Indian Journal of Dermatology
  Publication of IADVL, WB
  Official organ of AADV
Indexed with Science Citation Index (E) , Web of Science and PubMed
 
Users online: 6600  
Home About  Editorial Board  Current Issue Archives Online Early Coming Soon Guidelines Subscriptions  e-Alerts    Login  
    Small font sizeDefault font sizeIncrease font size Print this page Email this page


 
Table of Contents 
E-IJD® - CORRESPONDENCE
Year : 2022  |  Volume : 67  |  Issue : 2  |  Page : 207
Extraocular sebaceous carcinoma in muir-torre syndrome


1 From the Department of Plastic and Reconstructive Surgery, Başkent University Faculty of Medicine, Ankara, Turkey
2 From the Department of Pathology, Başkent University Faculty of Medicine, Ankara, Turkey

Date of Web Publication13-Jul-2022

Correspondence Address:
Abbas Albayati
From the Department of Plastic and Reconstructive Surgery, Başkent University Faculty of Medicine, Ankara
Turkey
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijd.ijd_2_21

Rights and Permissions



How to cite this article:
Albayati A, Ozkan B, Ayva ES, Uysal AC, Ertas NM. Extraocular sebaceous carcinoma in muir-torre syndrome. Indian J Dermatol 2022;67:207

How to cite this URL:
Albayati A, Ozkan B, Ayva ES, Uysal AC, Ertas NM. Extraocular sebaceous carcinoma in muir-torre syndrome. Indian J Dermatol [serial online] 2022 [cited 2022 Aug 9];67:207. Available from: https://www.e-ijd.org/text.asp?2022/67/2/207/350832




Sir,

A 58-year-old male patient presented to our clinic with a small mass on the lower eyelid of the left eye which enlarged over a period of 2 years. Examination showed a nodular lesion of 3 mm × 4 mm in size in the middle of the lower eyelid. In addition, the patient had a pigmented nevus of 4 mm × 4 mm on the left cheek for 6 months. This macular lesion was characterized by ulcerations in the middle with increased telangiectasia on dermoscopy [Figure 1]. There was no similar lesion elsewhere in the body and no history of radiation exposure. There was no localized or generalized lymphadenopathy. The first lesion was excised under local anesthesia. The pathology report showed a basal cell carcinoma (BCC). According to this report, the lesion on the cheek is removed on the provisional diagnosis of BCC [Figure 2]. The immunohistological staining showed strong positivity for Ber-EP4 monoclonal antibodies, EMA (epithelial membrane antigen) antibodies, and CD15 antibodies. The pathologic analysis revealed a basaloid differentiation of sebaceous cells with a foamy cytoplasm [Figure 3] and [Figure 4]. The diagnosis of sebaceous carcinoma (SC) was made. The surgical margin of the tumor was clear; re-excision was not made. The patient's medical history was relevant for intermittent rectal bleeding and a family history of colon cancer. The patient was referred to the gastroenterology department for investigation of possible internal organ neoplasms that may be a feature of Muir–Torre syndrome (MTS). Colonoscopy studies revealed multiple colorectal polyps of different sizes. Biopsy was performed, and the result was tubulovillous adenoma with varying degrees of dysplasia. The six-month follow-up of the patient did not show signs of recurrence or appearance of similar lesions.
Figure 1: BCC on the lower eyelid

Click here to view
Figure 2: Sebaceous carcinoma on the left cheek

Click here to view
Figure 3: Irregular epithelial lobules associated with epidermal ulceration and sebaceous cells in the center. H&E × 40

Click here to view
Figure 4: Differentiated sebaceous cells showing a foamy cytoplasm. H&E × 100

Click here to view


SC is a malignant tumor derived from the adnexal epithelium of sebaceous glands. It is classified into ocular and extraocular types. Extraocular SCs (ESCs) are less aggressive and account for 25% of the cases and are usually found in the head and neck region.[1] Surgical excision of the tumor with 5–6 mm is the most used treatment method.[2] The recurrence rate can be as high as 30%. The overall survival rate at 5 and 10 years is 71.1% ± 1.5% and 45.9% ± 2.1%, respectively.[3]

The diagnosis of such tumors should raise the suspicion of MTS, which is a rare autosomal dominant cancer syndrome characterized by an association of sebaceous gland tumors and a minimum of one internal malignancy. Internal malignancy includes gastrointestinal (47%), genitourinary (21%), and breast (12%).[4] Mayo score is accepted as a risk score to determine the possibility of MTS. Our patient had a Mayo score of 2 (age younger than 60 and a family history of Lynch-related cancer), which indicates a sensitivity of 100% and specificity of 81% for predicting a germ-line mutation.[5] The patient was referred to genetics for the detection of underlying germ-line mismatch repair gene mutations.

Regarding our case, the diagnosis of extraocular carcinoma was the key for early detection of premalignant colonic polyps before the transition to colonic cancer. It is necessary to be aware of the possibility of MTS whenever a SC is diagnosed. Internal malignancies within this syndrome may appear before or after the diagnosis of SC; thus, lifelong follow-up for internal organ neoplasms is required.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Nelson BR, Hamlet KR, Gillard M, Railan D, Johnson TM. Sebaceous carcinoma. J Am Acad Dermatol 1995;33:1–15.  Back to cited text no. 1
    
2.
Sawyer A, McGoldrick R, Mackey S, Powell B, Pohl M. Should extraocular sebaceous carcinoma be investigated using sentinel node biopsy?. Dermatol Surg 2009;35:704-8.  Back to cited text no. 2
    
3.
Dasgupta T, Wilson LD, Yu JB. A retrospective review of 1349 cases of sebaceous carcinoma. Cancer 2009;115:158–65.  Back to cited text no. 3
    
4.
Habif TP. Cutaneous manifestations of internal diseases. Chapter 26. Clinical Dermatology. 5th ed. p. 997.  Back to cited text no. 4
    
5.
Roberts ME, Riegert-Johnson DL, Thomas BC, Rumilla KM, Thomas CS, Heckman MG, et al. A clinical scoring system to identify patients with sebaceous neoplasms at risk for the Muir–Torre variant of Lynch syndrome. Genet Med 2014;16:711-6.  Back to cited text no. 5
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

Top
Print this article  Email this article
 
 
  Search
 
  
    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
    Article in PDF (1,276 KB)
    Citation Manager
    Access Statistics
    Reader Comments
    Email Alert *
    Add to My List *
* Registration required (free)  


    References
    Article Figures

 Article Access Statistics
    Viewed120    
    Printed12    
    Emailed0    
    PDF Downloaded0    
    Comments [Add]    

Recommend this journal