This is a clinicopathologic study of 71 hemangiomas of the choroid. The cases were divided into three groups: Group 1 consisted of 45 cases of solitary choroidal hemangiomas (not related to any systemic disease); Group 2 comprised 17 cases with unequivocal evidence of Sturge-Weber syndrome; and Group 3 included 9 cases, 6 of which were classified as "probably Sturge-Weber syndrome." Clinically, many differences were found that established a clear distinction between Groups 1 and 2. Histopathologically, the solitary hemangiomas were well-circumscribed tumors that showed a sharply demarcated pushing margin causing compression of melanocytes and choroidal lamellae. About 70% of these tumors were located temporally (at the posterior pole). In contrast, the lesions in the Sturge-Weber syndrome showed a diffuse angiomatosis involving more than one-half of the choroid, as well as the episcleral and intrascleral perilimbal plexuses. The cause and pathogenesis of these lesions are discussed.

Age Factors↗

PubMedDate not supplied

Persistent hyperplastic primary vitreous. A clinicopathologic study of 62 cases and review of the literature.

This is a clinicopathologic study of 62 cases of persistent hyperplastic primary vitreous (PHPV). The cases were divided into two main groups. Group 1 consisted of 55 unilateral cases not associated with any systemic abnormalities, including 36 eyes (58%) which were considered "pure cases" (Group 1A) and 19 (31%) which disclosed other ocular abnormalities in addition to PHPV (Group 1B). Group 2 consisted of 7 (11%) bilateral cases of PHPV accompanied by other ocular and systemic malformations. The most common presenting clinical signs are leukocoria, microphthalmia and cataract. The main histopathologic features of this condition are outlined, including those responsible for the disastrous results to the eye (retinal detachment, glaucoma, phthisis bulbi). Several clinical entities, usually mistaken for or associated with PHPV, such as retinoblastoma, congenital cataract, retinal dysplasia, trisomy 13 syndrome, and falciform retinal folds are discussed briefly.

Adolescent↗

PubMedDate not supplied

Diffuse malignant melanoma of iris with metastases.

A 66-year-old man had decreased visual acuity in the left eye. Slit-lamp examination showed hyperpigmentation of the periphery of the iris from 2:30 to 9 o'clock. Gonioscopy disclosed an ill-defined mass involving the angle and extending from 5 to 7:30 o'clock with diffuse pigmentation of the meshwork for 360 degrees. A sector iridectomy was performed inferiorly in his left eye. Histopathologic examination disclosed a diffuse malignant melanoma of the iris, mixed cell type. The patient was free of recurrence when examined at 18, 24 and 30 months following surgery. Two years and ten months following the iridectomy, the patient developed multiple metastatic bony lesions and a mass in the liver. Bone marrow aspiration from the sternum and biopsy from the iliac crest, respectively, showed metastatic malignant melanoma. He died shortly thereafter and an autopsy was not performed. The remote possibility of an occult, intraocular tumor such as a ciliary body melanoma or an internally located melanoma cannot be excluded. Assuming that this is indeed an iris melanoma, as our studies indicate, the rarity of this metastatic iris neoplasm is emphasized by noting that only 37 cases of iris melanomas with presumed metastases have been reported in the literature.

Aged↗