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CASE REPORT  
Year : 2013  |  Volume : 56  |  Issue : 4  |  Page : 408-410
Sebaceous carcinoma with apocrine differentiation: A rare entity with aggressive behavior


1 Department of Pathology, J N Medical College, Aligarh, Uttar Pradesh, India
2 Department of Ophthalmology, J N Medical College, Aligarh, Uttar Pradesh, India

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Date of Web Publication18-Jan-2014
 

   Abstract 

Sebaceous carcinomas are rare neoplasms but have aggressive behavior. Although they can be found anywhere in the body ocular region is the most common site which comprises 75% of all cases of sebaceous carcinomas. Due to their rarity, varied histopathological features, and diverse clinical presentation, their diagnosis is often delayed, sometimes by a year. They are divided on the basis of histological differentiation into well and poorly differentiated. Apocrine differentiation is a still rarer finding and only two cases have been reported in the literature so far. We report a case of sebaceous carcinoma with apocrine differentiation in a 60-year-old male who presented with a painless swelling in right upper eyelid for 2 months which was gradually progressive. Computed tomography (CT) scan was performed and a provisional diagnosis of hemangioma was made. The mass was excised and histopathological examination revealed it to be sebaceous carcinoma. However, there were areas with decapitation secretions and granular eosinophilic cytoplasm. These were positive for cytokeratin (CK) 7 and CK 19 which confirmed their apocrine nature. Therefore, a final diagnosis of sebaceous carcinoma with apocrine differentiation was made. Thus, it can be concluded that ocular sebaceous carcinomas with apocrine differentiation are extremely rare and have significant clinical importance since they can mimic a benign lesion and the nature of surgical intervention and follow up is more aggressive than that of simple sebaceous carcinoma alone.

Keywords: Apocrine differentiation, orbit, sebaceous carcinoma

How to cite this article:
Afroz N, Zaidi N, Rizvi SR. Sebaceous carcinoma with apocrine differentiation: A rare entity with aggressive behavior. Indian J Pathol Microbiol 2013;56:408-10

How to cite this URL:
Afroz N, Zaidi N, Rizvi SR. Sebaceous carcinoma with apocrine differentiation: A rare entity with aggressive behavior. Indian J Pathol Microbiol [serial online] 2013 [cited 2020 Oct 24];56:408-10. Available from: https://www.ijpmonline.org/text.asp?2013/56/4/408/125354



   Introduction Top


Sebaceous carcinoma (SC) is a rare and potentially aggressive cutaneous tumor. What is known about the incidence, risk factors, and prognosis of this disease is based on isolated case reports of <200 patients. [1] The face (particularly the eyelids) and scalp are the most commonly affected sites. [2] It is primarily periorbital and, whereas some authors consider it to be second most common, others consider it to be fourth most common neoplasms of the eyelid. [1],[3] This tumor usually arises in the  Meibomian gland More Detailss of tarsus, in the Zeis glands, and the sebaceous glands of caruncle or eyebrow. [3],[4] The upper eyelid is affected two to three times more often than the lower eyelid due to the greater number of Meibomian glands. [2] So SCs can be divided into ocular (periocular) and extraocular types, which respectively comprise approximately 75% and 25% of all SCs. [5] They exhibit such a variety of histologic growth patterns and diverse clinical presentations that the diagnosis is often delayed for months to years. [6] Both clinically and histologically, ocular SCs may masquerade as benign or less invasive conditions such as chalazion, blepharitis, conjunctivitis,  Meibomitis More Details, superior limbic keratoconjunctivitis, basal cell carcinoma, and carcinoma in situ, resulting in delayed diagnosis and treatment. [7] SCs can be categorized according to their degree of differentiation ranging from well differentiated (grade 1) with foamy cytoplasm in all cells to undifferentiated (grade 4) with little cytoplasm. [8] Few isolated case reports have described an apocrine variant of SC. Wang and Sarma, and Misago and Narisawa have described such a variant with glandular structures and decapitation secretions, in their case reports. [9],[10]

We hereby report a case, with brief review of the literature, of ocular SC with apocrine differentiation due to its extreme rarity and potential for aggressive behavior.


   Case Report Top


A 60-year-old male presented to the out-patient department with a swelling in the right upper eyelid since 2 months. The swelling was painless and was gradually progressive in nature. There was no other significant past, personal or family history. On examination, the patient was an elderly male of average built, with a 3 × 2 cm mass palpable in the superomedial part of right orbit. The mass was firm in consistency and was non-tender. There was no other swelling anywhere else. A computed tomography (CT) was advised. CT revealed a hypointense lesion in the superomedial aspect of right orbit [Figure 1] and based on this finding a provisional diagnosis of hemangioma was made. The mass was excised and was sent for histopathological examination.
Figure 1: Computed tomography scan showing a hypointense lesion in the superomedial aspect of right orbit (white arrow)

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Histopathological examination of the hematoxylin and eosin (HandE) stained slides revealed lobular arrangement of cells of variable size and differentiation. Focal vacuolated sebocytic differentiation and sebaceous ductal differentiation were seen with central areas showing more well-differentiated cells than periphery [Figure 2]a, 2b. The nuclei were oval with prominent nucleoli in many. Mitotic rate was high with 2-3 mitosis/high-power field. There was no evidence of peripheral palisading or keratinization. Focal (approximately 10-20% of tumor) areas of glandular differentiation were seen. These were lined by columnar cells with granular eosinophilic cytoplasm with evidence of decapitation secretions [Figure 2]c, 2d]. Immunostains were performed on the paraffin-embedded sections. The cells in the lobular areas were positive for epithelial membrane antigen (EMA). The areas of glandular differentiation showed positivity for cytokeratins (CK) 7 and 19 in the luminal cells [Figure 3]a, [Figure 3]b. Hence, a final diagnosis of ocular SC with apocrine differentiation was made.

Additional investigations were performed to detect any underlying malignancy to rule out Muir-Torre syndrome and they were unremarkable. The patient is on regular follow-up now.
Figure 2: (a) Invasive nests of malignant cells containing both sebocytes and apocrine cells (H and E secti on × 10×). (b) Higher magnifi cati on of 2A showing both sebaceous (left ) and apocrine (right) diff erenti ati on (H and E section × 40×). (c) Invasive glandular structures with decapitati on secreti ons (H and E secti on × 10×). (d) Higher magnifi cati on of figure 2C (H and E secti on × 40×)

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Figure 3: (a) Immunostaining for cytokerati n 7 (CK7) showing strong and diffuse cytoplasmic positi vity (IHC CK7 × 10×). (b) Immunostaining for cytokerati n 19 (CK19) exhibiti ng strong positi vity in cell nests as well as ducts (IHC CK19 × 10×)

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   Discussion Top


SC is a very rare malignant tumor primarily found in the area of the eyelid. It occurs predominantly in females (female:male ratio is 2:1) at an average age of 65 years. The tumor usually presents as a slowly growing, firm, and painless mass. Its prognosis is much worse than those of most cutaneous malignancies except for malignant melanoma due to local recurrences and distant metastases. [9] Ocular SCs are considered to have a poorer prognosis as they usually present with metastases. [2] However, according to few authors SCs are more common in males with no difference in prognosis between ocular and extraocular SCs. [1] In our case, the patient was a 60-year-old male and he presented with a painless and slowly growing mass in the right upper eyelid. There was no evidence of any metastasis.

According to Wolfe et al., SC can be divided into four grades depending on the degree of differentiation of the tumor cells. Grade 1 represents extremely well-differentiated SCs while grade 4 represents poorly differentiated ones where diagnosis requires special stains and ultra-structural studies. [8] Misago et al. divide well-differentiated SCs into secretory and non-secretory types, based on histopathology. The secretory type shows evidence of holocrine secretion whereas non-secretory type lacks it. [5] SCs are positive for epithelial membrane antigens (EMA) and androgen receptors but not for carcinoembryonic antigen (CEA) or S100. [11],[12] Apocrine differentiation in SC is a very rare finding. Very less is known about its clinical and pathological characteristics. However, according to the few case reports in the literature, its diagnosis is confirmed by positive immunostaining for CK 7, CK 19, CK 8, CK 8/18 and GCDFP-15. [9],[10]

In our case histopathological diagnosis of SC was confirmed by immunostaining for EMA. The apocrine differentiation was confirmed by positive immunostaining for CK 7 and CK 19. It is believed that pluripotent stem cells in the folliculosebaceous-apocrine unit can give rise to follicular stem cells, sebaceous stem cells and apocrine stem cells. Therefore, SCs with apocrine differentiation can originate from pluripotent stem cells destined to become sebaceous cells or from sebaceous stem cells which also have the ability to differentiate within apocrine glands. [10] According to Shintaku et al, apocrine adenocarcinomas of the ocular region have the potential for aggressive biological behavior, including distant metastasis. [13]

According to Martinelli et al., the histologic diagnosis of SC is not straightforward and other entities need to be considered in the pathologic differential diagnosis. The histologic differentiation of SC from sebaceous adenoma, sebaceous epithelioma, or normal mature sebaceous glands is based on the amount of cellular atypia, size, symmetry, degree of circumscription, and degree of differentiation. [14] In our case, due to evident apocrine differentiation, the diagnosis was quite straightforward.


   Conclusion Top


Apocrine differentiation in sebaceous adenocarcinoma is a rare entity with unfavorable prognostic behavior. Therefore, prompt and accurate clinical and/or histopathological diagnosis is necessary so that proper management is initiated before distant spread.

 
   References Top

1.Dasgupta T, Wilson LD, Yu JB. A retrospective review of 1349 cases of sebaceous carcinoma. Cancer 2009;115:158-65.  Back to cited text no. 1
    
2.Ho CK. Sebaceous carcinoma. Hong Kong Dermatol Venereol Bull 2002;10:129-32.  Back to cited text no. 2
    
3.De Potter P, Shields CL, Shields JA. Sebaceous gland carcinoma of the eyelids. Int Ophthalmol Clin 1993;33:5-9.  Back to cited text no. 3
    
4.Valenzuela-Flores G, Mozas-Dávila D, Rodríguez-Reyes AA, Gómez-Leal A. Sebaceous gland carcinoma of the eyelids. Cir Cir 2004;72:47-53.  Back to cited text no. 4
    
5.Misago N, Toda, NarisawaY. Two histopathologic patterns of well-differentiated extraocular sebaceous carcinoma. J Cutan Pathol 2011;38:767-74.  Back to cited text no. 5
    
6.Nelson BR, Hamlet KR, Gillard M, Railan D, Johnson TM. Sebaceous carcinoma. J Am Acad Dermatol 1995;33:1-15.  Back to cited text no. 6
    
7.Kass LG, Hornblass A. Sebaceous carcinoma of the ocular adnexa. Surv Ophthalmol 198;33:477-90.  Back to cited text no. 7
    
8.Wolfe JT 3rd, Yeatts RP, Wick MR, Campbell RJ, Waller RR. Sebaceous carcinoma of the eyelid. Errors in clinical and pathologic diagnosis. Am J Surg Pathol 1984;8:597-606.  Back to cited text no. 8
    
9.Wang B, Sarma DP. Sebaceous carcinoma with apocrine differentiation. Internet J Dermatol 2006;4:11.   Back to cited text no. 9
    
10.Misago N, Narisawa Y. Sebaceous carcinoma with apocrine differentiation. Am J Dermatopathol 2001;23:50-7.  Back to cited text no. 10
    
11.Moreno C, Jacyk WK, Judd MJ, Requena L. Highly aggressive extraocular sebaceous carcinoma. Am J Dermatopathol 2001;23:450-5.  Back to cited text no. 11
    
12.Ansai S, Takeichi H, Arase S, Kawana S, Kimura T. Sebaceous carcinoma: An immunohistochemical reappraisal. Am J Dermatopathol 2011;33:579-87.  Back to cited text no. 12
    
13.Shintaku M, Tsuta K, Yoshida H, Tsubura A, Nakashima Y, Noda K. Apocrine adenocarcinoma of the eyelid with aggressive biological behavior: Report of a case. Pathol Int 2002;52:169-73.  Back to cited text no. 13
    
14.Martinelli PT, Cohen PR, Schulze KE, Tschen JA, Nelson BR. Sebaceous Carcinoma. In: Nouri K, Editor. Skin cancer. 1 st ed. New York, NY: McGraw-Hill Medical; 2008. p. 240-50.  Back to cited text no. 14
    

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Correspondence Address:
Nishat Afroz
Department of pathology, J N Medical College, AMU, Aligarh - 202 002, Uttar Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0377-4929.125354

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