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Sebaceous carcinoma

Patricia Tai, MB, BS, DABR, FRCR, FRCPC
Section Editors
Robert S Stern, MD
June K Robinson, MD
Deputy Editor
Rosamaria Corona, MD, DSc


Sebaceous carcinoma is a rare malignant tumor of the sebaceous glands [1]. It can occur in any body site where sebaceous glands are present, but is most commonly found in the head and neck region, particularly in the periocular area [2]. Sebaceous carcinoma of the eyelid (picture 1) may be mistaken for inflammatory lesions, such as chalazion or blepharoconjunctivitis, resulting in a delayed diagnosis and poorer prognosis [3].

Sebaceous carcinomas can occur sporadically or may be associated with Muir-Torre syndrome, a subset of the hereditary nonpolyposis colorectal cancer syndrome (HNCCS, Lynch syndrome) characterized by single or multiple sebaceous neoplasms, keratoacanthomas, and internal malignancy [4].

This topic will review the pathogenesis, clinical presentation, diagnosis, and treatment of sebaceous carcinoma. Muir-Torre syndrome and Lynch syndrome are discussed separately. (See "Muir-Torre syndrome" and "Lynch syndrome (hereditary nonpolyposis colorectal cancer): Clinical manifestations and diagnosis" and "Lynch syndrome (hereditary nonpolyposis colorectal cancer): Screening and management".)


Sebaceous carcinoma is a rare tumor, with an estimated incidence rate of approximately 1 to 2 per 1,000,000 per year [2,4,5]. However, sebaceous carcinoma is the most common eyelid malignancy after basal cell carcinoma and squamous cell carcinoma [6,7].

A review of the Surveillance, Epidemiology and End Results (SEER) database from 1973 to 2004 identified 1349 cases of sebaceous carcinoma [2]. More than 90 percent of cases were seen in patients over 50 years old and the median age was 72 years. Sebaceous carcinoma is exceedingly rare in children [8,9].


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Literature review current through: Sep 2016. | This topic last updated: Oct 3, 2016.
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  1. Kyllo RL, Brady KL, Hurst EA. Sebaceous carcinoma: review of the literature. Dermatol Surg 2015; 41:1.
  2. Dasgupta T, Wilson LD, Yu JB. A retrospective review of 1349 cases of sebaceous carcinoma. Cancer 2009; 115:158.
  3. Muqit MM, Foot B, Walters SJ, et al. Observational prospective cohort study of patients with newly-diagnosed ocular sebaceous carcinoma. Br J Ophthalmol 2013; 97:47.
  4. Dores GM, Curtis RE, Toro JR, et al. Incidence of cutaneous sebaceous carcinoma and risk of associated neoplasms: insight into Muir-Torre syndrome. Cancer 2008; 113:3372.
  5. Kuzel P, Metelitsa AI, Dover DC, Salopek TG. Epidemiology of sebaceous carcinoma in Alberta, Canada, from 1988 to 2007. J Cutan Med Surg 2012; 16:417.
  6. Jakobiec FA, Mendoza PR. Eyelid sebaceous carcinoma: clinicopathologic and multiparametric immunohistochemical analysis that includes adipophilin. Am J Ophthalmol 2014; 157:186.
  7. Deprez M, Uffer S. Clinicopathological features of eyelid skin tumors. A retrospective study of 5504 cases and review of literature. Am J Dermatopathol 2009; 31:256.
  8. Mebazaa A, Boussofara L, Trabelsi A, et al. Undifferentiated sebaceous carcinoma: an unusual childhood cancer. Pediatr Dermatol 2007; 24:501.
  9. Omura NE, Collison DW, Perry AE, Myers LM. Sebaceous carcinoma in children. J Am Acad Dermatol 2002; 47:950.
  10. Izumi M, Tang X, Chiu CS, et al. Ten cases of sebaceous carcinoma arising in nevus sebaceus. J Dermatol 2008; 35:704.
  11. Ishida M, Iwai M, Yoshida K, et al. Sebaceous carcinoma associated with Bowen's disease: a case report with emphasis on the pathogenesis of sebaceous carcinoma. Int J Clin Exp Pathol 2013; 6:3029.
  12. Ishida M, Okabe H. Intraepidermal sebaceous carcinoma occurring concurrently with actinic keratosis. J Cutan Pathol 2012; 39:731.
  13. Nakashima K, Adachi K, Yamasaki A, et al. Sebaceous carcinoma with actinic keratosis. Acta Derm Venereol 2010; 90:196.
  14. Kraft S, Granter SR. Molecular pathology of skin neoplasms of the head and neck. Arch Pathol Lab Med 2014; 138:759.
  15. Harwood CA, Swale VJ, Bataille VA, et al. An association between sebaceous carcinoma and microsatellite instability in immunosuppressed organ transplant recipients. J Invest Dermatol 2001; 116:246.
  16. Landis MN, Davis CL, Bellus GA, Wolverton SE. Immunosuppression and sebaceous tumors: a confirmed diagnosis of Muir-Torre syndrome unmasked by immunosuppressive therapy. J Am Acad Dermatol 2011; 65:1054.
  17. Rajan Kd A, Burris C, Iliff N, et al. DNA mismatch repair defects and microsatellite instability status in periocular sebaceous carcinoma. Am J Ophthalmol 2014; 157:640.
  18. Shalin SC, Sakharpe A, Lyle S, et al. p53 staining correlates with tumor type and location in sebaceous neoplasms. Am J Dermatopathol 2012; 34:129.
  19. Hussain RM, Matthews JL, Dubovy SR, et al. UV-independent p53 mutations in sebaceous carcinoma of the eyelid. Ophthal Plast Reconstr Surg 2014; 30:392.
  20. Jayaraj P, Sen S, Sharma A, et al. Eyelid sebaceous carcinoma: a novel mutation in lymphoid enhancer-binding factor-1. Br J Dermatol 2015; 173:811.
  21. Liau JY, Liao SL, Hsiao CH, et al. Hypermethylation of the CDKN2A gene promoter is a frequent epigenetic change in periocular sebaceous carcinoma and is associated with younger patient age. Hum Pathol 2014; 45:533.
  22. Shields JA, Demirci H, Marr BP, et al. Sebaceous carcinoma of the eyelids: personal experience with 60 cases. Ophthalmology 2004; 111:2151.
  23. Shields JA, Demirci H, Marr BP, et al. Conjunctival epithelial involvement by eyelid sebaceous carcinoma. The 2003 J. Howard Stokes lecture. Ophthal Plast Reconstr Surg 2005; 21:92.
  24. Kumabe A, Kawase T, Miura K, et al. Sebaceous carcinoma of the parotid gland: F-18 FDG PET/CT findings. Clin Nucl Med 2010; 35:260.
  25. Gnepp DR. My journey into the world of salivary gland sebaceous neoplasms. Head Neck Pathol 2012; 6:101.
  26. Sawyer AR, McGoldrick RB, Mackey S, et al. Should extraocular sebaceous carcinoma be investigated using sentinel node biopsy? Dermatol Surg 2009; 35:704.
  27. Husain A, Blumenschein G, Esmaeli B. Treatment and outcomes for metastatic sebaceous cell carcinoma of the eyelid. Int J Dermatol 2008; 47:276.
  28. Ponti G, Losi L, Di Gregorio C, et al. Identification of Muir-Torre syndrome among patients with sebaceous tumors and keratoacanthomas: role of clinical features, microsatellite instability, and immunohistochemistry. Cancer 2005; 103:1018.
  29. Lazar AJ, Lyle S, Calonje E. Sebaceous neoplasia and Torre-Muir syndrome. Curr Diagn Pathol 2007; 13:301.
  30. Mulay K, White VA, Shah SJ, Honavar SG. Sebaceous carcinoma: clinicopathologic features and diagnostic role of immunohistochemistry (including androgen receptor). Can J Ophthalmol 2014; 49:326.
  31. Ansai S, Takeichi H, Arase S, et al. Sebaceous carcinoma: an immunohistochemical reappraisal. Am J Dermatopathol 2011; 33:579.
  32. Abbas O, Mahalingam M. Cutaneous sebaceous neoplasms as markers of Muir-Torre syndrome: a diagnostic algorithm. J Cutan Pathol 2009; 36:613.
  33. Shalin SC, Lyle S, Calonje E, Lazar AJ. Sebaceous neoplasia and the Muir-Torre syndrome: important connections with clinical implications. Histopathology 2010; 56:133.
  34. Edge SF, Byrd DR, Compton CC, et al. AJCC cancer staging manual, 7th ed, Springer, New York 2010.
  35. http://www.cancer.net/cancer-types/eyelid-cancer/stages-and-grades (Accessed on December 12, 2014).
  36. Hou JL, Killian JM, Baum CL, et al. Characteristics of sebaceous carcinoma and early outcomes of treatment using Mohs micrographic surgery versus wide local excision: an update of the Mayo Clinic experience over the past 2 decades. Dermatol Surg 2014; 40:241.
  37. Callahan EF, Appert DL, Roenigk RK, Bartley GB. Sebaceous carcinoma of the eyelid: a review of 14 cases. Dermatol Surg 2004; 30:1164.
  38. Snow SN, Larson PO, Lucarelli MJ, et al. Sebaceous carcinoma of the eyelids treated by mohs micrographic surgery: report of nine cases with review of the literature. Dermatol Surg 2002; 28:623.
  39. Spencer JM, Nossa R, Tse DT, Sequeira M. Sebaceous carcinoma of the eyelid treated with Mohs micrographic surgery. J Am Acad Dermatol 2001; 44:1004.
  40. Azad S, Choudhary V. Treatment results of high dose rate interstitial brachytherapy in carcinoma of eye lid. J Cancer Res Ther 2011; 7:157.
  41. Hata M, Koike I, Maegawa J, et al. Radiation therapy for primary carcinoma of the eyelid: tumor control and visual function. Strahlenther Onkol 2012; 188:1102.
  42. Pardo FS, Wang CC, Albert D, Stracher MA. Sebaceous carcinoma of the ocular adnexa: radiotherapeutic management. Int J Radiat Oncol Biol Phys 1989; 17:643.
  43. Lisman RD, Jakobiec FA, Small P. Sebaceous carcinoma of the eyelids. The role of adjunctive cryotherapy in the management of conjunctival pagetoid spread. Ophthalmology 1989; 96:1021.
  44. Russell HC, Chadha V, Lockington D, Kemp EG. Topical mitomycin C chemotherapy in the management of ocular surface neoplasia: a 10-year review of treatment outcomes and complications. Br J Ophthalmol 2010; 94:1316.
  45. Tumuluri K, Kourt G, Martin P. Mitomycin C in sebaceous gland carcinoma with pagetoid spread. Br J Ophthalmol 2004; 88:718.
  46. Shields CL, Naseripour M, Shields JA, Eagle RC Jr. Topical mitomycin-C for pagetoid invasion of the conjunctiva by eyelid sebaceous gland carcinoma. Ophthalmology 2002; 109:2129.
  47. Khong JJ, Muecke J. Complications of mitomycin C therapy in 100 eyes with ocular surface neoplasia. Br J Ophthalmol 2006; 90:819.
  48. Deo SV, Shukla NK, Singh M, et al. Locally advanced sebaceous cell carcinoma (T3) of eyelid: incidence and pattern of nodal metastases and combined modality management approach. Orbit 2012; 31:150.
  49. Jung YH, Woo IS, Kim MY, et al. Palliative 5-fluorouracil and cisplatin chemotherapy in recurrent metastatic sebaceous carcinoma: Case report and literature review. Asia Pac J Clin Oncol 2016; 12:e189.
  50. Orcurto A, Gay BE, Sozzi WJ, et al. Long-Term Remission of an Aggressive Sebaceous Carcinoma following Chemotherapy. Case Rep Dermatol 2014; 6:80.
  51. Pfeiffer ML, Yin VT, Myers J, Esmaeli B. Regional nodal recurrence of sebaceous carcinoma of the caruncle 11 years after primary tumor resection. JAMA Ophthalmol 2013; 131:1091.
  52. Rath S, Honavar SG, Reddy VA, et al. Sebaceous carcinoma of the eyelid metastasizing to the lacrimal sac after 5 years. Orbit 2009; 28:309.
  53. Murthy R, Honavar SG, Burman S, et al. Neoadjuvant chemotherapy in the management of sebaceous gland carcinoma of the eyelid with regional lymph node metastasis. Ophthal Plast Reconstr Surg 2005; 21:307.
  54. Priyadarshini O, Biswas G, Biswas S, et al. Neoadjuvant chemotherapy in recurrent sebaceous carcinoma of eyelid with orbital invasion and regional lymphadenopathy. Ophthal Plast Reconstr Surg 2010; 26:366.
  55. Eisen DB, Michael DJ. Sebaceous lesions and their associated syndromes: part I. J Am Acad Dermatol 2009; 61:549.
  56. Watanabe A, Sun MT, Pirbhai A, et al. Sebaceous carcinoma in Japanese patients: clinical presentation, staging and outcomes. Br J Ophthalmol 2013; 97:1459.
  57. Thomas WW, Fritsch VA, Lentsch EJ. Population-based analysis of prognostic indicators in sebaceous carcinoma of the head and neck. Laryngoscope 2013; 123:2165.