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Dr.D.P.Meshram, Dr.M.G.Panchal, Dr.S.V.Suvernakar,IntJScRT, MJ v2i3 2014, 01 - 04 SEBACEOUS CELL CARCINOMA OF THE EYELID IN A YOUNG PATIENT - A RARE CASE REPORT. Dr.D.P.Meshram1, Dr.M.G.Panchal2, Dr.S.V.Suvernakar3 1 (Assistant professor, Department of Pathology, Dr.S.C.G.M.C., Nanded, Maharashtra) (Assistant professor, Department of Pathology, Dr.S.C.G.M.C., Nanded, Maharashtra) 3 (Associate professor, Department of Pathology, Dr.S.C.G.M.C., Nanded, Maharashtra) Corresponding author email:[email protected], Ph: 8446935674 _____________________________________________________________________________________________ 2 Abstract: Sebaceous cell carcinoma or Sebaceous Gland Carcinoma (SGC) is amongst the most notorious tumors of the eyelid which commonly masqurades as many benign eye conditions like blepharo-conjunctivitis, mebomianitis, recurrent blepharitis etc. The tumor is most common in the 5th and 6th decade and seen frequently in female patients. Prognosis is good if diagnosed early in course since local excision can be performed. In late stages exenteration is the only option so as to prevent early metastasis. We here report a rare case of a young male patient who came with an extensive eye mass which was diagnosed as SGC based on cytological, histopathological and immunohistochemical (IHC) features. Key words: IHC, Masquarades, Sebaceous cell carcinoma (SGC). _____________________________________________________________________________________________ 1. Introduction Sebaceous Gland Carcinoma (SGC) is a rare eyelid tumor comprising overall about 0.2 – 1.2 % of all eyelid tumors but increased incidence is found in china and other asian countries.1 SGC is frequent in the 5th-6th decade of life with the male to female ratio of 1:2.1 In younger patients it is said to be associated with sunlight and radiation exposure. 2. Case report: A 17yr male presented came to OPD with complains of a painless mass protruding out of the right eye along with loss of vision since 3 months. Headache and other constitutional symptoms like fever, weight loss were also associated. Occular examination was performed. A globular mass arising from the right eye measuring around 8x6 cm was noted. Fig.1: Clinical photograph. The mass was hemorrhagic, firm and non tender. Also a single tiny 1x1 cm, firm, non tender, fixed left retro-mandibular lymph node was palpated. Systemic examination was normal. IJSRT | MAY - JUNE 2014 Available [email protected] 1 Dr.D.P.Meshram, Dr.M.G.Panchal, Dr.S.V.Suvernakar,IntJScRT, MJ v2i3 2014, 01 - 04 X-Ray Skull was performed which suggested a soft tissue mass involving the Rt orbit and surrounding sinuses. CT showed - An ill defined heterogeneous soft tissue density mass (6x5x4.5 cm) arising from the right orbit involving the infratemporal fossa, mandibular sinus, cavernous sinus and extending into the nasopharynx. FNAC was performed under all aseptic conditions with informed consent of the patient. FNAC of the mass revealed cellular smear showing scatteres and clusters of round to oval tumor cells having large pleomorphic and hyper chromatic nuclei with eosinophilic, vacuolated, bubbly cytoplasm arranged in clusters against a hemorrhagic background. Fig.3: FNAC of tumor mass showing cells with pleomorphic and hyper chromatic nuclei with eosinophilic , vacuolated cytoplasm. (PAP, 40x) Thus the provisional diagnosis of malignancy was made and excision biopsy was adviced for confirmation. FNAC of the retromandibular lymph node was done which showed deposits of tumor cells which had the same cytological features as the orbital mass. Thus the provisional diagnosis of secondary deposits was made. Fig.2: FNAC of tumor mass showing clusters of tumor cells. (PAP, 10x) Fig.4: FNAC of lymph node showing metastatic tumor cells. (PAP 10x) The patient was operated and the mass was removed. On gross a 7x6x4 cm exenterated eyeball specimen was received. Externally it was grayish, globular, along with attached adnexae and a grey white mass at one pole. On cut surface it was fleshy, homogenous and firm. IJSRT | MAY - JUNE 2014 Available [email protected] 2 Dr.D.P.Meshram, Dr.M.G.Panchal, Dr.S.V.Suvernakar,IntJScRT, MJ v2i3 2014, 01 - 04 Fig.5: Gross: Exenterated eye ball. Histopathological examination revelead round to oval tumor cells having large hyper chromatic and pleomorphic nuclei with moderate amount of granular, vacuolated cytoplasm arranged in lobular pattern separated by fibrous septae. The overlying stratified squamous epithelium shows severe dysplasia. Fig.6: HPE of tumor cells arranged in lobules separated by fibrous septae. Fig.7: HPE of tumor cells having hyperchromatic and pleomorphic nuclei along with granular, vacuolated cytoplasm. IJSRT | MAY - JUNE 2014 Available [email protected] 3 Dr.D.P.Meshram, Dr.M.G.Panchal, Dr.S.V.Suvernakar,IntJScRT, MJ v2i3 2014, 01 - 04 Thus on histopathology the diagnosis given was moderately differentiated Sebaceous carcinoma metastasizing to cervical lymph node. IHC was further performed. Positive markers were- Pan cytokeratin , EMA and BRST-1 Negative markers were- CEA, S100 Protein After performing Immunohistochemistry the final diagnosis given was Sebaceous Cell Carcinoma. 3. Discussion: Sebaceous carcinoma is a rare skin tumour which comprises to about less than 1% of malignancies of the skin adnexa.1 Median age of presentation is 73 years.2 Sebaceous carcinoma of the eyelid in younger patients is rare and is said to be associated with radiotherapy or radiation exposure by any means.3 SGC is difficult to diagnose since it is known to mimic various benign conditions like recurrent chalazion, mebomianitis, blepharitis & some malignancies like squamous cell carcinoma and basal cell carcinoma.4 Many a times it is found in association with the Muir-Torre Syndrome, which includes SGC with distant primary malignancies like Colon Carcinoma and GenitoUrinary malignancies.4 Spread of tumor is usually via lymphatics, but it can spread via hematogenous route and direct extension.5 Treatment include wide surgical excision with tumour free margin of at least 4 mm and in cases of diffuse and extensive involvement exenteration done.5 Prognosis of SGC is good (5-10 % mortality) if diagnosed before 6 months and size < 1 cm. In advanced condition mortality can reach to approximately 38 %.6 Hence the early diagnosis and prompt treatment is crusial so as to spare the eye. References: 1. Mirza Shafiq Ali Baig, Jameel A. Burney, Ataur Rehman, Syed Shab Ali Sebaceous Cell Carcinoma of the eyelids J. Med. Sci. 2010; Vol. 18(1): 11-14 2. Dasgupta T, Wilson LD, Yu JB: A retrospective review of 1349 cases of sebaceous carcinoma. Cancer 2009, 115(1):158-165. 3. Bhalla JH, Vashisht S, Gupta VK, et al. Meibomian gland carcinoma in a 20-year-old patient (letter). Am J Ophthalmol 1991;111:114–15. 4. Shalini Johnson, MD. Sebaceous Cell Carcinoma: A Masquerade Syndrome January 23, 2006 5. Song A, Carter KD, Syed NA, et al. Sebaceous cell carcinoma of the ocular adnexa: clinical presentations, histopathology, and outcomes. Ophthal Plast Reconstr Surg. 2008;24(3):194-200 6. Santosh G. Honavar, MD; Carol L. Shields, MD; Marlon Maus, MD;Primary Intraepithelial Sebaceous Gland clinicopathologic report(reprinted) arch opthalmol/vol 119. IJSRT | MAY - JUNE 2014 Available [email protected] 4