients who underwent orbital exenteration by the senior author and his colleagues. The underlying disease, method of exenteration, healing times, complications, cosmetic outcome, local tumor control, and patient survival were analyzed. RESULTS: Of 56 orbital exenterations, 47 were done by eyelid-sparing technique and 9 by eyelid-sacrificing technique. The 9 tumors of eyelid origin that required exenteration included basal cell carcinoma (4), sebaceous gland carcinoma (3), squamous cell carcinoma (1), and melanoma (1). Four of the eyelid lesions were managed by the eyelid-sparing technique and 5 by the eyelid-sacrificing method. The 24 tumors of conjunctival origin included melanoma (20) and squamous cell carcinoma (4). The eyelid-sparing method was used in 22 of the 24 cases. The 10 primary orbital lesions included melanoma (3), malignant lacrimal gland tumor (3), a rhabdoid tumor, meningioma, Wegener's granulomatosis, and aspergillosis. The eyelid-sparing method was used in 8 of the 10 cases. Among intraocular tumors were uveal melanomas (10) and retinoblastoma (1). The eyelid sparing method was used in all 11. With the eyelid-sparing technique, the mean time from exenteration to wound healing and readiness for a prosthesis was 4 weeks (median, 2 weeks; range, 2 to 24 weeks). With the eyelid-sacrificing technique, the mean time was 68 weeks (median, 34 weeks; range, 2 to 340 weeks). Complications were few and cosmetic results were satisfactory. Local tumor control with exenteration was achieved in 53 of 56 cases, but metastasis occurred in 11 of 20 cases of conjunctival melanoma (55%) and in 6 of 10 patients with uveal melanoma (60%). Metastasis has developed in none of the 3 patients with malignant epithelial tumors of lacrimal gland origin. CONCLUSIONS: Orbital exenteration is an appropriate procedure for orbital involvement by unresectable malignant tumors of the eyelids, conjunctiva, intraocular structures, and orbit. In most of these cases, including about half of tumors originating in the eyelids, an eyelid-sparing exenteration can be used to achieve more rapid healing.

Adolescent↗
PubMed2001

Locally invasive tumors arising from hyperplasia of the retinal pigment epithelium.

PURPOSE: Focal hyperplasia of the retinal pigment epithelium (RPE) is a common fundus condition that is generally stationary, with little or no tendency to enlarge or spawn neoplasms. The purpose of this report is to describe the unusual clinical features of two similar cases in which a nodular tumor of the RPE was documented to arise from a small focus of hyperplasia of the RPE. METHODS: Clinical and cytopathologic observations of two patients. RESULTS: Both patients were observed for approximately 25 years with an unusual progressive fundus tumor that originally arose from a small, flat, irregular focus of hyperplasia of the RPE. The originally observed pigmented lesion was attributed to toxoplasmosis in one patient and laser treatment for central serous chorioretinopathy in the other. In both patients, the tumor enlarged, invaded through the full-thickness sensory retina, and produced a characteristic retinal perforation with apposition of the mass to the vitreous. In both instances, fine-needle aspiration biopsy showed scant pigmented cells, but a definite diagnosis was not made. However, clinical observations in both patients suggested that these tumors were acquired neoplasms that arose from small foci of hyperplasia of the RPE. CONCLUSION: Focal hyperplasia of the RPE can give rise to unusual invasive tumors that invade and replace the overlying sensory retina. These tumors have unique clinical features that differentiate them from uveal melanoma and other pigmented fundus lesions.

Adult↗