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CASE REPORT
Peter Andersen, MD, Section Editor
Rare case study of a primary carcinoma of the petrous bone
and a brief literature review
Ihab Atallah, MD, PhD,1,3* Alexandre Karkas, MD, MSc,1,3 Christian Adrien Righini, MD, PhD,1,3 Sylvie Lantuejoul, MD, PhD,2,3
Sebastien Schmerber, MD, PhD1,3
1
Department of Otolaryngology, Grenoble University Hospital, Grenoble Cedex, France, 2Department of Pathology, Grenoble University Hospital, Grenoble Cedex, France,
Joseph Fourrier University, Grenoble, France.
3
Accepted 29 June 2014
Published online 25 September 2014 in Wiley Online Library (wileyonlinelibrary.com). DOI 10.1002/hed.23819
ABSTRACT: Background. Temporal bone carcinoma is an aggressive
patient shows complete remission on regular clinical and radiological follow-up.
Conclusion. Although no widely accepted strategy for managing temporal
bone tumors exists, a review of the literature showed that surgery with or without radiotherapy is the treatment of choice. Preoperative assessment and accurate staging are vital in ensuring that the treatment is adequate for each
C 2014 Wiley Periodicals, Inc. Head Neck 37: E45–E48, 2015
disease stage. V
tumor with multiple unconfirmed risk factors.
Methods and Results. Herein, we present a rare case of a primary
petrous bone carcinoma in a female patient (65 years old) with an
irrelevant medical history. She presented a postauricular swelling
that revealed a multilocular osteolytic cystic lesion of the mastoid
portion of the temporal bone on the CT scan. The patient underwent resection of the lesion and pathological analysis revealed
moderately to well-differentiated squamous cell carcinoma. Postoperative radiotherapy was carried out. Until the present time, the
scan
INTRODUCTION
CASE REPORT
Temporal bone carcinomas are rare malignancies that
present varying clinical courses and behaviors. The management of these tumors remains challenging because of
the complexity of temporal bone anatomy and due to the
proximity to the nearby neural and vascular structures.1
Temporal bone carcinoma staging is crucial for the choice
of the surgical procedure that must provide oncologic radical resection. In addition, the decision to perform postoperative radiation depends also upon tumor staging.2
Early detection and prompt treatment of temporal bone
carcinoma is essential because it is considered as an
aggressive disease with poor prognosis at late stages.
Although the current literature underlines the fact that
survival is related to stage presentation, some patients
with advanced stage survive and others succumb to their
illness.3–5 Herein, we present a rare case of a primary petrous bone carcinoma discovered at an advanced stage.
The treatment was in the form of surgery and radiotherapy with complete remission was achieved until the present time. In the third section of the article, in addition to
the case discussion, we also present an overview of temporal bone carcinoma pathogenesis, clinical presentation,
management, and prognosis.
A 65-year-old female patient was referred to our otolaryngology clinic in August 2011 for a right otalgia, hearing
loss, and dizziness associated with a retro-auricular swelling
that developed a few months before seeking medical
advice. The patient had an irrelevant medical history with
no known allergies. She had no history of smoking or alcohol consumption. Clinical examination revealed a small soft
right retro-auricular swelling which was mildly fluctuant.
Otoscopy imaging showed normal ear drum and external
auditory canal. There was no ataxia or nystagmus. Pure
tone audiometry as well as caloric tests did not show any
sign of middle or inner ear disease. A CT scan revealed a
5-cm multilocular osteolytic cystic lesion of the mastoid
portion of the temporal bone (Figure 1). The lesion had an
extension to the posterior cranial fossa and was in contact
with the right cerebellar hemisphere. MRI confirmed the
localization and the extension of the lesion, which showed
a heterogeneous signal on T1- and T2-weighted images
(Figure 2). The MRI also showed thrombosis of the transverse and sigmoid venous sinuses. Initially, the patient
underwent an extended canal wall-up mastoidectomy to
obtain sufficient specimens for microbiologic, cytologic,
and pathologic examinations. Intraoperative findings were
concordant with preoperative radiological findings. The
multilocular cystic lesion consisted of an inflammatory heterogeneous tissue filled with a serous, yellowish colored
fluid. No pathogen was identified on bacteriologic or parasitologic analyses. Cytology showed inflammatory and
*Corresponding author: I. Atallah, Department of Otolaryngology, Grenoble
University Hospital, Boulevard de la Chantourne, BP 217, 38043 Grenoble
Cedex 09, France. E-mail: IAtallah@chu-grenoble.fr
KEY WORDS: temporal bone, carcinoma, surgery, radiotherapy, CT
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ATALLAH ET AL.
FIGURE 1. Preoperative head CT scan. (A) CT scan axial section showing an osteolytic lesion (arrow) of the mastoid portion of the right temporal
bone. The posterior aspect of the petrous bone shows a large defect (dashed arrow) because of the lesion extension to the posterior cranial fossa.
(B) CT scan coronal section showing complete erosion of the superior aspect (arrow) of the petrous bone.
epithelial cells, whereas pathological analysis revealed moderately to well-differentiated squamous cell carcinoma with
a microscopic examination that showed a connective tissue
with small pseudocystic spaces filled with mucoid material
and coated with epithelial cells with a malignant histopathological aspect (Figure 3B). A total-body CT scan and an
18F-fluorodeoxyglucose-positron emission tomography scan
did not find any other primary or secondary malignant
lesion. In view of these findings, a retrolabyrinthine
approach was carried out to remove the residual disease,
which was in contact with the posterior fossa dura (Figure
3A). Hearing and cranial nerve VII were preserved. The
patient showed transient postoperative cerebellar ataxia that
regressed spontaneously. The patient received postoperative
chemoradiation with 60 to 66 Gy for the surgical bed and
50 Gy for the parotid lymph nodes as well as levels IIA,
IIB, and III of the ipsilateral cervical lymph nodes. Radiotherapy was complicated by middle ear effusion, which was
treated by myringotomy and ventilation tube placement, but
complete sensorineural hearing loss was settled down gradually and was considered as a delayed complication of the
radiotherapy. The patient underwent hearing rehabilitation
by a Cros-Wifi hearing aid. Until the present time, the
patient shows complete remission on regular clinical and
radiological follow-up.
DISCUSSION
Carcinoma of the petrous bone is a rare disease.
Although it is the most common temporal bone
FIGURE 2. Preoperative MRI. (A) T2-weighted axial slice showing multilocular cystic lesion of the mastoid portion of the temporal bone. The lesion
extends to the posterior cranial fossa and comes in contact (arrow) with the right cerebellar hemisphere. The lesion has a heterogeneous signal
ranging from moderately to highly intense. (B) T1-weighted coronal slice showing the same lesion, which is partly isointense to brain tissue. In this
slice, we can also observe that the lesion comes in contact with the temporal dura (arrow).
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TEMPORAL
BONE CARCINOMA
FIGURE 3. Macroscopic and microscopic appearance of the tumor. (A) Retrolabyrinthine approach showing a multiloculated tumor (star) in contact
with the posterior fossa dura (arrow). In this photograph, we can also observe the mastoidectomy cavity performed in the previous surgery (dashed
arrow). (B) Hematoxylin-eosin staining of the tumor which is composed of lobules with large polygonal cells showing squamous differentiation
with keratin pearl formation, disorganized architecture, hyperchromatic nuclei, pleomorphism, increased mitotic activity, and greatly altered
nuclear–cytoplasmic ratio (original magnification 310 and 320 objective lenses). [Color figure can be viewed in the online issue, which is available at wileyonlinelibrary.com.]
malignancy, its incidence is about 1 to 6 patients per million people annually.6–8 Most temporal bone carcinomas
occur between the fifth and seventh decade of life.4,5 The
common clinical symptoms are hearing loss, dizziness,
otorrhea, pain, facial nerve palsy, and external mass,
depending on tumor extension.5–7 Diagnosis is usually
delayed because symptoms mimic more common otologic
conditions. No confirmed risk factor has been identified
for the development of petrous bone carcinoma. Chronic
suppurative otitis media and radiotherapy have been widely
reported to have an association with this type of cancer,
but, in our case, the patient did not have any history of ear
disease or radiation therapy.9,10 Another risk factor, which
has to be confirmed, is the infection by human papillomavirus 16, which has been implicated in many head and
neck malignancies.9 A triggering factor that has to be taken
into consideration, especially in petrous bone carcinoma
arising from the external auditory meatus, is epithelial–
mesenchymal transition in which epithelial cells lose polarity and cell-to-cell adhesions. Consequently, they express
mesenchymal components like vimentin and gain a migratory phenotype, which is correlated with an extensive bone
erosion and a worse prognosis.11 Last, the role of activated
(phosphorylated) signal transducer and activator of transcription 3 in temporal bone squamous cell carcinoma warrants further investigation because it has been implicated
in cellular proliferation, anti-apoptosis, invasion, and angio-
genesis in various types of cancer, including head and
neck cancers.12
The most common temporal bone cancer is squamous
cell carcinoma, which accounts for 60% to 80% of cases.
Histological variants include clear cell type, spindle cell
type, and verrucous type. In addition, various degrees of
differentiation may be observed ranging from well to
poor differentiation. Verrucous squamous cell carcinoma
presents as a slowly growing indolent tumor.2,13 The second most common tumor is adenocarcinoma followed by
adenoid cystic carcinoma. Basal cell carcinomas and melanomas could also develop in the temporal bone. Last,
various forms of sarcoma have all been described as arising within the temporal bone.2,13
The radiologic workup of temporal bone carcinoma
should include a CT scan and MRI in order to evaluate
the extension in both bony structures and soft tissues
because the site of origin of the invading tumor may not
be obvious at presentation as the tumor progresses by
direct incursion into the temporal bone and adjacent structures like the parotid gland, the temporomandibular joint,
the dura, the brain, the carotid artery, and the jugular
foramen.14 In addition, like in any head and neck cancer,
we believe that at least a chest CT scan has to be performed to rule out secondary localizations. In addition, a
total body CT scan or a positron emission tomography
scan could be of great value to search for a primary or a
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secondary lesion, depending upon the stage and the clinical setting of the disease.
Concerning staging of temporal bone carcinoma, in
1985, a staging system based on the anatomic origin of a
tumor was designed.15 It takes into consideration the
extent of local spread, whether the tumor originates from
the external auditory canal or middle ear. Actually, the
Pittsburgh staging system is the most commonly utilized.
It allows an estimation of the prognosis and provides suggestions regarding treatment of cutaneous tumors originating from the external auditory canal; however, it is not a
practical staging system for primary tumors originating in
the middle ear or the mastoid portion of the temporal
bone.1,11,13,14
Although there is no widely accepted strategy for managing temporal bone tumors, surgery with or without
radiotherapy is the treatment of choice. Radiation alone is
ineffective for curative treatment.14 The minimum recommended surgery for small tumors (T1 and T2) is a lateral
temporal bone resection.16 Postoperative radiotherapy, in
these cases, could be performed in the presence of
adverse histological features or in case of positive tumor
margins. Some authors recommend radiotherapy systematically to T1 or T2 tumors in adjunct to surgery.2,11
Most authors agree for postoperative radiation for T3 or
T4 tumors in combination with extended temporal bone
resection.4,10,15 Because of the common anterior extension
of the temporal bone carcinoma, a superficial parotidectomy is frequently performed during surgery combined
with selective neck dissection of levels IIA, IIB, and
III.2,5,17 In our case, we did not perform parotidectomy or
neck dissection because the tumor originated primarily in
the mastoid without involvement of the external auditory
canal and had mainly a posterior extension. In addition,
in order to decrease postoperative morbidity, we tried to
avoid unnecessary prolonged operative time, as no clinical or radiological lymphadenopathy was detected preoperatively. Finally, chemotherapy could also have a place
in the treatment of temporal bone carcinoma, as induction
therapy in case of advanced unresectable tumors or as a
postoperative radiosensitizer in all tumor stages.2,18
Despite aggressive multimodal treatment, temporal
bone carcinoma is considered as a highly malignant disease associated with high morbidity and mortality. Parotid
and temporomandibular joint invasion, dural involvement,
facial nerve paralysis, node-positive neck disease, poorly
differentiated tumors, and positive tumor margins are considered as poor prognostic factors.5,7,8,14,16 Several studies
have been performed to evaluate the survival rate of
patients with temporal bone carcinoma. The 5-year survival rates of patients, in different series, were 83% to
100% for T1 tumors, 67% to 100% for T2 tumors, 21%
to 50% for T3 tumors, and 14% to 38% for T4
tumors.2,3,6–8,13,14,18,19 Survival had also been studied
according to the anatomic origin of temporal bone carcinoma with 59%, 56%, and 23% survival rates for tumors
originating, respectively, from the cartilaginous meatus,
the osseous meatus, and the middle ear.20
For patient follow-up after treatment of temporal bone
carcinoma, there is no formal need for routine postoperative
scans or MRI unless clinically warranted.16 From our point
of view, we believe that regular CT scans could be of great
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value only in primary tumors originating from the middle
ear or the mastoid because clinical examination might not
always be sufficient to rule out a tumor recurrence. After
treatment completion, patients could be reviewed every 3
months for the first 2 years. The follow-up interval should
be then increased to every 6 months until 5 years, and,
thereafter, patients could be reviewed annually.16
CONCLUSION
Temporal bone carcinoma remains an aggressive tumor
with a poor prognosis. Preoperative assessment and accurate staging are vital in ensuring that the treatment is
adequate for each stage of the disease. Because of the rarity of this disease, performance of multiple meta-analyses
will be of great significance to correlate between risk factors, disease stages, treatment modalities, and prognosis.
We also think that the staging system has to be revised in
order to differentiate between temporal bone carcinomas
arising from the auditory canal and those arising primarily
from the middle ear or the mastoid. In fact, we believe
that these 2 localizations have different physiopathologic
and prognostic entities.
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