Neoplasms of the Ear Canal

Mallory Raymond, MD*

KEYWORDS
 External auditory canal  Neoplasm  Bony  Ceruminous  Cutaneous

KEY POINTS
 Osteoma and exostoses are related in histology, often asymptomatic, diagnosed inciden-
tally and might not require intervention.
 Benign and malignant glandular EAC neoplasms are rare, can present similarly and can
share histologic characteristics.
 Squamous cell carcinoma is the most common malignant neoplasm of the EAC and
should be considered in patients with unresolving otitis externa or non-healing ulcerative
or friable lesions.
 Sleeve resection of the EAC skin has a limited role in the surgical treatment of cutaneous
malignancies.

INTRODUCTION

Neoplasms of the EAC can be described by their cell of origin and further divided into
lesions arising primarily from within the EAC or involving the EAC secondarily from a
separate primary location. Given the histologic components of the EAC, primary
neoplasms can be broadly classified into bony, glandular and cutaneous (Table 1).
Neoplasms that secondarily involve the EAC include metastasis and those arising
from within the middle ear, mastoid or jugular foramen. This article will review these
classifications, focusing on histopathology, clinical characteristics, prognosis, and
management.

Bony
Exostoses are benign generally broad-based rounded bony growths that arise circum-
ferentially as multiple lesions in the medial EAC, often bilateral and symmetric, and
consisting of layers of subperiosteal bone containing no bone marrow.1 Their forma-
tion is correlated with cold water exposure and thought to arise due to cold-induced
periostitis. Clinical presentation varies with the degree of EAC occlusion and can

Department of Otolaryngology – Head and Neck Surgery, Mayo Clinic Florida, 4500 San Sablo
Drive, Jacksonville, FL 32224, USA
* Corresponding.author.
E-mail address: [email protected]

Otolaryngol Clin N Am 56 (2023) 965–976

https://doi.org/10.1016/j.otc.2023.06.003 oto.theclinics.com
0030-6665/23/ª 2023 Elsevier Inc. All rights reserved.
966 Raymond

Table 1
Neoplasms of the ear canal classified by cell of origin

Bony
Benign Osteoma
Exostoses
Glandular
Benign Ceruminous gland adenoma
Ceruminous pleomorphic adenoma
Ceruminous syringocystadenoma papilliferum
Malignant Ceruminous adenoid cystic carcinoma
Ceruminous adenocarcinoma NOS
Ceruminous mucoepidermoid carcinoma
Cutaneous
Benign Squamous papilloma
Pilomatrixoma
Malignant Squamous cell carcinoma
Basal cell carcinoma
Melanoma
Merkel cell carcinoma
Metastatic Parotid, colorectal, bronchogenic, esophageal adenocarcinomas;
small cell, hepatocellular, prostate, renal cell carcinoma
Arising from Paraganglioma, sarcoma, schwannoma, hemangioma,
secondary site Langerhans cell histiocytosis, lymphoma, extramedullary
plasmacytoma, leukemia, solitary fibrous tumor

include conductive hearing loss and otitis externa.2 High resolution computed tomog-
raphy (CT) of the temporal bone will delineate the depth of bony involvement as well
as the presence of any medial cholesteatoma formation. Management includes obser-
vation for asymptomatic individuals or exostectomy and canalplasty for symptomatic
individuals. A variety of approaches using the drill or osteotome have been described.
Advantages of the osteotome include decreased risk of sensorineural hearing loss and
postoperative stenosis, while the drill might reduce the risk of tympanic membrane
perforation.2 Preservation of native canal skin is paramount to reducing the risk of
stenosis.3
Osteomata are benign generally solitary, pedunculated smooth, round lesions
arising along the tympanomastoid and tympanosquamous suture line. Histologically
they consist of lamellar bone with outer cortical and inner bone marrow spaces, differ-
entiating osteomata from exostoses.1 Osteomata are often diagnosed incidentally.
Most individuals are asymptomatic. For symptomatic individuals, surgical removal
can done be via a transcanal or postauricular approach using a drill or osteotome. Pre-
operative CT imaging can delineate the medial extent of the osteoma and the pres-
ence of medial debris (Fig. 1). Similar to exostectomy, skin flaps are created with
the intention of skin preservation and the bony lesion is drilled to its base. Complete
removal of the base is recommended to prevent recurrence.3

Glandular
Glandular neoplasms of the EAC are uncommon. Formerly called ceruminomas, they
are thought to arise from ceruminous glands, which are concentrated in the lateral
membranous portion of the EAC. The World Health Organization (WHO) currently clas-
sifies both benign and malignant EAC glandular tumors.1
 Neoplasms of the Ear Canal 967

Fig. 1. High resolution CT temporal bone of the right external auditory canal in the axial (A)
and coronal (B) planes demonstrating a large superiorly based osteoma originating from a
relatively narrow stalking, causing near complete canal occlusion.

Benign
Benign EAC glandular tumors include ceruminous gland adenoma, ceruminous pleo-
morphic adenoma and syringocystoma papilliferum. Patients are often asymptomatic
but found by otologic exam to have a soft well-circumscribed lesion occupying the
membranous EAC, not associated with ulceration or destruction. Rarely are these le-
sions identified in pediatric patients.4,5 On imaging, benign lesions will be well-
circumscribed with homogeneous enhancement with or without cystic changes and
no bony infiltration.6 Once biopsy confirms the histology, a wide local excision and
reconstruction is recommended.
Ceruminous gland adenomas, or more recently termed adenoma not-otherwise-
specified (NOS), are the most common benign glandular EAC tumors.1 They are
firm nonencapsulated masses with smooth surfaces and are composed of well-
differentiated glandular structures lined by epithelium. The average age of presenta-
tion is in the 6th decade.5,7 There is no sex predilection.
Ceruminous pleomorphic adenoma (CPA) is the second most common EAC benign
glandular neoplasm.7 Tumors are firm, nonencapsulated, and well-circumscribed and
are composed of both epithelial and mesenchymal elements, similar to pleomorphic
adenomas in the head and neck.7 Typically, primary neoplasms present as enlarging
masses with occlusive and compressive symptoms. There appears to be a slight male
predominance, though the average age of presentation is less than that of patients
with adenoma NOS.6
Ceruminous syringocystadenoma papilliferum (SCAP) is an extremely rare tumor of
the EAC with only a few case reports in the literature.8–12 Tumors can be polypoid,
lobulated or ulcerated, containing multiple short, thick papillae.13 Presenting symp-
toms are similar to those of other benign EAC neoplasms. Most reported cases
were diagnosed in the 7th to 8th decade of life.

Malignant
There is clinical and histologic overlap between benign and malignant EAC glandular
tumors, though malignant neoplasms appear to be slightly more common.13 In
descending order of frequency, these include ceruminous adenoid cystic carcinoma,
adenocarcinoma NOS, and mucoepidermoid carcinoma.13 Patients can be asymp-
tomatic or present with canal occlusion, otalgia and facial weakness. Tumors are
968 Raymond

more likely to ulcerate and exhibit perineural invasion. They can extend through the
fissures of Santorini into the parotid gland, into periauricular soft tissue, through the
tympanic membrane or into the bony and cartilaginous EAC. Adequate depth of bi-
opsy is needed to distinguish between benign and malignant neoplasms. CT and
MRI should be obtained to delineate the extent of invasion.

Ceruminous adenoid cystic carcinoma

The origin cell-type of ceruminous adenoid cystic carcinoma of the EAC is unknown
but it shares features with malignant salivary gland tumors. Tumors are unencapsu-
lated, diffusively infiltrative and invasive into deep tissue and perineurium, and made
up of monomorphic basaloid cells arranged in tubular, cribriform or solid patterns.
The solid pattern carries the worse prognosis.14,15 Alterations of the MYB transcription
factors seen in salivary gland adenoid cystic carcinoma might also occur in cerumi-
nous adenoid cystic carcinoma,16,17 however tumors that do not stain for MYB might
have a worse prognosis.18
Ceruminous adenoid cystic carcinoma is approximately twice as common in fe-
males as males, and presents earlier in life than other ceruminous gland neoplasms.19
Histologic features which correlate with worse prognosis include perineural or bone
invasion, solid pattern, involvement of the parotid gland, duration of symptoms for
greater than 2 years and positive resection margins.19 Regional lymph node and
distant metastases are not uncommon, but of all distant sites, the lungs are the
most common.20,21 Treatment is a lateral temporal bone resection and superficial
parotidectomy. A selective neck dissection is indicated for imaging consistent with
regional lymphadenopathy. Adjuvant radiation is recommended for close or positive
margins, perineural or lymphovascular invasion, bone invasion, solid pattern histology,
or lymph node involvement. In one series of 43 patients, the 5-year survival rates for
patients with clear surgical margins was 89% and for patients with positive margins
was 54%. The 5-year survival rate for patients who received radiation was 62% and
those who did not was 86%.22 Over-all survival is estimated to be approximately
75%.23

Ceruminous gland adenocarcinoma not-otherwise-specified

Ceruminous adenocarcinoma is nearly identical to ceruminous adenoma. Microscopic
examination will show irregular clusters, nests and sheets of atypical diffusively inva-
sive epithelial cells.13 Tumors can be classified as high-grade and low-grade tumors
depending on the extent of glandular differentiation, but no specific histologic feature
has been shown to correlate with patient outcomes.
Ceruminous adenocarcinoma appears to be more common in males and presents
most commonly in the 6th to 7th decades of life. Treatment includes complete surgical
resection, but it is unknown whether adjuvant radiation improves overall or disease-
free survival.24 Recurrence and metastasis occur frequently despite adequate surgical
resection with negative margins.25 Over-all survival is estimated to be approximately
50%.23

Ceruminous mucoepidermoid carcinoma

Ceruminous mucoepidermoid carcinoma of the EAC is extremely rare with only case
reports documented in the literature.26–32 Histologically, it is identical to its salivary
gland counterpart. Tumors can be low, intermediate, or high-grade depending on
the growth pattern; infiltrative tumors with lymphovascular or perineural invasion, tu-
mor necrosis, high mitotic rate, and cellular pleomorphism all indicate higher grades.
 Neoplasms of the Ear Canal 969

Complete surgical resection is recommended. Robust quantitative survival data is

lacking given the rarity of the disease.23
Cutaneous
Benign
Squamous papilloma. Squamous papilloma is a benign lesion rarely found in the EAC
thought to be caused by the human papilloma virus type 6 and 11.33,34 The route of
transmission is unknown.34–36 Tumors are fungiform or polypoid with variably
sized bases and comprised of well-differentiated stratified squamous epithelium ar-
ranged in stalks with a central fibrous core.33,34 Complete surgical resection is
recommended.37
Pilomatrixoma. Pilomatrixoma, previously described as calcifying epithelioma of Mal-
herbe, is a benign cutaneous neoplasm arising from primitive hair matrix cells.38,39
Occurring in the membranous canal, they are solitary firm, cystic, and well-
circumscribed. They consist of basaloid cells intermixed with “ghost cells” that have
distinct borders but a central unstained area.40 The majority of diagnoses occur in pe-
diatric patients. Treatment is complete excision.
Malignant
The most common cause of malignancy in the EAC is from extension from
the pinna, followed by primary squamous cell carcinoma, basal cell carcinoma,
melanoma and Merkel cell carcinoma. Patients can present with symptoms of
chronic otitis externa, leading to delays in diagnosis. A high degree of suspicion
is warranted, and biopsy should be considered for patients with unresolving
symptoms.
Squamous cell carcinoma
Squamous cell carcinoma (SCC) on the helix arises secondary to actinic exposure.
SCC arising from the EAC is thought to be related to chronic inflammatory states.41
Tumors are friable or ulcerated scaly, irregular, and raised. They are characterized
by pleomorphic polygonal cells with eosinophilic cytoplasm and intercellular
bridging.42 Tumors are locally invasive and can spread through the cartilaginous canal
into the parotid gland and infratemporal fossa, into the postauricular sulcus, through
the tympanic membrane and into the middle ear, mastoid, inner ear, and jugular
foramen.
The mean age at presentation of lesions on the pinna is the 6th decade of life and for
primary EAC lesions, the 5th decade of life. Chronic bloody otorrhea, deep otalgia,
facial palsy or sensorineural hearing loss should raise suspicion for invasive malig-
nancy. A timely biopsy followed by a CT to assess for bony invasion and MRI to eval-
uate for the extent of soft tissue involvement, depth of invasion and perineural invasion
are warranted. Regional metastases are common for advanced tumors so regional or
full-body imaging are usually performed.
There is no universally accepted staging system for SCC of the ear and temporal
bone, however the modified University of Pittsburgh staging system is the most
used.43 In general, tumors limited to the EAC (T1 and T2) have a better prognosis
than tumors with the involvement of the middle ear, mastoid, or facial nerve (T3
and T4). Surgery is the standard of care and should involve a lateral temporal
bone resection, subtotal temporal bone resection or total temporal bone dissection
depending on disease extent. Complete excision with adequate margins is favored,
and there is no literature supporting the use of en bloc versus piecemeal resection
for either subtotal or total temporal bone resection. Sleeve resections have
970 Raymond

fallen out of favor because of a high associated recurrence rate.43 Direct tumor
involvement of the parotid gland necessitates a parotidectomy but both elective
parotidectomy and neck dissection remain controversial.43 Adjuvant radiation is
recommended for positive margins, perineural invasion, bone invasion and lymph
node involvement. Chemotherapy and immunotherapy are both emerging as poten-
tial alternatives to surgery followed by adjuvant radiation, but neither are widely uti-
lized yet.43 The reported 5-year disease free survival rates for combined T1 and T2
tumors and combined T3 and T4 tumor range from 67% to 100% and 41% to 59%,
respectively.

Basal cell carcinoma

Basal cell carcinoma (BCC) is the second most common EAC malignancy but ac-
counts for less than 30% of tumors in most series.44 Actinic exposure is thought to
be the primary etiology. Lesions are typically well-circumscribed, displaying a nodular
irregularity with rolled edges and a central crusting ulcer but can extend subcutane-
ously, lacking well-defined margins. Tumors display palisading basaloid cells margin-
ally with central necrosis and ulceration. Histologic subtypes include nodular,
sclerosing, morpheaform, superficial spreading, and infiltrative, but tumors can
display a mix of subtypes. Most of the BCC of the ear are of the nodular and invasive
subtype, but up to 25% might be of morpheaform or sclerosing subtypes.44 Morphea-
form lesions have a propensity for deeper infiltration.
Patients often present in the 6th decade of life with symptoms related to EAC occlu-
sion, but up to one-third might be asymptomatic.44 Males are affected more
commonly than females.44 Biopsy is required to obtain a diagnosis, and both CT
and MRI are used to assess for bony invasion and depth and spread of soft tissue in-
vasion (Fig. 2). Though the modified Pittsburgh staging system is commonly used to
direct treatment, it appears that BCC overall portends a better prognosis than that of
SCC. Surgical resection is the standard of treatment with the choice of limited resec-
tion with skin graft, lateral temporal bone resection or composite resection with neck
dissection be made based on tumor size and surrounding soft tissue involvement (see
Fig. 2).44 Adjuvant radiation should be considered for perineural invasion. The 5-year

Fig. 2. Preoperative CT temporal bone in the coronal plane (A), demonstrating left superior
bony external auditory canal soft tissue thickening in a patient diagnosed with primary
basal cell carcinoma of the external auditory canal. She underwent a left lateral temporal
bone resection, external auditory canal closure and temporalis rotational flap (B).
 Neoplasms of the Ear Canal 971

reported disease-free survival is approximately 80%, and the 5-year overall survival is
estimated at 78%.44

Melanoma
Malignant melanoma arises from melanocytes, derivatives of neural crest cells. Mela-
nomas arising primarily from the EAC make up approximately 5% of primary EAC ma-
lignancies.45 Tumors can be pigmented with changes in color or size or ulceration
(Fig. 3). Each of five subtypes - superficial spreading, nodular, lentigo, desmoplastic
and mucosal – has variable gross and histologic appearances as well as behavior. Su-
perficial spreading is the most common and nodular is the most aggressive. Lentigo
maligna has variable pigmentation and desmoplastic may be amelanotic. Tumors
are comprised of atypical melanocytes and stain positive for HMB-45, Melan-A,
S-100 protein, and vimentin.45
Patients often present in the 5th decade of life.46 The incidence is higher among
males than females. Sun exposure is a known risk factor, but there might also be
a genetic predisposition.47 Excisional biopsy of any suspicious lesion should be
undertaken. Complete surgical resection is recommended with appropriate
margins. Frozen section pathology cannot reliably detect tumor free margins.
Primary melanomas might have a propensity for higher stage at diagnosis than
melanomas extending from the auricle. En bloc lateral temporal bone resection is
recommended.46 Additionally, current guidelines recommend consideration of a
sentinel lymph node biopsy for lesions that are 0.8 mm thick with adverse
features and strongly recommend performance of sentinel lymph node biopsy
(SLNB) for all lesions greater than 1.0 mm (T2a).48 Advanced melanoma of the
EAC is often treated with adjuvant radiation with improved locoregional control.49

Fig. 3. Clinical photograph of a patient presenting with a discolored, raised lesion centered
at the right helical root with extension from the concha cavum toward the meatus (A),
consistent with malignant melanoma from a dermatologic shave biopsy. He underwent a
wide local excision with 1 cm margins involving the meatus and was reconstructed with a
split thickness skin graft (B).
972 Raymond

Several immunotherapeutic agents have been approved for use as well. Distant
recurrence is common and disease-free and overall survival outcomes at 1 year
are dismal.46

Merkel cell carcinoma

Merkel cell carcinoma (MCC) is a rare, aggressive cutaneous neuroendocrine carci-
noma.50 Tumors appear as solitary cutaneous or subcutaneous nodules. Histological-
ly, tumors are comprised of cords, strands or clusters of small, large or intermediate
cell sizes, with variably distinct borders, high nucleus to cytoplasmic ratio, salt and
pepper appearing nuclei with a small nucleolus. Immunohistochemical staining will
differentiate these tumors from other cutaneous malignancies.50 Risk factors include
sun exposure, immunocompromised states or exposure to the polyomavirus.51,52
The mean age at presentation is in the 7th decade and males are affected more than
females.53 In addition to developing a rapidly growing, painless nodule of varying
hues, approximately 30% of patients will present with regional metastasis.53 Prompt
biopsy and imaging are necessary. Tumor size, infiltrative pattern, thickness and lym-
phovascular invasion, as well as the presence of regional and distant metastasis are
associated with poor prognosis. Recommended treatment includes a wide local exci-
sion with 1 to 2 cm margins along with an SLNB or therapeutic neck dissection,
followed by adjuvant radiation.54–57 Local recurrence rates have been reported to
be between 40% and 50%.57,58 Immunotherapy is emerging as a durable option
for recurrent and metastatic disease.57 The estimated 5-year overall survival rates
for MCC of all sites is 50% for local disease, 36% for regional metastasis and 14%
for distant metastasis.59

Metastatic
Metastatic EAC lesions are rare but should be considered in patients with symptoms
of chronic non-resolving otitis externa and EAC occlusion with a history of both sys-
temic and nonsystemic malignancies. Metastatic EAC lesions have been reported
to arise from parotid, colorectal, bronchogenic, esophageal, rectal, and hepatocellular
adenocarcinoma, extrapulmonary small cell carcinoma, prostate carcinoma and renal
cell carcinoma.60–69 Metastatic lesions, most commonly from the breast, lung and
prostate, to other regions of the temporal bone might also present as EAC lesions.70
Up to one-third of patients might be asymptomatic, but of those who are symptomatic,
hearing loss, facial paresis and otalgia are the most common symptoms.70

Rare neoplasms and those with secondary involvement of the external auditory
canal
Primary neoplasms of the middle ear, mastoid, and jugular foramen can present
initially as EAC lesions. These include paraganglioma, sarcoma, schwannoma, hem-
angioma, Langerhans cell histiocytosis, lymphoma, extramedullary plasmacytoma,
leukemia, and solitary fibrous tumor. Clinical presentation varies by tumor cell type
and extent of disease at the primary site.

SUMMARY

Primary EAC neoplasms include benign and malignant lesions of bony, glandular or
cutaneous origin. Benign bony lesions are often asymptomatic, diagnosed incidentally
and might not require intervention. Both malignant and benign neoplasms of cuta-
neous and glandular origin can present with symptoms of chronic otitis externa, lead-
ing to delays in diagnosis. Prompt biopsy of soft tissue nodules or lesions associated
 Neoplasms of the Ear Canal 973

with non-resolving otitis externa are warranted. Because of the thin EAC skin, even
early-stage malignant neoplasms require aggressive surgical treatment. Metastatic
neoplasms and lesions arising from other regions of the temporal bone can present
in the EAC as well. Therefore, in addition to prompt biopsy, local and regional imaging
is helpful to understand disease extent and origin.

CLINICS CARE POINTS

 Osteoma and exostoses do not warrant intervention unless leading to canal obstruction and
conductive hearing loss.
 Wide local excision with canalplasty is acceptable management for symptomatic benign
cutaneous and glandular neoplasms.
 Cutaneous malignancy should be considered for patients with unresolving otitis externa or
non-healing ulcerative or friable lesions.
 Sleeve resection of the EAC skin has a limited role in the management of cutaneous EAC
malignancies.

DISCLOSURE

The author has no financial disclosures.

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