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Sebaceous carcinoma with apocrine differentiation arising in a known case of basal cell carcinoma: A rare entity

 Department of Pathology and Plastic Surgery, Maulana Azad Medical College and Associated Hospitals, New Delhi, India

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Date of Submission29-Sep-2021
Date of Decision26-Apr-2022
Date of Acceptance28-Apr-2022
Date of Web Publication27-Jan-2023


Sebaceous carcinoma is a ra malignant tumor of adnexal origin arising from sebaceous glands. It is most commonly seen arising from the eyelids and head and neck. It is predominantly seen in females with an average age of around 65 years. Apocrine differentiation in sebaceous carcinomas is rare but has been reported in the literature. Here, we present a case of sebaceous carcinoma with apocrine differentiation in a 62-year- old female who was a diagnosed case of basal cell carinoma.

Keywords: Apocrine differentiation, sebaceous carcinoma, Basal cell carcinoma

How to cite this URL:
Ahuja M, Mandal S, Singh M, Khurana N, Bhandari PS. Sebaceous carcinoma with apocrine differentiation arising in a known case of basal cell carcinoma: A rare entity. Indian J Pathol Microbiol [Epub ahead of print] [cited 2023 Mar 27]. Available from:

   Introduction Top

Basal cell carcinoma (BCC) is a common malignant tumor in adults which follows an aggressive course. It originates from interfollicular or follicular epithelium. BCC is known to show sebaceous and apocrine differentiation, but sebaceous carcinoma arising from BCC is rare. Hereby, we present a rare case of sebaceous carcinoma (SC) with apocrine differentiation occurring in a known case of BCC. Extensive literature research has failed to show any such case. Sebaceous carcinoma is a rare high-grade malignant adnexal neoplasm arising from sebaceous glands. Sebaceous carcinomas have been traditionally classified into ocular and extraocular types. The ocular type most commonly occurs on the eyelids, typically originating from the meibomian glands and less commonly from the glands of Zeis. Extraocular sites include most commonly the head and neck. Rare cases have been reported on the vulva, penis, and rarely other locations.[1]

   Case Report Top

A 62-year-old female presented with complaints of ulcerated swelling over the left side of the nose and cheek. The patient had a similar swelling over the Ala of the nose 2 years back. Grossly a globular bit was received measuring 1.5 × 1 × 0.5 cm, and a diagnosis of basal cell carcinoma (nodular BCC) was made [Figure 1]a. All the resected margins of the tumor were involved by the tumor. Two months later, a completion surgery was done in which all resected margins were reported to be uninvolved. The patient was kept under follow-up. After a year, the patient presented with swelling over the nose at the similar site. On examination, an ill-defined swelling measuring roughly 4 × 3 cm was present over the left lateral border of the nose which was extending over the left cheek. The swelling was firm to cystic in consistency. Magnetic resonance imaging (MRI) revealed an enhancing well-defined lobulated soft lesion suggestive of mitotic etiology.
Figure 1: (a) Tumor cells showing basaloid appearance diagnosed as basal cell carcinoma on biopsy (H and E × 20×). (b) Tumor tissue showing both sebaceous and apocrine differentiation with decapitation secretions (H and E × 40×). (c) High power view showing sebaceous differentiation (H and E × 40 ×). (d) Tumor tissue in sheets with areas of necrosis (H and E × 20×)

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An excision biopsy was performed. A skin-covered, suture-oriented soft tissue mass along with bony chips measuring 5.5 × 4 × 2 cm was received. On the cut section, a grey-white growth was noted measuring 1.5 × 1.1 cm. The growth was firm in consistency. Areas of hemorrhage and cystic changes were also noted. Multiple sections examined from the growth revealed tumor tissue composed of nests of cells having scant cytoplasm and hyperchromatic nuclei with cystic spaces containing eosinophilic material and lined by columnar luminal cells [Figure 1]b. The center of the nests showed cells having clear to vacuolated cytoplasm [Figure 1]c. Mitosis was frequent, and necrosis was also noted [Figure 1]d. The tumor was seen infiltrating into the surrounding tissue and cartilage. On immunohistochemistry (IHC), the tumor cells expressed positivity for androgen receptor (AR) and epithelial membrane antigen (EMA), the apocrine areas were positive for CK 7 and CK19 [Figure 2]. The tumor was just short of the medial resected margin and deep resected plane. All the other margins were free of tumor. No typical areas of BCC were noted. A diagnosis of sebaceous carcinoma with apocrine differentiation was rendered.
Figure 2: (a) Tumor cells showing nuclear positivity for androgen receptor (IHC × 40 ×). (b) Tumor cells showing membranous positivity for EMA in sebaceous cells (IHC × 40 ×). (c) Strong immunopositivity for CK 19 in apocrine areas (IHC × 40 ×). (d) Strong and diffuse immunopositivity for CK 7 (IHC × 40 ×)

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   Discussion Top

BCC is a common malignant tumor of the skin and accounts for 75% of nonmelanotic skin tumors.[2] Basal cell carcinoma is a malignant neoplasm derived from abnormal folliculosebaceous-apocrine germinative cells.[3] Studies have been reported in the literature on BCC with apocrine and sebaceous differentiation. An extensive literature search has failed to show SCs arising in BCC. Hereby, we present a rare and the first case of SC arising in a known case of BCC. Sebaceous carcinoma is a very rare malignant tumor primarily found in the area of the eyelid with approximately 25% occurring in the extraocular location.[4] It occurs predominantly in females (the ratio of female to male is 2:1) and is seen at an average age of 65 years.[5] In our study, the case was a 62-year-old female patient. 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.

Certain strains of human papillomavirus have been implicated in the pathogenesis of sebaceous carcinoma.[6] Mutations in the p53 gene have been seen in invasive but not in situ sebaceous carcinoma. Immunohistochemical staining for proliferating cell nuclear antigen and p53 seem to have some prognostic value.

The tumor cells in sebaceous carcinoma are often large and resemble squamous cells or show basaloid differentiation with lipidization of the cells. Therefore, it is important to differentiate them from squamous cell carcinomas with clear cell change and basal cell carcinomas with sebaceous change. The tumor cells in sebaceous carcinoma do not show the basaloid appearance of cells like those of basal cell carcinoma or characteristic keranitinization like squamous cell carcinoma.[7],[8] Also, immunohistochemical markers like EMA and CAM 5.2 can be helpful in distinguishing sebaceous carcinoma from basal cell carcinoma and squamous cell carcinoma.[9] Even AR is helpful in the diagnosis of SCs. EMA and AR are positive in sebaceous carcinoma. The apocrine differentiation is a rare finding and can be 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.[9]

Thus, SCs with apocrine change show aggressive behavior and therefore require an early diagnosis. It is vital also to make the pathologist aware of this rare entity, and a definite diagnosis can be made after all the differentials have been excluded.

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Conflicts of interest

There are no conflicts of interest.

   References Top

Carlson JW, McGlennen RC, Gomez R, Longbella C, Carter J, Carson LF. Sebaceous carcinoma of the vulva: A case report and review of the literature. Gynecol Oncol 1996;60:489-91.  Back to cited text no. 1
Bickers DR, Lim HW, Margolis D, Weinstock MA, Goodman C, Faulkner E, et al. The burden of skin diseases: 2004 a joint project of the American Academy of Dermatology Association and the Society for Investigative Dermatology. J Am Acad Dermatol 2006;55:490-500.  Back to cited text no. 2
Misago N, Mihara I, Ansai S, Narisawa Y. Sebaceoma and related neoplasms with sebaceous differentiation: A clinicopathologic study of 30 cases. Am J Dermatopathol 2002;24:294-304.  Back to cited text no. 3
Natarajan K, Rai R, Pillai S B. Extra ocular sebaceous carcinoma: A rare case report. Indian Dermatol Online J 2011;2:91-3.  Back to cited text no. 4
[PUBMED]  [Full text]  
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.  Back to cited text no. 5
[PUBMED]  [Full text]  
Hayashi N, Furihata M, Ohtsuki Y, Ueno H. Search for accumulation of p53 protein and detection of human papillomavirus genomes in sebaceous gland carcinoma of the eyelid. Virchows Arch 1994;424:503-9.  Back to cited text no. 6
Friedman KJ, Boudreau S, Farmer ER. Superficial epithelioma with sebaceous differentiation. J Cutan Pathol 1987;14:193-7.  Back to cited text no. 7
Kuo T. Clear cell carcinoma of the skin. A variant of the squamous cell carcinoma that simulates sebaceous carcinoma. Am J Surg Pathol 1980;4:573-83.  Back to cited text no. 8
Pinheiro TA, Lopes JM. Periocular sebaceous carcinoma with apocrine differentiation: A case report and review of the literature. Int J Surg Pathol 2019;27:432-6.  Back to cited text no. 9

Correspondence Address:
Shramana Mandal,
Department of Pathology, 642- Pocket E, Mayur Vihar Phase II, New Delhi
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijpm.ijpm_972_21


  [Figure 1], [Figure 2]


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