Cases reported • Neoplasms, Multiple Primary; Neoplasms, Synchronous; Neoplasms, Synchronous Multiple Primary

1/56. Multifocal choroidal malignant melanoma: at least 3 melanomas in one eye.

OBJECTIVES: To describe a case of an enucleated eye harboring multiple choroidal malignant melanomas. methods: Clinical, ultrasonographic, and histopathological evaluations. RESULTS: Meticulous sectioning of the globe did not disclose any structural continuity between the 3 choroidal melanomas. Fourth and fifth masses were connected to one of the tumors by fibrous tissue and therefore were not considered as additional distinct lesions. CONCLUSION: Although very rare, a single eye can harbor multiple distinct choroidal melanomas.

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keywords = choroid
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2/56. dermatofibrosarcoma protuberans in an infant with a history of choroid plexus papilloma.

We describe an infant with a dermatofibrosarcoma protuberans (DFSP) who also had a choroid plexus papilloma. This report underscores the occurrence of DFSP in this age group and may support a neural pathogenesis of this tumor.

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keywords = choroid
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3/56. Malignant melanoma of the choroid in neurofibromatosis.

A 60-year-old white woman with generalized neurofibromatosis and multiple melanocytic hamartomas of the iris developed an unusual choroidal mass, with secondary sensory retinal separation in the left eye. Ophthalmoscopically the tumor had a peculiar donut configuration that was caused by a large focus of central necrosis within a spindle B melanoma.

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keywords = choroid
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4/56. Choroidal melanoma with oculodermal melanocytosis in Hispanic patients.

PURPOSE: To describe three Hispanic patients with oculodermal melanocytosis and uveal melanoma. METHOD: Case series. RESULTS: Three Hispanic patients with oculodermal melanocytosis and uveal melanoma underwent enucleation. The diagnosis of choroidal melanoma was confirmed by histopathologic examination. CONCLUSION: In the Hispanic population, uveal melanoma can occur in the presence of oculodermal melanocytosis.

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ranking = 0.14285714285714
keywords = choroid
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5/56. Three choroid plexus papillomas in a patient with Aicardi syndrome. A case report.

Aicardi syndrome is an X-linked dominant disorder primarily defined by the triad of corpus callosum agenesis, infantile spasms and a pathognomonic lacunar chorioretinopathy. Papillomas of the choroid plexus have been reported in affected patients. We report an Aicardi syndrome patient who had three separate choroid plexus papillomas and associated hydrocephalus. A dizygotic twin was unaffected. Staged resection of the tumors was safely accomplished, with improvement in seizure control. Imaging between procedures revealed rapid tumor growth.

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keywords = choroid
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6/56. Coincident choroid plexus carcinoma and adrenocortical carcinoma with elevated p53 expression: a case report of an 18-month-old boy with no family history of cancer.

We describe a young patient with no known family history of cancer who presented at 18 months with 2 advanced primary tumors, choroid plexus carcinoma and adrenal cortical carcinoma. Immunohistochemical studies demonstrated high levels of nuclear p53 protein expression in both tumors, as well as in the adjacent normal-appearing adrenal cortical cell nuclei of the adrenal gland. The immunohistologic distribution of elevated p53 expression suggests that this individual has a de novo germline mutation affecting p53 gene expression.

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keywords = choroid
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7/56. Two discrete choroidal melanomas in an eye with ocular melanocytosis.

We report a case of multifocal choroidal melanoma arising in an eye with ocular melanocytosis and review the pertinent literature. A 63-year-old Caucasian male with ocular melanocytosis in the left eye was found to have two discrete choroidal melanomas in the same eye. Histopathology of the enucleated eye confirmed the diagnosis of two discrete choroidal melanomas of mixed cell type within a region of choroidal melanocytosis. It is estimated that 1 in 160,000 patients with unilateral ocular melanocytosis may develop two uveal melanomas, based on the reported data. On the basis of random chance, patients with two melanomas in the same eye would be expected to have approximately 1000-fold greater likelihood of underlying ocular melanocytosis than the general Caucasian population. In conclusion, multifocal choroidal melanoma is rare and may be related to underlying ocular melanocytosis.

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ranking = 1.2857142857143
keywords = choroid
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8/56. Two primary intracranial tumors of different histology: report of a case with a choroid plexus papilloma and a concurrent vestibular schwannoma in the cerebellopontine angle.

A very rare case of multiple primary intracranial tumors is reported. A 41-year-old female patient was referred for surgery with a cerebellopontine angle (CPA) tumor. Medical history and MRI study showed typical findings of a right acoustic neuroma with a hydrocephalus. Neurological, dermatological, and ocular examinations revealed no evidence of neurofibromatosis. During surgery, a red-colored cauliflower like mass was found in the right CPA. The roof of the fourth ventricle could be seen through the lateral recess after removal of the tumor. Another mass, a 1.5-cm sized schwannoma protruding through the right internal auditory meatus, was removed by the transmeatal approach. Although the tumor masses were in contact and compressed against each other, there was a clear demarcation between them. Histological examination confirmed that the first mass was a typical choroid plexus papilloma with fibrovascular core, and that the second was a schwannoma. The patient recovered without any new neurological deficit. Result of a medline search indicated that this rare combination of multiple primary tumors has not been reported previously.

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keywords = choroid
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9/56. Ipsilateral orbital cavernous hemangioma and choroidal hemangioma.

PURPOSE: A unique case of simultaneous ipsilateral choroidal and orbital hemangiomas is described. methods: A 71-year-old man was found to have a left orbital intraconal mass while being worked up for intractable headache. He also had a left juxtapapillary choroidal hemangioma which did not cause any leakage to macular area. RESULTS: The left intraconal tumor was removed by medial transconjunctival orbitotomy. Histopathological examination revealed cavernous hemangioma. The ipsilateral choroidal hemangioma was managed by observation only. During 15 months of follow-up, there has been no growth or accumulation of subretinal fluid. CONCLUSIONS: The occurrence of orbital cavernous and choroidal hemangiomas on the same side suggests common pathogenic derangement acting on two different sites.

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keywords = choroid
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10/56. retinoblastoma, microphthalmia, coloboma, and neuroepithelioma of the pineal body.

A one-month-old infant boy was examined early in life because his mother had bilateral retinoblastoma and his father had bilateral microphthalmia. The ophthalmologist found his right eye was normal size with a coloboma of the iris, choroid, and retina. The left eye was microphthalmic with a coloboma of the uveal tract and retina. A vascularized fluffy white mass in the posterior pole was diagnosed clinically as a retinoblastoma. The tumor regressed with radiation. When the patient was four years of age, a large tumor was found in the region of the pineal recess, causing hydrocephalus and seizures. A biopsy showed an undifferentiated malignant neuroepithelial neoplasm. The patient died within three months of diffuse central nervous system tumor. The unusual findings of a retinoblastoma in a microphthalmic eye with bilateral colobomas and a neuroepithelial neoplasm of the pineal gland are discussed.

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keywords = choroid
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