AM ER IC AN JOURNAL OF OT OLA RYNGOLOGY– H E A D A N D NE CK M E D ICI N E AN D S U RGE RY 3 6 (2 0 1 5) 2 90 – 2 9 1
Available online at www.sciencedirect.com
ScienceDirect www.elsevier.com/locate/amjoto
Cholesteatoma of the maxillary sinus: a case report and review of the literature☆,☆☆ Jeanie Sozansky, BS a , Jordan S. Josephson, MD b,⁎ a b
Northeast Ohio Medical University, 4209 Ohio 44, Rootstown, Ohio Clinical Assistant Professor, Hofstra North Shore-LIJ School of Medicine, 500 Hofstra University, Hempstead, New York
ARTI CLE I NFO
A BS TRACT
Article history:
Cholesteatoma is a relatively common disease entity within the middle ear cavity, but it is
Received 28 July 2014
rarely found in the paranasal sinuses. We describe a rare case of cholesteatoma in the maxillary sinus of an elderly man presenting with recurrent sinus infections and nasal obstruction refractory to medical treatment. The patient was treated using functional endoscopic sinus surgery with successful removal of the cholesteatoma. He has no recurrence of the cholesteotoma at a 13 year follow-up. We review the literature and history on maxillary cholesteatomas and discuss the theories on the pathogenesis of cholesteatoma formation. We propose functional endoscopic sinus surgery with maxillary antrostomy and marsupialization of the cholesteatoma as a primary treatment option for a maxillary cholesteatoma. © 2015 Elsevier Inc. All rights reserved.
1.
Introduction
Cholesteatoma is a relatively common disease entity within the middle ear cavity, but it is rarely found in the paranasal sinuses. A review of the English literature reported less than 30 cases of a cholesteatoma in a paranasal sinus [1]. Of these limited cases, the most common location was the frontal sinus, followed by the ethmoid sinus, and finally the maxillary sinus [2]. Paranasal cholesteatomas are generally unilateral [3]. The origin of the term cholesteatoma traces back to Müller who in 1838 described the presence of cholesterol crystals in a cavity lined with squamous epithelium and filled with layers of dense, squamated keratin [2,4]. This term is a misnomer as Virchow noted in 1854 that cholesterol is not an essential component of this entity [2,4]. More appropriate terminology that is used to refer to a cholesteatoma is keratoma, keratocyst, epidermoid tumor, or epidermoid cyst [5,6].
☆
2.
Case report
A 72-year-old Caucasian male presented with recurrent sinus infections. No facial deformity was noted. He complained of chronic nasal obstruction and post nasal drip with colored mucopus. He had been treated with antibiotics, steroids, nasal sprays, and irrigation with no improvement of symptoms. The CT scan showed right maxillary sinus opacification consistent with nasal polyposis and erosion of the right medial maxillary wall. As medical treatment failed to control the symptoms, the patient opted for surgery. Functional endoscopic sinus surgery was performed and a mass was visualized eroding the right medial wall of the maxillary sinus with purulent material and keratinaceous debris present. The mass was biopsied and specimens were sent to pathology, which revealed keratinaceous material consistent with cholesteatoma as well as some filamentous organisms, possibly Actinomyces. A middle meatal
Financial Disclosures: None. Grant support: None. ⁎ Corresponding author at: 205 East 76th Street, Suite M-1, New York, NY 10021. Tel.: +1 212 717 1773. E-mail addresses: [email protected] (J. Sozansky), [email protected] (J.S. Josephson).
☆☆
http://dx.doi.org/10.1016/j.amjoto.2014.10.025 0196-0709/© 2015 Elsevier Inc. All rights reserved.
AM ER IC AN JOURNAL OF OT OLARYNGOLOGY – H E A D A N D NE CK M E D IC IN E A ND S U RGE RY 3 6 (2 0 1 5) 2 90– 2 9 1
antrostomy with marsupialization of the cholesteatoma and removal of maxillary sinus debris were carried out. Infected bone pertaining to the ethmoid sinus, sphenoid sinus, and frontal recess was also observed, so endoscopic ethmoidectomy, sphenoidotomy, and frontal recess dissection were performed. The patient has not had a recurrence of his cholesteotoma since his surgery 13 years ago, although he is still a chronic sinus sufferer who requires medical therapy.
3. History and diagnosis of maxillary sinus cholesteatoma The first documented case of a maxillary sinus cholesteatoma was made by Hutcheon in 1941 [2,5]. Although benign, cholesteatomas have an uncontrolled, erosive nature of growth, which led Hutcheon to consider them malignant [2,5]. Accordingly, the clinical presentation and radiologic characteristics of a cholesteatoma in the maxillary sinus are difficult to distinguish from those of a malignancy. Clinical features include nasal obstruction, rhinorrhea, headache, cheek pain, and cheek and buccal swelling; all symptoms resulting from expansion of the lesion [5]. Before significant cheek swelling develops, maxillary sinus lesions generally first cause obstruction that leads to sinusitis, so cholesteatomas are often complicated by a concomitant infection [6]. Pro-inflammatory TNFα levels have been shown to correlate with the severity of bone erosion in temporal bone cholesteatomas [4]. On radiological examination, a cholesteatoma appears as a sharply circumscribed round or ovoid bony defect with smooth marginal sclerosis [2,4]. This presentation is very similar to that of a mucocele [2]. On CT, a non-enhancing, homogenous lesion with irregular borders that is less dense than brain tissue will be seen [2,6]. The diagnosis of cholesteatoma is confirmed on histological examination which shows fully differentiated, stratified squamous epithelium with a central core composed of lamellar sheets of anucleate keratin squames on connective tissue infiltrated with inflammatory cells [2,4]. The differential diagnosis of a maxillary sinus cholesteatoma includes nonneoplastic lesions (mucocele, mucus retention cyst, pseudocyst, pyocele), benign neoplastic lesions (papilloma, mucin impaction tumor, meningioma, schwannoma, chondroma, hemangioma, chordoma, juvenile nasal angiofibroma, fibrous dysplasia), and malignant neoplastic lesions (squamous cell carcinoma, esthesioneuroblastoma, salivary gland tumor, sarcoma, ameloblastoma) [2].
4.
Pathophysiology of cholesteatoma formation
Cholesteatomas can be congenital or acquired. Congenital cholesteatomas are thought to be a result of misplaced epithelial
291
cell rests that develop during the embryonic stage [2–4]. The formation of these rests is proposed to result from failure of two ectodermal surfaces to separate completely, leaving an epithelial remnant that fails to involute and later proliferates [3,6]. The most widely accepted theory on the pathogenesis of an acquired cholesteatoma is traumatic implantation of squamous epithelium. Numerous reports have documented the occurrence of paranasal sinus cholesteatomas at the site of previous injury or after nasal or sinus surgery [4,5]. Two other theories on the origin of acquired cholesteatomas for which there is scarce supporting scientific evidence are migration of squamous epithelium to an area that does not inherently contain squamous epithelium and epithelial metaplasia following chronic inflammation [4].
5.
Treatment of maxillary sinus cholesteatoma
The treatment for a maxillary sinus cholesteatoma is surgery, specifically the Caldwell-Luc procedure [2,5]. However, with advancements in techniques, functional endoscopic sinus surgery can afford a successful outcome, as was the case in this patient. It is suggested that endoscopic surveillance be incorporated after surgical excision as is done with inverted papilloma. The wall of the mass must be completely removed to prevent further erosion of surrounding structures and to prevent recurrence. Cholesteatomas in the temporal bone have reoccurred due to the presence of residual epithelium, and it is presumed that the pathophysiologic mechanisms of growth of a cholesteatoma are independent of the lesion location [2]. One case of a recurrence in the frontal sinus after incomplete removal has been reported in the literature [5]. Adequate drainage and sinusotomy are also recommended to prevent long-term complications. CT serves a main role in follow-up as it can exclude recurrence.
REFERENCES
[1] Hammami B, Mnejja A, Chakroun A, et al. Cholesteatoma of the frontal sinus. Eur Ann Otorhinolaryngol Head Neck Dis 2010;127:213–6. [2] Viswanatha B, Nayah L, Karthik S. Cholesteatoma of the maxillary sinus. Ear Nose Throat J 2007;86:351–3. [3] Hartman JM, Stankieewicz JA. Cholesteatoma of the paranasal sinuses: case report and review of the literature. Ear Nose Throat J 1991;70:719–25. [4] Hansen S, Sørensen CH, Stage J, et al. Massive cholesteatoma of the frontal sinus: case report and review of the literature. Auris Nasus Larynx 2007;34:387–92. [5] Vaz F, Callanan V, Leighton S, et al. Congenital maxillary sinus cholesteatoma. Int J Pediatr Otorhinolaryngol 2000;52:283–6. [6] Yağci AB, Kara CO, Karabulut N, et al. Horseshoe maxillary sinus: CT of a unique case with cholesteatoma. Eur J Radiol 2003;48:5–7.
Available online at www.sciencedirect.com
ScienceDirect www.elsevier.com/locate/amjoto
Cholesteatoma of the maxillary sinus: a case report and review of the literature☆,☆☆ Jeanie Sozansky, BS a , Jordan S. Josephson, MD b,⁎ a b
Northeast Ohio Medical University, 4209 Ohio 44, Rootstown, Ohio Clinical Assistant Professor, Hofstra North Shore-LIJ School of Medicine, 500 Hofstra University, Hempstead, New York
ARTI CLE I NFO
A BS TRACT
Article history:
Cholesteatoma is a relatively common disease entity within the middle ear cavity, but it is
Received 28 July 2014
rarely found in the paranasal sinuses. We describe a rare case of cholesteatoma in the maxillary sinus of an elderly man presenting with recurrent sinus infections and nasal obstruction refractory to medical treatment. The patient was treated using functional endoscopic sinus surgery with successful removal of the cholesteatoma. He has no recurrence of the cholesteotoma at a 13 year follow-up. We review the literature and history on maxillary cholesteatomas and discuss the theories on the pathogenesis of cholesteatoma formation. We propose functional endoscopic sinus surgery with maxillary antrostomy and marsupialization of the cholesteatoma as a primary treatment option for a maxillary cholesteatoma. © 2015 Elsevier Inc. All rights reserved.
1.
Introduction
Cholesteatoma is a relatively common disease entity within the middle ear cavity, but it is rarely found in the paranasal sinuses. A review of the English literature reported less than 30 cases of a cholesteatoma in a paranasal sinus [1]. Of these limited cases, the most common location was the frontal sinus, followed by the ethmoid sinus, and finally the maxillary sinus [2]. Paranasal cholesteatomas are generally unilateral [3]. The origin of the term cholesteatoma traces back to Müller who in 1838 described the presence of cholesterol crystals in a cavity lined with squamous epithelium and filled with layers of dense, squamated keratin [2,4]. This term is a misnomer as Virchow noted in 1854 that cholesterol is not an essential component of this entity [2,4]. More appropriate terminology that is used to refer to a cholesteatoma is keratoma, keratocyst, epidermoid tumor, or epidermoid cyst [5,6].
☆
2.
Case report
A 72-year-old Caucasian male presented with recurrent sinus infections. No facial deformity was noted. He complained of chronic nasal obstruction and post nasal drip with colored mucopus. He had been treated with antibiotics, steroids, nasal sprays, and irrigation with no improvement of symptoms. The CT scan showed right maxillary sinus opacification consistent with nasal polyposis and erosion of the right medial maxillary wall. As medical treatment failed to control the symptoms, the patient opted for surgery. Functional endoscopic sinus surgery was performed and a mass was visualized eroding the right medial wall of the maxillary sinus with purulent material and keratinaceous debris present. The mass was biopsied and specimens were sent to pathology, which revealed keratinaceous material consistent with cholesteatoma as well as some filamentous organisms, possibly Actinomyces. A middle meatal
Financial Disclosures: None. Grant support: None. ⁎ Corresponding author at: 205 East 76th Street, Suite M-1, New York, NY 10021. Tel.: +1 212 717 1773. E-mail addresses: [email protected] (J. Sozansky), [email protected] (J.S. Josephson).
☆☆
http://dx.doi.org/10.1016/j.amjoto.2014.10.025 0196-0709/© 2015 Elsevier Inc. All rights reserved.
AM ER IC AN JOURNAL OF OT OLARYNGOLOGY – H E A D A N D NE CK M E D IC IN E A ND S U RGE RY 3 6 (2 0 1 5) 2 90– 2 9 1
antrostomy with marsupialization of the cholesteatoma and removal of maxillary sinus debris were carried out. Infected bone pertaining to the ethmoid sinus, sphenoid sinus, and frontal recess was also observed, so endoscopic ethmoidectomy, sphenoidotomy, and frontal recess dissection were performed. The patient has not had a recurrence of his cholesteotoma since his surgery 13 years ago, although he is still a chronic sinus sufferer who requires medical therapy.
3. History and diagnosis of maxillary sinus cholesteatoma The first documented case of a maxillary sinus cholesteatoma was made by Hutcheon in 1941 [2,5]. Although benign, cholesteatomas have an uncontrolled, erosive nature of growth, which led Hutcheon to consider them malignant [2,5]. Accordingly, the clinical presentation and radiologic characteristics of a cholesteatoma in the maxillary sinus are difficult to distinguish from those of a malignancy. Clinical features include nasal obstruction, rhinorrhea, headache, cheek pain, and cheek and buccal swelling; all symptoms resulting from expansion of the lesion [5]. Before significant cheek swelling develops, maxillary sinus lesions generally first cause obstruction that leads to sinusitis, so cholesteatomas are often complicated by a concomitant infection [6]. Pro-inflammatory TNFα levels have been shown to correlate with the severity of bone erosion in temporal bone cholesteatomas [4]. On radiological examination, a cholesteatoma appears as a sharply circumscribed round or ovoid bony defect with smooth marginal sclerosis [2,4]. This presentation is very similar to that of a mucocele [2]. On CT, a non-enhancing, homogenous lesion with irregular borders that is less dense than brain tissue will be seen [2,6]. The diagnosis of cholesteatoma is confirmed on histological examination which shows fully differentiated, stratified squamous epithelium with a central core composed of lamellar sheets of anucleate keratin squames on connective tissue infiltrated with inflammatory cells [2,4]. The differential diagnosis of a maxillary sinus cholesteatoma includes nonneoplastic lesions (mucocele, mucus retention cyst, pseudocyst, pyocele), benign neoplastic lesions (papilloma, mucin impaction tumor, meningioma, schwannoma, chondroma, hemangioma, chordoma, juvenile nasal angiofibroma, fibrous dysplasia), and malignant neoplastic lesions (squamous cell carcinoma, esthesioneuroblastoma, salivary gland tumor, sarcoma, ameloblastoma) [2].
4.
Pathophysiology of cholesteatoma formation
Cholesteatomas can be congenital or acquired. Congenital cholesteatomas are thought to be a result of misplaced epithelial
291
cell rests that develop during the embryonic stage [2–4]. The formation of these rests is proposed to result from failure of two ectodermal surfaces to separate completely, leaving an epithelial remnant that fails to involute and later proliferates [3,6]. The most widely accepted theory on the pathogenesis of an acquired cholesteatoma is traumatic implantation of squamous epithelium. Numerous reports have documented the occurrence of paranasal sinus cholesteatomas at the site of previous injury or after nasal or sinus surgery [4,5]. Two other theories on the origin of acquired cholesteatomas for which there is scarce supporting scientific evidence are migration of squamous epithelium to an area that does not inherently contain squamous epithelium and epithelial metaplasia following chronic inflammation [4].
5.
Treatment of maxillary sinus cholesteatoma
The treatment for a maxillary sinus cholesteatoma is surgery, specifically the Caldwell-Luc procedure [2,5]. However, with advancements in techniques, functional endoscopic sinus surgery can afford a successful outcome, as was the case in this patient. It is suggested that endoscopic surveillance be incorporated after surgical excision as is done with inverted papilloma. The wall of the mass must be completely removed to prevent further erosion of surrounding structures and to prevent recurrence. Cholesteatomas in the temporal bone have reoccurred due to the presence of residual epithelium, and it is presumed that the pathophysiologic mechanisms of growth of a cholesteatoma are independent of the lesion location [2]. One case of a recurrence in the frontal sinus after incomplete removal has been reported in the literature [5]. Adequate drainage and sinusotomy are also recommended to prevent long-term complications. CT serves a main role in follow-up as it can exclude recurrence.
REFERENCES
[1] Hammami B, Mnejja A, Chakroun A, et al. Cholesteatoma of the frontal sinus. Eur Ann Otorhinolaryngol Head Neck Dis 2010;127:213–6. [2] Viswanatha B, Nayah L, Karthik S. Cholesteatoma of the maxillary sinus. Ear Nose Throat J 2007;86:351–3. [3] Hartman JM, Stankieewicz JA. Cholesteatoma of the paranasal sinuses: case report and review of the literature. Ear Nose Throat J 1991;70:719–25. [4] Hansen S, Sørensen CH, Stage J, et al. Massive cholesteatoma of the frontal sinus: case report and review of the literature. Auris Nasus Larynx 2007;34:387–92. [5] Vaz F, Callanan V, Leighton S, et al. Congenital maxillary sinus cholesteatoma. Int J Pediatr Otorhinolaryngol 2000;52:283–6. [6] Yağci AB, Kara CO, Karabulut N, et al. Horseshoe maxillary sinus: CT of a unique case with cholesteatoma. Eur J Radiol 2003;48:5–7.
