Case Report
Cholesteatoma of the Maxillary Sinus
Watcharaporn Bourchom and Pariyanun Jaruchinda*
Department of Otorhinolaryngology, Phramongkutklao Hospital, Thailand
*Corresponding author: Pariyanun Jaruchinda, Department of Otorhinolaryngology, Phramongkutklao Hospital, 315 Ratchawithi Rd, Ratchathewi, Bangkok 10400, Thailand
Published: 15 Aug, 2017
Cite this article as: Bourchom W, Jaruchinda P.
Cholesteatoma of the Maxillary Sinus.
Ann Clin Case Rep. 2017; 2: 1417.
Abstract
Cholesteatoma is a relatively common pathology in the middle ears and mastoid cavity, but is a rare disease for the paranasal sinuses, especially in the maxillary sinus. It should be considered in the differential diagnosis of slowly expanding lesions of the sinuses and is difficult to distinguish from malignancy. The author describes a rare case of cholesteatoma in the maxillary sinus of an elderly woman presenting swelling at hard palate over 3 months. The swelling began at the right side hard palate and gradually enlarged over 2 months. She had right side facial swelling and nasal obstruction. Several biopsies were performed under local anesthesia using the transnasal, sinuscope and sublabial approaches (Caldwell-luc). The last histopathological report showed consistency with cholesteatoma. The patient was operated by medial maxillectomy and complete tumor removal. The patient experienced disease free survival about 18 months and afterwards she died from cerebrovascular disease. This is the second case in Asia and first case reported in Southeast Asia.
Keywords: Cholesteatoma; Maxillary sinus; Medial maxillectomy; Caldwell-luc
Introduction
Cholesteatoma of the maxillary sinus occurs when the normal respiratory epithelium (ciliated pseudostratified columnar epithelium) that lines the sinus is replaced by keratinized squamous epithelium. The first reported cholesteatoma of the maxillary sinus was by Hutcheon et al. [1] in 1941. He considered it a malignant lesion because of its erosive nature and rapid uncontrolled growth. Therefore, the differential diagnosis of any lesion within the maxillary sinus is important. Current theories of pathogenesis suggest that cholesteatoma can be either congenital or acquired.
Case Presentation
A 74-year-old woman with underlying dyslipidemia, gouty arthritis, hypertension and deep
vein thrombosis (DVT) of the left leg presented with swelling at right side hard palate over three
months. She had right side facial swelling and nasal obstruction. She visited the dentist clinic
because she could not fit the obtulator in her mouth and lost one tooth about four month earlier. She
had no history of facial trauma, nasal discharge, chronic sinusitis or sinonasal surgery. On physical
examination, she presented right facial swelling, a small ulcer on the right side of her face (Figure 1) and bulging hard palate on the right side 3 cm. in diameter. Examination of the right nasal cavity
revealed that the medial wall had encroached on the septum and had completely obstructed the
nasal cavity. Eye examination and facial sensation were normal. The other ENT examinations and
blood tests were normal.
Computed Tomography (CT) scan of the paranasal sinuses
demonstrated a large heterogenous enhanced lesion at the right hard
palate and maxillary sinus with bony destruction measuring 5.5 cm x
5.3 cm x 5 cm in transverse, AP and vertical diameters, respectively
(Figure 2). Medially, the expanded sinus had encroached the nasal
cavity abutting the nasal septum and contralateral side. Laterally,
involvement and bony destruction was observed of the right maxillary
sinus. Inferiorly, a bony density lesion in the right maxillary sinus
involved the right side of the hard palate. Anteriorly, it extended
beyond the right maxilla and maxillary sinus, from the nasal cavities
to the subcutaneous tissue. No extension in the orbit was seen, and
the other sinuses were normal.
Several biopsies were performed under local anesthesia
using transnasal and sinuscope through the inferior meatus. The
histopathological report showed squamous mucosal hyperplasia,
acute and chronic inflammation and no neoplasm or dysplastic
change was seen. The last biopsy using the sublabial approach
(Caldwell-luc) was performed. The histopathological report showed
consistency with cholesteatoma (Figure 3). A medial maxillectomy
was performed under general anesthesia. Grayish-white debris was
seen in the right maxillary sinus protruding through the eroded
anteromedial wall and floor of the maxillary sinus. The anterior wall
of the maxillary sinus was opened and one tooth was found in the
right maxillary sinus. The tooth, sac and its contents were removed.
The patient was followed up for 18 months and no evidence of
recurrence was observed. Afterwards she died from cerebrovascular
disease, so the patient had disease free survival for about 18 months.
Figure 1
Figure 2
Figure 3
Figure 3
The histopathological report show hyperplastic squamous epithelium with hyperkeratosis, focal hypergranulosis, acute and chronic inflammation, consistent with cholesteatoma of maxillary sinus.
Discussion
Cholesteatoma of the maxillary sinus is a rare condition.
Hutcheon [1] reported the first case of maxillary sinus in 1941. 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. Haeggstrom
[2] reported the first case of frontal sinus cholesteatoma in a patient
with proptosis and diplopia in 1961. The frontal sinus is the most common site of origin followed by the ethmoid sinus, while maxillary sinus involvement is rare [3-6]. This patient is the first reported in
Southeast Asia and the second case in Asia.
The pathogenesis of cholesteatoma [7] can be either congenital
or acquired. Four basic theories have been reported as described
below. First, the theory of congenital epithelial rests (1854), Remark
and Bucy statet that cholesteatomas arise from misplaced epithelial
rests that develop during the embryonic stage. Second, the metaplasia
theory (1873), Wendt theorized that the nonkeratinizing squamous
epithelium that lines a cavity undergoes a metaplastic change,
possibly in response to infection and begins to produce keratin.
Third, the immigration theory (1888), Habermann hypothesized that
cholesteatoma is caused by the migration of keratinizing squamous
epithelium in an area where it would not usually be found. Last, the
implantation theory (1928), Ewing proposed that cholesteatomas
arise secondary to the direct entry of epithelium during trauma.
Cholesteatomas had been reported to occur at the site of a previous
injury and after nasal or sinus surgery.
Regarding other characteristics, the cholesteatomas are not
biologically neoplastic. It has the capacity to erode bone and expand
into adjacent areas. The capacity to erode bone has been attributed
to enzymes [3-5,8]. In 1962, Harris [9] demonstrated two proteolytic
enzymes; leucine aminopeptidase and a nonspecific esterase in the
subepithelial layer of cholesteatomas. Recently, further investigations
in bony erosions of cholesteatomas had been highlighted the role of
cytokines such as TNFα. These cytokines were thought to act directly
on bone and indirectly by stimulating the release of proteolytic
enzymes. These studies had only been performed on temporal
bone cholesteatomas and further investigation of paranasal sinus
cholesteatomas is required [15]. The cause of cholesteatoma in
this patient should have been from immigration or implantation
theory because the tooth was founded in the maxillary sinus. The
keratinize squamous epithelium in the oral cavity may migrate to the
maxillary sinus. The clinical presentations are difficult to distinguish
from those of malignancy [3]. The presenting symptoms, possibly
occurring as a result of the interaction between bone erosion and
infection, determined by the anatomic relationship of the maxillary antrum (including the orbit, nasal cavity, teeth and mouth) and the pterygomaxillary space [5]. Symptoms are caused by the expansion
of the lesion. As the cholesteatoma grows, pain becomes more
severe. The differential diagnosis includes both non-neoplastic
lesions (mucocele, mucus retention cyst, pyocele and pseudocyst)
and neoplastic lesions may be benign or malignant. The appropriate
treatment for cholesteatoma is surgery. The procedure of choice for
maxillary sinus cholesteatoma is Caldwell-Luc surgery [3-5]. The wall
of the cholestealoma should be completely removed to stop further
erosion of the surrounding structures and to prevent recurrence [2].
To avoid long term complications, adequate drainage and sinusotomy
for postoperative follow-up are recommended [6].
This patient’s right medial maxillectomy was operated on because
one time she received the Caldwell-luc operation and we found rather
large tumor involved superiorly nearly at the floor of orbit and had a
tooth in the lateral part of maxillary sinus. The small ulcer on the skin
was cut at the surgical wound and the cholesteatoma sac was removed
including one tooth and open drainage for follow up.
Conclusion
Cholesteatoma of the maxillary sinus is a rare condition. Remembering and considering the differential diagnosis of slowly expanding and erosive lesions of the maxillary sinus is important. Distinguishing from paranasal sinus malignancy remains difficult. Appropriate treatment of cholesteatoma is surgery, complete removal and adequate drainage.
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