Advanced sebaceous gland carcinoma: A Case Report

Sebaceous carcinoma is a highly aggressive, uncommon potentially lethal tumour arising from the sebaceous glands in the skin. An advanced case of sebaceous carcinoma with distant metastasis is even rarer as most of them present in the periocular area and are easily visible. Here we present an elderly woman who presented with two exophytic masses over the right orbital region and neck that was confirmed on histopathological examination to be sebaceous carcinoma.


Introduction
Sebaceous gland carcinoma is an aggressive, uncommon cutaneous tumour accounting for 3.2% of all cutaneous malignancies and was first well described by Allaire in 1891. 1 75% of these tumours arise in the periocular region an area rich in a variety of types of sebaceous glands 2 . The cell of origin is often not known 3 . The periorbital primaries are reported to metastasize early with a significant mortality in most series 4 , where as those arising elsewhere on the skin infrequently cause metastasis 5 .

Case report
A 55 year old female presented with history of progressively increasing swelling in front of right lower eyelid for 3yrs associated with another swelling in the right side of face and neck for 1yr. Copious, foul smelling, blood tinged discharge was present. Local examination revealed 2 exophytic, pinkish, fungating masses in the right orbital region measuring 8 x7 cm and the right side of neck below right ear easuring 15 x10 cm. Large areas of ulceration and necrotic slough were noted ( Figure-1). Severe pallor was present.

Abstract
Sebaceous carcinoma is a highly aggressive, uncommon potentially lethal tumour arising from the sebaceous glands in the skin. An advanced case of sebaceous carcinoma with distant metastasis is even rarer as most of them present in the periocular area and are easily visible. Here we present an elderly woman who presented with two exophytic masses over the right orbital region and neck that was confirmed on histopathological examination to be sebaceous carcinoma. Keywords: Sebaceous carcinoma, eyelid, Meibomian glands Basic investigations revealed a microcytic hypochromic anaemia with haemoglobin of 4.8g/dl and an ESR of 95 mm/hr. Renal and thyroid function tests were normal. Liver function tests showed severe hypoalbuminemia. CT scan of neck revealed a large, irregular, heterogenous mass lesion over the orbital region ( Figure-2 & 3) and right side of neck with multiple necrotic areas suggestive of neoplastic growth.   (12) www.ssjournals.com FNAC showed individual tumour cells with irregular hyperchromatic nuclei, conspicuous nucleoli and moderate amount of cytoplasm with vacuoles suggestive of a poorly differentiated carcinoma confirmed later on histopathological examination (Figures-6,7) and with special fat stains to be sebaceous carcinoma. Patient is currently awaiting radiotherapy.

Discussion
Sebaceous gland carcinoma is a very rare malignant tumour primarily found in the area of eyelid. Most of these carcinomas originate in the tarsal Meibomian glands 4 . Tumours of the upper eyelid are 2-3 times more common than lesions of lower eyelid 5 . The clinical presentation is non-specific and is unusually described as a nodule that is pink to yellow red 2 . In the present case an elderly lady presented with 2 pinkish exophytic growths over right orbital region and right side of neck that initially began in front of lower eyelid.
The rate of metastasis in extraocular and ocular sebaceous carcinoma is thought to be similar occurring in 14-25% of cases, first to the draining lymphnode and then to distant sites like liver, lungs, bones and brain 5 . The present case had features suggesting metastasis to skull bones, cervical lymphnodes and lungs on CT scan. FNAC suggested a poorly differentiated carcinoma confirmed on histopathological exam to be sebaceous gland carcinoma. Sebaceous gland carcinoma demonstrates disordered invasion of the dermis by lobules of poorly defined sebaceous cells or basaloid/squamous cells 6 . Sebocytes tend to have multivacuolated clear cytoplasm causing the nucleus to be scalloped from the lipid invasion. In many cases, moderate to severe atypia can be found as well as high nuclear cytoplasmic ratio and perinuclear halo 3 . Sebaceous gland carcinoma can be stained positively with Oil red O or Sudan black which are specific for cytoplasmic fat 7 . The therapy of choice for sebaceous carcinoma is primarily a surgical one 4 , but in view of such extensive lesions and distant metastasis patient is being considered for radiotherapy.

Conclusion
Sebaceous carcinoma is an uncommon and aggressive malignant cutaneous tumor. Most are typically about 10 mm in size at presentation. This neoplasm is thought to arise from sebaceous glands in the skin and, therefore, may originate anywhere in the body where these glands are found. Because the periocular region is rich in this type of gland, this region is a common site of origin. The etiologies of these lesions are, in the vast majority of cases, unknown.
This type of cancer usually has a poor prognosis because of a high rate of metastasis. This type of carcinoma is commonly managed by local resection, cryotherapy, topical chemotherapy, and radiotherapy. Multimodal therapy has been shown to improve both visual prognosis and survival.