The human eyelid is a complex structure that is frequently subject to pathological diseases such as inflammatory and benign and/or malignant tumors with similar clinical presentation. The sebaceous gland tumors originate in the Meibomian glands, glands of Zeiss, caruncle, and skin of the eyebrow [1]. The sebaceous gland carcinoma (SGC) being the third most common malignancy in the eyelid frequently occurs in elderly women [2]. It can be misdiagnosed as blepharitis or chalazion or conjunctivitis and result in significant morbidity and mortality [3]. We report a case of an eyelid sebaceous gland carcinoma of a young boy who had undergone incision and curettage (I and C) several times. The importance of reporting this case is based on a misdiagnosis of SGC in young boys and the need for full-thickness biopsy in case of recurrent chalazion.

Case Report 

A 21-year-old boy, presented in the oculoplastic outpatient department with a chief complaints of swelling in the left upper lid for 6 months. He gave history Incision and curettage done elsewhere for the same condition thrice. However, an incision biopsy done in one of the tertiary centers showed sebaceous cell carcinoma and was referred for wide excision and reconstruction in our oculoplastic and oncology department. 

On external examination, an oval mass approximately 3 cm x 2.5 cm in diameter was visible in the upper lid with mass effect causing mechanical ptosis [Figure 1a]. The mass was firm to hard in consistency with adherent to deep tissues associated with distorted lid margin and loss of cilia. The skin overlying tumor revealed an ulcerative lesion. The eyelid eversion revealed scar of previous surgery, thickening of the tarsal plate, marked distortion of anatomy, and vascular engorgement around the lesion [Figure 1b]. On ophthalmic examination, Visual acuity, Hertel’s Exophthalmometry measurements, and extraocular muscle motility were normal. There was no regional lymphadenopathy and systemic workup revealed no metastasis.    

Figure 1: Preoperative photographs of patient with SGC. (a) Round to oval mass with hyperemia and skin ulceration producing ptosis. (b) Eversion of eyelid showing scar of previous surgery, distorted tarsal plate and vascular engorgement around lesion

Figure 2: Intraoperative photographs of patient undergoing wide local excision and eyelid reconstruction by cutler-beard technique. (a) Large eyelid defect after tumor and plus all layers of eyelid excision. (b) Posterior lamellar reconstruction with autologous post-auricular cartilage. (c) Passage of the right lower eyelid myocutaneous flap under the lower eyelid border. (d) Completion of 1^st step of cutler beard procedure

The tumor was removed en-block with a 5 mm margin and sent for histopathological reconfirmation. Double freeze-thaw cryotherapy was applied to all the skin and conjunctival margins as well as cut end of the canaliculus. The large upper lid defect was reconstructed in the same sitting with 1st step cutler beard technique wherein post auricular ear cartilage was harvested for posterior lamella reconstruction (Figure 2a, 2b, 2c and 2d). He was prescribed oral antibiotics and proteolytic enzymes for a week and topical antibiotics for a month. Histopathological examination showed tumor consisting of nests and irregular lobules of oval cells involving the full thickness of eyelid with foci of necrosis and coarse nuclear chromatin and prominent nuclei with foamy, multivacuolated cytoplasm (Figure 3a and 3b). There was no lymphatic, vascular and perineural invasion.

At the 6 weeks, post-operative visit, a 2nd step cutler beard procedure was performed to separate the eyelids, and conjunctiva was sutured to reconstructed the upper eyelid. The lower eyelid wound margins were freshened, underlying tissues, and the skin sutured to the surrounding tissue. Postoperatively after one week after 2nd step of the cutler beard procedure there was no 

Figure3: Hematoxylin and eosin stained section from a full-thickness wedge resection. (a) The tumor consisted numerous of nests and irregular lobules of invasive disease throughout the eyelid. (b) High magnification shows poorly differentiated tumor with foamy, multivacuolated cytoplasm and abnormal mitotic figure.

Figure 4: Post-operative photographs of patients after 2 weeks of 2^nd step of cutler beard procedure. (a) Maintenance of eyelid contour (b) Absence of lagophthalmos

lagophthalmos or exposure keratitis, and no residual periocular malpositioning.

After 2 months it remains with excellent eyelid closure and good aesthetic results [ Figure 4a and 4b]. He is advised to follow up every 3 months for functional and aesthetic evaluation as well as regional or distant metastasis workup.

The sebaceous gland carcinoma can occur anywhere in the body where sebaceous glands are found. However, the upper eyelid is the most common site due to the presence of more Meibomian glands [4,5]. The incidence of sebaceous gland carcinoma on the eyelid varies from geographical areas accounting for approximately 5% in the USA [5],7.9% in Taiwan [6] and 28% to 60 % in Asia [7]. The sebaceous gland carcinoma of the eyelid is more commonly occurs in old aged over 55-years [8,9]. The patient in our study is young which contradicts other studies in which most of patients were from older age group. There were no justifiable causes like Muir-Torre Syndrome, HIV, or retinoblastoma for the early onset of the disease [10,11]. The male patient in our study also contradicts other studies in which most of the patients are women [2,12]. However, the occurrence of eyelid sebaceous gland carcinoma was reported in 39 -years –old males in a Brazilian study [12]. In the context of our country, no case has been reported occurrence of sebaceous carcinoma of the eyelid in young males. Our case was a young boy without previous history of irradiation or genetic predisposition who presented with a tumor size of about 2cm x 2cm that resembled a giant chalazion. On eversion of the eyelid, we noticed a scar of previous surgery, thickening of the tarsal plate, marked distortion of anatomy, and vascular engorgement around the lesion. He had undergone incision and curettage thrice in different hospitals for misdiagnosis of chalazion. However, sebaceous carcinoma of the eyelid commonly masquerades as recurrent chalazion or blepharitis, or chronic blepharoconjunctivitis leading to clinical misdiagnosis [8,13]. We should examine the lid margin, conjunctiva, and tumor carefully to avoid clinical misdiagnosis. It is recommended that all types of malignant eyelid tumors should undergo wide excision biopsy under frozen section or Moh’s micrographic surgery control [8,14]. In our study, we performed wide excision with a 5 mm margin. We couldn’t do a mapping biopsy at primary sitting due to lack of facility. However, double freeze-thaw cryotherapy was applied on all margins of skin and conjunctiva to prevent the local spread of the tumor [15]. The upper eyelid defect was reconstructed at the same sitting with post-auricular ear cartilage and cutler beard technique since the tumor was limited to the eyelid, and there was no regional lymphadenopathy. In our case, the histopathological report revealed poorly differentiated sebaceous carcinoma with the margins free of tumor invasion and no lymphatic, vascular, and perineural invasion TNM (AJCC) stage: pT2cNx. We advised our patients to consult with an oncologist for further systemic metastasis work up and needful. However, the patient didn’t require any postoperative adjuvant therapy and sentinel lymph node biopsy in accordance with the prognostic value of the staging system for eyelid tumors American Joint Committee on Cancer Staging Manual ,7th edition [16].

This case report aims to highlight the need for a high degree of suspicion, sound clinical skills, and good pathology backup for successful diagnosis and treatment of sebaceous carcinoma of the eyelid in the young population.

Acknowledgment

We would like to express our sincere thanks to Prof. Dr. Gita Sayami, Prof. Dr Ram Chandra Adhikari and the Department of Pathology, HAMS hospital for their kind cooperation.

Conflict of Interest

None

Consent

Informed written consent from Patient was obtained

1. Pe’er, J. “Pathology of Eyelid Tumors.” Indian Journal of Ophthalmology, vol. 64, no. 3, 2016, p. 177.

2. Snow, S.N. “Sebaceous Carcinoma of the Eyelids Treated by Mohs Micrographic Surgery: Report of Nine Cases with Review of the Literature.” Dermatologic Surgery, vol. 28, no. 7, 2002, pp. 623–631.

3. Kass, L.G., and A. Hornblass. “Sebaceous Carcinoma of the Ocular Adnexa.” Survey of Ophthalmology, vol. 33, no. 6, 1989, pp. 477–490.

4. Mahipathy, S.R.R.V. et al. Sebaceous Gland Carcinoma of the Lower Eyelid Reconstructed with a Composite Flap: A Case Report. Journal of Clinical and Diagnostic Research: JCDR, vol. 10, no. 6, 2016, pp. PD16–PD18.

5. Shields, J.A. et al. “Sebaceous Carcinoma of the Ocular Region: The 2014 Professor Winifred Mao Lecture.” The Asia-Pacific Journal of Ophthalmology, vol. 4, no. 4, 2015, pp. 221–227.

6. Lin, H.Y. et al. “Incidence of Eyelid Cancers in Taiwan: A 21-Year Review.” Ophthalmology, vol. 113, no. 11, 2006, pp. 2101–2107.

7. Sihota, R. et al. “Malignant Eyelid Tumors in an Indian Population.” Archives of Ophthalmology, vol. 114, no. 1, 1996, pp. 108–109.

8. Shields, J.A. et al. “Sebaceous Carcinoma of the Ocular Region: A Review.” Survey of Ophthalmology, vol. 50, no. 2, 2005, pp. 103–122.

9. While, B. et al. “Excision and Delayed Reconstruction with Paraffin Section Histopathological Analysis for Periocular Sebaceous Carcinoma.” Ophthalmic Plastic & Reconstructive Surgery, vol. 30, no. 2, 2014, pp. 105–109.

10. Hussain, R.M. et al. “UV-Independent p53 Mutations in Sebaceous Carcinoma of the Eyelid.” Ophthalmic Plastic & Reconstructive Surgery, vol. 30, no. 5, 2014, pp. 392–395.

11. Burns, S.J. et al. “Outcome of Periocular Sebaceous Gland Carcinoma.” Ophthalmic Plastic & Reconstructive Surgery, vol. 21, no. 5, 2005, pp. 353–355.

12. Veras, D.D.O. et al. “Reconstrução Total de Pálpebra Superior no Tratamento do Carcinoma de Glândula Sebácea.” Revista Brasileira de Oftalmologia, vol. 79, no. 3, 2020, pp. 203–206.

13. Shields, J.A. et al. “Sebaceous Carcinoma of the Eyelids: Personal Experience with 60 Cases.” Ophthalmology, vol. 111, no. 12, 2004, pp. 2151–2157.

14. Jakobiec, F.A. “Sebaceous Tumors of the Ocular Adnexa.” Principles and Practice of Ophthalmology, vol. 3, 1993, pp. 1745–1770.

15. Kaliki, S. et al. “Neoadjuvant Systemic Chemotherapy in the Management of Extensive Eyelid Sebaceous Gland Carcinoma: A Study of 10 Cases.” Ophthalmic Plastic and Reconstructive Surgery, vol. 32, no. 1, 2016, pp. 35–39.

16. Ford, J. et al. “Prognostic Value of the Staging System for Eyelid Tumors in the 7th Edition of the American Joint Committee on Cancer Staging Manual.” Ophthalmic Plastic and Reconstructive Surgery, vol. 33, no. 5, 2017, pp. 317–324.