medicina

Case Report
Pleomorphic Adenoma of External Auditory Canal:
Case Report of First Endoscopic Resection and
Literature Review
Sven Beckmann 1 , Matthias S. Dettmer 2 , Marco D. Caversaccio 1 , Roland Giger 1, *,†
and Lukas Anschuetz 1
1 Department of Otorhinolaryngology, Head and Neck Surgery, Inselspital, Bern University Hospital,
University of Bern, 3010 Bern, Switzerland; sven.beckmann@insel.ch (S.B.);
marco.caversaccio@insel.ch (M.D.C.); lukas.anschuetz@insel.ch (L.A.)
2 Institute of Pathology, University of Bern, 3010 Bern, Switzerland; matthias.dettmer@pathology.unibe.ch
* Correspondence: roland.giger@insel.ch; Tel.: +41-31-632-29-31; Fax: +41-31-632-88-09
† Equal last shared authorship.




Received: 7 April 2020; Accepted: 15 May 2020; Published: 20 May 2020


Abstract: Ceruminous pleomorphic adenoma is a very rare, mostly benign tumor originating
from the ceruminal glands in the external auditory canal. Histologically, it is a mixed tumor with
epithelial and stromal parts of different proportions, and is recognized today by the World Health
Organization (WHO) as a ceruminous adenoma. Similar to the pleomorphic adenoma of salivary
glands, recurrence or malignant degeneration with cellular atypia and metastasis can occur on rare
occasions. Here, we describe an 87-year old female patient with a growing spherical mass in the
right external auditory canal. After exclusive endoscopic tumor resection, a ceruminous pleomorphic
adenoma was histologically diagnosed. Due to the absence of nuclear pleomorphism, no increased
mitotic rate, no perineural invasion and no fusion transcripts of the MYB or MYBL1 gene loci,
an adenoid cystic carcinoma could be excluded. The postoperative course was without any evidence
of complications. A literature review identified 44 articles with 49 patients that were considered.
Hearing loss and ear sensations were the most commonly reported symptoms. Most cases underwent
an excision via an endaural or retroauricular approach. Recurrences were described in four patients,
three of which had a malignant transformation.

Keywords: Pleomorphic adenoma; external auditory canal; ceruminal gland tumor; ceruminous
adenoma; endoscopic ear surgery

1. Introduction
Pleomorphic adenoma is one of the most common tumors in the head and neck area. It usually
occurs in the large salivary glands, although numerous other localizations have been described [1].
With an incidence of 4.3/100,000 persons per year, pleomorphic adenoma in the parotid gland is
the most common salivary gland tumor; it should be resected because of its growth tendency and
possible malignant transformation [2]. Therapeutically, a superficial or total parotidectomy is typically
performed in most cases, although recently, partial superficial parotidectomy and extracapsular
dissection as limited surgery are a suitable alternative in selected patients [3]. There is even the
possibility of an endoscopically assisted extracapsular dissection with cosmetically favorable outcomes,
although the long-term outcomes of this procedure are not well known [4].
A much more rarely affected area for pleomorphic adenomas is the external auditory canal
(EAC), as first described in 1951 [5]. Since then, only a few cases have been reported in the literature.

Medicina 2020, 56, 248; doi:10.3390/medicina56050248 www.mdpi.com/journal/medicina

Medicina 2020, 56, 248 2 of 11

The manifestation of pleomorphic adenomas in the EAC is not completely understood, although the
ceruminal glands may be the origin of the tumors.
With the spread of endoscopic surgery for middle ear pathologies, this technique has also been
used successfully for diseases in the EAC [6]. To our knowledge, this is the first reported case of
exclusive endoscopic resection of a pleomorphic adenoma of the EAC.

2. Presentation of Case Report

An 87-year-old female patient underwent a superficial biopsy of a 1.2 × 0.4 cm spherical mass in
the right EAC at the transition from the cartilaginous to the bony part in an external setting. The mass
grew in the five subsequent months and was treated again in an external setting with a second,
more extensive biopsy of the 1.3 × 1.0 × 0.6 cm actual-size lesion.
The initial biopsy revealed a ceruminous adenoma, whereby a ceruminous pleomorphic adenoma
or ceruminous adenocarcinoma could not be excluded due to the superficial nature of the biopsy.
The second biopsy of the growing mass identified a basaloid biphasic neoplasia with chondromyxoid
stroma. In addition to a ceruminous pleomorphic adenoma, a ceruminous adenoid cystic carcinoma
was another differential diagnosis in the second biopsy.
The patient was then allocated to our ENT-Department. Apart from having a known sensorineural
hearing loss on both sides, she was asymptomatic. Otalgia, otorrhea, facial paralysis or taste disorders
were absent. Otoendoscopy revealed a mass at the posterior wall of the EAC with otherwise normal
ear findings (Figure 1). Computed tomography imaging (CT) showed a soft tissue-like exophytic mass
without bony erosion of the EAC (Figure 2).

Figure 1. Endoscopic view on the tumor at the right posterior external auditory canal.
Medicina 2020, 56, 248 3 of 11

Figure 2. Computed tomography imaging (coronal plane) shows a homogeneous tumor in the right
external auditory canal without evidence of bone erosion.

Therefore, it was decided that an exclusive endoscopic tumor resection should be performed up to
the cartilaginous and bony part of the EAC, including perichondrium and periosteum, while covering
the defect using a biomembrane (Bio Design, COOK Medical® ). The tumor was resected according to
its macroscopic aspect, and subsequently, resections in all directions were performed during the same
operation. This comprised a complete resection.
Finally, the existence of a ceruminous pleomorphic adenoma was assumed due to a lack of nuclear
pleomorphism, no increased mitotic rate, no perineural invasion and no fusion transcripts of the MYB
or MYBL1 gene loci for an adenoid cystic carcinoma (Figure 3).

Figure 3. Admixture of ductules/tubules and matrix stroma including myxoid and hyaline foci.
Some spindle shaped cells in loose connective tissue and fibrous backgrounds. No evidence of
malignant growth pattern, no perineuralor vascular invasion. No nuclear pleomorphism or increased
mitotic rate.
Medicina 2020, 56, 248 4 of 11

The postoperative course showed a completely re-epithelialized EAC at 6 weeks and without any
evidence of complications or regrowth up to 4.5 months after the operation.

3. Discussion
The rarity of this pathology in this localization led us to conduct a literature review on current
diagnostic and therapeutic management. Included were case reports of pathologically confirmed
pleomorphic adenoma in the area of the EAC in English from any time period. Case reports published
in other languages were excluded, as were localizations other than the EAC, as well as tumor invasion
to the EAC from the parotid gland.
The search and review process is illustrated in Figure 4. A total of 44 articles describing 49 patients
with pleomorphic adenoma of the EAC were included. Summaries of the included patients are
presented in Table 1.

Figure 4. Flow diagram of case report selection.

Pleomorphic adenoma in the EAC usually occur in the posterior part (34.7%, n = 17), seem to
be unrelated to gender and are usually symptomatic, e.g., through hearing loss or ear sensations
(40.8% each, n = 20). The entity requires adequate surgical therapy with sufficient safety margins
as well as regular and long-term follow-up due to the potential risk of recurrence in 8.2% (n = 4)
and malignancy in 6.1% (n = 3) of cases. So far, only endaural and retroauricular approaches and
one transcanal approach have been described in the literature; our case reports the first endoscopic
approach, as described above. We chose endoscopic resection due to the minimal invasiveness of the
procedure with short postoperative pain and healing times for the comparable cholesteatoma of the
EAC [6]. Compared to the microscopic approach, the endoscopic approach makes it possible to obtain
a continuous overview of the surgical area in the EAC through one-handed endoscopic handling,
although this requires a one-handed surgical technique. Notably, bleeding control as a one-handed
technique requires practice, but can safely be achieved by topical and injected epinephrine [49].
Medicina 2020, 56, 248 5 of 11

Table 1. Patient’s characteristics, type of surgery and outcome.

Author Number/Age/Gender EAC Symptoms Size Therapy Relapse

Mark et al., 1951 [5] 1/37/f anterior LM* 25 × 13 × 17 mm Exc No rec
Fink, 1953 [7] 1/57/m N/A OR 5 mm Diameter Exc No rec
Nandi et al., 1961 [8] 1/79/f posterior OL/OR/H/HL/FP/D 17 × 13 mm Mast/En-Exc No rec
Smith et al., 1962 [9] 1/60/f superior LM*/OL 35 × 10 mm Exc/Rad No rec
Tung et al., 1963 [10] 1/33/m N/A LM*/OR 15 × 8 × 9 mm Exc No rec
Cankar et al., 1964 [11] 1/27/f N/A N/A N/A Exc No FU
Batsakis et al., 1967 [12] 1/71/f posterior N/A 20 × 20 × 15 mm Exc/Rad Rec
Nail et al., 1967 [13] 1/70/f posterior OL/OR/TI N/A Exc No rec
Pahor et al., 1975 [14] 1/53/m superior HL/TI 25 × 20 × 15 mm En-Exc No rec
Baker et al., 1977 [15] 1/66/f posterior LM*/OR/HL 15 mm Diameter Exc No rec
Botha et al., 1978 [16] 1/15/f N/A N/A N/A Exc Rec (malignant)
Goldenberg et al., 1980 [17] 1/20/f anterior OL/HL N/A S-Par No rec
Dehner et al., 1980 [18] 1/74/m N/A N/A 20 mm Diameter Exc No FU
Chen, 1982 [19] 1/27/m N/A FB*/OR 10 mm Diameter Exc No rec
Hicks, 1983 [20] 1/44/m N/A F*/P*/IF 10 mm Diameter Exc No FU
Tanaka et al., 1984 [21] 1/52/m posterior OB* 10 mm Diameter En-Exc No rec
Collins et al., 1989 [22] 1/55/f posterior HL 8 mm Diameter Re-Exc No rec
Suzuki et al., 1991 [23] 1/51/f anterior OR 9 × 7 × 6 mm Exc No rec
1/45/m superior HL/TI 15 mm Diameter En-Exc No rec
Mansour et al., 1992 [24]
1/52/m anterior TI 10 mm Diameter En-Exc No rec
Tang et al., 1994 [25] 1/39/m posterior LM*/HL 20 × 10 × 10 mm Exc No rec
Haraguchi et al., 1996 [26] 1/38/m anterior LM* 12 × 8 × 5 mm En-Exc No rec
Gerber et al., 1999 [27] 1/43/m superior OL/HL 22 × 18 × 17 mm En-Exc No rec
Goh et al., 2001 [28] 1/12/f posterior N/A 30 mm Diameter Exc Rec (malignant)
Medicina 2020, 56, 248 6 of 11

Table 1. Cont.

Author Number/Age/Gender EAC Symptoms Size Therapy Relapse

Masumara et al., 2003 [29] 1/62/m N/A LM*, F* 12 mm Diameter En-Exc No FU
Thompsen et al., 2004 [30] 4/N/A N/A N/A N/A N/A N/A
Koyuncu et al., 2005 [31] 1/58/f anterior HL/TI/IT 16 × 8 × 4 mm En-Exc No rec
Kaushik et al., 2005 [32] 1/57/f posterior OB*/HL 11 × 10 × 10 mm En-Exc No rec
Karnwal et al., 2006 [33] 1/40/m posterior LM* 15 × 20 × 17 mm Exc No FU
Kuwabara et al., 2006 [34] 1/69/f posterior LM* 9 mm Diameter Re-Exc No rec
Tsukahara et al., 2006 [35] 1/36/m posterior HL/OL 18 × 14 × 21 mm Re-Exc No FU
Granell et al., 2008 [36] 1/38/m inferior OR/HL 17 × 11 × 8 mm Exc No FU
López Campos et al., 2008 [37] 1/68/m N/A HL N/A Exc No rec
Markou et al., 2008 [38] 1/60/f posterior LM*/OL/TI/HL N/A Mast/Re-Exc No rec
Ayers et al., 2010 [39] 1/32/f anterior LM*/OL N/A Tra-Exc No rec
Chadha et al., 2011 [40] 1/37/f N/A OB*/OR/HL N/A Re-Exc No rec
Kuo et al., 2011 [1] 1/N/A N/A N/A N/A Exc No FU
Kanaan et al., 2011 [41] 1/23/f inferior F*/OL 10 × 7 × 7 mm Re-Exc No rec
Vasileiadis et al., 2011 [42] 1/34/m posterior LM*/HL 15 × 8 mm Exc No rec
Lee et al., 2012 [43] 1/40/f N/A N/A N/A Exc Rec (malignant)
Wadhara et al., 2013 [44] 1/25/m posterior OB*/HL 12 mm Diameter En-Exc No rec
Maruyama et al., 2014 [45] 1/40/m posterior HL/TI 18 × 12 × 12 mm En-/Re-Exc No rec
Saito et al., 2014 [46] 1/40/m posterior FB*/HL 23 × 21 × 18 mm Re-Exc No rec
Jaber et al., 2015 [47] 1/55/m N/A OR/HL 10 mm Diameter Re-Exc No rec
Mohan et al., 2015 [48] 1/40/m N/A OL/HL 20 × 17 mm N/A No rec
Our case 1/87/f posterior A 13 × 10 × 6 mm End-Exc No rec
f: female; m: male; A: asymptomatic; D: dizziness; EAC: external ear canal; F*: fullness; FB*: foreign body sensation; FP: facial palsy; H: headache; HL: hearing loss; IF: infection; IT: itchiness;
LM*: lump/mass; N/A: not available; OB*: obstruction/blocking; OL: otalgia; OR: otorrhea; P: pressure; TI: tinnitus; *Ear sensations (lump/mass/obstruction/blocking/fullness/foreign body
sensation), Biop: Biopsy; En-Exc: Endaural excision; Exc: Excision; Mast: Mastoidectomy; Rad: Radiation; Re-Exc: Retroauricular excision; S-Par: superficial parotidectomy with tumor
exstirpation parapharyngeal; Tra-Exc: transcanalic excision; End-Exc: Endoscopic excision; No FU: No follow-up: No rec: No recurrence; Rec: Recurrence.
Medicina 2020, 56, 248 7 of 11

Tumors emerging from the ceruminal glands were first described in 1894 by Haug [50], and were
previously referred to as ceruminoma in the literature, irrespective of their type [51]. Nowadays,
ceruminous pleomorphic adenoma are recognized by the WHO as ceruminous adenoma. In total,
only 2.4% of all tumors in the EAC are tumors of the ceruminal glands [52]. Squamous cell carcinomas
in the area of the EAC occur much more frequently, although other tumors such as basal cell carcinoma
and malignant melanoma may occur there too [53]. Immunohistochemical staining makes it possible to
differentiate among these tumor pathologies, in addition to their clinical, radiological and histological
properties [54]. Other benign lesions such as osteoma, exostosis, inflammatory polyps of the middle
ear and EAC-cholesteatoma are further possible differential diagnoses [41].
An initial classification of the ceruminal gland tumors was proposed by Cankar and Crowley [11],
and later by Welti et al. [55], into the following groups: (1) ceruminous adenoma, (2) pleomorphic
adenoma, (3) ceruminous adenocarcinoma, and (4) adenoid-cystic carcinoma. This was later
extended by Mansour et al. [24], as advocated today by the WHO (Table 2) [56]. After ceruminous
syringocystadenoma papilliferum, ceruminous pleomorphic adenoma is the second rarest benign
tumor of the ceruminal glands [30]. In addition, mixed tumors from adnexal structures in other skin
areas can appear and are called chondroid syringoma [57]. The term “chondroid syringoma” can
therefore be used synonymously with pleomorphic adenoma in the area of the EAC.

Table 2. Ceruminous neoplasms of the EAC according to the WHO classification [56].

Ceruminous Neoplasms
Benign Malignant
Ceruminous adenoma Ceruminous adenocarcinoma
Ceruminous pleomorphic adenoma Ceruminous adenoid cystic carcinoma
Ceruminous syringocystadenoma papilliferum Ceruminous mucoepidermoid carcinoma

The histogenetical origin of pleomorphic adenoma in the EAC is controversial in the literature.
In addition to an originating from ceruminal glands [22,31], an ectopic salivary gland tissue origin was
discussed [19]. However, in contrast to ectopic salivary gland tissue in the middle ear [58], this evidence
could never be proven for the EAC [39]. The ingrowth of a pleomorphic adenoma from the parotid
gland into the EAC by the Foramen Huschke, invading the fallopian canal, cartilaginous fissures of
Santorini and petrotympanic sutures are other possible routes [41,59]. Therefore, parotid gland origin
should be always excluded using clinical exams and imaging studies.
The diagnosis of a pleomorphic adenoma can be confirmed by FNAC [27]. Benign and malignant
forms can be distinguished using FNAC [28]. If FNAC is not diagnostic, incision biopsy is the method
of choice [36]. Computed tomography imaging in these cases shows no bony erosion [36,37,44].
In magnetic resonance imaging, pleomorphic adenoma of the EAC have well-defined margins with
hypointensity on T1-weighted images and hyperintensity on T2-weighted images, as in the parotid
gland [29]. Due to the possible malignancy of every lesion in the EAC, excision with a complete
histopathological exam should be carried out in each case [60].
In the present case, the diagnosis of a pleomorphic adenoma of the external auditory canal was
delayed by the initially superficial and nonrepresentative biopsy. After a second biopsy of the growing
mass, a ceruminous pleomorphic adenoma was identified, with ceruminous adenoid cystic carcinoma as
the differential diagnosis. As adenoid-cystic carcinomas can feature genetic alterations in the MYB and
MYBL1 genes, in addition to typical perineural invasion [61,62], further molecular pathological analyses
of the tissue were performed. Since no detection of fusion transcripts and no perineural invasion
could be demonstrated, the diagnosis of a pleomorphic adenoma was assumed. Further development
of detection methods for genetic alterations in adenoid cystic carcinoma and pleomorphic adenoma
may increase the diagnostic reliability in such cases [63]. Furthermore, these genetic alterations might,
in future, also serve as the basis for molecular targeted therapies in malignant cases.
Medicina 2020, 56, 248 8 of 11

Similar to the pleomorphic adenoma of the salivary glands, relapses may occur in the area of the
EAC if resection is inadequate or if the tumor ruptures during surgery [41]. A local relapse 6 years after
resection, which recurred within 1 year after irradiation, was described in 1967 by Batsakis [12]. The first
malignant relapse with satellite nodules, cellular atypia and mitotic activity was described in 1978 by
Botha et al. [16], and the first metastasizing pleomorphic adenoma of the EAC was described in 2001
by Goh et al. [28]. Even the development of an epithelial-myoepithelial carcinoma from a pleomorphic
adenoma in the EAC has been described [43]. Furthermore, cases of metastatic pleomorphic adenoma
without malignant transformation have been described in salivary glands [64], although there is no
known occurrence of such a case in the area of the EAC so far. Therefore, we recommend complete
excision according to the macroscopic aspect with subsequent resections in all directions in the same
operation to ensure negative margins.
Sufficient surgical therapy with wide margins if possible should be performed with regular
and long-term follow-ups [28], especially as recurrence is also possible after complete local excision.
However, recurrence and malignancy rates need to be interpreted with caution, as they are widely
variable and based on case reports only. Apart from the endaural and retroauricular approaches
described above, our case reports the first endoscopic resection using a minimally invasive approach.

4. Conclusions
Pleomorphic adenoma in the EAC is the second rarest ceruminal gland tumor after
syringocystadenoma papilliferum. The tumor is benign and frequently occurs at the posterior
wall of the EAC. Symptoms include hearing loss or ear sensations. In addition to a thorough clinical
examination, confirmation using FNAC or biopsy, and a CT to determine the extent and exclude bone
erosion are recommended. The treatment of small neoplasms of the EAC should comprise excisional
biopsy. Due to possible recurrence and malignant transformation, appropriate safety margins are
necessary. For the same reasons, long-term follow-up is recommended.

Author Contributions: All authors contributed to the design of the study and writing of the manuscript. S.B.,
L.A. and R.G. contributed equally to all aspects of the case report and literature review. M.S.D. performed
the histological examination. S.B., M.S.D., M.D.C., R.G. and L.A. contributed to the manuscript and revisions.
All authors approved the final version of the manuscript.
Funding: This research received no external funding.
Conflicts of Interest: The authors declare no conflict of interest.

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