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Biomedical subjects

C L Shields

Publications and source records attributed to C L Shields.

At least 55 records · Page 3Linked to original sources

Prognostic factors in uveal melanoma.

Uveal melanoma is the most common primary intraocular malignant tumour, with an annual incidence of approximately six cases per million per year. Approximately 40% of patients with posterior uveal melanoma develop metastatic melanoma to the liver within 10 years after initial diagnosis. Despite high accuracy of diagnosis and availability of various methods of treatment; the mortality due to uveal melanoma has remained unchanged. The prognosis in uveal melanoma depends on clinical, histopathological and cytological factors. Clinical factors that relate to prognosis include location, size, and configuration of the tumour. Uveal melanoma can arise in the iris, the ciliary body or the choroid. Iris melanomas have the best prognosis and ciliary body melanomas have the worst prognosis. Based on retrospective studies, the mortality rates for uveal melanoma for comparable sized tumours treated by enucleation or other globe conserving methods such as radiotherapy appear to be similar. Histopathological factors such as cell type, mitotic activity, microcirculation architecture, tumour-infiltrating lymphocytes and the presence of extrascleral extension are also significant predictors of survival. More recently, cytological factors such as cell proliferation, cytogenic, and molecular genetic prognostic markers have been identified with the hope of detecting high risk cases for adjuvant systemic immune therapy or chemotherapy. At present, the role of these therapeutic methods is not clearly established.

Cell Division↗

Factors associated with elevated intraocular pressure in eyes with iris melanoma.

AIM: To identify clinical factors associated with secondary elevated intraocular pressure (IOP) in eyes with iris malignant melanoma METHODS: A retrospective case series of 169 consecutive patients with microscopically confirmed iris malignant melanoma. The main outcome measure was the presence of tumour induced secondary elevated IOP. Cox proportional regression models were used to calculate the relation of clinical features to elevated IOP. RESULTS: Of 169 patients with microscopically proved iris melanoma, 50 (30%) presented with tumour induced secondary elevated IOP. The mean pressure in those eyes with elevated IOP at diagnosis was 33 mm Hg (median 31 mm Hg, range 23-65 mm Hg). The tumour configuration was nodular in 23 (46%) and diffuse in 27 (54%) with a mean base dimension of 7.4 mm and thickness of 2.0 mm. Invasion of the angle structures by melanoma seeds was visible for a mean of 7 clock hours (median 7, range 0-12 clock hours). The mechanism of elevated IOP was judged to be outflow obstruction from tumour invasion into the trabecular meshwork. There were no cases of neovascular glaucoma. The tumour was ultimately managed with enucleation in 30 patients (60%), local resection (iridectomy, iridocyclectomy, or iridocyclogoniectomy) in 11 (22%), and plaque radiotherapy in five (10%). In four cases (8%), observation of cytologically low grade tumour was the patient's preference. Using multivariate analysis, the clinical factors at initial evaluation associated with tumour induced secondary elevated IOP from iris melanoma included increasing extent of tumour seeding in the anterior chamber angle (p=0.01) and poor visual acuity at presentation (p=0.02). CONCLUSIONS: Microscopically confirmed iris melanoma demonstrates tumour related elevated IOP in 30% cases at the time of presentation, usually secondary to tumour involvement of the trabecular meshwork obstructing aqueous outflow. Enucleation is necessary in the majority of these patients (60%) as opposed to those cases without elevated intraocular pressure (18%).

Adolescent↗

Central retinal vascular obstruction secondary to melanocytoma of the optic disc.

A 35-year-old black man developed abrupt visual loss in his left eye. Ophthalmic examination revealed a deeply pigmented mass obscuring the optic disc, hemorrhagic retinopathy, and signs of central retinal vascular obstruction. Fluorescein angiography disclosed sluggish filling of the retinal blood vessels; ultrasonography disclosed an acoustically solid mass in the optic nerve head. Cytopathologic findings of a fine needle aspiration biopsy specimen demonstrated probable benign tumor cells, but melanoma could not be excluded. Histopathologic findings in the enucleated eye revealed a large, necrotic melanocytoma of the optic disc and hemorrhagic necrosis of the retina secondary to obstruction of the central retinal artery and vein. Melanocytoma of the optic nerve can undergo spontaneous necrosis and induce central retinal vascular obstruction. Abrupt visual loss in a patient with a melanocytoma does not necessarily imply malignant transformation.

Adult↗

Adenoma of the retinal pigment epithelium simulating a juxtapapillary choroidal neovascular membrane.

We report a case of an adenoma of the retinal pigment epithelium that clinically simulated a juxtapapillary choroidal neovascular membrane in a 60-year-old man. Fluorescein angiography supported the diagnosis of a juxtapapillary choroidal neovascular membrane in his left eye. After 13 years the lesion become slightly pigmented and the optic disc became swollen. The possibility of choroidal melanoma with optic disc invasion was considered, and the eye was enucleated. The lesion proved histopathologically to be an adenoma of the retinal pigment epithelium.

Adenoma↗

Primary ophthalmic rhabdomyosarcoma in 33 patients.

PURPOSE: To review the findings, management, and outcome in 33 cases of primary ophthalmic rhabdomyosarcoma. METHODS: The records of 33 consecutive patients from a single ocular oncology center were analyzed retrospectively for outcomes of final visual acuity, local recurrence, and distant metastasis. RESULTS: Rhabdomyosarcoma was primarily located in the orbit m 25 cases (76%), conjunctiva in 4 (12%), eyelid in 1 (3%), and uveal tract in 3 (9%). Findings had been present for a mean of 5 weeks and included proptosis in 10 patients (30%), eyelid swelling in 7 (21%), and blepharoptosis in 6 (18%). The initial diagnoses before referral to us included primarily rhabdomyosarcoma in 8 cases (24%), conjunctivitis in 5 (15%), cellulitis in 5 (15%), and pseudotumor in 4 (12%). Tumors were classified according to the Intergroup Rhabdomyosarcoma Study Group staging and treatment protocols as group I in 4 cases (12%), group II in 12 (36%), group III in 16 (48%), and group IV in 1 case (3%). Treatment included surgical debulking, chemotherapy, and radiotherapy. Local tumor recurrence was detected in 6 patients (18%), lymph node spread in 2 (6%), and distant metastasis in 2 (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 (18%), and 20/200 or worse in 12 (43%). Mean follow-up was 8.3 years; tumor-related death occurred in 1 patients (3%). CONCLUSIONS: Rhabdomyosarcoma can present in the orbit, eyelid, conjunctiva, and uveal tract. Following treatment, local tumor recurrence occurs in 18% of cases, metastasis in 6%, and death in 3%.

Adolescent↗

Protracted survival after resection of metastatic uveal melanoma.

BACKGROUND: The objective of this study was to evaluate the usefulness of resection of metastatic uveal melanoma and to analyze the characteristics of patients who may benefit from surgical intervention. PATIENTS AND METHODS Twelve patients underwent surgical removal of metastasis between 1976 and 1998. Data regarding primary uveal melanoma, systemic metastasis, surgical procedures, and outcomes were reviewed retrospectively. RESULTS: There were seven patients with liver metastases, two with lung metastases, one with brain metastasis, and two patients with metastases in the liver and other organs. Median time to systemic metastasis was 8 years. Seven of 12 patients were asymptomatic when they were found to have metastasis. Ten patients underwent complete resection of metastasis. No significant surgical complications were experienced. Median recurrence free and overall survival periods after complete resection were 19 months (range, 6-78 months) and greater than 27 months (range, 11-86 months), respectively. Recurrence free and overall 5-year survival rates of those patients were 15.6% and 53.3%, respectively. Three of these patients had no further systemic recurrence. All patients whose time to systemic metastasis was within 5 years developed further systemic recurrence within 2 years after surgery. In contrast, in 8 patients whose time to systemic metastases was greater than 5 years, 4 patients either were recurrence free or developed second metastasis more than 4 years after surgery. CONCLUSIONS: Complete surgical removal of metastatic uveal melanoma provided unexpectedly long survival without significant morbidity for the selected patients. These results are encouraging and justify a trial in which patients eligible for resection are randomized between standard treatment and surgery.

Adolescent↗

Conjunctival melanoma: risk factors for recurrence, exenteration, metastasis, and death in 150 consecutive patients.

OBJECTIVE: To identify the risk factors of conjunctival malignant melanoma that predict local tumor recurrence, orbital exenteration, distant metastasis, and tumor-related mortality. DESIGN: The clinical parameters of the patient, tumor, and treatment were analyzed in a nonrandomized fashion for their relation to 4 main outcome measures using Cox proportional hazards regression models. PARTICIPANTS: One hundred fifty consecutive patients. MAIN OUTCOME MEASURES: Local tumor recurrence, orbital exenteration, distant metastasis, and death from conjunctival melanoma. RESULTS: The Kaplan-Meier estimates of local tumor recurrence was 26% at 5 years, 51% at 10 years, and 65% at 15 years. The mean number of recurrences per patient was 1 (median, 0 recurrences). There was no recurrence in 98 patients (65%), 1 recurrence in 28 patients (19%), 2 recurrences in 11 patients (7%), 3 recurrences in 5 patients (3%), and 4 or more recurrences in 8 patients (5%). Using multivariate analysis, the factors correlated with local tumor recurrence were melanoma location (not touching the limbus) (P =.01) and pathological tumor margins (lateral margin involved) (P =.02). Multivariate analysis for features correlated with ultimate exenteration included initial visual acuity (20/40 OU or worse) (P<. 001), melanoma color red (P =.01), and melanoma location (not touching the limbus) (P =.02). Tumor metastasis was present in 16% of patients at 5 years, 26% of patients at 10 years, and 32% of patients at 15 years. Metastasis was first located in the regional lymph nodes in 17 cases, the brain in 4 cases, the liver in 3 cases, the lung in 2 cases, and was disseminated in 1 case. The risks for metastases using multivariate analysis included pathological tumor margins (lateral margin involved) (P =.002) and melanoma location (not touching limbus) (P =.04). Tumor-related death occurred in 7% patients at 5 years' follow-up and 13% at 8 years' follow-up. The risk factors for death using multivariate analysis included initial symptoms (lump) (P =.004) and pathologic findings (de novo melanoma without primary acquired melanosis) (P =.05). The technique of initial surgery was shown to be an important factor in preventing eventual tumor recurrence (P =.07), metastasis (P =.03), and death (P =.006) in the univariate analysis, but did not reach significance in the multivariate analysis. CONCLUSIONS: Conjunctival malignant melanoma is a potentially deadly tumor. In the present study, metastasis was detected in 26% of patients, and death occurred in 13% of patients at 10 years. Extralimbal melanoma and tumor involvement of the surgical margins were especially poor prognostic factors. Meticulous surgical planning using wide microsurgical excisional biopsy working with the "no touch" technique and supplemental alcohol corneal epitheliectomy and conjunctival cryotherapy is advised. Arch Ophthalmol. 2000;118:1497-1507

Adolescent↗

Malignant melanoma arising from unusual conjunctival blue nevus.

Cellular blue nevus is an uncommon pigmented tumor in the conjunctiva, where it generally appears as a deep, circumscribed, pigmented conjunctival mass. We report a case of conjunctival blue nevus that clinically resembled primary acquired melanosis and gave rise to conjunctival melanoma. A 41-year-old man developed a diffuse pigmented mass in the inferior fornix of his left eye. Over a 20-year period, he noted slight progression of the pigment. Foci of epibulbar pigmentation were also present. The lesion resembled primary acquired melanosis. Excisional biopsy and adjuvant cryotherapy were performed. Histopathologic examination disclosed an intense infiltrate of heavily pigmented dendritic melanocytes with aggregates of less pigmented plump cells in the substantia propria. The conjunctival epithelium was normal. Malignant cellular features consistent with melanoma were observed in some foci. Cellular blue nevus of the conjunctiva can simulate primary acquired melanosis and can give rise to malignant melanoma. Arch Ophthalmol. 2000;118:1581-1584

Adult↗

Observations on 17 patients with retinocytoma.

OBJECTIVE: To study the clinical features and natural history of 17 patients with retinocytoma. DESIGN: A retrospective case series. SETTING: Tertiary referral center. PATIENTS: Data on 17 patients with retinocytoma were reviewed for clinical features. The natural history of retinocytoma and its risk for malignant transformation were also evaluated. RESULTS: Among 920 consecutive patients who had retinoblastoma, retinocytoma, or both, we identified 24 tumors in 17 patients (1.8%) with clinical features compatible with retinocytoma. The median age at diagnosis was 15 years (range, 4-45 years). Of the 24 tumors, the retinocytoma was bilateral in 3 cases (13%) and the family history of retinoblastoma was positive in 3 cases (13%). Seventeen (71%) of the tumors were extramacular in location, and 7 (29%) were located in the macular area. Ophthalmoscopic features characteristic of retinocytoma included the presence of a translucent retinal mass in 21 (88%), calcification in 15 (63%), and retinal pigment epithelial alteration in 13 (54%) of the 24 tumors. A combination of all 3 features was observed in 8 (33%) of the 24 tumors. In 13 (54%) of the tumors, a zone of chorioretinal atrophy could be observed. In 1 patient, subtle tumor regression was documented photographically. Only 1 retinocytoma (4%) underwent malignant transformation into retinoblastoma. At the last follow-up visit, none of the patients had developed a pineoblastoma or another second malignant neoplasm. CONCLUSIONS: Retinocytoma is a rare benign retinal tumor that has characteristic clinical features. The areas of chorioretinal atrophy were suggestive of tumor regression. In our series, the risk for malignant transformation of retinocytoma into retinoblastoma was 4%; therefore, patients with a presumed diagnosis of retinocytoma should be closely observed.

Adolescent↗

Metastatic neoplasms in the optic disc: the 1999 Bjerrum Lecture: part 2.

BACKGROUND: Little information is available on metastatic tumors to the optic disc. OBJECTIVE: To determine the clinical features and prognosis of patients with optic disc metastasis. DESIGN: Retrospective chart review. RESULTS: Of 660 consecutively evaluated patients with intraocular metastasis, 30 (4.5%) (31 eyes) had metastatic cancer to the optic disc; 24 (80%) were women and 6 (20%) were men. Mean age at the time of ocular diagnosis was 55 years. The primary neoplasm was in the breast in 13 patients (43%), in the lung in 8 (27%), in the intestine in 1 (3%), in the kidney in 1 (3%), and in the prostate in 1 (3%); the primary neoplasm was never determined in 6 patients (20%). The optic disc metastasis was unilateral in 29 patients (97%) and bilateral in 1 (3%). Ophthalmoscopically, the disc metastasis appeared as a diffuse enlargement of the optic disc in 26 eyes (84%) and as a distinct nodule in 5 (16%). There was an adjacent juxtapapillary choroidal component to the metastatic disc lesion in 23 eyes (74%), and the optic disc was involved without a retinal or choroidal component in 8 (26%). Other associated findings included some degree of secondary disc edema in all eyes, buried disc blood vessels in 23 (74%), and splinter hemorrhages in 13 (42%). Fine needle aspiration biopsy was useful in establishing the diagnosis in all 5 eyes in which it was performed. Mean survival was 13 months after diagnosis of the disc metastasis. CONCLUSIONS: Metastasis to the optic disc accounts for 5% of all intraocular metastases. It can occur as invasion from a juxtapapillary choroidal metastasis or as isolated optic disc metastasis. Breast and lung cancers are the most common primary neoplasms that account for metastasis to the optic disc. The primary site is never determined in 20% of patients. The characteristic clinical features of optic disc metastasis should help differentiate it from other causes of swollen optic disc. Patient prognosis is poor.

Adult↗