J Korean Ophthalmol Soc.  2011 Mar;52(3):355-358. 10.3341/jkos.2011.52.3.355.

A Case of Muir-Torre Syndrome

Affiliations
  • 1Department of Ophthalmology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea. eyeminded@hanmail.net

Abstract

PURPOSE
To present a rare case of Muir-Torre syndrome characterized by the association of sebaceous skin tumors and systemic malignancies.
CASE SUMMARY
A 65-year-old female visited our clinic with an irregular nodular mass of the right lower eyelid, which developed 1 year earlier. An excisional biopsy and lower lid reconstruction with Tenzel's semicircular rotational flap was performed under local anesthesia. Histopathologic examination showed well-differentiated sebaceous cells, consistent with sebaceous adenoma. The patient had undergone total abdominal hysterectomy and lower anterior resection due to endometrial cancer and sigmoid colon cancer 5 years before, and nephroureterectomy due to papillary urothelial carcinoma 3 years before. Based on the history of systemic malignancy and sebaceous skin cancer, a diagnosis of Muir-Torre syndrome was made.
CONCLUSIONS
When a sebaceous gland tumor of the eyelids is detected, Muir-Torre syndrome should be included in the differential diagnosis, and systemic work-up for the internal malignancy must be performed.

Keyword

Eyelid tumor; Muir-Torre syndrome; Sebaceous; Systemic malignancy

MeSH Terms

Adenoma
Aged
Anesthesia, Local
Biopsy
Diagnosis, Differential
Endometrial Neoplasms
Eyelids
Female
Humans
Hysterectomy
Muir-Torre Syndrome
Sebaceous Glands
Sigmoid Neoplasms
Skin
Skin Neoplasms

Figure

  • Figure 1. Clinical photograph showing an irregular nodular mass in the lower tarsal conjunctiva of the right eye.

  • Figure 2. Sebaceous gland adenoma. Microscopically the tumor exhibited a lobular pattern with basaloid cells at the periphery of the lobules with central areas of sebaceous differentiation (hematoxylin and eosin, original magnification ×100 (A), ×400 (B)).

  • Figure 3. The patient's photograph taken 18 months after surgery shows no recurrence.


Reference

References

1. Muir EG, Bell AJ, Barlow KA. Multiple primary carcinomata of the colon, duodenum, and larynx associated with kerato-acantho-mata of the face. Br J Surg. 1967; 54:191–5.
Article
2. Torre D. Multiple sebaceous tumors. Arch Dermatol. 1968; 98:549–51.
Article
3. Rishi K, Font RL. Sebaceous gland tumors of the eyelids and conjunctiva in the Muir-Torre syndrome: a clinicopathologic study of five cases and literature review. Ophthal Plast Reconstr Surg. 2004; 20:31–6.
4. Font RL, Rishi K. Sebaceous gland adenoma of the tarsal conjunctiva in a patient with Muir-Torre syndrome. Ophthalmology. 2003; 110:1833–6.
Article
5. Cohen PR, Kohn SR, Kurzrock R. Association of sebaceous gland tumors and internal malignancy: the Muir-Torre syndrome. Am J Med. 1991; 90:606–13.
Article
6. Stockl FA, Dolmetsch AM, Codère F, Burnier MN Jr. Sebaceous carcinoma of the eyelid in an immunocompromised patient with Muir-Torre syndrome. Can J Ophthalmol. 1995; 30:324–6.
7. Tay E, Schofield JB, Rowell NP, Jones CA. Ophthalmic pre-sentation of the Muir Torre syndrome. Ophthal Plast Reconstr Surg. 2003; 19:402–4.
Article
8. Akhtar S, Oza KK, Khan SA, Wright J. Muir-Torre syndrome: case report of a patient with concurrent jejunal and ureteral cancer and a review of the literature. J Am Acad Dermatol. 1999; 41:681–6.
Article
9. Schwartz RA, Torre DP. The Muir-Torre syndrome: a 25-year retrospect. J Am Acad Dermatol. 1995; 33:90–104.
Article
10. Tillawi I, Katz R, Pellettiere EV. Solitary tumors of meibomian gland origin and Torre's syndrome. Am J Ophthalmol. 1987; 104:179–82.
Article
11. Kruse R, Rütten A, Lamberti C, et al. Muir-Torre phenotype has a frequency of DNA mismatch-repair-gene mutations similar to that in hereditary nonpolyposis colorectal cancer families defined by the Amsterdam criteria. Am J Hum Genet. 1998; 63:63–70.
Article
12. Lynch HT, Leibowitz R, Smyrk T, et al. Colorectal cancer and the Muir-Torre syndrome in a Gypsy family: a review. Am J Gastroenterol. 1999; 94:575–80.
Article
13. Fusaro RM, Lemon SJ, Lynch HT. Muir-Torre syndrome and de-fective DNA mismatch repair genes. J Am Acad Dermatol. 1996; 35:493–4.
Article
14. Demirci H, Nelson CC, Shields CL, et al. Eyelid sebaceous carcinoma associated with Muir-Torre syndrome in two cases. Ophthal Plast Reconstr Surg. 2007; 23:77–9.
Article
15. Cohen PR, Kohn SR, Davis DA, Kurzrock R. Muir-Torre syndrome. Dermatol Clin. 1995; 13:79–89.
Article
Full Text Links
  • JKOS
Actions
Cited
CITED
export Copy
Close
Share
  • Twitter
  • Facebook
Similar articles
Copyright © 2024 by Korean Association of Medical Journal Editors. All rights reserved.     E-mail: koreamed@kamje.or.kr