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Muir-Torre Syndrome (muir-torre + syndrome)

Distribution by Scientific Domains
Medical Sciences100%

Selected Abstracts

Sebaceous Carcinoma, Basal Cell Carcinoma, Trichoadenoma, Trichoblastoma, and Syringocystadenoma Papilliferum Arising within a Nevus Sebaceus

DERMATOLOGIC SURGERY, Issue 12p2 2004
Christopher J. Miller MD
Background. Nevus sebaceus has a well-documented potential to develop a wide variety of neoplasms of both epidermal and adnexal origins. It is highly unusual for more than three tumors to arise simultaneously within a single nevus sebaceus. Sebaceous carcinoma arising within a nevus sebaceus is a rare occurrence. Objective. The objective was to report the case of a patient with a nevus sebaceus that simultaneously developed five distinct neoplasms of epidermal and various adnexal origins and to report the fourth case of sebaceous carcinoma arising within a nevus sebaceus. Methods. A 45-year-old woman presented with a nevus sebaceusthat contained five separate neoplasms, including sebaceous carcinoma, basal cell carcinoma, trichoadenoma, trichoblastoma, and syringocystadenoma papilliferum. Results. Complete excision of the nevus sebaceus and the five tumors was performed. Systemic work-up showed no evidence of metastatic disease or association with Muir-Torre syndrome. Conclusion. This case report highlights the diverse neoplastic potential of nevus sebaceus and demonstrates the capacity of this hamartoma to develop aggressive tumors, such as sebaceous carcinoma. Prophylactic excision or at least close clinical surveillance for sudden development of new growths is warranted in all cases of nevus sebaceus. [source]

Muir-Torre syndrome: A case of two brothers

INTERNATIONAL JOURNAL OF DERMATOLOGY, Issue 9 2006
Krisztián Gáspár MD
No abstract is available for this article. [source]

Cutaneous sebaceous neoplasms as markers of Muir-Torre syndrome: a diagnostic algorithm

JOURNAL OF CUTANEOUS PATHOLOGY, Issue 6 2009
Ossama Abbas
Sebaceous gland neoplasms such as adenoma, epithelioma, and carcinoma are uncommon cutaneous tumors. Although sporadic, their occurrence is clinically significant because of their association with Muir-Torre syndrome (MTS). MTS is a rare autosomal dominant genodermatosis characterized by the occurrence of sebaceous gland neoplasms and/or keratoacanthomas associated with visceral malignancies that include gastrointestinal and genitourinary cancers. MTS is usually the result of germline mutation in one or more of the DNA mismatch repair (MMR) genes. MMR genes commonly implicated include MSH -2 and MLH -1 and, more recently, MSH -6. Recent evidence suggests that immunohistochemistry is very sensitive and effective in detecting these defects in cutaneous tumors in MTS. In addition, the genetic instability of cutaneous and visceral tumors in MTS caused by the defects in MMR genes can also be detected, using polymerase chain reaction (PCR)-based techniques, for microsatellite instability (MSI). Given that some sebaceous neoplasms represent cutaneous markers of MTS, what should we as dermatopathologists be advocating? Should we be looking for absence/loss of MMRs in all sebaceous neoplasms? When should we recommend assaying for MSI? This review attempts to address all of these issues with a view to streamlining the work-up of a patient presenting for the first time with a sebaceous neoplasm and no prior personal or family history of internal malignancies. [source]