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A unique case of an epidermoid cyst of the pterygopalatine fossa and its management
International Journal of Pediatric Otorhinolaryngology (2003) 67, 1259—1261
CASE REPORT
A unique case of an epidermoid cyst of the pterygopalatine fossa and its management Enver Özer a,*, Muzaffer Kanlıkama a , Yıldırım A. Bayazıt a , Semih Mumbuç a , ˙Ibrahim Sarı b , Abdülvahap Gök c a
Department of Otolaryngology, Faculty of Medicine, Gaziantep University, Kolejtepe, Gaziantep, Turkey b Department of Pathology, Faculty of Medicine, Gaziantep University, Kolejtepe, Gaziantep, Turkey c Department of Neurosurgery, Faculty of Medicine, Gaziantep University, Kolejtepe, Gaziantep, Turkey Received 18 January 2003 ; received in revised form 18 July 2003; accepted 21 July 2003
KEYWORDS Epidermoid cyst; Pterygopalatine fossa
Summary Epidermoid cysts are rare benign tumors that are derived from the development of abnormally situated ectodermal tissue. Although they may originate from any part of the body head and neck region is occasionally affected. Epidermoid cyst originating from pterygopalatine fossa has not been reported previously in the literature. We report the first case of epidermoid cyst of pterygomaxillary fossa in this study. © 2003 Elsevier Ireland Ltd. All rights reserved.
1. Introduction Epidermoid cysts are relatively rare tumors. They may occur any where in the body. About 7% of them are found in the head and neck region. All flat bones of the calvarium, paranasal sinuses, maxilla, temporal bone, and sphenoid bone could be the site of the origin [1]. This is the first case of epidermoid cyst in the literature that originated from the pterygopalatine fossa.
2. Case report A 4-year-old female child came to our clinic with a 6-month history of progressively worsening headache of the right side of the face. *Corresponding author. Tel.: +90-342-336-5400; fax: +90-342-3365505. E-mail address: [email protected] (E. Özer).
The physical examination was unremarkable. Cranial nerves were functionally intact. Computed tomography (CT) and magnetic resonance imaging (MRI) revealed a 3 cm × 3 cm encapsulated mass of the pterygomaxillary fossa. The mass was destructing the roof of the fossa and exposing the cranial base duramater (Figs. 1 and 2). The tumor was totally excised via trans-maxillary approach. After en-block removal of the anterior surface of the maxillary sinus, posterior wall of the sinus was removed and the tumor was easily dissected from the surrounding tissues and totally excised. At the end of the operation, anterior maxillary wall was reconstructed with the help of mini-plates. On gross examination the tumor measured 3 cm in diameter and it was whitish in color. Histopathology revealed that the tumor was a simple epidermal cyst (Fig. 3). In the 1 year follow-up, the patient was free of disease.
0165-5876/$ — see front matter © 2003 Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.ijporl.2003.07.009
1260
E. Özer et al.
Fig. 1 Computed tomographic view of epidermal cyst in the pterygopalatine fossa. Note the erosion of the roof of the fossa and exposed duramater.
3. Discussion Epidermoid cysts are rare benign tumors that are derived from the development of abnormally situated ectodermal tissue and are often an incidental finding. They may occur any where in the body. About 7% of them are found in the head and neck region and only 1.6% are located within the oral cavity [1]. Intradiploic epidermoids are very rare lesions that may occur in all flat bones of the calvarium and in the paranasal sinuses, maxilla, temporal bone, and sphenoid bone [2]. A case of temporomandibular joint have also been described [3]. The first case of epidermoid cyst of pterygomaxillary fossa is reported in this study. Although it is extremely rare malignant change in epidermoid cysts in several cases have been
Fig. 2
reported in the literature [4,5]. Epidermoid cysts sometimes show relatively different clinical presentations. An intradiploic epidermoid cyst of the frontal bone may present with tension pneumocephalus [6], a life-threatening epidermoid cyst of the floor of the mouth [7] or an unusual cause of obstructive sialadenitis [8] could be the presentation. Although most of the epidermal cysts are congenital in origin, trauma could be a major factor in the etiology of epidermoid cysts in some cases. Head injury that caused the inclusion of epidermal cells into the diploe of the bone was present in the medical history of a patient with an intradiploic epidermoid cyst of calvarium in the literature [9]. Sometimes this trauma could be a surgical procedure [3]. Previous trauma or surgical intervention was not encountered in our case; it was a congenital epidermal cyst. Pre-operative imaging of these lesions provides important diagnostic and anatomical information with regard to the patient’s therapeutic management. The most common computed tomographic finding is a low density lesion. Magnetic resonance imaging reveals varying signal intensities (high signal intensity being associated with hemorrhagic fluid in the cyst). Epidermoid cysts are consistently hyperintense on T2 -weighted images [10]. Surgical excision is the treatment of choice and the tool for the definitive diagnosis. An epidermoid cyst should be distinguished from dermoid cyst, hemangioma, lipoma, eosinophilic granuloma, cholesterol granuloma, neurofibroma, hydatid cyst, teratoma, fibrous dysplasia, aneurysmal bone cyst, and meningioma [11].
Magnetic resonance imaging of epidermal cyst in the pterygopalatine fossa.
A unique case of an epidermoid cyst of the pterygopalatine fossa and its management
1261
Fig. 3 Histopathological view of the epidermal cyst. Note the simple stratified squamous keratinized epithelium of the cyst.
Epidermoid cysts that have been excised and examined histologically reveal simple stratified squamous keratinized epithelium. After complete excision careful follow-up is essential, as recurrence rate of 8.3—25% have been reported [12]. In conclusion, although epidermal cysts are rather rare in head and neck region, particularly in the pterygopalatine fossa, it should be considered in the differential diagnosis of the tumors of this region. Complete surgical excision is the treatment of choice.
References [1] K. Turetschek, H. Hospodka, E. Steiner, Case report: epidermoid cyst of the floor of the mouth: diagnostic imaging bysonography, computed tomography and magnetic resonance imaging, Br. J. Radiol. 68 (806) (1995) 205— 207. [2] A.K. White, H.A. Jenkins, N.J. Coker, Intradiploic epidermoid cyst of the sphenoid wing, Arch. Otolaryngol. Head Neck Surg. 113 (9) (1987) 995—999. [3] S. Weinberg, B. Kryshtalskyj, Epidermoid cyst in a temporomandibular joint dermal graft: report of a case and review of the literature, J. Oral Maxillofac. Surg. 53 (3) (1995) 330—332.
[4] C. Hoeffel, N. Heldt, C. Chelle, M. Claudon, J.C. Hoeffel, Malignant change in an intradiploic epidermoid cyst, Acta Neurol. Belg. 97 (1) (1997) 45—49. [5] T.H. Wong, A.K. Khoo, P.H. Tan, B.H. Ong, Squamous cell carcinoma arising in a cutaneous epidermal cyst–—a case report, Ann. Acad. Med. Singapore 29 (6) (2000) 757—759. [6] E. Jakubowski, E. Kirsch, T. Mindermann, D. Ettlin, O. Gratzl, E.W. Radu, Intradiploic epidermoid cyst of the frontal bone presenting with tension pneumocephalus, Acta Neurochir. (Wien) 139 (1) (1997) 86—87. [7] N. Zachariades, C. Skoura-Kafoussia, A life-threatening epidermoid cyst of the floor of the mouth: report of a case, J. Oral Maxillofac. Surg. 48 (4) (1990) 400—403. [8] C.N. Dohvoma, Epidermoid cyst: an unusual cause of obstructive sialadenitis, Br. J. Oral Maxillofac. Surg. 30 (2) (1992) 125—127. [9] D. Karadag, A. Tuba Karagulle, A. Erden, E. Colpan, I. Erden, S. Caglar, et al., Post-traumatic intradiploic epidermoid cyst. A case report, J. Neurosurg. Sci. 45 (4) (2001) 224—227. [10] L.A. Posey, B.A. Sterman, W. Milwaaukee, Intradiploic epidermoid cyst: quiz case, Arch. Otolaryngol. Head Neck Surg. 125 (1999) 913—915. [11] J.G. Smirniotopoulous, M. Chiecki, Teratomas, dermoids, and epidermoids of the head and neck, Radiographics 15 (1995) 1437—1455. [12] Y. Yanai, R. Tsuji, S. Ohmori, N. Tatara, S. Kubota, C. Nagashima, Malignant change in an intradiploic epidermoid: report of a case an review of the literature, Neurosurgery 16 (2) (1985) 252—256.
CASE REPORT
A unique case of an epidermoid cyst of the pterygopalatine fossa and its management Enver Özer a,*, Muzaffer Kanlıkama a , Yıldırım A. Bayazıt a , Semih Mumbuç a , ˙Ibrahim Sarı b , Abdülvahap Gök c a
Department of Otolaryngology, Faculty of Medicine, Gaziantep University, Kolejtepe, Gaziantep, Turkey b Department of Pathology, Faculty of Medicine, Gaziantep University, Kolejtepe, Gaziantep, Turkey c Department of Neurosurgery, Faculty of Medicine, Gaziantep University, Kolejtepe, Gaziantep, Turkey Received 18 January 2003 ; received in revised form 18 July 2003; accepted 21 July 2003
KEYWORDS Epidermoid cyst; Pterygopalatine fossa
Summary Epidermoid cysts are rare benign tumors that are derived from the development of abnormally situated ectodermal tissue. Although they may originate from any part of the body head and neck region is occasionally affected. Epidermoid cyst originating from pterygopalatine fossa has not been reported previously in the literature. We report the first case of epidermoid cyst of pterygomaxillary fossa in this study. © 2003 Elsevier Ireland Ltd. All rights reserved.
1. Introduction Epidermoid cysts are relatively rare tumors. They may occur any where in the body. About 7% of them are found in the head and neck region. All flat bones of the calvarium, paranasal sinuses, maxilla, temporal bone, and sphenoid bone could be the site of the origin [1]. This is the first case of epidermoid cyst in the literature that originated from the pterygopalatine fossa.
2. Case report A 4-year-old female child came to our clinic with a 6-month history of progressively worsening headache of the right side of the face. *Corresponding author. Tel.: +90-342-336-5400; fax: +90-342-3365505. E-mail address: [email protected] (E. Özer).
The physical examination was unremarkable. Cranial nerves were functionally intact. Computed tomography (CT) and magnetic resonance imaging (MRI) revealed a 3 cm × 3 cm encapsulated mass of the pterygomaxillary fossa. The mass was destructing the roof of the fossa and exposing the cranial base duramater (Figs. 1 and 2). The tumor was totally excised via trans-maxillary approach. After en-block removal of the anterior surface of the maxillary sinus, posterior wall of the sinus was removed and the tumor was easily dissected from the surrounding tissues and totally excised. At the end of the operation, anterior maxillary wall was reconstructed with the help of mini-plates. On gross examination the tumor measured 3 cm in diameter and it was whitish in color. Histopathology revealed that the tumor was a simple epidermal cyst (Fig. 3). In the 1 year follow-up, the patient was free of disease.
0165-5876/$ — see front matter © 2003 Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.ijporl.2003.07.009
1260
E. Özer et al.
Fig. 1 Computed tomographic view of epidermal cyst in the pterygopalatine fossa. Note the erosion of the roof of the fossa and exposed duramater.
3. Discussion Epidermoid cysts are rare benign tumors that are derived from the development of abnormally situated ectodermal tissue and are often an incidental finding. They may occur any where in the body. About 7% of them are found in the head and neck region and only 1.6% are located within the oral cavity [1]. Intradiploic epidermoids are very rare lesions that may occur in all flat bones of the calvarium and in the paranasal sinuses, maxilla, temporal bone, and sphenoid bone [2]. A case of temporomandibular joint have also been described [3]. The first case of epidermoid cyst of pterygomaxillary fossa is reported in this study. Although it is extremely rare malignant change in epidermoid cysts in several cases have been
Fig. 2
reported in the literature [4,5]. Epidermoid cysts sometimes show relatively different clinical presentations. An intradiploic epidermoid cyst of the frontal bone may present with tension pneumocephalus [6], a life-threatening epidermoid cyst of the floor of the mouth [7] or an unusual cause of obstructive sialadenitis [8] could be the presentation. Although most of the epidermal cysts are congenital in origin, trauma could be a major factor in the etiology of epidermoid cysts in some cases. Head injury that caused the inclusion of epidermal cells into the diploe of the bone was present in the medical history of a patient with an intradiploic epidermoid cyst of calvarium in the literature [9]. Sometimes this trauma could be a surgical procedure [3]. Previous trauma or surgical intervention was not encountered in our case; it was a congenital epidermal cyst. Pre-operative imaging of these lesions provides important diagnostic and anatomical information with regard to the patient’s therapeutic management. The most common computed tomographic finding is a low density lesion. Magnetic resonance imaging reveals varying signal intensities (high signal intensity being associated with hemorrhagic fluid in the cyst). Epidermoid cysts are consistently hyperintense on T2 -weighted images [10]. Surgical excision is the treatment of choice and the tool for the definitive diagnosis. An epidermoid cyst should be distinguished from dermoid cyst, hemangioma, lipoma, eosinophilic granuloma, cholesterol granuloma, neurofibroma, hydatid cyst, teratoma, fibrous dysplasia, aneurysmal bone cyst, and meningioma [11].
Magnetic resonance imaging of epidermal cyst in the pterygopalatine fossa.
A unique case of an epidermoid cyst of the pterygopalatine fossa and its management
1261
Fig. 3 Histopathological view of the epidermal cyst. Note the simple stratified squamous keratinized epithelium of the cyst.
Epidermoid cysts that have been excised and examined histologically reveal simple stratified squamous keratinized epithelium. After complete excision careful follow-up is essential, as recurrence rate of 8.3—25% have been reported [12]. In conclusion, although epidermal cysts are rather rare in head and neck region, particularly in the pterygopalatine fossa, it should be considered in the differential diagnosis of the tumors of this region. Complete surgical excision is the treatment of choice.
References [1] K. Turetschek, H. Hospodka, E. Steiner, Case report: epidermoid cyst of the floor of the mouth: diagnostic imaging bysonography, computed tomography and magnetic resonance imaging, Br. J. Radiol. 68 (806) (1995) 205— 207. [2] A.K. White, H.A. Jenkins, N.J. Coker, Intradiploic epidermoid cyst of the sphenoid wing, Arch. Otolaryngol. Head Neck Surg. 113 (9) (1987) 995—999. [3] S. Weinberg, B. Kryshtalskyj, Epidermoid cyst in a temporomandibular joint dermal graft: report of a case and review of the literature, J. Oral Maxillofac. Surg. 53 (3) (1995) 330—332.
[4] C. Hoeffel, N. Heldt, C. Chelle, M. Claudon, J.C. Hoeffel, Malignant change in an intradiploic epidermoid cyst, Acta Neurol. Belg. 97 (1) (1997) 45—49. [5] T.H. Wong, A.K. Khoo, P.H. Tan, B.H. Ong, Squamous cell carcinoma arising in a cutaneous epidermal cyst–—a case report, Ann. Acad. Med. Singapore 29 (6) (2000) 757—759. [6] E. Jakubowski, E. Kirsch, T. Mindermann, D. Ettlin, O. Gratzl, E.W. Radu, Intradiploic epidermoid cyst of the frontal bone presenting with tension pneumocephalus, Acta Neurochir. (Wien) 139 (1) (1997) 86—87. [7] N. Zachariades, C. Skoura-Kafoussia, A life-threatening epidermoid cyst of the floor of the mouth: report of a case, J. Oral Maxillofac. Surg. 48 (4) (1990) 400—403. [8] C.N. Dohvoma, Epidermoid cyst: an unusual cause of obstructive sialadenitis, Br. J. Oral Maxillofac. Surg. 30 (2) (1992) 125—127. [9] D. Karadag, A. Tuba Karagulle, A. Erden, E. Colpan, I. Erden, S. Caglar, et al., Post-traumatic intradiploic epidermoid cyst. A case report, J. Neurosurg. Sci. 45 (4) (2001) 224—227. [10] L.A. Posey, B.A. Sterman, W. Milwaaukee, Intradiploic epidermoid cyst: quiz case, Arch. Otolaryngol. Head Neck Surg. 125 (1999) 913—915. [11] J.G. Smirniotopoulous, M. Chiecki, Teratomas, dermoids, and epidermoids of the head and neck, Radiographics 15 (1995) 1437—1455. [12] Y. Yanai, R. Tsuji, S. Ohmori, N. Tatara, S. Kubota, C. Nagashima, Malignant change in an intradiploic epidermoid: report of a case an review of the literature, Neurosurgery 16 (2) (1985) 252—256.