Cholesteatoma of the maxillary sinus.

Abstract

Cholesteatoma of the maxillary sinus is a rare condition, but it should be considered in the differential diagnosis of any slowly expanding lesion of the maxillary sinus. Because the keratinizing squamous epithelium continues to desquamate and expand, thereby causing erosion of the surrounding structures, the cholesteatoma sac must be removed completely. We describe a case of cholesteatoma of the maxillary sinus in an 18-year-old woman.

Introduction

Cholesteatoma of the maxillary sinus occurs when the normal respiratory epithelium that lines the sinus is partially or totally replaced by hyperkeratotic squamous epithelium; this in turn leads to formation of lamellar sheets of keratin--that is, a cholesteatoma. In the maxillary sinus, cholesteatoma--which is also known as keratoma, primary epidermoid tumor, epidermoid cyst, and keratocyst--is rare. (1-3) In a review of the literature published in 1992, Storper and Newman found only 10 such cases. (1) One year earlier, Hartman and Stankiewicz reported that they found only 20 cases of cholesteatoma of any paranasal sinus. (4) In this article, we report a new case of maxillary sinus cholesteatoma.

Case report

An 18-year-old woman presented with a painless swelling in the left cheek of 1 year's duration. The swelling had begun slowly in the anterolateral part of the left cheek, and it had become gradually larger over the 4 months preceding her visit. Her edema was associated with a left-sided nasal obstruction also of 1 year's duration. The obstruction was mild initially, but it eventually became complete. The patient also reported a 2-year history of intermittent headaches that were relieved with medication. She had no history of nasal discharge, facial trauma, or nasal or sinus surgery.

On physical examination, a soft 3 x 3-cm swelling could be seen over the anterolateral part of the left cheek (figure 1, A). The swelling was nontender and not very mobile. Examination of the left nasal cavity revealed that the lateral wall had encroached onto the septum and had completely obstructed the nasal cavity. Inspection of the oral cavity detected a bulging of the hard palate on the left side (figure 1, B). Findings on the remainder of the ENT examination and systemic examination were normal, as were the results of routine blood and urine testing.

[FIGURE 1 OMITTED]

Computed tomography (CT) of the paranasal sinuses demonstrated a large, hypodense, expansile lesion in the left maxilla (figure 2). Fluid density was uniform. Marked expansion and remodeling of the bony walls of the sinus were seen. All sinus walls were severely thinned. The lateral wall of the left maxilla was deficient, and the mass bulged into the soft tissue of the cheek. Medially, the expanded sinus had encroached into the nasal fossa abutting the nasal septum. The left maxillary alveolus was severely thinned, and the roots of the teeth were eroded. The expanded maxillary sinus had obliterated the left infundibulum, the hiatus semilunaris, the middle turbinate, and the middle meatus. The left nasal turbinates were compressed and remodeled. No extension into the orbit was seen, and the other sinuses were normal.

[FIGURE 2 OMITTED]

With the patient under general anesthesia, the swelling was approached through a left sublabial incision. A grayish-white mass was seen protruding through the eroded anterolateral wall of the maxillary sinus. The anterior wall of the maxillary sinus was opened, revealing that this sinus was filled with a cyst that contained grayish-white flakes (figure 3). The sac and its contents were removed in their entirety. A wide inferior meatal antrostomy was performed.

[FIGURE 3 OMITTED]

Histopathologic examination of the mass revealed a fibrocollagenous wall that was lined with stratified squamous epithelium. The epithelium was overlaid with lamellar layers of keratin (figure 4). The histopathologic diagnosis was a cholesteatoma.

[FIGURE 4 OMITTED]

The patient was followed for 6 months, and no evidence of recurrence was seen.

Discussion

In addition to the paranasal sinuses, cholesteatomas also arise elsewhere in the cranium (including the temporal bone, orbit, and mandible), as well as in the skin, breast, kidney, and central nervous system. (1) Cholesteatoma was first described by Cruveilhier in 1829 as a "pearly tumor" by virtue of its highly refractive and nodular surface. (5) The term cholesteatoma was coined by Muller in 1838 to describe the presence of cholesterol crystals in a cavity lined with squamous epithelium and filled with layers of dense, squamated keratin. (6) In 1854, Virchow noted that cholesterol is not a necessary component of this entity. (7) In 1928, Critchley and Ferguson proposed that the term epidermoid more suitably conveys the epithelial character of this growth. (8)

In 1916, Haeggstrom reported the first case of frontal sinus cholesteatoma in a patient with proptosis and diplopia. (9) The first case of maxillary sinus cholesteatoma was reported by Hutcheon in 1941. (10) He noted that "[one of] the interesting features of this case is that the symptoms and signs were more suggestive of carcinoma of the antrum and adjacent nasal wall." This finding means that despite its rarity, this non-neoplastic entity should at least be considered in the differential diagnosis of any erosive lesion of the maxillary sinus. Of all the sinuses, the most common site of origin is the frontal sinus followed by the ethmoid sinus; as mentioned, maxillary sinus involvement is rare. (1-4)

Pathogenesis. The pathogenesis of cholesteatoma--which can be either congenital or acquired--has been debated for more than century. Four basic theories have been proposed to explain its development:

* The theory of congenital epithelial rests (1854). Proponents of this theory--notably Remark (11) and Bucy (12)--believe that cholesteatomas arise from misplaced epithelial rests that develop during the embryonic stage.

* The metaplasia theory (1873). According to this theory proposed by Wendt, the nonkeratinizing squamous epithelium that lines a cavity undergoes a metaplastic change, possibly in response to infection, and it begins to produce keratin. (13)

* The immigration theory (1888). Habermann theorized that cholesteatoma is caused by the migration of keratinizing squamous epithelium into an area where it is not usually found. (14)

* The implantation theory (1928). Ewing proposed that cholesteatomas arise secondary to the direct entry of epithelium during trauma. (15) Indeed, cholesteatomas have been reported to occur at the site of a previous injury and after nasal or sinus surgery. The epithelium continues to desquamate and form an expanding, destructive cyst.

Other characteristics. While cholesteatomas are not biologically neoplastic, they have the capacity to erode bone and expand into adjacent areas. The capacity to erode bone has been attributed to enzymes. (1-3,16)

Macroscopically, cholesteatomas appear as cyst-like structures. Microscopically, cholesteatoma capsules exhibit fully differentiated, stratified squamous epithelium on connective tissue. The central core of a cholesteatoma is made up of anucleate keratin squamae. The subepithelial connective tissue is of varying thickness, and it contains inflammatory infiltrate and fibroblasts. (17)

Symptoms and signs. The clinical presentation and radiologic characteristics of maxillary sinus cholesteatoma are difficult to distinguish from those of malignancy (1):

* Clinicalpresentation. The complications and presenting symptoms of cholesteatoma, which may occur as a result of the interplay between bone erosion and infection, are determined by the anatomic relationship of the maxillary antrum (including the orbit, nasal cavity, teeth, and mouth) and the pterygomaxillary space. (3) Symptoms are caused by the expansion of the lesion. As the cholesteatoma grows, pain becomes more severe. As the lesion extends toward the ostiomeatal complex, rhinorrhea and sinusitis may develop. As it erodes the anterolateral wall of the antrum, facial swelling becomes noticeable and tenderness increases. Inferior erosion produces palatal swelling, and superior erosion produces proptosis, chemosis, erythema, and restriction of extraocular movements. Erosion into the nasal vestibule produces nasal obstruction. (1)

* Radiologic characteristics. Atypical cholesteatoma appears as a sharply circumscribed bony defect with smooth marginal sclerosis. This appearance closely mimics that of the maxillary mucocele, which must be considered in the differential diagnosis. (18) On CT, a cholesteatoma appears as a nonenhancing, expansile, homogenous lesion. On magnetic resonance imaging, it exhibits fairly low signal intensity on Tl-weighted imaging and high intensity on T2-weighted imaging. (19)

Differential diagnosis. The differential diagnosis of a maxillary sinus cholesteatoma includes both non-neoplastic lesions (mucocele, mucus retention cyst, pseudocyst, and pyocele) and neoplastic lesions. Neoplastic lesions may be benign or malignant. Benign lesions include papilloma, mucin impaction tumor, meningioma, schwannoma, chondroma, hemangioma, chordoma,juvenile nasal angiofibroma, and fibrous dysplasia. Malignant lesions include squamous cell carcinoma, esthesioneuroblastoma, salivary gland tumor, sarcoma, and ameloblastoma. (1,3,18)

Treatment. The appropriate treatment for cholesteatoma is surgery. The procedure of choice for maxillary sinus cholesteatoma is Caldwell-Luc surgery. (l-3) The wall of the cholesteatoma mass--which is the only living, growing portion of the mass--should be completely removed to stop further erosion of the surrounding structures and to prevent reccurence. (2) Although no case of recurrence of a paranasal sinus cholesteatomahas actually been reported, it is assumed that its pathophysiologic mechanisms of growth are similar to those of a temporal bone cholesteatoma; because the presence of residual epithelium is the usual cause of recurrence of a temporal bone cholesteatoma, it is likely that a residual focus will cause a recurrence in the maxillary sinus, as well. (1)

To avoid long-term complications, adequate drainage and sinusotomy for postoperative follow-up are recommended. CT plays an important role in follow-up. (4)

References

(1.) Storper IS, Newman AN. Cholesteatoma of the maxillary sinus. Arch Otolaryngol Head Neck Surg 1992;118:975-7.

(2.) Campanella RS, Caldarelli DD, Friedberg SA. Cholesteatoma of the frontal and ethmoid areas. Ann Otol Rhinol Laryngol 1979;88:518-23.

(3.) Pogorel BS, Budd EG. Cholesteatoma of the maxillary sinus: A case report. Arch Otolaryngol 1965;82:532-4.

(4.) Hartman JM, Stankiewicz JA. Cholesteatoma of the paranasal sinuses: Case report & review of the literature. Ear Nose Throat J 1991;70: 719-25.

(5.) Cruveilhier LJB. Anatomie Pathologique du Corps Humain. Vol. 1. Paris: J.B. Bailliere; 1829.

(6.) Muller J. Von der geschichteten perlmutter glanzenden Fettgeschwulst. In: Uber den feineren Bau und die Formen der Krankhaften Geschwulste. Berlin: G. Reimer; 1838:49-54.

(7.) Virchow R. Uber Perlgeschwulste. Arch Klin Med 1854;8:371.

(8.) Critchley M, Ferguson FR. The cerehrospinal epidermoids (cholesteatoma). Brain 1928;51:336-41.

(9.) Haeggstrom AA. Cholesteatoma removed from the maxillary sinus. Hygiea 1916;78:1122.

(10.) Hutcheon JR. A case of rhinolith combined with cholesteatoma of the maxillary antrum. Med J Aust 1941;1:451.

(11.) Remark R. Ein Beitrag zur Entwickel ungsgesch ichte der Krebshafter Geschwulste. Arch Klin Med 1854;6:170.

(12.) Bucy PC. Intradiploic epidermoid (cholesteatoma) of the skull. Arch Surg 1935;31:190-7.

(13.) Wendt H. Desquamative Entzundung des Mittelohrs ("Cholesteatom des Felsen beines" der Auroren). Arch Heilkd 1873; 14:428-46.

(14.) Habermann J. Zur Entstehung des Cholesteatoms des Mittelohres. Arch Ohren Nasen Kehlkopfheilkd 1888;27:42-50.

(15.) Ewing J. A Treatise on Tumors. Philadelphia: W.B. Saunders; 1928.

(16.) Chole RA, Choo MJ. Chronic otitis media, mastoiditis, and petrositis. In: Cummings CW, Fredrickson JM, Harker LA, et al, eds. Otolaryngology-Head and Neck Surgery. 3rd ed. St. Louis: Mosby; 1998:3026-46.

(17.) Youngs R. Chronic suppurative otitis media--cholesteatoma. In: Ludman H, Wright T, eds. Diseases of the Ear. 6th ed. London: Arnold; 1998:386-97.

(18.) Wenig BL, Sciubba JJ, Zielinski BZ, et al. Mucin impaction tumor of the paranasal sinuses: A new clinical entity? Laryngoscope 1983;93:621-6.

(19.) Valvassori GE. Imaging of the temporal bone. In: Glasscock ME, Gulya AJ, eds. Shambaugh's Surgery of the Ear. 5th ed. Hamilton, Ont.: B.C. Decker; 2003:227-59.

From the ENT Department, Victoria Hospital, Bangalore Medical College, Bangalore, Karnataka, India.

Reprint requests: Dr. Borlingegowda Viswanatha, #716, 10th Cross, 5th Main, MC Layout, Vijayangar, Bangalore-560 040, Karnataka, India. Phone: [phone]; fax: [phone]; e-mail: [e-mail]