Sebaceous gland tumors of the eyelids and conjunctiva in the Muir-Torre syndrome: a clinicopathologic study of five cases and literature review.
OBJECTIVE
To study the sebaceous tumors of eyelid/conjunctiva associated with Muir-Torre syndrome (MTS) and to determine the role of immunohistochemical markers (MSH2, mismatch repair gene) in the diagnosis of patients with MTS.


METHODS
A retrospective, noncomparative case series of 5 patients diagnosed with MTS from our laboratory. We also reviewed all previously reported cases of sebaceous eyelid tumors with a visceral malignancy.


RESULTS
Four of the 5 patients were men, with a mean age of 55 years (range, 41 to 76 years). Four of the 5 patients had gastrointestinal carcinoma. On histopathological examination, 4 of the 5 tumors were classified as sebaceous adenomas that exhibited a distinct lobular pattern with prominent basaloid cells at the periphery of the lobules. One tumor was classified as a well-differentiated sebaceous gland adenocarcinoma. The diagnosis of MTS in all 5 patients was made after the diagnosis of the eyelid lesions. Immunohistochemical stains showed a lack of MSH2 expression in two tumors, which is highly consistent with MTS.


CONCLUSIONS
Muir-Torre syndrome should be considered in patients who develop sebaceous tumors of the ocular adnexa. Immunohistochemistry for MSH2 is a practical initial approach for screening MTS in patients with sebaceous tumors.
Rishi
Kirtee
K
Department of Ophthalmology, The Methodist Hospital, Baylor College of Medicine, Houston, Texas, U.S.A.
Font
Ramon L
RL
D018266:Adenocarcinoma, Sebaceous / Q000737:chemistry / Q000473:pathology*; D000236:Adenoma / Q000737:chemistry / Q000473:pathology*; D000328:Adult; D000368:Aged; D014408:Biomarkers, Tumor / Q000032:analysis; D003230:Conjunctival Neoplasms / Q000737:chemistry / Q000473:pathology*; D004268:DNA-Binding Proteins; D005142:Eyelid Neoplasms / Q000737:chemistry / Q000473:pathology*; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D008875:Middle Aged; D051718:MutS Homolog 2 Protein; D009386:Neoplastic Syndromes, Hereditary / Q000473:pathology*; D011518:Proto-Oncogene Proteins / Q000032:analysis; D012626:Sebaceous Gland Neoplasms / Q000737:chemistry / Q000473:pathology*; D013577:Syndrome
D014408:Biomarkers, Tumor; D004268:DNA-Binding Proteins; D011518:Proto-Oncogene Proteins; C497172:MSH2 protein, human; D051718:MutS Homolog 2 Protein