Association of sebaceous gland tumors and internal malignancy: The muir-torre syndrome

Abstract

The Muir-Torre syndrome (MTS) is an autosomal dominant genodermatosis characterized by: (1) at least a single sebaceous gland tumor (either an adenoma, an epithelioma, or a carcinoma) and (2) a minimum of one internal malignancy. To date, 120 patients with MTS have been reported. The most commonly associated neoplasms were colorectal (51%) and genitourinary (25%). Unlike colorectal neoplasms in the general population, the majority (58%) of these tumors in MTS patients occurred proximal to or at the splenic flexure. Nearly half of the MTS patients had more than one primary malignancy. Cutaneous lesions occurred before or concurrent with the diagnosis of the initial cancer in 41% of these patients. The median age for the appearance of the skin lesions was 53 years (range, 23 to 89 years); the median age for the detection of the initial visceral neoplasm was 50 years (range, 23 to 81 years). The cancers appear to have an indolent course in many of the MTS patients; the median survival has not been reached and the median follow-up is 10+ years. Patients with an MTS-associated cutaneous lesion should have a complete evaluation for gastrointestinal or genitourinary cancers. Although the penetrance of this disease is variable, its autosomal dominant inheritance suggests that relatives should be examined for sebaceous gland tumors and internal malignancy.

Muir-Torre Syndrome

“…4,7 Sebaceous tumors precede visceral malignancy diagnosis in 22%, occur simultaneously in 6%, and develop subsequently in 56% of reported MTS cases. [8][9][10] Muir-Torre syndrome-associated sebaceous gland neoplasms include sebaceous adenoma, sebaceoma/sebaceous epithelioma, sebaceous carcinoma, keratoacanthoma with sebaceous differentiation, and basal cell carcinoma (BCC) with sebaceous differentiation. 4 These neoplasms can arise in any body site containing sebaceous glands and, in sporadic cases, show a predilection for areas with abundant sebaceous glands, such as the nose and eyelid.…”

An Overview of Autosomal Dominant Tumour Syndromes with Prominent Features in the Oral and Maxillofacial Region

“…Cutaneous sebaceous tumours and internal malignancies present over a similar age range, with medians of 50 and 53 years respectively [60]. Muir-Torre syndrome is most commonly associated with colorectal carcinomas as well as urothelial carcinomas and carcinomas of the endometrium [53,60,61].…”

“…Muir-Torre syndrome is most commonly associated with colorectal carcinomas as well as urothelial carcinomas and carcinomas of the endometrium [53,60,61]. A recent case report found an association with a parotid sebaceous carcinoma, although a metastasis could not entirely be excluded [62].…”

A jéghegy csúcsa: multiplex faggyúmirigy-eredetű bőrtumor coloncarcinomában. Muir–Torre-szindróma

“…Egy-két sporadikus esetet leszámítva a kór-kép családi halmozódást mutat, átlagosan 55 éves korban alakul ki, a férfi -nő arány 3:2. Viszonylag hosszú kórlefo-lyású betegségről van szó, az átlagos túlélés 10 év [1,2,7].…”