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E-CORRESPONDENCE
Year : 2013  |  Volume : 58  |  Issue : 3  |  Page : 248
Synchronous peri-ocular and extra ocular sebaceous carcinomas


1 Department of Surgical Oncology, Cancer Institute (WIA), Adyar, Chennai, India
2 Department of Pathology, Cancer Institute (WIA), Adyar, Chennai, India

Date of Web Publication20-Apr-2013

Correspondence Address:
Arvind Krishnamurthy
Department of Surgical Oncology, Cancer Institute (WIA), Adyar, Chennai
India
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Source of Support: None, Conflict of Interest: None


PMID: 23723528

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How to cite this article:
Krishnamurthy A, Majhi U. Synchronous peri-ocular and extra ocular sebaceous carcinomas. Indian J Dermatol 2013;58:248

How to cite this URL:
Krishnamurthy A, Majhi U. Synchronous peri-ocular and extra ocular sebaceous carcinomas. Indian J Dermatol [serial online] 2013 [cited 2019 May 25];58:248. Available from: http://www.e-ijd.org/text.asp?2013/58/3/248/110905


Sir,

Sebaceous carcinoma (SC) is an aggressive, uncommon cutaneous malignancy which exhibits an aggressive clinical course with an increased tendency for both local recurrences and distant metastasis. [1] This tumor can arise anywhere in the body; approximately 75% of these tumors arise in the peri-ocular region. [2] Extra-ocular SC represents the remaining 25% of the tumors and usually localizes in the head and the neck region. An individual immunosuppressed due to human immunodeficiency virus (HIV) infection is at an increased risk for several cancers. HIV infected patients have a modestly elevated risk of common skin cancers i.e., melanomas and squamous carcinomas and a greatly elevated risk of 2 rare non-keratinocytic skin cancers i.e., merkel cell carcinomas and sebaceous carcinomas. We report a case of synchronous peri-ocular and extra-ocular SC in a HIV infected patient; to the best of our knowledge there are no similar reports in the English literature.

A 59-year-old HIV infected male on HAART (Highly Active Anti-Retroviral Therapy), was referred to our center for evaluation of 2 asymptomatic ulcerative skin lesions of the face, one a narrow based pedunculated ulcer (4 × 4 cm) in the left cheek along the nasolabial fold of about 6 months duration and the other a 1 × 1 cm ulcer in the middle of the left lower eyelid of about 4 months duration [Figure 1], Both the lesions were of insidious onset, gradually increased in size, were well demarcated and had a crusted surface with a tendency to bleed on minimal trauma. The visual acuity and ocular movements were normal in both eyes. There was no significant pre-auricular or cervical adenopathy, the same was confirmed by a neck ultrasound. The family history and physical examination of the other organ systems did not reveal anything suggestive of a possible associated internal malignancy. A formal evaluation for a possible association with Muir-Torre syndrome (MTS) was done following a biopsy confirmation of a SC from both the ulcerative lesions [Figure 2]. The tests included an ultrasound of the abdomen and pelvis, urine cytology, a motion examination for occult blood and a complete colonoscopy; all of which were normal. He was taken up for surgery and a wide excision of both the ulcerative lesions was done, negative margins were confirmed by frozen section examination. The resultant defects were closed primarily by local advancement flaps [Figure 3]. The patient continues to be on regular follow up for the past one year with a good cosmetic and functional outcome.
Figure 1: Pre-operative clinical photograph showing synchronous peri-ocular and extra-ocular sebaceous carcinomas

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Figure 2: Section shows a tumor in the dermis continuous with the basal cell layer. The cells are large, round, oval to polygonal and have a moderate cytoplasm, large round hyperchromatic nuclei and are arranged in irregular sheets and alveolar masses. (a) H and E , ×40 - Section demonstrating sebaceous differentiation and mitosis. (b,c) H and E , ×40 - Section demonstrating squamoid areas and necrosis. (d) IHC - Section demonstrating 40% of cells showing nuclear positivity to Ki 67

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Figure 3: Immediate post-operative clinical photograph showing the resultant defects were closed primarily by local advancement flaps

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The etiology of sebaceous carcinomas remains unclear. Genetic factors clearly play a role, because sebaceous gland carcinomas are part of the genodermatosis MTS. [3] SC generally presents as a gradually enlarging firm nodule, it can mimic more common ophthalmologic or dermatologic conditions leading to occasional diagnostic delays; the definitive diagnosis is only by pathology. [4]

Surgery remains the primary and the only standard treatment modality for sebaceous carcinomas, which typically involves excision of the visible tumor plus 5-6 mm of healthy-appearing tissue in all directions. [5] The use of the fresh tissue Mohs technique has been successful in a number of case series. [5] Radiation and topical and systemic therapies also have been suggested as useful in the treatment of sebaceous carcinoma, although experience is limited.

In conclusion, awareness of the association of sebaceous carcinomas with HIV among clinicians is essential to ensure early diagnosis of this highly aggressive neoplasm, there is clearly a need for better guidelines aimed at prevention and effective management of skin cancers in HIV-infected individuals.

 
   References Top

1.Song A, Carter KD, Syed NA, Song J, Nerad JA. Sebaceous cell carcinoma of the ocular adnexa: Clinical presentations, histopathology, and outcomes. Ophthal Plast Reconstr Surg 2008;24:194-200.  Back to cited text no. 1
    
2.Shields JA, Demirci H, Marr BP, Eagle RC Jr, Shields CL. Sebaceous carcinoma of the ocular region: A review. Surv Ophthalmol 2005;50:103-22.  Back to cited text no. 2
    
3.Sung D, Kaltreider SA, Gonzalez-Fernandez F. Early onset sebaceous carcinoma. Diagn Pathol 2011;6:81.  Back to cited text no. 3
    
4.Ansai S, Mitsuhashi Y, Kondo S, Manabe M. Immunohistochemical differentiation of extra-ocular sebaceous carcinoma from other skin cancers. J Dermatol 2004;31:998-1008.  Back to cited text no. 4
    
5.Thomas CJ, Wood GC, Marks VJ. Mohs micrographic surgery in the treatment of rare aggressive cutaneous tumors: The Geisinger experience. Dermatol Surg 2007;33:333-9.  Back to cited text no. 5
    


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  [Figure 1], [Figure 2], [Figure 3]



 

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