Sebaceous Gland Carcinoma of Lid: Masquerading as a Recurring Chalazion.

Recurrent chalazion may be associated with bad lid hygiene but can have clinical changes which lead to the suspicion of a more dangerous entity. Particularly in elderly patients, recurrence of chalazion should be suspected to have carcinomatous changes. We present the case of a 52-year-old woman with presentation of recurrent chalazion which turned out to be sebaceous gland carcinoma of the lid. Sebaceous gland carcinoma usually presents as a small firm nodule resembling chalazion, and majority of the premalignant and malignant lesions misdiagnosed as chalazion are primary cases. Hence, the need of histopathological examination in every such case cannot be undermined. Copyright:

Introduction

Chalazion is a localized chronic granulomatous inflammation particularly affecting the meibomian glands of the eyelids. It usually arises secondary to noninfectious obstruction of sebaceous gland duct.[1] Granulation tissue containing giant cells, plasma cells, histiocytes, and polymorphonuclear leukocytes replaces normal glandular tissue, probably due to chronic irritation. Patients often notice a hard, painless swelling in either lid, increasing very gradually in size and without inflammatory symptoms. Chalazion is the most common inflammatory lesion of the eyelids.[2] Although it seems to be easy to be diagnosed clinically, chalazion can simulate many benign, premalignant, and malignant lesions. It is always important to remember that few malignant conditions such as sebaceous gland carcinoma, Merkel cell tumor, desmoplastic malignant melanoma, microcystic adnexal carcinoma, and neurilemoma have already been reported masquerading as chalazion. Out of all these, sebaceous cell carcinoma can particularly be misdiagnosed as chalazion.[345] A limited number of single case reports and small series discussing misdiagnosis of lesions masquerading as chalazion have been reported in the literature.[4567]

Case Report

A 52-year-old woman presented to us with a well-defined nontender swelling of the left upper lid for 1 month. She had a history of similar swelling 2 months back, and after a minor surgery, it was resolved. There was no history of any trauma, systemic disease, or malignancy. Examination revealed a well-defined nontender, palpable nodule in the left upper eyelid of size 4 mm × 6 mm. The skin over the nodule was freely movable, not associated with blepharitis, meibomitis, thickening of the lid margin, or focal loss of eyelashes [Figure 1]. No local lymphadenopathy was found. Best-corrected visual acuity was 20/20, N6 in both eyes. Anterior and posterior segments were within normal limits. The lacrimal apparatus was also normal with an intraocular pressure of 14 mmHg in both the eyes. Routine investigations were normal, and hemoglobin level was 14 g/dl. Initial plan of management was incision and curettage of the lesion. However, during the procedure, the whole mass could not be adequately scooped, so a part was incised and send for histopathological examination. Histopathology revealed irregular lobular pattern, with each lobule containing disorderly admixture of basophilic sebaceous cells with variable amount of lipid in it. Marked nucleocytoplasmic pleomorphism was seen and was diagnosed as sebaceous cell carcinoma [Figure 2a and b]. Hence, as a part of further management, excision of the tumor with 3 mm clinically uninvolved tissue followed by reconstruction of eyelids was done. Such excision created a tissue defect of 15 mm (>50% of the eyelid tissue). Hence, to reconstruct such a large detect, a lower lid switch flap was done. Computed tomography scan orbit was done which was normal. After 1-year of follow-up, no recurrence was seen [Figure 3].

Figure 1

Well-defined freely mobile nodule of size 4 mm × 6 mm on the left upper eyelid

Figure 2

(a) Histopathological section of the mass (×10 magnification showing irregular lobular pattern. (b) Histopathological section (×40 magnification) showing disorderly admixture of basophilic sebaceous cells with marked nucleocytoplasmic pleomorphism

Figure 3

Postoperative photograph showing no recurrence 1 year after surgery

Discussion

Chalazion is a chronic, lipogranulomatous inflammatory lesion caused by retained sebaceous secretion. Common in the adult age group as sebum viscosity increase with androgen, it usually is found to be associated with seborrhea, chronic blepharitis, acne rosacea, and poor lid hygiene. The upper eyelid is found to be the most common site.

Around 6% of cases of chalazion are clinically misdiagnosed, out of which 75% are benign, 22% premalignant, and 3% are malignant. More than one-third of the misdiagnosed malignant tumors turn out to be sebaceous gland carcinoma.[8] Sebaceous gland carcinoma is the most common malignancy found in recurrent chalazion. It is a highly malignant and potentially lethal tumor, common after 50 years of age and more frequent in females, twice more common in the upper lid – mainly two types: Extraocular and periocular. Periocular variety accounts for 75% of the total cases and is relatively more aggressive. Sebaceous carcinoma accounts for 1%–5% of lid malignancy in developed countries, but in India, the incidence is much higher (37%–60%), and in some studies, it is the most common lid malignancy.[9]

Etiology is still unknown. The possible implicating factors are ultraviolet rays, human papillomavirus infection (especially in Asians), mutation of P53 gene, and increased expression of c-erB-2 oncogene. Histological examination shows differentiated variety where each lobule consists of disorderly admixture of basophilic germinative sebaceous cells; each cell contains round/oval nuclei and lightly eosinophilic “bubbly” cytoplasm and shows irregular lobular pattern and poorly differentiated type where tumor cells are more hyperchromatic and contain less amount of lipid material with marked nucleocytoplasmic pleomorphism. Management of these cases includes wide surgical excision leaving clear cancer-free margins. Orbital exenteration may be considered for recurrent or large tumors invading through the orbital septum. In metastatic disease, systemic chemotherapy needed as it is relatively radioresistant, so radiotherapy is usually ineffective. With metastasis, the 5-year survival rate is only ~50%. Hence, metastasis and mortality rate can be lowered with early detection and treatment. Poor prognostic factors are multicentricity, size >1 cm in diameter, poorly differentiated, extensive tissue involvement, and vascular or lymphatic involvement. Differentiation between chalazion and sebaceous carcinoma is possible via noninvasive meibography (NIM) where chalazion shows low reflectivity in NIM images due to replacement of normal meibomian gland tissue by granulomatous tissue comprising infiltrated inflammatory cells and accumulated extracellular matrix. Chalazion associated with nodule may show high reflectivity due to the presence of fatty granules in the nodular lesion. On the other hand, sebaceous gland carcinoma with its associated lipid is visualized as a poorly marginated irregular lesion of high reflectivity with or without associated nodular structure.[10]

Conclusion

Further investigation and histopathological examination is a must in cases with atypical symptomatology, recurrence, elderly age group, unilateral therapy-resistant keratoconjunctivitis, and regional lymph node involvement. The most common malignancy in recurrent chalazion is sebaceous gland carcinoma. It is highly malignant and potentially lethal; early diagnosis and treatment is key to optimum survival. Hence, it is wiser to send a biopsy for a histopathological examination even in a benign lesion taking into account all the abovementioned factors.

Declaration of patient consent

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

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Conflicts of interest

There are no conflicts of interest.