Sebaceous Carcinoma: a Rare Presentation in a Child

Title: Sebaceous Carcinoma: A Rare Presentation in a Child

Authors: Seyedmehdi Jadali, MD, David D. Zabel , MD, and Katrina

Conard, MD

Sebaceous carcinoma is a rare malignant tumor arising from the epithelium of sebaceous glands (1). This type of tumor is usually located peri-ocularly.

Sebaceous carcinoma is very unusual in the pediatric population. To our knowledge, this is the third case of extra-ocular sebaceous carcinoma in a child.

CASE REPORT:A 6-year-old boy presented with a history of a lesion over the medial aspect of his left elbow that had increased in size over the past year. The lesion was red, raised, and non-ulcerative. Because of the abnormal growth and appearance of this lesion, an excisional biopsy was performed. (Fig 1 &2)

Tumor cells were positive for Kerlix, EMA, Ber-Ep4, and Vimentin. S-100 protein was also positive. Sebaceous differentiation areas appeared positive with CEA staining.

The patient was referred to a plastic surgeon for re-excision of the area. The scar was removed with 1-cm margin and the defect was covered with full thickness skin graft. The patient recovered uneventfully. Pathology report on this specimen was scar tissue without residual tumor.

A pediatric oncologist is following the patient, and thus far, no other primary tumor has been identified.

DISCUSSION:Extra-ocular sebaceous carcinoma is an extremely rare tumor of the skin. Because of the diverse clinical presentation, diagnosis of sebaceous carcinoma is often delayed for months or years.

There is not a pathognomonic presentation for sebaceous carcinoma (1). Lesions can be pink to dark red, raised or flat, ulcerative, pedunculated, or sessile. Diagnosis is even more complicated by lack of a uniform pathologic terminology (7). There is almost always a delay in diagnosis for this reason, with reported delays ranging from 6 months to several years after presentation (2,3).

Sebaceous carcinoma is locally aggressive and can have regional or distant metastases (2). Only 2 cases of metastatic sebaceous carcinoma have been reported in the literature, but in general, 15% of extra-ocular sebaceous carcinomas have lymph node involvement and 10% are associated with distant metastases (2).

Sebaceous carcinoma can be associated with Muir-Torre syndrome, an autosomal dominant disease with sebaceous adenoma, epithelioma, or carcinoma along with one or more low-grade visceral malignancy (6,8). The most common visceral malignancy is colorectal cancer, but Muir-Torre syndrome has never been reported in children.

Immunohistochemical studies could also help with distinguishing biopsies of sebaceomas from carcinoma. Expression of p53, Ki67, and p21 occurs in topologically distinct compartments in normal sebaceous glands. The proliferative compartment in sebaceous adenoma and sebaceous hyperplasia is expanded but remains in the periphery of the gland. This contrasts greatly with sebaceous carcinoma, in which there is complete loss of compartmentalization, suggesting loss of topological control of mediators of the cell cycle (11).

Histologically, sebaceous carcinoma is in the dermis and nonencapsulated (12). Based on growth pattern, Rao and coworkers divided sebaceous carcinoma into lobular, comedo-carcinoma, papillary, and mixed types (12). There are also classifications based on degree of differentiation (10).

Sebaceous carcinoma has bubbly cells with abundant, vacuolated cytoplasm, associated with cellular features of malignancy such as nuclear pleomorphism, hyperchromasia, and infiltration (1,3). Special staining for lipid can differentiate sebaceous carcinoma from other lesions with little or no sebaceous components.

Treatment recommendations of sebaceous carcinoma is wide excision with a significant margin (5 to 20 mm). The patient might need a local or distant flap or a skin graft to cover the area (3,6).

Mohs microscopic surgery has been used for peri-ocular sebaceous carcinomas, but no long-term follow-up is available to compare the results of this kind of treatment with those from traditional surgical procedures (13). Two practical factors are responsible for failure of Mohs technique: multicentricity and pagetoid pathology.

Radiotherapy has been used for treatment. Radiation is a good modality for palliation or for treatment of metastases, especially in tumors with parotid involvement.

Regional and distant metastases are predictive of a poor prognosis (15,16). Size of the tumor and pagetoid spread are also considered important factors in prognosis (12). In general, extra-ocular sebaceous carcinoma has better prognosis (2,17,18). Although the number of cases is so small that the conclusion might be biased (18).

CONCLUSION:This unique case is the third case of extra-ocular sebaceous carcinoma reported in a child. The patient had no family history and negative evaluation for Muir-Torre syndrome and did not have regional or distant involvement. Treatment consisted of resection of the scar with a margin of at least 5 mm and local flap coverage.

This presentation reiterated the need for early biopsy of any unusual skin lesion

especially if changes in size or color are present. Physicians need to be aware of the unusual presentation like this kind to prevent delay in diagnosis and insure timely and safe management, which can make a difference in the patient’s prognosis.

REFERENCES:

1. Omura,N.E.,Collison, D.W.,Perry,A.E.,Myers,L.M.. Sebaceous carcinoma in children. J Am Acad Dermatol. 47(6):950-3, 2002

2. Nelson,B.R., Hamlet,K.R.,Gillard,M, Railan,D, Johnson,T.M..Sebaceous carcinoma. J Am Acad Dermatol. 33:1-15, 1995

3. Pricolo,V.E., Jesus,M.D., Rodil,V., Vezeridis, M.P.. Extra-orbital Sebaceous carcinoma. Arch Surgery.120:853-5,1985

4. Cramer,S.F.,Gnepp,D.R.,kiehn,C.L.. Sebaceous differentiation in adenoid cystic carcinoma of the parotid gland. Cancer.46:1405-10, 1980

5. Martinez-Madrigal,F,Casiraghi,O.Khattech,A. Hypopharyngeal sebaceous carcinoma: a case report. Human pathology.22:929-31, 1991

6. Giorgi,D.E., Brunasso,M.D., Mannone,G.,Soyer,F, Carli,H.P. Sebaceous carcinoma arising from nevus sebaceous: a case report. Dermatol surg. 29(1):105-7, 2003

7. Brownstein,M.H., Shapiro,L. The pilosebaceous tumors. Dermatology.16:340-52, 1977

8. Eschie,C.,Kruse,R.,Lamberti,C.,Friedl,W.,Propping,P.Lehmann,P.,Ruzicka.T. Muir- Torre syndrome: Clinical features and molecular genetic analysis. Brit J of Dermatol. 136(6): 913-17, 1997

9. Lemos,L.B.,Santa Cruz,D.J.Baba, N..Sebaceous carcinoma of the eyelid following radiation therapy. Am J Surg Pathol, 2:305-11, 1978

10.Sebaceous carcinoma of the eyelid: error in clinical and pathologic diagnosis. Am J Surg Pathol. 8:597-606, 1984

11. McBride,S.R.,Leonard,N. Reynold,N.J. Loss of p21waf1 compartmentalisation in sebaceous carcinoma compared with sebaceous hyperplasi and sebaceous adenoma. J Clin Pathol. 55(10):763-66. 2002

13. Dzubow,L.M. Sebaceous carcinoma of the eyelid: treatment with Mohs Surgery. J Dematol Surg Oncol, 11:40-4, 1985

14. Pardo,F.S.,Wang,C.C.,Albert,D. Sebaceous carcinoma of the ocular adnexa: radiotherapeutic management. Int J Radiat Oncol Biol Phys. 17:643-7, 1989

15. King,D.T.,Hirose,F.M.,Gurevtich,A.W. Sebaceous carcinoma of the skin with visceral metastases. Arch Dermatol, 115:862-63, 1979

16. Hernandez-Perez,E. Banos,E. Sebaceous carcinoma: Report of two cases with metastases. Dematology. 156:184-88, 1978

17. Rao,NA.Hidayat,A.A.McLean,J.W. Sebaceous carcinomas of the ocular adnexa: a clinicopathologic study of 104 patients with five year follow-up data. Hum Pathol, 13:113-22, 1982

18. Jensen,M.L. Extra-ocular sebaceous carcinoma of the skin with visceral metastases: case report. J Cutan Pathol, 17:117-21, 1990