Case Report Korean J Otorhinolaryngol-Head Neck Surg 2018;61(1):47-51 / pissn / eissn

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Case Report Korean J Otorhinolaryngol-Head Neck Surg 2018;61(1):47-51 / pissn 2092-5859 / eissn 2092-6529 https://doi.org/10.3342/kjorl-hns.2016.

1 Case Report Korean J Otorhinolaryngol-Head Neck Surg 2018;61(1):47-51 / pissn / eissn A Case of Dermatofibrosarcoma Protuberans was Misdiagnosed as Keloid of the Anterior Neck Jin Hyun Seo 1, Jin Pyeong Kim 1, and Seung Hoon Woo 1,2 1 Departments of Otorhinolaryngology, 2 Institute of Health Sciences, College of Medicine, Gyeongsang National University, Jinju, Korea 켈로이드로오인된전경부융기성피부섬유육종 서진현 1 김진평 1 우승훈 1,2 경상대학교의과대학이비인후과학교실, 1 건강과학원 2 Received May 18, 2016 Revised August 2, 2016 Accepted August 10, 2016 Address for correspondence Seung Hoon Woo, MD Department of Otorhinolaryngology, College of Medicine, Gyeongsang National University, 79 Gangnam-ro, Jinju 52727, Korea Tel Fax lesaby@hanmail.net Dermatofibrosarcoma protuberans is a rare type of cancer, a soft tissue sarcoma that develops in the deep layers of skin. It is a locally aggressive tumor with a high recurrence rate but the metastasis rarely occurs. The first sign of dermatofibrosarcoma protuberans is often a small bump on the skin. It may resemble a deep-seated pimple of rough patch of skin. The tumor typically grows slowly and can become a raised nodule. Therefore, it is likely to be misdiagnosed as keloid, basal cell carcinoma and scleroderma. We recently experienced a case of dermatofibrosarcoma protuberans in a 63-year-old man, who presented with anterior lower neck mass. We present this case with a review of the literature. Korean J Otorhinolaryngol-Head Neck Surg 2018;61(1):47-51 Key WordsZZDermatofibrosarcoma protuberans ㆍ Keloid ㆍ Operation ㆍ Sarcoma. 서 론 This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License ( which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. 증례 Copyright 2018 Korean Society of Otorhinolaryngology-Head and Neck Surgery 47

8 4 cm sized, erythematous protruding mass

Frontal view (A). Right lateral view (B).

2 Korean J Otorhinolaryngol-Head Neck Surg 2018;61(1):47-51 A B C Fig cm sized, erythematous protruding mass was noted on the anterior lower neck. Frontal view (A). Right lateral view (B). Left lateral view (C). A B Fig. 2. Pharynx CT shows 5.5 cm enhancing mass on the anterior lower neck subcutaneous area. Axial view, Suspicious invasion of right strap muscle (A). Coronal view, 5.5 cm enhancing mass on the anterior lower neck (B)

Dermatofibrosarcoma Protuberans of the Anterior Neck Seo JH, et al.

4) 증상은 주위 피부 의 모세혈관확장증이 동반된 분홍색의 판(plaque)형태로 시 작하여 결절(nodule)의 형태로 성장한다.

5) 융기성 피부섬유육종의 병인은 확실히 알 수 없으나 외상의 경력을 10~20%에서 찾아볼 수 있으며,6) 화상을 입은 부위나 수술 후 반흔조직에서 이 종양이 발생되었다는 보고들이 있

The resection margins were cancer positive. Fig. 4.

Histologic finding shows slender spindle cells arranging in storiform pattern (H&E stain 100) (A).

(B). This photograph shows negative for S-100 protein (S-100 immunostain 200) (C).

3 Dermatofibrosarcoma Protuberans of the Anterior Neck Seo JH, et al. 고 찰 융기성 피부섬유육종은 비교적 드문 중등도의 악성 종양으 로 0.5 cm에서부터 20 cm에 이르기까지 다양한 크기로 보고 되며, 전체 종양의 0.1% 이하를 차지한다.4) 증상은 주위 피부 의 모세혈관확장증이 동반된 분홍색의 판(plaque)형태로 시 작하여 결절(nodule)의 형태로 성장한다. 서서히 자라기 때문 에 진단되기까지 대부분 오랜 시간이 경과된다.5) 융기성 피부섬유육종의 병인은 확실히 알 수 없으나 외상의 경력을 10~20%에서 찾아볼 수 있으며,6) 화상을 입은 부위나 수술 후 반흔조직에서 이 종양이 발생되었다는 보고들이 있 다.7) 따라서 켈로이드와 감별이 어렵다. 융기성 피부섬유육종은 자라면서 점차 피부뿐만 아니라 피 Fig. 3. After mass excision. The resection margins were cancer positive. Fig. 4. Histopathology and immunochemistry showing dermatofibrosarcoma protuberans. Histologic finding shows slender spindle cells arranging in storiform pattern (H&E stain 100) (A). Immunohistochemical stain reveals tumor cells showing positive reaction for CD34 (CD34 immunostain 100) (B). This photograph shows negative for S-100 protein (S-100 immunostain 200) (C). This photograph shows negative for anti-sma antibody (SMA immunostain 100) (D). SMA: smooth muscle actin. Fig. 5. Intraoperative photograph taken after wide excision, both strap muscle resection, both sternocleidomastoid muscle medial side resection, thyroid both lobe medial side and isthmus resection, both neck level III, IV, VI lymph nodes dissection. A B C D 49

4 Korean J Otorhinolaryngol-Head Neck Surg 2018;61(1):47-51 Fig. 6. The pectoralis major myocutaneous flap is laid in the recipient bed. - REFERENCES 1) Le Vay J, O Sullivan B, Catton C, Cummings B, Fornasier V, Gullane P, et al. An assessment of prognostic factors in soft-tissue sarcoma of the head and neck. Arch Otolaryngol Head Neck Surg 1994;120 (9): ) Gayner SM, Lewis JE, McCaffrey TV. Effect of resection margins on dermatofibrosarcoma protuberans of the head and neck. Arch Otolaryngol Head Neck Surg 1997;123(4): ) Laskin WB. Dermatofibrosarcoma protuberans. CA Cancer J Clin 1992;42(2): ) Lee PK, Kim HT, Kim YJ. A case of Dermatofibrosarcoma Protuberans in a breast. J Korean Soc Plast Reconstr Surg 2002;29(3): ) Lindner NJ, Scarborough MT, Powell GJ, Spanier S, Enneking WF. Revision surgery in dermatofibrosarcoma protuberans of the trunk and extremities. Eur J Surg Oncol 1999;25(4): ) Taylor HB, Helwig EB. Dermatofibrosarcoma protuberans. A study of 115 cases. Cancer 1962;15: ) Simstein NL, Tuthill RJ, Sperber EE, Kovalcik PJ, Mullen JT. 50

5 Dermatofibrosarcoma Protuberans of the Anterior Neck Seo JH, et al. Dermatofibrosarcoma protuberans--case reports and review of literature. South Med J 1977;70(4): ) Aiba S, Tabata N, Ishii H, Ootani H, Tagami H. Dermatofibrosarcoma protuberans is a unique fibrohistiocytic tumour expressing CD34. Br J Dermatol 1992;127(2): ) Blackburn WR, Cosman B. Histologic basis of keloid and hypertrophic scar differentiation. Clinicopathologic correlation. Arch Pathol 1966; 82(1): ) Vásconez HC, Sengezer M, McGrath PC. Flap coverage of a large defect after excision of a massive dermatofibrosarcoma protuberans. Plast Reconstr Surg 1995;95(1): ) Smola MG, Soyer HP, Scharnagl E. Surgical treatment of dermatofibrosarcoma protuberans. A retrospective study of 20 cases with review of literature. Eur J Surg Oncol 1991;17(5): ) Kang SY, Yang WY, Yoo YC, Park J. Treatment of wide dermatofibrosarcoma protuberans with tissue expander. J Korean Soc Plast Reconstr Surg 2003;30(3): ) Mendenhall WM, Zlotecki RA, Scarborough MT. Dermatofibrosarcoma protuberans. Cancer 2004;101(11): ) Bowne WB, Antonescu CR, Leung DH, Katz SC, Hawkins WG, Woodruff JM, et al. Dermatofibrosarcoma protuberans: a clinicopathologic analysis of patients treated and followed at a single institution. Cancer 2000;88(12): ) McArthur G. Molecularly targeted treatment for dermatofibrosarcoma protuberans. Semin Oncol 2004;31(2 Suppl 6):

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