Correspondence
Recurrent sebaceous carcinoma of the scalp in a young male treated with adjuvant radiotherapy ABSTRACT Sebaceous carcinoma is a rare malignancy of the skin appendages. It tends to occur in elderly patients. Orbital region is the most commonly involved site seen in 75% of cases. The involvement of extra-orbital sites is infrequent. Herein, a case of extra-ocular sebaceous carcinoma arising in the scalp of a 20-year-old man is described.The patient developed tumor relapse after excision biopsy. He was treated with wide local excision of the tumor. However, the patient developed local recurrence after an interval of four months for which he again underwent wide local excision. He did not manifest any regional or distant metastases. In view of the locally aggressive tumor, he received adjuvant radiation therapy. The patient was successfully treated with no evidence of any local recurrence seen after a follow-up period of one year. The timely recognition of sebaceous carcinoma is imperative so as to execute the primary treatment i.e., wide local excision. Adjuvant radiotherapy may be considered to improve the clinical outcome for recurrent tumors. KEY WORDS: Adjuvant radiotherapy, recurrence, scalp, sebaceous carcinoma, sebaceous gland neoplasms
INTRODUCTION Sebaceous carcinoma is a rare malignancy of the skin appendages. It tends to occur in elderly patients. Orbital region is the most commonly involved site seen in 75% of cases. The involvement of extra-orbital sites is infrequent. Herein, a case of extra-ocular sebaceous carcinoma arising in the scalp of a young man is described. The clinical behavior, pathological details, and the management strategy are discussed.
infiltrated by the tumor with involvement of the lateral and deep resected margins. Based on the aforementioned findings, a pathological diagnosis of malignant skin appendageal tumor was made.
Puja Sahai, Karuna Singh, Aman Sharma, Ranajoy Ghosh1, Prerna Nembang2, Chitra Sarkar1, Bidhu Kalyan Mohanti Departments of Radiation Oncology, 1 Pathology, All India Institute of Medical Sciences, 2Department of Plastic Surgery, Safdarjang Hospital, New Delhi, India For correspondence: Dr. Puja Sahai, Department of Radiation Oncology, Dr. B. R. Ambedkar Institute Rotary Cancer Hospital, All India Institute of Medical Sciences, Ansari Nagar, New Delhi - 110 029, India. E-mail: [email protected]
A 20-year-old man presented in the Dermatology clinic with a 7-month history of swelling on scalp. An incisional biopsy of skin was performed, which revealed a proliferating trichilemmal tumor. Five months later, he again noticed a swelling at the same site. On physical examination, a nodular swelling on scalp measuring 3 × 3 cm was seen without any associated regional lymphadenopathy. An excision biopsy of the swelling was performed. Histopathological examination of the specimen demonstrated a poorly circumscribed tumor in the dermis composed of clear looking cells and few polyhedral cells arranged in trabeculae, nests, and cords in a hyalinized stroma. The tumor cells showed mitotic activity and central areas of necrosis. The skin adnexal structure was seen
Subsequently, he developed a swelling in the tissue defect area after remaining asymptomatic for four months. On examination, there was a nodular swelling of 3 × 3 cm dimension on the scalp overlying the left parietal region. The surface of the swelling was irregular and the margins were well-defined. He was subjected to radiological investigations for evaluation. There was no evidence of any lytic or sclerotic lesion on the skull X-ray film. Magnetic resonance imaging (MRI) of the brain revealed a small focal area of altered signal within the scalp in left posterior parietal region. The region appeared hypointense on T1-weighted and fluid attenuated inversion recovery (FLAIR) images. It showed mild focal heterogeneous enhancement on postcontrast images. The underlying bone showed normal cortical outline and marrow signal. The patient was then referred to the Plastic Surgery clinic for evaluation. A wide local excision of the scalp swelling with local template flap was performed. The histopathological examination of the resected specimen revealed features of a malignant skin appendageal tumor of low grade. The resected margins and the deep resected plane were free of tumor.
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Journal of Cancer Research and Therapeutics - October-December 2013 - Volume 9 - Issue 4
CASE REPORT
Access this article online Website: www.cancerjournal.net DOI: 10.4103/0973-1482.126472 PMID: 24518729 Quick Response Code:
Sahai, et al.: Sebaceous carcinoma of scalp
Figure 1: Axial section of CECT head shows an enhancing soft tissue mass lesion in scalp overlying the occipital region
The patient was advised to visit at regular follow-up intervals. Four months later, he developed a swelling on the scalp overlying the left occipital region. The swelling was of 5 × 4 cm dimension with involvement of skin. Contrast enhanced computed tomography (CECT) of head, neck, chest, and abdomen was performed. The scan revealed multiple soft tissue mass lesions in the scalp overlying the left occipital and parietal area [Figure 1]. There was no abnormality seen in the scanned images of brain, neck, chest, and abdomen. A wide local excision of the scalp swelling with rotation advancement flap was performed. Histopathological examination of the resected specimen demonstrated a tumor of 3.2 × 2.8 × 2.5 cm dimension. On immumohistochemistry, the cells showed positivity for cytokeratin and focal positivity for epithelial membrane antigen (EMA). The microscopic features demonstrated a malignant skin appendageal tumor compatible with sebaceous carcinoma [Figure 2]. The resected margins were free of tumor. With the intent of decreasing the chances of local recurrence, adjuvant radiotherapy was planned for the patient. Consequently, a dose of 50 Gy in 20 fractions over 4 weeks was delivered using electron beam therapy. He completed the course of radiation therapy with associated grade 2 cutaneous morbidity. Currently, he has completed a follow-up period of one year after therapy and has no evidence of disease. DISCUSSION Sebaceous carcinoma is a disease of the sixth and seventh decade of life.[1] The most frequent extra-orbital site for this tumor is skin in the head and neck region wherein sebaceous glands are most abundant.[1] It has been associated with Muir– Torre syndrome. The Muir–Torre syndrome[2,3] is an autosomal dominant genodermatosis consisting of sebaceous neoplasms viz. sebaceous adenoma, sebaceous carcinoma, or sebaceous epithelioma with or without keratoacanthomas associated with one or more visceral malignancies. The visceral malignancies associated with the Muir–Torre syndrome are often of low-grade.
Figure 2: Photomicrograph shows (a) islands of raggedly infiltrating tumor cells in dermis (H and E stain, ×40), (b) tumor islands with areas of central necrosis (H and E stain, ×40), (c) cells with large bizarre nuclei and prominent nucleolus (H and E stain, ×100), (d) cells with finely vacuolated cytoplasm (H and E stain, ×200), (e) epithelial membrane antigen positive immunostaining (×200), (f) cytokeratin positive immunostaining (×100)
Sebaceous carcinoma presents as a firm and painless slowgrowing mass. The main histological criterion is sebaceous differentiation. The cells show nuclear pleomorphism, lobular architecture and foamy vacuolization of the cytoplasm.[4] The primary treatment for sebaceous carcinoma is excision with wide margins. In a review of 91 patients with extra-ocular sebaceous carcinoma, Bailet et al.[4] reported local recurrence rates of 29%, regional nodal metastasis in 15%, and a disease-related mortality of 20%. Adverse prognostic indicators that have been postulated include poor differentiation, infiltrative growth pattern, multicentric origin of the tumor, delay in diagnosis of over 6 months, and size of the tumor more than 10 mm. Samarasinghe et al.[5] reported a case of sebaceous carcinoma of scalp with nodal metastasis at presentation. With regard to the patient concerning this case study, the disease presentation was in the third decade, which differs from the usual presentation in elderly patients. He did not have a family history of malignancy or associated visceral malignancy. The patient developed tumor relapse after excision biopsy. He was treated with wide local excision of the tumor. However, the patient developed local recurrence after an interval of four months for which he again underwent wide local excision. He did not manifest any regional or distant metastasis. The present case highlights the locally aggressive nature of this tumor. The role of adjuvant radiotherapy is not defined for sebaceous carcinoma. However, it was incorporated in the management of this patient considering the clinical presentation. The patient was successfully treated with no evidence of any local recurrence seen after a follow-up period of one year. The presentation of sebaceous carcinoma may precede the manifestation of visceral malignancy by many
Journal of Cancer Research and Therapeutics - October-December 2013 - Volume 9 - Issue 4
731
Sahai, et al.: Sebaceous carcinoma of scalp
years. Therefore, these patients require life-long surveillance for malignancy. Sebaceous carcinoma needs to be considered as differential diagnosis for the cutaneous malignancies in all age groups. The timely recognition enables execution of the primary treatment i.e., wide local excision. Adjuvant radiotherapy may be considered to improve the clinical outcome of recurrent tumors. REFERENCES 1.
732
Pickford MA, Hogg FJ, Fallowfield ME, Webster MH. Sebaceous carcinoma of the periorbital and extraorbital regions. Br J Plast Surg 1995;48:93-6.
2. 3. 4. 5.
Cohen PR, Kohn SR, Davis DA, Kurzrock R. Muir-Torre syndrome. Dermatol Clin 1995;13:79-89. Schwartz RA, Torre DP. Muir-Torre syndrome: A 25-year retrospect. J Am Acad Dermatol 1995;33:90-104. Bailet JW, Zimmerman MC, Arnstein DP, Wollman JS, Mickel RA. Sebaceous carcinoma of the head and neck. Case report and literature review. Arch Otolaryngol Head Neck Surg 1992;118:1245-9. Samarasinghe V, Marsden J, Roberts C. Sebaceous carcinoma of the scalp presenting with nodal metastasis. J Plast Reconstr Aesthet Surg 2010;63:2193-4.
Cite this article as: Sahai P, Singh K, Sharma A, Ghosh R, Nembang P, Sarkar C, et al. Recurrent sebaceous carcinoma of the scalp in a young male treated with adjuvant radiotherapy. J Can Res Ther 2013;9:730-2. Source of Support: Nil, Conflict of Interest: None declared.
Journal of Cancer Research and Therapeutics - October-December 2013 - Volume 9 - Issue 4
Copyright of Journal of Cancer Research & Therapeutics is the property of Medknow Publications & Media Pvt. Ltd. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
Recurrent sebaceous carcinoma of the scalp in a young male treated with adjuvant radiotherapy ABSTRACT Sebaceous carcinoma is a rare malignancy of the skin appendages. It tends to occur in elderly patients. Orbital region is the most commonly involved site seen in 75% of cases. The involvement of extra-orbital sites is infrequent. Herein, a case of extra-ocular sebaceous carcinoma arising in the scalp of a 20-year-old man is described.The patient developed tumor relapse after excision biopsy. He was treated with wide local excision of the tumor. However, the patient developed local recurrence after an interval of four months for which he again underwent wide local excision. He did not manifest any regional or distant metastases. In view of the locally aggressive tumor, he received adjuvant radiation therapy. The patient was successfully treated with no evidence of any local recurrence seen after a follow-up period of one year. The timely recognition of sebaceous carcinoma is imperative so as to execute the primary treatment i.e., wide local excision. Adjuvant radiotherapy may be considered to improve the clinical outcome for recurrent tumors. KEY WORDS: Adjuvant radiotherapy, recurrence, scalp, sebaceous carcinoma, sebaceous gland neoplasms
INTRODUCTION Sebaceous carcinoma is a rare malignancy of the skin appendages. It tends to occur in elderly patients. Orbital region is the most commonly involved site seen in 75% of cases. The involvement of extra-orbital sites is infrequent. Herein, a case of extra-ocular sebaceous carcinoma arising in the scalp of a young man is described. The clinical behavior, pathological details, and the management strategy are discussed.
infiltrated by the tumor with involvement of the lateral and deep resected margins. Based on the aforementioned findings, a pathological diagnosis of malignant skin appendageal tumor was made.
Puja Sahai, Karuna Singh, Aman Sharma, Ranajoy Ghosh1, Prerna Nembang2, Chitra Sarkar1, Bidhu Kalyan Mohanti Departments of Radiation Oncology, 1 Pathology, All India Institute of Medical Sciences, 2Department of Plastic Surgery, Safdarjang Hospital, New Delhi, India For correspondence: Dr. Puja Sahai, Department of Radiation Oncology, Dr. B. R. Ambedkar Institute Rotary Cancer Hospital, All India Institute of Medical Sciences, Ansari Nagar, New Delhi - 110 029, India. E-mail: [email protected]
A 20-year-old man presented in the Dermatology clinic with a 7-month history of swelling on scalp. An incisional biopsy of skin was performed, which revealed a proliferating trichilemmal tumor. Five months later, he again noticed a swelling at the same site. On physical examination, a nodular swelling on scalp measuring 3 × 3 cm was seen without any associated regional lymphadenopathy. An excision biopsy of the swelling was performed. Histopathological examination of the specimen demonstrated a poorly circumscribed tumor in the dermis composed of clear looking cells and few polyhedral cells arranged in trabeculae, nests, and cords in a hyalinized stroma. The tumor cells showed mitotic activity and central areas of necrosis. The skin adnexal structure was seen
Subsequently, he developed a swelling in the tissue defect area after remaining asymptomatic for four months. On examination, there was a nodular swelling of 3 × 3 cm dimension on the scalp overlying the left parietal region. The surface of the swelling was irregular and the margins were well-defined. He was subjected to radiological investigations for evaluation. There was no evidence of any lytic or sclerotic lesion on the skull X-ray film. Magnetic resonance imaging (MRI) of the brain revealed a small focal area of altered signal within the scalp in left posterior parietal region. The region appeared hypointense on T1-weighted and fluid attenuated inversion recovery (FLAIR) images. It showed mild focal heterogeneous enhancement on postcontrast images. The underlying bone showed normal cortical outline and marrow signal. The patient was then referred to the Plastic Surgery clinic for evaluation. A wide local excision of the scalp swelling with local template flap was performed. The histopathological examination of the resected specimen revealed features of a malignant skin appendageal tumor of low grade. The resected margins and the deep resected plane were free of tumor.
730
Journal of Cancer Research and Therapeutics - October-December 2013 - Volume 9 - Issue 4
CASE REPORT
Access this article online Website: www.cancerjournal.net DOI: 10.4103/0973-1482.126472 PMID: 24518729 Quick Response Code:
Sahai, et al.: Sebaceous carcinoma of scalp
Figure 1: Axial section of CECT head shows an enhancing soft tissue mass lesion in scalp overlying the occipital region
The patient was advised to visit at regular follow-up intervals. Four months later, he developed a swelling on the scalp overlying the left occipital region. The swelling was of 5 × 4 cm dimension with involvement of skin. Contrast enhanced computed tomography (CECT) of head, neck, chest, and abdomen was performed. The scan revealed multiple soft tissue mass lesions in the scalp overlying the left occipital and parietal area [Figure 1]. There was no abnormality seen in the scanned images of brain, neck, chest, and abdomen. A wide local excision of the scalp swelling with rotation advancement flap was performed. Histopathological examination of the resected specimen demonstrated a tumor of 3.2 × 2.8 × 2.5 cm dimension. On immumohistochemistry, the cells showed positivity for cytokeratin and focal positivity for epithelial membrane antigen (EMA). The microscopic features demonstrated a malignant skin appendageal tumor compatible with sebaceous carcinoma [Figure 2]. The resected margins were free of tumor. With the intent of decreasing the chances of local recurrence, adjuvant radiotherapy was planned for the patient. Consequently, a dose of 50 Gy in 20 fractions over 4 weeks was delivered using electron beam therapy. He completed the course of radiation therapy with associated grade 2 cutaneous morbidity. Currently, he has completed a follow-up period of one year after therapy and has no evidence of disease. DISCUSSION Sebaceous carcinoma is a disease of the sixth and seventh decade of life.[1] The most frequent extra-orbital site for this tumor is skin in the head and neck region wherein sebaceous glands are most abundant.[1] It has been associated with Muir– Torre syndrome. The Muir–Torre syndrome[2,3] is an autosomal dominant genodermatosis consisting of sebaceous neoplasms viz. sebaceous adenoma, sebaceous carcinoma, or sebaceous epithelioma with or without keratoacanthomas associated with one or more visceral malignancies. The visceral malignancies associated with the Muir–Torre syndrome are often of low-grade.
Figure 2: Photomicrograph shows (a) islands of raggedly infiltrating tumor cells in dermis (H and E stain, ×40), (b) tumor islands with areas of central necrosis (H and E stain, ×40), (c) cells with large bizarre nuclei and prominent nucleolus (H and E stain, ×100), (d) cells with finely vacuolated cytoplasm (H and E stain, ×200), (e) epithelial membrane antigen positive immunostaining (×200), (f) cytokeratin positive immunostaining (×100)
Sebaceous carcinoma presents as a firm and painless slowgrowing mass. The main histological criterion is sebaceous differentiation. The cells show nuclear pleomorphism, lobular architecture and foamy vacuolization of the cytoplasm.[4] The primary treatment for sebaceous carcinoma is excision with wide margins. In a review of 91 patients with extra-ocular sebaceous carcinoma, Bailet et al.[4] reported local recurrence rates of 29%, regional nodal metastasis in 15%, and a disease-related mortality of 20%. Adverse prognostic indicators that have been postulated include poor differentiation, infiltrative growth pattern, multicentric origin of the tumor, delay in diagnosis of over 6 months, and size of the tumor more than 10 mm. Samarasinghe et al.[5] reported a case of sebaceous carcinoma of scalp with nodal metastasis at presentation. With regard to the patient concerning this case study, the disease presentation was in the third decade, which differs from the usual presentation in elderly patients. He did not have a family history of malignancy or associated visceral malignancy. The patient developed tumor relapse after excision biopsy. He was treated with wide local excision of the tumor. However, the patient developed local recurrence after an interval of four months for which he again underwent wide local excision. He did not manifest any regional or distant metastasis. The present case highlights the locally aggressive nature of this tumor. The role of adjuvant radiotherapy is not defined for sebaceous carcinoma. However, it was incorporated in the management of this patient considering the clinical presentation. The patient was successfully treated with no evidence of any local recurrence seen after a follow-up period of one year. The presentation of sebaceous carcinoma may precede the manifestation of visceral malignancy by many
Journal of Cancer Research and Therapeutics - October-December 2013 - Volume 9 - Issue 4
731
Sahai, et al.: Sebaceous carcinoma of scalp
years. Therefore, these patients require life-long surveillance for malignancy. Sebaceous carcinoma needs to be considered as differential diagnosis for the cutaneous malignancies in all age groups. The timely recognition enables execution of the primary treatment i.e., wide local excision. Adjuvant radiotherapy may be considered to improve the clinical outcome of recurrent tumors. REFERENCES 1.
732
Pickford MA, Hogg FJ, Fallowfield ME, Webster MH. Sebaceous carcinoma of the periorbital and extraorbital regions. Br J Plast Surg 1995;48:93-6.
2. 3. 4. 5.
Cohen PR, Kohn SR, Davis DA, Kurzrock R. Muir-Torre syndrome. Dermatol Clin 1995;13:79-89. Schwartz RA, Torre DP. Muir-Torre syndrome: A 25-year retrospect. J Am Acad Dermatol 1995;33:90-104. Bailet JW, Zimmerman MC, Arnstein DP, Wollman JS, Mickel RA. Sebaceous carcinoma of the head and neck. Case report and literature review. Arch Otolaryngol Head Neck Surg 1992;118:1245-9. Samarasinghe V, Marsden J, Roberts C. Sebaceous carcinoma of the scalp presenting with nodal metastasis. J Plast Reconstr Aesthet Surg 2010;63:2193-4.
Cite this article as: Sahai P, Singh K, Sharma A, Ghosh R, Nembang P, Sarkar C, et al. Recurrent sebaceous carcinoma of the scalp in a young male treated with adjuvant radiotherapy. J Can Res Ther 2013;9:730-2. Source of Support: Nil, Conflict of Interest: None declared.
Journal of Cancer Research and Therapeutics - October-December 2013 - Volume 9 - Issue 4
Copyright of Journal of Cancer Research & Therapeutics is the property of Medknow Publications & Media Pvt. Ltd. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
