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CASE REPORT
Year : 2016  |  Volume : 22  |  Issue : 2  |  Page : 127-129  

Unusual presentation of ulcerative postauricular swelling as sebaceous cell carcinoma


Department of General Surgery, Government Medical College, Patiala, Punjab, India

Date of Web Publication24-Aug-2016

Correspondence Address:
Prem Chand
Department of General Surgery, Government Medical College, Patiala - 147 001, Punjab
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/1117-6806.169819

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  Abstract 

Sebaceous glands have high concentration over head and neck region. Despite high concentration, sebaceous cell adenoma and carcinomas are infrequent. Sebaceous cell carcinoma is an uncommon, cutaneous aggressive tumor arising from the sebaceous glands and seen almost exclusively on the eyelids (75%). It accounts for just 0.2-0.7% of all eyelid tumors in the USA and very few cases that have originated in areas other than the eyelids have been reported. A 67-year-old male presented with swelling (3 cm Χ 4 cm), on the right postauricular region, since about 1-month. The swelling became ulcerative and associated with progressive tinnitus and hoarseness of voice. The patient was investigated. Fine-needle aspiration cytology suggested sebaceous cell carcinoma. Then excision biopsy was done, and histopathological examination of excised tissue confirmed the diagnosis. Extraorbital sebaceous cell carcinoma is an aggressive and invasive malignancy. It clinically mimics other diseases and is difficult to diagnose. Hence, an accurate and prompt diagnosis is crucial because of its fulminant course, serious associations with Muir-Torre syndrome and high potential for regional and distant metastasis.

Keywords: Extraocular, sebaceous cell carcinoma, sebaceous gland


How to cite this article:
Chand P, Kumar A, Singh P, Singla L. Unusual presentation of ulcerative postauricular swelling as sebaceous cell carcinoma. Niger J Surg 2016;22:127-9

How to cite this URL:
Chand P, Kumar A, Singh P, Singla L. Unusual presentation of ulcerative postauricular swelling as sebaceous cell carcinoma. Niger J Surg [serial online] 2016 [cited 2019 Sep 19];22:127-9. Available from: http://www.nigerianjsurg.com/text.asp?2016/22/2/127/169819


  Introduction Top


Sebaceous cell carcinoma is an uncommon, cutaneous tumor, arising from the sebaceous glands. It was first well-described by Allaire in 1891. [1] Mostly these tumors have no obvious etiology, and only a few are associated with Muir-Torre syndrome (MTS). Sebaceous gland tumor may crop up anywhere on the body, where these glands exist, including the genitalia. [2] Despite high concentration, true neoplasm, that is, sebaceous cell adenomas and carcinomas are infrequent and very few cases have been reported. [3] This tumor has a fulminant clinical course, with a considerable propensity for local recurrence and distant metastasis. Diagnosis and therapy tend to be delayed because sebaceous carcinoma is frequently mistaken for more common benign entities, further complicating treatment of this aggressive malignancy. [4] In addition to its varied clinical appearance, a varied histologic appearance may occur, and delayed diagnosis or misdiagnosis following a biopsy is not uncommon. [5] The ocular region accounts for nearly 75% of all reported cases, as head and neck region of the body has the greatest density of sebaceous glands and its ectopias. Classically, it occurs in females and the older population at sixth to a seventh decade, and arises in the ocular region from the  Meibomian gland More Details of the tarsal plate and upper eyelid. [6] The parotid gland is most common site outside the ocular region, accounting for about 20% of cases. [7] Overall, it is uncommon tumor with orbital sebaceous carcinoma accounting for only 0.2-0.7% of all eyelid tumors. [8]


  Case Report Top


A 67-year-old male, presented with complaints of swelling, on the right postauricular region [Figure 1], since about 1-month. The swelling increased in size (3 cm × 4 cm) rapidly and developed discharging ulcer over the swelling associated with progressive tinnitus and hoarseness of voice. The lesion was nontender, fixed to overlie skin and underlying tissue with no regional lymph-adenopathy. Ear-nose-throat examination and systemic examination was unremarkable. All investigations of blood and urine were within normal limits. No abnormality was detected on radiological investigations, that is X-ray skull, X-ray chest, and USG whole abdomen.
Figure 1: Postauricular swelling (3 cm × 4 cm)

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Fine needle aspiration cytology suggested sebaceous cell carcinoma and excision biopsy was performed. Cut surface revealed yellowish-grey tumor with an area of necrosis and hemorrhages [Figure 2]. Microscopic examination [Figure 3] and [Figure 4] exhibited lobules of tumor cells separated by fibrovascular stroma. The nuclei showed pleomorphism, hyperchromasia and cytoplasm of the cell were characteristically finely vacuolated to foamy. Some areas showed focal necrosis with the comedo-like pattern [Figure 3], while other areas displayed several mitiotic figures, globules and gland-like structure formation. The distinctive microscopic findings confirmed the diagnosis of sebaceous cell carcinoma, and as the extent of the lesion precluded further surgical treatment, radiotherapy was advised for palliation.
Figure 2: Cut surface of tumour-yellowishgrey with area of necrosis and hemorrhages

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Figure 3: Cells arranged in sheets with finely vacuolated to foamy cytoplasm (H and E stain: ×40)

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Figure 4: Section showing lobules of tumor cells separated by fibrovascular stroma, cytoplasm of the tumor cells within the lobules is finely vacuolated to foamy (H and E stain: ×10)

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  Discussion Top


Sebaceous gland carcinoma is an aggressive, uncommon, cutaneous tumor, may arise anywhere on the body where these glands exist, first well described by Allaire in 1891. [1] It may appear on preexisting dermatosis, such as nevus sebaceous and actinic keratosis or may follow radiation therapy for other diseases. It may also occur in MTS, characterized by the occurrence of sebaceous tumors in association with visceral malignancies. Sebaceous carcinoma is traditionally classified into two groups: Tumor arising from the ocular adenexa, and those arising in extra ocular sites. Extraocular sebaceous carcinoma most commonly involve the head and neck region, the parotid and submandibular glands, the external auditory canal, the trunk and upper extremity, sole, the dorsum of the great toe, and laryngeal or pharyngeal cavities. The sex distribution of extra-orbital sebaceous carcinoma appears to be about equal for male and female patients, and the mean age of occurrence is 63 years. [9]

The disease exhibits such a variety of clinical presentations, that the clinical appearance of extraocular sebaceous carcinoma is not pathognomic, but the lesion may be a pink to the red­yellow nodule. Diagnosis and therapy tend to be delayed because sebaceous carcinoma is frequently mistaken for more common benign entities, further complicating treatment of this aggressive malignancy. [4] In addition to its varied clinical appearance, a varied histologic appearance may occur, and delayed diagnosis or misdiagnosis following a biopsy is not uncommon. [5] Extraocular sebaceous carcinoma, in association with nevus sebaceous, in the postauricular region or in the external auditory canal is very rarely described in the literature. [10] Although extraocular sebaceous carcinoma, compared to orbital sebaceous carcinoma, is generally considered less aggressive, visceral metastasis has been reported. [11] Draining lymph nodes may be involved in few cases. The possibility of MTS must be considered in every case of sebaceous tumor. Criteria for diagnosis of MTS include the presence of at least one sebaceous adenoma, sebaceous epithelioma, or sebaceous carcinoma and at least one visceral cancer. [12]

In our patient, there was neither any regional lymph adenopathy nor any evidence of other internal malignancies as associated with MTS. Metastases have been reported to occur as late as 5 years after the initial diagnosis, lending support to the continual surveillance of patients with sebaceous carcinoma. [9]

Histologically, sebaceous carcinomas are often poorly differentiated neoplasms mainly within the dermis. Multiple lobules of basaloid undifferentiated cells are present within the dermis. In the central portion of lobules, more mature cells are present. Marked nuclear atypia, pleomorphism, and mitosis are common. [13] This neoplasm may be confused with tumors composed of basal cells, squamous cells (mucoepidermoid and spindle cell carcinoma), clear and balloon nevus cells as well as other sebaceous neoplasm. [14]


  Conclusions Top


Sebaceous carcinoma is an uncommon tumor. Extraorbital sebaceous cell carcinoma is an aggressive and invasive malignancy. It clinically mimics other diseases and is difficult to diagnose. Further, correct preoperative diagnosis of sebaceous cell carcinoma is rarely made and its malignant potential is usually underestimated by the surgeon. Hence, an accurate and prompt diagnosis is crucial because of its fulminant course, serious associations with MTS and high potential for regional and distant metastasis.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Kass LG, Hornblass A. Sebaceous carcinoma of the ocular adnexa. Surv Ophthalmol 1989;33:477-90.  Back to cited text no. 1
    
2.
Gomes CC, Lacerda JC, Pimenta FJ, do Carmo MA, Gomez RS. Intraoral sebaceous carcinoma. Eur Arch Otorhinolaryngol 2007;264:829-32.  Back to cited text no. 2
    
3.
Batsakis JG, Littler ER, Leahy MS. Sebaceous cell lesions of the head and neck. Arch Otolaryngol 1972;95:151-7.  Back to cited text no. 3
    
4.
Doxanas MT, Green WR. Sebaceous gland carcinoma. Review of 40 cases. Arch Ophthalmol 1984;102:245-9.  Back to cited text no. 4
    
5.
Song A, Carter KD, Syed NA, Song J, Nerad JA. Sebaceous cell carcinoma of the ocular adnexa: Clinical presentations, histopathology, and outcomes. Ophthal Plast Reconstr Surg 2008;24:194-200.  Back to cited text no. 5
    
6.
Johnson S, Nerad JA, Syed NA. Sebaceous cell carcinoma: A masquerade syndrome. EyeRounds.org January 23, 2007. Available from: http://www.webeye.ophth.uiowa.edu/eyeforum/62-Sebaceous-Cell-Carcinoma-A-Masquerade-Syndrome.htm.[Last cited on 2012 Jan 20].  Back to cited text no. 6
    
7.
Constant E, Leahy MS. Sebaceous cell carcinoma. Plast Reconstr Surg 1968;41:433-7.  Back to cited text no. 7
    
8.
Bhavarajua VM, Shamim SE, Naik VR, Shaari S. Sebaceous cell carcinoma of scalp - A rare presentation. Malays J Med Sci 2007;14:67-70.  Back to cited text no. 8
    
9.
Ghosh SK, Bandyopadhyay D, Gupta S, Chatterjee G, Ghosh A. Rapidly growing extraocular sebaceous carcinoma occurring during pregnancy: A case report. Dermatol Online J 2008;14:8.  Back to cited text no. 9
    
10.
Rinaggio J, McGuff HS, Otto R, Hickson C. Postauricular sebaceous carcinoma arising in association with nevus sebaceus. Head Neck 2002;24:212-6.  Back to cited text no. 10
    
11.
Jensen ML. Extraocular sebaceous carcinoma of the skin with visceral metastases: Case report. J Cutan Pathol 1990;17:117-21.  Back to cited text no. 11
    
12.
Schwartz RA, Torre DP. The muir-torre syndrome: A 25-year retrospect. J Am Acad Dermatol 1995;33:90-104.  Back to cited text no. 12
    
13.
Akkari D, Naraghi Z, Emadi N, Ghaninejad H. Extraocular sebaceous carcinoma arising from nevus sebaceous. Acta Med Iran 2005;43:227-9.  Back to cited text no. 13
    
14.
Kim SY, Chi JG, Lee YS. Extraocular sebaceous carcinoma - A case report. J Med 1987;28:47-50.  Back to cited text no. 14
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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