Multiple Sebaceous Gland Tumours, with Multiple Tumours of Internal Organs

Abstract

The authors present a case of multiple sebaceous tumours and kerato acanthomata of the skin in a man who in a period of 21 years was also treated twice for a carcinoma of the colon and once for a carcinoma of the stomach. In the literature only two comparable cases have been found. It is suggested that these three cases represent examples of a rare syndrome.

Clinical, dermoscopic and reflectance confocal microscopy features of sebaceous neoplasms in Muir–Torre syndrome

“…1,2 A diagnosis of MTS can be made when concurrent or sequential sebaceous skin tumors (adenoma, epithelioma or carcinoma) are found together with at least one VC or when a family history of MTS occurs together with a personal history of multiple KAs and visceral malignancies. [1][2][3][4][5][6][7][8][9][10] MTS is thought to be a phenotypic variant of Lynch syndrome (LS) [11][12][13][14] also known as hereditary non-polyposis colorectal cancer or cancer family syndrome. 15 LS is an autosomal dominant condition with incomplete penetrance, predisposing to colorectal cancer and other malignancies and caused by a germline mutation in one of the mismatch repair (MMR) genes (MLH1, MSH2, MSH6 and PMS2).…”

Sebaceous lesions and their associated syndromes: Part II

“…50,51 Criteria to define the syndrome have been proposed as concurrent or sequential diagnosis of a sebaceous neoplasm (adenoma, epithelioma, seboacanthoma, or carcinoma), and a minimum of one internal malignancy or a family history of MTS with a personal history of multiple KAs and visceral malignancies (Table I). [50][51][52][53][54][55][56][57][58][59] Sebaceous neoplasms may occur without internal malignancy, but are rare, and MTS should be considered when they are found. 52 MTS is thought to be a phenotypic variant of Lynch syndrome.…”

The genodermatopathology of adnexal tumors

“…Sebaeeous adenoma (15) is an uneommon lesion that usually appears on (he face as a solitary non-distinctive or yellowish nodule measuritig less than 5 mm in maximum ditnension. Multiple sebaceous adenomas, usually on the (runk, may occur in associa(ion with visceral malignancy (16)(17)(18)(19).…”

“…The associatioti of tnultiple sebaceous tumors of skin with visceral tnalignancy was first rccogtiized as a possible tiew sytidrotne by Bakkcr and Tjon A .loc at a tnecting of the Netherlands Society of Dertnatology in The Hague in 1969 (16)(17). Bakkcr and Tjon A Joe described a patient with 18 sebaceous gland tumors and 4 keratoacanthotnas associated with 2 carcinotnas of the colon and 1 eareinotna of the stomach.…”