No abstract

A rare variant of adenoid cystic carcinoma is the dedifferentiated sarcomatoid form, which has previously been reported in the hard and soft palate, maxillary sinus, submandibular glands, and nasal cavity. The authors report the first case of a dedifferentiated sarcomatoid adenoid cystic carcinoma occurring in the lacrimal gland, that of a 52-year-old man. The patient presented with a 4-month history of diplopia, decreased vision, and right upper eyelid swelling. Radiographic imaging showed a soft tissue mass in the extraconal compartment superolateral to the right eye. The patient subsequently underwent surgical debulking. Histologic examination of the tissue revealed classic cribiform adenoid cystic carcinoma and a sarcomatous component consisting of malignant spindle cells and fusiform cells arranged in whorls. Dedifferentiation is a well-established phenomenon in salivary gland tumors that is associated with aggressive behavior and poor prognosis; however, the exact nature of such dedifferentiated neoplasms remains unclear.

PurposeThe purpose of this study is to report a case of Kyrieleis plaques (segmental retinal periarteritis) associated with cytomegalovirus (CMV) retinitis.MethodsA 47-year-old female with recently diagnosed human immunodeficiency virus and a CD4 count of 55 cells/µl presented with decreased vision and floaters in her left eye. Ophthalmic examination revealed an advancing border of white granular CMV retinitis extending into the macula. Intraocular aqueous specimen contained 420,000 copies/ml of CMV DNA by polymerase chain reaction. The patient was treated with intravitreal foscarnet and oral valganciclovir.ResultsKyrieleis plaques involving the retinal arteries were noted on presentation and increased during the first 6 weeks of treatment as the retinitis faded. The plaques on fluorescein angiography did not leak fluorescein dye and slowly faded over 5 months.ConclusionsKyrieleis plaques can be seen in the setting of CMV retinitis. These plaques can be differentiated from vascular sheathing and frosted branch angiitis by its occurrence only in the retinal arteries and the absence of leakage of fluorescein dye.

Background-Choroideremia is an X-linked recessive disorder characterized by vision loss with progressive atrophy of the retinal photoreceptors, retinal pigment epithelium (RPE), and choriocapillaris. Ectodermal dysplasia is a heterogeneous group of disorders characterized by a deficiency of two or more ectodermal derivatives. We report on the phenotypic and genetic characteristics of a 29-year-old woman with both choroideremia and ectodermal dysplasia.