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Multiple dermoid cysts of the external auditory canal
International Journal of Pediatric Otorhinolaryngology Extra (2009) 4, 158—161
www.elsevier.com/locate/ijporl
CASE REPORT
Multiple dermoid cysts of the external auditory canal Sung Hyun Boo a,*, Min Jung Heo b, Chi-Sung Han c a
Department of Otorhinolaryngology-Head and Neck Surgery, Masan Samsung Hospital, Sungkyunkwan University School of Medicine, Hapsung 2-dong, Masan, Gyeongsangnam-do 630-723, Republic of Korea b Department of Special education, Pusan National University, Busan, Republic of Korea c Department of Otorhinolaryngology, Wallace Memorial Baptist Hospital, Busan, Republic of Korea Received 22 October 2008; received in revised form 25 November 2008; accepted 27 November 2008 Available online 15 January 2009
KEYWORDS Dermoid cyst; External auditory canal
Summary A dermoid cyst is an ectodermal cyst that contains an epithelial lining as well as adnexal structures. It may occur in numerous areas of the body. Dermoid cysts of the head and neck region are relatively rare. Only a few cases of dermoid cysts in the auriculotemporal area have been reported but not in the external auditory canal previously. We present the case of an 11-year-old girl with multiple dermoid cysts of external auditory canal. Because of its location and rarity, we report this case with brief review of literature. # 2008 Elsevier Ireland Ltd. All rights reserved.
Introduction Dermoid cysts are congenital anomalies that are derived from both ectoderm and mesoderm. A keratinized squamous epithelium is typically present together with dermal derivatives such as hair follicles, sebaceous glands, and eccrine glands. Dermoid cysts of the head and neck are uncommon. More than 80% of these are located in the orbit, oral cavity or nasal cavity [1]. The most common location in the head and neck is the lateral third of the eyebrow [2]. Dermoid cysts of the auricle are rare, and only a few cases have been reported in the literature [3—5]. Within the auricu* Corresponding author. Tel.: +82 55 290 6068; fax: +82 55 299 8652. E-mail address: [email protected] (S.H. Boo).
lar area, most dermoids are found in the postauricular area. The external auditory canal (EAC) is extremely rare place for its occurrence. Also, multiple occurrence of dermoid cysts is quite uncommon. Here we report a rare case of an 11-year-old girl with multiple dermoid cysts of the EAC. The lesions were successfully removed under local anesthesia. To the best of our knowledge, this is the first report of multiple dermoid cysts occurring in the ear canal in English literatures.
Case report An 11-year-old girl presented to our clinic with slow growing mass of left EAC for several years. Although her parents had observed a swelling behind the left tragus, they did not seek medical attention. She did
1871-4048/$ — see front matter # 2008 Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.pedex.2008.11.001
Multiple dermoid cysts of the EAC
159
Fig. 3 Gross finding of the two dermoid cysts after surgical excision. The specimen consists of two ovoid yellowish cystic mass, measuring 9 mm 6 mm 5 mm and 12 mm 7 mm 6 mm in size. Fig. 1 A clinical photograph showing the mass nearly obstructing the left external auditory canal.
not complain any symptoms except aural fullness. Her past history was unremarkable. Physical examination showed a smooth surfaced ovoid mass obstructing the left EAC and tympanic membrane could not be visualized (Fig. 1). High resolution computed tomographic (CT) scan of the temporal bone demonstrated a soft tissue mass filling in the left EAC, which originated from the anterior wall (Fig. 2). There was no bony erosion adjacent to the mass and no abnormal finding in the middle ear and mastoid cavity. The patient underwent surgical resection under local anesthesia in an operating room. After the incision over the mass was done, the well-encapsulated yellowish ovoid mass was dissected from a
surrounding soft tissue and removed. However, there was another mass in more medial side. The second mass was completely removed and no remaining mass was confirmed (Fig. 3). Postoperatively, the patient did not show any evidence of complications and there was no recurrence of cyst during 12-month follow up. On histopathology, 2 specimens from each mass revealed a cystic wall lined by a keratinized stratified squamous epithelium with sebaceous glands and hair follicles, which finding is consistent with dermoid cyst (Fig. 4).
Discussion Dermoid cysts are developmental cystic lesions that occur primarily in the testes and ovaries.
Fig. 2 Preoperative axial view of temporal bone CT scan shows a soft tissue mass in left external auditory canal.
Fig. 4 Histopathologic finding of the dermoid cyst. Cystic wall is lined by stratified squamous epithelium and laminated keratin material with a dermoid structure containing hair follicles, sebaceous glands and eccrine glands (hematoxylin and eosin stain, 40).
160 However, they can occur anywhere in the body at an embryologic fusion point including the head and neck areas. Dermoid cysts of the head and neck account for 6.9% of all dermoid cysts. Within the head and neck area, 49.5% of these occur in the orbital area, 23.3% occur in the oral cavity, and 12.6% occur in the nasal cavity [1]. Recently, Pryor et al. [6] presented a series of 49 dermoid cysts of the head and neck of children and found that the most common location was periorbital (30 patients), followed by the neck (9 patients), scalp (4 patients), nose (3 patients), ear (2 patients), and forehead (1 patients). Dermoid cysts rarely involve the ear including auricle and temporal bone. Up to now, isolated cases have been reported such as middle ear, mastoid, eustachian tube, auricle and retroauricular area [3—5,7—9]. Dermoid cysts of the EAC are extremely rare and have been described in only one case in Russian by literature search of Pubmed [10]. In addition, dermoid cysts rarely exhibit multiple occurrence pattern [11]. Multiple dermoid cysts of the auriculotemporal area have never been reported previously. Dermoid cysts are subdivided into three distinct histologic types [12]. (1) Epidermoid cysts are cavities lined with simple squamous epithelium with fibrous wall and no attached structures. (2) True dermoid cysts are lined with epithelium showing keratinization and with identifiable adnexal structures such as hair follicles, sebaceous glands, and sweat glands. (3) Teratomas contain derivatives of all three embryonic germ layers (ectoderm, mesoderm and endoderm). All three subtypes may contain keratinous material. Dermoid cysts can be congenital or acquired even if the presentation or histology is similar [1,6]. Congenital cysts are dysembryogenetic lesions that arise from ectoderm elements entrapped during fusion of the first and second branchial arches. Acquired cysts derived from traumatic or iatrogenic inclusion of epithelial cells or from the occlusion of a sebaceous gland duct. Dermoid cysts of the head and neck are thought to be the congenital inclusion cyst [6]. Because of the few patients reported, the pathogenesis of dermoid cysts of the EAC is not well established. Embryologically, the auricle grows by fusion of the first and second branchial arches. The external auditory meatus develops from the dorsal end of the first branchial cleft. Ikeda et al. [3] described that the dermoid cyst of the auricle might result from inclusion at the time of the first branchial arch fusion. It is thought that dermoids of the EAC have similar developmental process of auricular dermoids.
S.H. Boo et al. The natural history of dermoid cysts is slow and progressive growth and they remain asymptomatic unless they enlarge. The cyst’s fast growth is associated with infectious processes [2,13]. The size and location of the cyst are the cause of the clinical manifestations. In this case, the cyst nearly obstructed the ear canal so, it produced ear fullness. The differential diagnosis depends on the location of the mass. Any of the cystic or solid tumors occurring in the EAC should be considered in the differential diagnosis. It includes epidermal inclusion cyst, sebaceous cyst, first branchial cleft cyst, and lipoma. These lesions can be differentiated by only histologic examination. It is difficult to distinguish between the dermoid cyst and the first branchial cleft cyst. Otorrhea, the presence of external opening, and clinical suspicion would be helpful to diagnose and to plan excision. CT, MRI, ultrasonogram and/or fine needle aspiration biopsy can be performed to help a diagnosis of dermoid cyst. CT and MRI can show the exact location of the mass and the relationship to the surrounding structure but cannot give a final preoperative diagnosis. But dermoid cysts of EAC are usually small and look like a benign lesion, above diagnostic methods are not necessarily needed. The treatment of choice is surgical excision. Dermoid cysts of ear canal do not require urgent removal but, excision is generally done to obtain a pathologic diagnosis, correct a cosmetic deformity or prevent infection [6]. The prognosis is usually favorable. The cysts are benign in nature, but as with most cysts, they can recur unless the entire wall of the cyst is removed.
References [1] G.B. New, J.B. Erich, Dermoid cysts of the head and neck, Surg. Gynecol. Obstet. 65 (1937) 48—55. [2] R.C. King, B.R. Smith, J.L. Burk, Dermoid cyst in the floor of the mouth. Review of the literature and case reports, Oral Surg. Oral Med. Oral Pathol. 78 (1994) 567—576. [3] M. Ikeda, J. Muto, S. Omachi, Dermoid cyst of the auricle: report of two cases, Auris Nasus Larynx 16 (1990) 193—197. [4] A. Samper, R. Ruiz de Erenchun, L. Yeste, A. Bazan, Dermoid cyst on the auriculotemporal area, Plast. Reconstr. Surg. 106 (2000) 947—948. [5] K.C. Prasad, S. Karthik, S.C. Prasad, A comprehensive study on lesions of the pinna, Am. J. Otolaryngol. 26 (2005) 1—6. [6] S.G. Pryor, J.E. Lewis, A.L. Weaver, L.J. Orvidas, Pediatric dermoid cysts of the head and neck, Otolaryngol. Head Neck Surg. 132 (2005) 938—942. [7] P.E. Farris, W.L. Meyerhoff, F. Vuitch, Congenital dermoid cyst of the middle ear, Skull Base Surg. 8 (1998) 77—80. [8] M.P. Fried, D.M. Vernick, Dermoid cyst of the middle ear and mastoid, Otolaryngol. Head Neck Surg. 92 (1984) 594—596.
Multiple dermoid cysts of the EAC [9] J.Y. Sichel, I. Dano, D. Halperin, R. Chisin, Dermoid cyst of the eustachian tube, Int. J. Pediatr. Otorhinolaryngol. 48 (1999) 77—81. [10] T.S. Khudainazarov, Sh.K. Khodzhamkulyev, Kh.N. Niiazova, O.K. Kurbanov, Dermoid cyst of the external auditory canal, Vestnik Otorinolaringol. Mar—Apr (2) (1981) 77—78.
161 [11] J.O. Akinosi, Multiple sublingual dermoid cysts, Br. J. Oral Surg. 12 (1974) 235—239. [12] I. Meyer, Dermoid cysts of the floor of the mouth, Oral Surg. 8 (1955) 1149—1164. [13] J.L. Vargas Femandez, J. Lorenzo Rojas, J. Aneiros Femandez, M. Sainz Quevedo, Dermoid cyst of the floor of the mouth, Acta Otorrinolaringol. Esp. 58 (2007) 31—33.
Available online at www.sciencedirect.com
www.elsevier.com/locate/ijporl
CASE REPORT
Multiple dermoid cysts of the external auditory canal Sung Hyun Boo a,*, Min Jung Heo b, Chi-Sung Han c a
Department of Otorhinolaryngology-Head and Neck Surgery, Masan Samsung Hospital, Sungkyunkwan University School of Medicine, Hapsung 2-dong, Masan, Gyeongsangnam-do 630-723, Republic of Korea b Department of Special education, Pusan National University, Busan, Republic of Korea c Department of Otorhinolaryngology, Wallace Memorial Baptist Hospital, Busan, Republic of Korea Received 22 October 2008; received in revised form 25 November 2008; accepted 27 November 2008 Available online 15 January 2009
KEYWORDS Dermoid cyst; External auditory canal
Summary A dermoid cyst is an ectodermal cyst that contains an epithelial lining as well as adnexal structures. It may occur in numerous areas of the body. Dermoid cysts of the head and neck region are relatively rare. Only a few cases of dermoid cysts in the auriculotemporal area have been reported but not in the external auditory canal previously. We present the case of an 11-year-old girl with multiple dermoid cysts of external auditory canal. Because of its location and rarity, we report this case with brief review of literature. # 2008 Elsevier Ireland Ltd. All rights reserved.
Introduction Dermoid cysts are congenital anomalies that are derived from both ectoderm and mesoderm. A keratinized squamous epithelium is typically present together with dermal derivatives such as hair follicles, sebaceous glands, and eccrine glands. Dermoid cysts of the head and neck are uncommon. More than 80% of these are located in the orbit, oral cavity or nasal cavity [1]. The most common location in the head and neck is the lateral third of the eyebrow [2]. Dermoid cysts of the auricle are rare, and only a few cases have been reported in the literature [3—5]. Within the auricu* Corresponding author. Tel.: +82 55 290 6068; fax: +82 55 299 8652. E-mail address: [email protected] (S.H. Boo).
lar area, most dermoids are found in the postauricular area. The external auditory canal (EAC) is extremely rare place for its occurrence. Also, multiple occurrence of dermoid cysts is quite uncommon. Here we report a rare case of an 11-year-old girl with multiple dermoid cysts of the EAC. The lesions were successfully removed under local anesthesia. To the best of our knowledge, this is the first report of multiple dermoid cysts occurring in the ear canal in English literatures.
Case report An 11-year-old girl presented to our clinic with slow growing mass of left EAC for several years. Although her parents had observed a swelling behind the left tragus, they did not seek medical attention. She did
1871-4048/$ — see front matter # 2008 Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.pedex.2008.11.001
Multiple dermoid cysts of the EAC
159
Fig. 3 Gross finding of the two dermoid cysts after surgical excision. The specimen consists of two ovoid yellowish cystic mass, measuring 9 mm 6 mm 5 mm and 12 mm 7 mm 6 mm in size. Fig. 1 A clinical photograph showing the mass nearly obstructing the left external auditory canal.
not complain any symptoms except aural fullness. Her past history was unremarkable. Physical examination showed a smooth surfaced ovoid mass obstructing the left EAC and tympanic membrane could not be visualized (Fig. 1). High resolution computed tomographic (CT) scan of the temporal bone demonstrated a soft tissue mass filling in the left EAC, which originated from the anterior wall (Fig. 2). There was no bony erosion adjacent to the mass and no abnormal finding in the middle ear and mastoid cavity. The patient underwent surgical resection under local anesthesia in an operating room. After the incision over the mass was done, the well-encapsulated yellowish ovoid mass was dissected from a
surrounding soft tissue and removed. However, there was another mass in more medial side. The second mass was completely removed and no remaining mass was confirmed (Fig. 3). Postoperatively, the patient did not show any evidence of complications and there was no recurrence of cyst during 12-month follow up. On histopathology, 2 specimens from each mass revealed a cystic wall lined by a keratinized stratified squamous epithelium with sebaceous glands and hair follicles, which finding is consistent with dermoid cyst (Fig. 4).
Discussion Dermoid cysts are developmental cystic lesions that occur primarily in the testes and ovaries.
Fig. 2 Preoperative axial view of temporal bone CT scan shows a soft tissue mass in left external auditory canal.
Fig. 4 Histopathologic finding of the dermoid cyst. Cystic wall is lined by stratified squamous epithelium and laminated keratin material with a dermoid structure containing hair follicles, sebaceous glands and eccrine glands (hematoxylin and eosin stain, 40).
160 However, they can occur anywhere in the body at an embryologic fusion point including the head and neck areas. Dermoid cysts of the head and neck account for 6.9% of all dermoid cysts. Within the head and neck area, 49.5% of these occur in the orbital area, 23.3% occur in the oral cavity, and 12.6% occur in the nasal cavity [1]. Recently, Pryor et al. [6] presented a series of 49 dermoid cysts of the head and neck of children and found that the most common location was periorbital (30 patients), followed by the neck (9 patients), scalp (4 patients), nose (3 patients), ear (2 patients), and forehead (1 patients). Dermoid cysts rarely involve the ear including auricle and temporal bone. Up to now, isolated cases have been reported such as middle ear, mastoid, eustachian tube, auricle and retroauricular area [3—5,7—9]. Dermoid cysts of the EAC are extremely rare and have been described in only one case in Russian by literature search of Pubmed [10]. In addition, dermoid cysts rarely exhibit multiple occurrence pattern [11]. Multiple dermoid cysts of the auriculotemporal area have never been reported previously. Dermoid cysts are subdivided into three distinct histologic types [12]. (1) Epidermoid cysts are cavities lined with simple squamous epithelium with fibrous wall and no attached structures. (2) True dermoid cysts are lined with epithelium showing keratinization and with identifiable adnexal structures such as hair follicles, sebaceous glands, and sweat glands. (3) Teratomas contain derivatives of all three embryonic germ layers (ectoderm, mesoderm and endoderm). All three subtypes may contain keratinous material. Dermoid cysts can be congenital or acquired even if the presentation or histology is similar [1,6]. Congenital cysts are dysembryogenetic lesions that arise from ectoderm elements entrapped during fusion of the first and second branchial arches. Acquired cysts derived from traumatic or iatrogenic inclusion of epithelial cells or from the occlusion of a sebaceous gland duct. Dermoid cysts of the head and neck are thought to be the congenital inclusion cyst [6]. Because of the few patients reported, the pathogenesis of dermoid cysts of the EAC is not well established. Embryologically, the auricle grows by fusion of the first and second branchial arches. The external auditory meatus develops from the dorsal end of the first branchial cleft. Ikeda et al. [3] described that the dermoid cyst of the auricle might result from inclusion at the time of the first branchial arch fusion. It is thought that dermoids of the EAC have similar developmental process of auricular dermoids.
S.H. Boo et al. The natural history of dermoid cysts is slow and progressive growth and they remain asymptomatic unless they enlarge. The cyst’s fast growth is associated with infectious processes [2,13]. The size and location of the cyst are the cause of the clinical manifestations. In this case, the cyst nearly obstructed the ear canal so, it produced ear fullness. The differential diagnosis depends on the location of the mass. Any of the cystic or solid tumors occurring in the EAC should be considered in the differential diagnosis. It includes epidermal inclusion cyst, sebaceous cyst, first branchial cleft cyst, and lipoma. These lesions can be differentiated by only histologic examination. It is difficult to distinguish between the dermoid cyst and the first branchial cleft cyst. Otorrhea, the presence of external opening, and clinical suspicion would be helpful to diagnose and to plan excision. CT, MRI, ultrasonogram and/or fine needle aspiration biopsy can be performed to help a diagnosis of dermoid cyst. CT and MRI can show the exact location of the mass and the relationship to the surrounding structure but cannot give a final preoperative diagnosis. But dermoid cysts of EAC are usually small and look like a benign lesion, above diagnostic methods are not necessarily needed. The treatment of choice is surgical excision. Dermoid cysts of ear canal do not require urgent removal but, excision is generally done to obtain a pathologic diagnosis, correct a cosmetic deformity or prevent infection [6]. The prognosis is usually favorable. The cysts are benign in nature, but as with most cysts, they can recur unless the entire wall of the cyst is removed.
References [1] G.B. New, J.B. Erich, Dermoid cysts of the head and neck, Surg. Gynecol. Obstet. 65 (1937) 48—55. [2] R.C. King, B.R. Smith, J.L. Burk, Dermoid cyst in the floor of the mouth. Review of the literature and case reports, Oral Surg. Oral Med. Oral Pathol. 78 (1994) 567—576. [3] M. Ikeda, J. Muto, S. Omachi, Dermoid cyst of the auricle: report of two cases, Auris Nasus Larynx 16 (1990) 193—197. [4] A. Samper, R. Ruiz de Erenchun, L. Yeste, A. Bazan, Dermoid cyst on the auriculotemporal area, Plast. Reconstr. Surg. 106 (2000) 947—948. [5] K.C. Prasad, S. Karthik, S.C. Prasad, A comprehensive study on lesions of the pinna, Am. J. Otolaryngol. 26 (2005) 1—6. [6] S.G. Pryor, J.E. Lewis, A.L. Weaver, L.J. Orvidas, Pediatric dermoid cysts of the head and neck, Otolaryngol. Head Neck Surg. 132 (2005) 938—942. [7] P.E. Farris, W.L. Meyerhoff, F. Vuitch, Congenital dermoid cyst of the middle ear, Skull Base Surg. 8 (1998) 77—80. [8] M.P. Fried, D.M. Vernick, Dermoid cyst of the middle ear and mastoid, Otolaryngol. Head Neck Surg. 92 (1984) 594—596.
Multiple dermoid cysts of the EAC [9] J.Y. Sichel, I. Dano, D. Halperin, R. Chisin, Dermoid cyst of the eustachian tube, Int. J. Pediatr. Otorhinolaryngol. 48 (1999) 77—81. [10] T.S. Khudainazarov, Sh.K. Khodzhamkulyev, Kh.N. Niiazova, O.K. Kurbanov, Dermoid cyst of the external auditory canal, Vestnik Otorinolaringol. Mar—Apr (2) (1981) 77—78.
161 [11] J.O. Akinosi, Multiple sublingual dermoid cysts, Br. J. Oral Surg. 12 (1974) 235—239. [12] I. Meyer, Dermoid cysts of the floor of the mouth, Oral Surg. 8 (1955) 1149—1164. [13] J.L. Vargas Femandez, J. Lorenzo Rojas, J. Aneiros Femandez, M. Sainz Quevedo, Dermoid cyst of the floor of the mouth, Acta Otorrinolaringol. Esp. 58 (2007) 31—33.
Available online at www.sciencedirect.com