Department of Otolaryngology-Head & Neck Surgery, Seoul St. Mary’s Hospital, The Catholic University of Korea School of Medicine, Seoul, South Korea
Soonil Yoo,MD, PhD, Eun Hye Shin,MD, PhD, Ji Sun Kong,MD, PhD, KyoungHo Park, MD, PhD
J Korean Skull Base Society 11권 2호 : 70~73, 2016
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종설1 원저1 원저2
원저4 원저3
원저5 원저6 원저7 원저8 원저9 증례1 증례2 증례3 증례4 증례5 증례6 증례7
The chondroid syringoma, known to be a mixed tumor of the skin, is an extremely rare benign cutaneous neoplasm of the external auditory canal.
We encountered a 47-year-old man with a mass in his left external auditory canal, and the mass was histologically diagnosed as chondroid syringoma, having both epithelial and mesenchymal stromal components. There were no signs of recurrence after surgery during a 15 month follow-up examination.
The chondroid syringoma in the external auditory canal is an interesting case, and we discuss this benign tumor of the sweat glands in the context of the relevant literature.
A Case of benign chondroid syringoma of external auditory canal
논문 접수일 : 2016년 8월 5일 논문 완료일 : 2016년 8월 30일
주소 : Department of Otolaryngology Head & Neck Surgery Seoul St. Mary’s Hospital, The Catholic University of Korea
222, Banpo-daero, Seocho-gu, Seoul, Korea Tel : 82-2-2258-6213
Fax : 82-2-595-1354 E-mail : khpent@catholic.ac.kr
Kyoung Ho Park, MD, PhD
교신저자
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A Case of benign chondroid syringoma of external auditory canal
▒ INTRODUCTION
Chondroid syringoma(CS) was described by Stout and Gorman in 1959 and known as a benign cutaneous neoplasm.
1-2) Among all primary skin tumors, the incidence of CS is under 0.01%, and the peak occurance of this tumor is in the 4th and 5th decade of life with an occurrence rate of 1.2 for female-to-male ratios.2-5) CS usually presents as a single mass that is asymptomatic, slow-growing, arises frequently in the nose, cheek, scalp, and forehead. In this case, we report a CS of the external auditory canal, and review its clinical characteristics.
Case report
A 47-year-old man was referred to a tertiary referral center with complaints of a painless mass in his left external auditory canal. He complained of ear symptoms including hearing impairment, ear fullness and otorrhea, for 3months.
Upon physical examination, the left tympanic membrane could not be examined due to obstruction of the external auditory canal by the mass(Fig. 1). The threshold of tonal audiometry showed 65dB mixed type hearing loss(Fig. 2) and speech audiometry, SRT 55dB, SDS 72% at 90dB, checked on the left side. The temporal bone CT and MRI were performed to define the extension of the lesion, and showed a round mass of about 15×9mm in size, arising from the superoposterior wall of the left external auditory canal(Fig. 3). There was also soft tissue
density in the antrum, additus and middle ear cavities, but the ossicles were intact.
A biopsy was carried out of the left external auditory canal mass. The result was acute suppurative inflammation. To evaluate further, we planned a mass excision and operation for chronic otitis media. Under general anesthesia, we did resection of a 15mm sized encapsulated mass in the cartilage segment of the external auditory canal, with a posterior auricular approach, and performed canal wall up mastoidectomy and tympanoplasty using temporalis deep
Endoscopic views show the mass has obstructing the left external auditory canal.
Fig. 1
Fig. 2
A. Pure tone audiometry before mass excision
B. Pure tone audiometry 3 months after mass excision
A B
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fascia. Granulation tissue, filling the middle ear cavity, antrum and additus, was removed. Ossicles were intact, presenting normal mobility. The subcutaneous layer and skin were primary closed with vicryl and surgiseal, and there was no the skin defect. After packing the external auditory canal with furacin gauze and compression dressing, the operation was finished, and the patient was discharged the next day.
On POD 3, the compression dressing and packing furacin gauze were removed, and the operation wound was healed well without hematoma or infection. The pathological result
was CS, mixed tumour of the skin showing both epithelial and stromal component and immunohistochemical staining was positive for EMA, S-100, SMMHC(Fig. 4). There were no other ear symptoms of problematic hearing recovery, and no signs of recurrence during a 15 month follow-up.
▒ DISCUSSION
A chondroid syringoma is a rare mixed tumor of sweat- gland origin. Hirsch and Helwig defined CS specifically as
Fig. 3
A B
Temporal bone CT (A) and MRI (B)
The enhancing mass is approximately 15×9mm in size, arising from superoposterior wall, obstructed the external auditory canal Fig. 4
Immunohistochemical results of the tumour show positivity for EMA, S-100, SMMHC
A. CEA, B. EMA, C. S-100, D. SMMHC (x400)
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A Case of benign chondroid syringoma of external auditory canal
a neoplasm that originated from epithelial cells, with the capacity to make cutaneous adnexal-like structures, and also, to produce chondroid matrix. According to them, the differentiation of epithelial cells consisting of tumors to various structures follows a morphological pattern, specific to their anatomical region. The most common site of a tumor is the head and neck region. Head and neck tumors occur in descending order of nose, cheek, upper lip, and scalp.2) However, CS of the external auditory canal is very rare.
Histologically, CS has both epithelial and mesenchymal stromal components. CS presents a circumscribed nodule, consisting of bland epithelial cells. They are arranged in cords, ducts, or tubules. The majority of the stromal component is often chondromyxoid; however, other components are fibrous, fatty, hyalinised, osteoid, or may even be absent.
The stromal cells show myoepithelial differentiation and immunohistochemically, they are positive for S100, vimentin, neuronespecific enolase, glial fibrillary acid protein, smooth muscle actin and others.1, 2, 6) In our case, CS was located in the dermis with well-defined margin. It had both epithelial and stromal components. In the epithelial component, tubuloaveolar structures had myoepithelial cells. The stromal component was fibrous, particularly chondroid stroma. Some components had plasma-cystoid changes, and immunohistochemically, in our case, was positive to S-100, EMA, SMMHC.
There have been few reported that the malignant CS (MCS) is transformed from CS. Unlike benign CS, MCS is predominantly found on the head and necks of middle-aged males and it tends to occur on the extremities and trunks of females. Common locations of metastases are regional lymph nodes, lung and bone. Histologically, there is cellular atypia, increased mitotic activity, infiltrative margins, satellite tumor nodules, and tumor necrosis.7) Excision is the best choice of treatment and thereby it is important that early detection of tumors. In our case we did not find malignant potential.
▒ CONCLUSION
We experienced a chondroid syringoma case of external auditory canal, which is a rare mixed tumor, and removed mass though wide excision with clear margin. The patient was improved conductive hearing loss and otorrhea after surgery and satisfied clinical outcome and now, he is healthy with no signs of recurrence during 15 month follow-up.
References
1. Stout AP, Gorman JG. Mixed tumours of the skin of the salivary gland type.
Cancer. 1959;12:537-43.
2. Hirsch P, Helwig EB. Chondroid syringoma. Mixed tumor of skin, salivary gland type. Arch Dermatol. 1961;84:835-47.
3. Obaidat NA, Alsaad KO, Ghazarian DJ. Skin adnexal neoplasms.- part 2: an approach to tumours of cutaneous sweat glands. Clin Pathol. 2007;60:145- 59.
4. Yavuzer R, Basterzi Y, Sari A, Bir F, Sezer C. Chondroid syringoma: a diagnosis more frequent than expected. Dermatol Surg. 2003;29:179-81.
5. Watarai A, Amoh Y, Aki R, Takasu H, Katsuoka K. Malignant chondroid syringoma: report of a case with lymph node metastasis 12 years after local excision. Dermatol Online J. 2011;17:5.
6. Argenyi ZB, Balogh K, Goeken JA. Immunohistochemical characterisation of chondroid syringomas. Am J Clin Pathol. 1988;90:662-.9.
7. Bates AW, Baithun SI. Atypical mixed tumor of the skin: histologic, immunohistochemical, and ultrastructural features in three cases and a review of the criteria for malignancy. Am J Dermatopathol. 1998;20:35-.40.
