Cholesteatoma of the Ian S.

Storper, MD,

of the maxillary sinus, also known as keratoma, primary epidermoid tumor, epidermoid cyst, and \s=b\ Cholesteatoma

keratocyst, is a rare entity, with a clinical presentation and radiologic findings that are difficult to distinguish from those of malignancy. We report a case of cholesteatoma of the maxillary antrum that occurred in a 12-year-old boy without any history of predisposing factors. Multiple theories of pathogenesis have been proposed throughout history; currently there are two accepted ones. We discuss these theories and present, along with the differential diagnosis,

a

review of the literature and the characteristic

radiologic and pathologic findings. While it is a rare entity, the diagnosis of cholesteatoma should be considered for any slowly expanding lesion of the maxillary antrum. (Arch Otolaryngol Head Neck Surg. 1992;118:975-977) the

of maxillary Choiesteatoma only reported Cruveilhier11 with

knowledge).110

10

cases

antrum is a rare entity, to date (to our may have been the first to de¬

scribe this lesion, terming it pearly tumor, in 1829. In 1838, Müller12 termed this lesion choiesteatoma and noted that it consists of layers of squamous epithelium, surrounding a mass of desquamated keratin. In 1854, Virchow13 realized that cholesterol is not a necessary part of this lesion, and suggested that the name be changed back to pearly tumor, as choiesteatoma is a misnomer. The fact that this is a mis¬ nomer has been echoed throughout the years, most recently in the otolaryngology literature by Hopp and Montgom¬ ery,14 who suggested abolishing the term and continuing the name keratoma, which is a more accurate and also com¬ monly used term. The first case of choiesteatoma of the maxillary antrum was reported by Hutcheon1 in 1941. His finding that "the most interesting feature of this case is that the symptoms and signs were much more suggestive of carcinoma of the antrum and adjacent nasal wall" make this nonneoplastic entity an important consideration in the differential diag¬ nosis of an erosive lesion of the maxillary sinus. Current theories of pathogenesis suggest that cholesteatomas may occur secondary to an inciting factor or a congenital rest.15"19 The age of our patient and the lack of predisposing factors suggest that his choiesteatoma may

Accepted

for publication June 2, 1992. From the Division of Head and Neck Surgery, UCLA Medical Center, Los Angeles, Calif. Reprint requests to Division of Head and Neck Surgery, UCLA Medical Center, 62-132 CHS, 10833 Le Conte Ave, Los Angeles, CA 90024\x=req-\ 1624 (Dr Storper).

Maxillary Sinus

Anita N. Newman, MD

have arisen from a congenital rest. One previous report10 has referenced a possible congenital origin for cho¬ iesteatoma of the maxillary sinus; this lesion, however, was found in a 55-year-old woman, and her complete medical history was not addressed. REPORT OF A CASE A 12-year-old boy was in his usual, excellent state of health un¬ til 1 month before presentation to his pediatrician, when his mother noted that he had left-sided facial swelling. He com¬ plained of pain in the region of his left maxilla and hard

palate. During

the 2 weeks before

presentation,

he

developed

¡eft-sided nasal obstruction, clear rhinorrhea, and intermittent

headaches. There was no history of epistaxis, fe¬ or chronic sinusitis. The patient was carried to term and his birth was uncom¬ plicated. The patient, his mother, and his father have never ingested illicit drugs. The patient's mother refrained from all known toxins during her pregnancy. The family history was unremarkable aside from diabetes mellitus type II on the pa¬ ternal side. On presentation to the Head and Neck Surgery Service, the pa¬ tient appeared healthy, with a moderate amount of left-sided fa¬ cial swelling and tenderness. He was afebrile with normal vital signs. A reddish, nonbleeding mass was seen in the left nasal vestibule, below the inferior turbinate. There was no chemosis, erythema, or proptosis of the left eye. The patient's extraocular movements, as well as facial sensation, were normal. The results of the remainder of the physical examination were unremarkable. A complete blood cell count, electrolyte levels, serologie test re¬ sults, and a roentgenogram of the chest were all normal. A computed tomographic (CT) scan was performed of the pa¬ tient's brain and paranasal sinuses, which revealed a large, inhomogeneous, expansile mass centered in the left maxillary an¬ trum, eroding the anterior, medial, and lateral walls, and extending into the infratemporal fossa (Fig 1). An upper left molar tooth was rotated, secondary to erosion of its root, and the floor of the orbit was slightly elevated. A few central calcifications were present. There was no intracranial ex¬ tension. Because of the rapid growth and aggressive appearance of this lesion on CT scan, it was originally thought that a neoplas¬ tic process was occurring. After informed consent was obtained from his mother, the patient was promptly taken to the operating room, where a left Caldwell-Luc antrostomy was performed without diffi¬ culty. Approximately 20 cc of keratinaceous debris was ob¬ tained from the left maxillary antrum; a thin surrounding wall was noted. Frozen section of the wall and its contents revealed squamous epithelium with keratinaceous debris, consistent with choiesteatoma. Permanent section confirmed the diagno¬ sis (Fig 2). There was no other lesion in the sinus by inspection and pal-

generalized

ver, facial trauma,

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been used to describe this lesion include keratocyst, epider¬ moid tumor, epidermoid cyst, and dysembryodysplasia. While most often occurring in the temporal bone, cholesteatomas have been found to originate in the kidney, testicle, skin, breast, central nervous system, cranial vault, orbit, mandi¬ ble, and paranasal sinuses.9 Of the sinuses, the most com¬ mon site of origin is the frontal sinus, with about 30 reported cases,14 then the ethmoidal sinus, and, rarely, the

maxillary sinus.9

Fig 1.—Computed tomogram revealing

erosive mass in left maxillary antrum, causing destruction of anterior, medial, and lateral walls. Mass extends into nasal vestibule. Left-sided facial enlargement is obvious.

A choiesteatoma consists of three components: a keratin debris core, squamous epithelium, and fibrous stroma, with the last two components being termed the matrix.20'™ While not biologically neoplastic, these lesions have the capacity to erode bone and expand into adjacent areas. The capacity to erode bone has been attributed to enzymes. In 1962, Harris21 demonstrated two proteolytic enzymes— leucine aminopeptidase and a nonspecific esterase—in the subepithelial layer of cholesteatomas. These enzymes are located at the squamous epithelial-subepithelial junction.22-23In the temporal bone, cholesteatomas have been shown to spread via active and inactive phases, the active phase consisting of an osteitic component as well as the enzymatic component.19,20 In the maxillary sinus, the method of spread has not been recorded in any more de¬ tail than cystic expansion, because of the rarity of its occurrence.

Cholesteatomas may be divided into primary and sec¬ ondary subtypes.24 A primary lesion appears to occur without any implantation or migration of squamous epi¬ thelium into the site of origin. A secondary lesion occurs as a result of squamous epithelium invading an area in which it did not originate. The pathogenesis of cholesteato¬

has been debated for more than a century. Current theories of generation include congenital epithelial rests, differentiation of pluripotential congenital embryonic mesenchyme, and implantation or epithelial migration. The theory of congenital epithelial rests, proposed by von Remak17 in 1854, may be used to explain primary cho¬ lesteatomas. Proponents of this theory believe that cho¬ lesteatomas develop from misplaced epithelial rests that occurred at the embryonic stage. This theory has been confirmed in laboratory research.25 The unremarkable medical history of our patient suggests that the cho¬ iesteatoma may have developed from a congenital rest. It is also possible, however, that inciting factors not noticed by the patient, ie, asymptomatic maxillary sinusitis, could have been present. The theory of differentiation of embryonic mesenchyme is similar to that of congenital rests, but states that cholesteato¬ mas occur secondary to the differentiation of embryonic mas

Fig 2.—Histologie section of left maxillary antrum contents. Right side of lesion shows stratified squamous epithelium. Left side shows kerati¬ naceous debris (hematoxylin-eosin, X40).

pation. The epithelium was carefully stripped from the surround¬ ing wall and removed in toto. The patient's postoperative course was unremarkable. He was still asymptomatic 8 months after his surgery.

COMMENT The term choiesteatoma was coined by Müller12 in 1838 to describe a cavity, lined with squamous mucosa filled with layers of desquamated keratin, in which cholesterol crys¬ tals were identified. Virchow13 noted in 1854 that choles¬ terol is not a necessary component of this entity. A com¬ pletely different entity from choiesteatoma, cholesterol granuloma, does contain cholesterol, which is generated from the breakdown of blood and other biological tissues.19 As has been suggested throughout recent medical his¬ tory, the term choiesteatoma is therefore a misnomer; kera¬ toma would be a better term.14·19 Other names that have

mesenchymal rests into epidermal tissue in inappropriate areas.14 While plausible, there is little scientific evidence to support this theory. If this theory were true, one would ex¬

pect to find lesions consisting of more tissue types than just squamous epithelium, which have not been found. The theory of epithelial invasion, first proposed by Habermann18 in 1888, states that the cause of cholesteato¬ mas is the migration of keratinizing squamous epithelium into an area in which it is not usually found. Causes of this migration include trauma and destruction of a natural barrier secondary, for example, to infection. Mills and Sy¬ camore3 have proposed that a cause of maxillary cho¬ lesteatomas may be the traumatic implantation of buccal mucosa into the maxillary antrum. Another theory was proposed in 1873 by Wendt,26 in which it was stated that

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the nonkeratinizing squamous epithelial lining of a cavity could undergo metaplastic change, possibly in response to infection, and begin to produce keratin. This theory has been refuted.14 If metaplasia of the squamous epithelium were to occur in response to infection, the maxillary sinus would be involved more frequently than the frontal; as mentioned earlier, the opposite holds true. Symptoms of maxillary cholesteatomas are caused by the expansile nature of the lesions. Pain increases in sever¬ ity as the lesion grows. As the lesion approaches the osteomeatal complex, rhinorrhea and sinusitis may de¬ velop. As it erodes the anterior and lateral walls of the an¬ trum, facial swelling becomes noticeable, and tenderness increases. Erosion inferiorly produces palatal swell¬ ing. Erosion superiorly produces proptosis, chemosis, erythema, and restriction of extraocular movements. Ero¬ sion into the nasal vestibule produces nasal obstruction; cribriform plate erosion produces frontal lobe signs.14 Radiographie studies may not be diagnostic, but are helpful in establishing the differential diagnosis and extent of involvement. At our institution, the first-line radiographic study for any paranasal sinus lesion is the CT scan. It allows visualization of bone in detail, as well as soft-tissue extension, and is cost-effective when compared with plain roentgenograms. On CT scan, a choiesteatoma appears as a nonenhancing, expansile lesion, less dense than brain, with irregular borders. It is usually inhomogeneous. On magnetic resonance images, cholesteatomas appear low intensity on Ti and high intensity on T2 sequences.24 Differential diagnosis of a maxillary sinus le¬ sion includes both nonneoplastic and neoplastic lesions.27·28 For the nonneoplastic lesions, mucocele, choiesteatoma, mucous retention cyst, pseudocyst, odontogenic keratocyst, and pyocele should be considered. Neoplastic lesions may be benign or malignant. Benign lesions include papilloma, inverting papilloma, mucin impaction tumor, men-

ingioma, schwannoma, hemangioma, chordoma, juvenile nasopharyngeal angiofibroma, osteoma, and fibrous dys¬ plasia. Malignant lesions include squamous cell carci¬ noma, esthesioneuroblastoma, salivary gland tumor, melanoma, sarcoma, and ameloblastoma. While symptom¬

atology, age, and CT findings can often provide a likely diagnosis, biopsy must be performed except under exten¬ uating circumstances (eg, obvious juvenile nasopharyn¬ geal angiofibroma) to establish a diagnosis of a mass lesion in the maxillary sinus. This point is exemplified by the fact that our patient was thought to have either rhabdomyosarcoma or lymphoma prior to biopsy. The surgical approach to a maxillary sinus mass lesion should be one that allows visibility, along with complete removal if the anatomic configuration and pathologic di¬ agnosis reveal a surgically amenable entity. The best exposure for biopsy is the Caldwell-Luc approach.2"10 For more aggressive lesions, establishment of the diagnosis by permanent section is recommended before resection is planned, if the time required to obtain the result is accept¬

able. A biopsy may be performed on lesions that extend into the nose or through the palate if a vascular lesion has been excluded from the differential diagnosis. Surgical approaches to cholesteatomas of the frontal, ethmoidal, and maxillary sinuses have been reviewed.29 We agree that appropriate treatment of a maxillary cho¬ iesteatoma is complete excision. While the incidence of re¬ currence of cholesteatomas of the paranasal sinuses has not been reported because of the rare occurrence of this lesion,

it is assumed that pathophysiologic mechanisms of growth are similar to those of cholesteatomas of the temporal bone. As residual epithelium is the usual reason for recurrence in temporal bone cholesteatomas, a residual focus will likely cause recurrence in the maxillary sinus. A

case

CONCLUSIONS of choiesteatoma of the maxillary sinus has been

presented. Etiologic theories, differential diagnosis, patho¬ logic findings, indicated radiologie studies, and surgical approaches have been discussed. It is important to remem¬ ber this rare entity in treating patients with erosive lesions of the maxillary antrum, as this lesion is easily treated and nonneoplastic, while it often appears as a high-grade ma¬ lignancy on radiographie imaging studies. 1. Hutcheon JR. A

case

References of rhinolith combined with cholesteatoma of the

antrum. Med J Aust. 1941;1:451. 2. Knapp RG. Cholesteatoma of the maxilla. J Oral Surg. 1946;4:236-237. 3. Mills PC, Sycamore EMK. Cholesteatoma of the maxillary antrum. J

maxillary

Laryngol Otol. 1958;72:580-583.

4. Fendel K. Cholesteatoma of the maxillary sinuses. Arch Ohr Nas Kehlkopfheilkd. 1962;179:320-326. 5. Pogorel BS, Budd EG. Cholesteatoma of the maxillary sinus. Arch Otolaryngol. 1965;82:532-534. 6. Baxter JJR. Cholesteatoma of the maxillary antrum. J Laryngol Otol. 1966;80:1059-1061. 7. Das SK. Cholesteatoma of the maxillary antrum. JLaryngolOtol. 1971;

85:397-400. 8. Flach M, Baerthold W, Rietschel M.

Entartung eines Oberkiefercholesteatoms. Monatsschr Ohrenheilkd. 1973;107:511-515. 9. Paaske PB. Cholesteatoma of the maxillary sinus. J Laryngol Otol.

1984;98:539-541. 10. Sadoff RS, Pliskin A. Cholesteatoma (keratoma) of the maxillary sinus: report of a case. J Oral Maxillofac Surg. 1989;47:873-876. 11. Cruveilhier LJB. Anatomie pathologique du corpus humani. Paris, France: Bailliere; 1829;2:1(book 2, plate 6). 12. M\l=u"\llerJ. Von der geschichteten perlmutterglanzenden Fettgeschwulst. In: Ueber den feinern Bau und die Krankhaften Geschwalste. Berlin, Germany: G Reimer; 1838:49-54. 13. Virchow R. \l=U"\berPerlgeschwulste. Arch Klin Med. 1854;8:371. 14. Hopp ML, Montgomery WW. Primary and secondary keratomas of the frontal sinus. Laryngoscope. 1984;94:628-632. 15. Cawthorne T. Congenital cholesteatoma. Arch Otolaryngol. 1963;78: 248-252. 16. Derlacki EL, Clemis JD. Congenital cholesteatoma of the middle ear and mastoid. Ann Otol Rhinol Laryngol. 1965;74:706-727. 17. von Remak R. Ein Beitrag zur Entwickelungsgeschichte der kreb-

shaften Geschwulste, Deutsches. Arch Klin Med. 1854;6:170-176. 18. Habermann J. Zur Entstehung des Cholesteatoms des Mittelohrs. Arch Klin Exp Ohr Nas Kehlkopfheilkd. 1888;27:42-50. 19. Schuknecht HF. Pathology of the Ear. Cambridge, Mass: Harvard Uni-

versity Press;

1976:225-228. 20. Friedmann I. Pathology of the Ear. Boston, Mass: Blackwell Scientific Publications Inc; 1974. 21. Harris AJ. Cholesteatosis and chronic otitis media. Laryngoscope.

1962;72:954-980. 22. Abramson MJ. Collagenolytic activity in middle ear cholesteatoma. Ann Otol Rhinol Laryngol. 1969;78:112-124. 23. Abramson MJ, Gross J. Further studies on a collagenase in middle ear cholesteatoma. Ann Otol Rhinol Laryngol. 1971;80:177-185. 24. Glasscock ME III, Shambaugh GE Jr. Pathology and clinical course of inflammatory diseases of the middle ear. In:Glasscock ME III, Shambaugh GE Jr, eds. Surgery of the Ear. 4th ed. Philadelphia, Pa: WB Saunders Co; 1991: 167-193. 25. Cushing H. A

large epidermoid cholesteatoma of the parieto-temporal

region deforming the left hemisphere without cerebral symptoms. Surg Gynecol Obstet. 1922;34:557-566. 26. Wendt H. Desquamative Entzundung des Mittelohrs ('Cholesteatom

des Felsen beins' der Autoren). Arch Heilkd. 1873;14:428-446. 27. Chole RA. Acute and chronic infection of the temporal bone including otitis media with effusion. Otolaryngol Head Neck Surg. 1986;4:2963\x=req-\

2988. 28. Wenig BL, Sciubba JJ, Zielinski BZ, Stegnjajic A, Abramson AL. Mucin impaction tumor of the paranasal sinuses: a new clinical entity? Laryngoscope. 1983;93:621-626. 29. Hartman JM, Stankiewicz JA. Cholesteatoma of the paranasal sinuses: case report and review of the literature. Ear Nose Throat J. 1991;70: 719-725.

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Cholesteatoma of the maxillary sinus.

Cholesteatoma of the maxillary sinus, also known as keratoma, primary epidermoid tumor, epidermoid cyst, and keratocyst, is a rare entity, with a clin...
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