UNDERSTANDING SEBACEOUS CARCINOMA OF THE EYELIDS
Clinical scenario:
A 75-year-old female patient is referred to the ophthalmology clinic for a second opinion. She reports a small lesion on her right upper eyelid six months ago, initially treated conservatively with a diagnosis of chalazia, leading to surgical excision. Three weeks post-lesion removal, recurrence occurred, now more significant in size, prompting a second evaluation and excision with the same diagnosis of chalazia. One month after this second intervention, the patient notices further lesion growth, escalating over the next months, eventually causing mechanical ptosis of the upper eyelid. This is the primary reason for seeking a second opinion. On presentation, yellowish, elevated, poorly demarcated lesion is noted at the upper eyelid margin, which is non-mobile. The adjacent eyelid margin shows keratinization, and there is madarosis or absence of eyelashes in the same region. Eversion reveals the lesion involving the superior tarsal conjunctival (see images). Now, let us delve into crucial details about this condition, especially when to suspect it. Remember, the goal is to enhance your understanding and know when to refer to a specialist. This review focuses on that, and we will not delve into surgical techniques and specific treatment at this moment, although it could be a topic for future discussion.
Introduction
Sebaceous carcinoma, a rare and aggressive skin tumor, commonly affects the eyelids, originating from sebaceous glands. While predominantly found in the upper eyelid due to higher gland density, it can occur in other body areas. Diagnosis prompts consideration of Muir-Torre Syndrome (MTS), an inherited condition linked to various tumors. Sebaceous carcinomas in MTS cases display less aggressiveness and lower metastatic potential than solitary occurrences.¹
Key clinical features¹,²:
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High index of suspicion: Recognizing atypical presentations such as recurrent chalazia, yellow thickening of the eyelid, and unresponsive keratoconjunctivitis or blepharoconjunctivitis.
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Physical examination: Yellow appearance due to lipid content, typically arising at the lid margin and extending to the palpebral conjunctiva. Eversion of the lid is crucial for examination.
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Mimicry of other lesions: Sebaceous carcinoma can mimic various benign lesions, including chalazia, chronic blepharitis, basal cell carcinoma, squamous cell carcinoma, superior limbic keratoconjunctivitis, and ocular cicatricial pemphigoid.
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Difficulty in clinical diagnosis: Studies highlight the challenge in clinically diagnosing sebaceous carcinoma, often misdiagnosed as other conditions such as basal cell carcinoma or labeled with vague terms like "lesion" or "mass."
General Treatment:
Surgical excision remains the cornerstone of treatment, emphasizing the importance of obtaining a full-thickness specimen and conducting map biopsies of the conjunctiva. Invasive cases may necessitate orbital exenteration, and staging is assessed using TNM definitions. Sentinel lymph node biopsies are recommended for cases with specific tumor characteristics as per American Joint Committee on Cancer (AJCC) guidelines³.
Surgery:
Mohs micrographic surgery demonstrates efficacy in treating sebaceous carcinoma, aiding in precise tumor removal. Detailed history-taking, age-appropriate cancer screening, and immunohistochemical staining help identify patients requiring geneticist referral for Muir-Torre Syndrome work-up.Wide local excision and, in advanced cases, exenteration may be warranted, especially for deeply invasive or large lesions involving significant bulbar conjunctival tissue¹,³.
Adjunctive Therapies:
Cryotherapy and topical mitomycin C may be considered as adjunctive therapies, particularly in cases of conjunctival epithelial involvement or pagetoid spread².
Role of Radiation Therapy:
The role of radiation therapy for primary sebaceous carcinomas remains uncertain. While some small studies have reported favorable outcomes, surgical resection remains the primary treatment modality⁴.
Prognosis:
Sebaceous carcinoma exhibits a potential for recurrence in up to 33% of cases, with metastasis occurring in approximately 25%. Higher T categories, particularly T2b or worse, correlate with regional lymph node metastases, while T3a or worse categories are associated with distant metastases and mortality. Notably, tumors smaller than 12mm have shown no association with distant metastases or mortality, as per AJCC findings¹,³.
EYEQ-POINTS:
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Clinical Presentation: Recognize atypical signs like recurrent chalazia and yellowish eyelid thickening.
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Physical Examination: Look for a yellowish lesion at the lid margin, extending to the conjunctiva.
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Differential Diagnosis: Be aware of mimicking conditions such as chalazia and other malignancies.
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Treatment Approach: Surgery, including Mohs, is primary; consider adjuvants like cryotherapy.
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Prognosis: Understand recurrence and metastasis risks based on tumor size and extent.
References
1. Buitrago W, Joseph AK. Sebaceous carcinoma: the great masquerader. Dermatol Ther. 2008;21(6):459-466. doi:10.1111/j.1529-8019.2008.00247.x
2. Shields JA, Demirci H, Marr BP, Eagle RC, Stefanyszyn M, Shields CL. Conjunctival Epithelial Involvement by Eyelid Sebaceous Carcinoma. The 2003 J. Howard Stokes Lecture. Ophthalmic Plast Reconstr Surg. 2005;21(2):92-96. doi:10.1097/01.IOP.0000152494.47038.0E
3. Esmaeli B, Nasser QJ, Cruz H, Fellman M, Warneke CL, Ivan D. American Joint Committee on Cancer T Category for Eyelid Sebaceous Carcinoma Correlates with Nodal Metastasis and Survival. Ophthalmology. 2012;119(5):1078-1082. doi:10.1016/j.ophtha.2011.11.006
4. Azad S, Choudhary V. Treatment results of high dose rate interstitial brachytherapy in carcinoma of eye lid. J Cancer Res Ther. 2011;7(2):157. doi:10.4103/0973-1482.82922