Clinicopathologic reports, case reports, and small case series: acute myeloid leukemia manifesting initially as a conjunctival mass in a patient with acquired immunodeficiency syndrome.

Muir-Torre syndrome is a rare cancer predisposition syndrome characterized by unusual cutaneous tumors and internal malignancy. The cutaneous tumors associated with Muir-Torre syndrome include mainly sebaceous gland neoplasms (sebaceous adenoma and sebaceous carcinoma), keratoacanthoma, and basal cell carcinoma. Colorectal and genitourinary carcinoma are the common types of internal malignancies that occur in Muir-Torre syndrome. Although Muir-Torre syndrome is characterized by autosomal dominant inheritance, sporadic cases are known to occur. Significant variation can occur in the phenotypic manifestations of Muir-Torre syndrome, and in some cases Muir-Torre syndrome may resemble hereditary nonpolyposis colorectal carcinoma. Recent investigations into the molecular genetics of Muir-Torre syndrome have revealed genomic replication errors, known as microsatellite instabilities, due to mutations in the mismatch repair genes, hMSH2 and hMLH1. In this report, we present the clinical and histologic features of a patient with MuirTorre syndrome who had multiple facial lesions, some of which involved the eyelid, in association with an internal malignancy.