15SatyartheeGuru_Primary
Romanian Neurosurgery | Volume XXX | Number 4 | 2016 | October - December
Article
Primary giant myxoma of the temporal bone
with major intracranial extension: presenting
with hearing impairment and ear polyp
Guru Dutta Satyarthee1, Luis Rafael Moscote-Salazar2
1INDIA, 2COLOMBIA
DOI: 10.1515/romneu-2016-0087
540 | Satyarthee, Moscote-Salazar - Primary giant myxoma of the temporal bone
DOI: 10.1515/romneu-2016-0087
Primary giant myxoma of the temporal bone with major
intracranial extension: presenting with hearing
impairment and ear polyp
Guru Dutta Satyarthee1, Luis Rafael Moscote-Salazar2
1Associate Professor, Department of neurosurgery, All India Institute of Medical Sciences, New
Delhi, INDIA
2Neurosurgeon-Critical Care, RED LATINO, Latin American Trauma & Intensive Neuro-Care
Organization, Bogota, COLOMBIA
Abstract: Myxomas are mesenchymal origin, benign tumor, constituting approximately
half of the benign cardiac tumors. Occasionally, it may also occurs at other locations,
though the intracranial location of a myxoma is considered exceptionally rare. Only
isolated few cases of intracranial myxoma are reported in the literature, almost all were
locally confined within the originating bone. The extensive Pubmed and Medline search
yielded only eight cases of primary myxoma arising in the temporal bone with extension
into intracranial compartment. However intracranial extension is limited as early
detection, however, Osterdock et al reported a case also arising from temporal bone with
extensive intracranial extension. Author report an interesting case of intracranial
myxoma in 27- year- old- male, involving the temporal bone associated with extensive
bony erosion and also extending into infratemporal fossa, mastoid, and frontoparietal
region and a polypoidal mass protruding into external ear. To the best of knowledge of
authors, temporal myxoma presenting with external ear polypoidal mass, which
underwent successful surgical excision is not reported and represent first case in the
world literature.
Key words: intracranial primary myxoma, temporal bone, surgery, outcome
Introduction
Primary intracranial myxoma is an
extremely uncommon lesion, however,
majority of reported cases represent
metastatic intracranial myxoma. [1] It
originates from mesenchymal tissue with
predilection in the diminishing frequency i e.
heart, skin, bone or genitalia. [2, 5-8] Primary
myxoma is a benign but locally invasive
tumour [2,7]. Surgical en-block excision of
the lesion along with surrounding soft tissue is
considered as the treatment of choice. [9-13]
Incomplete excision may lead to early
recurrence and further myxoma is not
responsive to radiotherapy. Although radical
resection is goal but always may not be
possible especially in cases of intracranial
Romanian Neurosurgery (2016) XXX 4: 540 – 546 | 541
myxoma and further challenges due to
gelatinous consistency, local invasiveness of
lesion and nature of neoplasm.
Case illustration
A-27-year- old male presented with 5- year
history of progressive hearing loss, protruding
soft mass through right external acoustic meatus
associated with sero-sanguinous discharge . He
developed progressive bulging over the right
temporal region for last four years, causing
cosmetic facial distortion. He also developed
slowly progressive worsening headache, which
was not associated with vomiting, visual decline
or double vision. He consulted
otolaryngologist, who advised computed
tomography scan of head with bone window
view and coronal reconstruction and referred to
our neurosurgery outpatient services. On
examination, he was of average built and height.
The swelling over right temporal and
infratemporal region was hard and bony in
consistency. A soft fleshy mass with sero-
sanguinous discharge was protruding through
external auditory meatus with intact surfaces
except raw area at one place. His visual acuity
was normal. He had no field defect on
confrontation test. The pupils were normal with
brisk light reflex. The optic fundi were normal.
Right sided conductive deafness was also
present. Other cranial nerves were
unremarkable. Muscle strength of extremities
was good and there were no pathological
reflexes. The haematological and biochemical
profiles were within normal limits.
The cranial computed tomography scan
revealed a large heterogeneous calcified mass
located in the right middle cranial fossa
extending and invading into the petrous and
squamus portion of right temporal bone,
causing the erosion of middle cranial fossa
base with extension into right infratemporal
fossa causing swelling in the temporal and
infratemporal fossa of, its size was 6x4.5x 5
cm. (Figure 1).
Large inhomogeneous mass lesion of size
6x4.5 x 5cm, with rim of calcification
extending and invading the petrous and
squamus portion of temporal bone, also
causing the erosion of middle cranial fossa
base with extension into infratemporal fossa.
A large hypodense area with calcified rim
was noted that was extending into
frontoparietal region and infratemporal
region in the bone window view. (Figure 2)
HRTCT of temporal bone also confirmed
destruction of squamus and petrous temporal
bone (Figure 3) along with middle and inner
ear and associated extension into the
Eustachian tube. (Figure 4)
Radiologically, the possible diagnosis of
chondrosarcoma was entertained and
possibility of other primary skull base tumour
was also considered as differentials. He was
planned for surgical management.
He underwent right-sided frontotemporal
craniotomy. The tumour was causing
remoulding and expansion of squamus and
petrous temporal bone with erosion through
outer table and extension into middle ear. It
was primarily located extra-durally. It was
firmly attached to dura. However, no dural
infiltrated was observed during intraoperative
period. It had variable consistency, areas of
gelatinous consistency corresponding to
hypodense areas in the CT scan were present.
The rim was solid and more fibrous.
Gelatinous part was relatively lesser vascular
unlike solid part. Tumour was also eroded
through middle fossa to extend into sub-
542 | Satyarthee, Moscote-Salazar - Primary giant myxoma of the temporal bone
temporal region. Piecemeal dissection of
tumour along with surrounding infiltrated
muscle was carried out. External auditory
meatus was closed after excision of polypoidal
extension. The histopathological examination
of resected specimen revealed presence of
sparsely cellular tumour consisting of stellate
to spindle shaped cells. These cells did not
exhibit hyperchromasia, pleomorphism or
mitotic activity. No mitosis or necrosis was
observed. The bony specimen shows bony
trabecullae, no evidence of tumor infiltration
was observed. The tumour histopathology was
interpreted as benign myxoma. In the post-
operative period, cranial CT scan showed
excision with subsidence of mass effect and no
hematoma in the surgical resected cavity. He
was discharged from the hospital on eighth
post-operative day.
Figure 1 - Non-contrast enhanced computed
tomography of head, axial section image showing
large inhomogeneous mass lesion of size 6x4.5 x5cm,
with rim of calcification extending and invading the
petrous and squamus portion of temporal bone, also
causing the erosion of middle cranial fossa base with
extension into infratemporal fossa
Figure 2 – Computed tomography head, bone
window view image showing extra-large mass
lesion with irregular rim of calcification causing
erosion of middle cranial fossa and extending upto
the right internal ear
Figure 3 - Non-contrast enhanced computed
tomography of head, coronal section image demonstrating
large area of hypodense with calcified rim causing
destruction of squamous part of the right temporal bone
with extension into the infratemporal fossa
Figure 4 - Computed tomography head, bone
window view, coronal section re-constructed image
of 27-year male showing destruction of middle and
inner ear with complete loss of mastoid air cells
Romanian Neurosurgery (2016) XXX 4: 540 – 546 | 543
Figure 5 - Post-operative computed tomography scan
of head showing excision of temporal myxoma
Discussion
A myxoma is a benign neoplasm, however,
biologically it behaves like locally invasive
tumour. Myxoma originates from tissues of
mesenchymal origin. However, most of
primary myxoma is commonly located in the
heart, skin, genitalia and aponeurotic tissue.
Although myxoma arising in bone,
development begins in bone- marrow,
subsequently causes expansion of dipole and
ultimately expansion, destruction and
remoulding of involved bone caused by aseptic
pressure necrosis.
Primary myxoma of head and neck usually
involve maxilla and mandible. [16-22]
Primary myxoma involving skull base and
vault is very rare. Primary intracerebral
myxoma is not reported; even secondary
intracranial myxoma is also uncommon.
Intracerebral monastic myxoma, which
usually originates as a result of metastatic
deposit or remobilization from primary
cardiac myxoma. Only few isolated case report
of primary intracranial myxoma is reported.
[1- 7, 11-13, 21, 23] The etiology of myxomas
occurring in the head and neck region still
remains unclear, although postulated about
head and neck myxomas, these may arise from
the tooth germ cells, because of presence in
the maxillary and mandibular region. [22]
Kleinsasser postulated primitive mesodermal
tissue filling the middle ear space in the
embryo and in the newborn may give rise to
temporal bone myxomas. [17, 18, 23]
Myxoma can occur at any age and both
sexes are at equal risk. Radiologically CT scan
shows hypodense to isodense mass lesion
with variation in pattern of enhancement
following contrast administration. Even there
is variation in degree of bony involvement,
extensive bony involvement is considered
extremely uncommon and our case was
unique having huge intracranial extension,
although only one such case was reported by
Osterdock et al [3].
The management of myxoma is mainly
surgical excision with wide margins of
surrounding healthy soft tissue. Myxomas
possess a strong tendency to recurrence,
especially in the bones. [24] In cases of
temporal bone myxomas, where healthy
margins cannot be achieved, drilling and
piecemeal removal as much as tissue are
possible, can be seen as needs to be adequate.
The radical surgery requirement must be
evaluated in light of safety and preservation of
facial nerve and inner ear function. Windfuhr
and Schwerdtfeger [25] advocated en bloc
excision with wide margins is not possible in
view of ill-defined margins of tumor and
complex anatomy of temporal bone in cases
harbouring temporal bone myxomas.
Therefore, it is wise to operate thoroughly but
544 | Satyarthee, Moscote-Salazar - Primary giant myxoma of the temporal bone
without sacrificing vital structures. Regular
follow-up is required, both clinically and
radiologically, to detect recurrence early. The
treatment of myxoma is surgery, although en
bloc resection of intracranial myxoma is
considered extremely difficult due to presence
of important neurovascular structures
difficult. [table 1] practically impossible to
maintain its intactness during surgical
dissection of these tumours, if cannot be
excised with surplus surrounding tissue. But it
is impossible in cranial skull-base and
temporal bone [2, 4]. Due to associated danger
of en-block resection, in relation to
intracranial myxoma, piecemeal removal is
preffered. Despite operative difficulties, the
treatment of myxoma is surgical as myxoid
tumours are generally insensitive to radiation
therapy. Due to its locally invasiveness,
complete surgical excision is difficult. Charabi
et al reported left temporal myxoma which was
involving mastoid, antrum and epitympenum
but without any intracranial extension [2].
Klein et al reported a primary intracranial
myxoma located in the posterior fossa without
extensive bony involvement and
intraoperatively tumour was relatively peeled
from cerebellum easily [4]. However, in few
cases myxoma grows beyond confines of
dipole and extends into adjoining spaces. In
these cases with significant intracranial
extension and associated with extensive bone
destruction, where attempt of radical excision
may not be possible. Nagatani et al reported a
case of primary myxoma of pituitary fossa,
which had extensive bone erosion with spread
into suprasellar cistern and inferiorly into
sphenoid sinus after eroding sellar floor [16].
TABLE I
Published report of primary intracranial myxoma originating in the Temporal bone
S. No. Series/ (Ref. no.) Year Site Intracranial extension
1. Richarth & Terrache (6) 1969 Temporal bone Not mentioned
2. Bulghov et al (7.) 1980 Temporal bone Not mentioned
3. Charabi et al. (2) 1989 Temporal bone No
4. Osterdock et al (3 ) 2001 Temporal bone Extensive
5 Hsieh et al. (11) 2006 Temporal bone small
6 Oruckaptan et al (1) 2010 Temporal bone small
7 Sareen et al21 2010 Temporal bone external auditory canal,
middle ear, mastoid antrum
nil
8 Zhang (15) 2006 Lateral skull Details not available
Current case 2016 Temporal bone extensive
Osterdock et al [3] reported a 17- year -old
male with left temporal bone invading petrous
bone myxoma associated with a large
intracranial extradural component containing
central gelatinous part surrounded by
hyperdense rim extending up to frontoparietal
region and inferiorly up to posterior fossa. Our
case also had extensive spread extending to
frontoparietal region and posteriorly invading
mastoid, middle ear, Eustachian tube and
polyploidy like extension through middle year
after perforating tympanic membrane into
Romanian Neurosurgery (2016) XXX 4: 540 – 546 | 545
external auditory canal. To best of our
knowledge this is first case of primary
intracranial myxoma with extensive bony
erosion, which was also further extending to
infratemporal fossa, mastoid, and
frontoparietal region with mass protruding
into external ear.
Recurrences are common, up to 25 % case
may show recurrence if radical excision was
not carried out [9]. The recurrence after
surgery can occur as early as months and can
delay up to 10 years.
Mueller et al reported a 12-year- girl, a case
of surgically resected medulloblastoma,
received adjuvant radiotherapy, presented
with a mass lesion at the left transverse sinus
during the follow-up [13]. Further added
close relation to the radiation field of the
posterior fossa medulloblastoma treatment,
developed myxoma as a secondary radiation
induced neoplasm.
Conclusion
The primary intracranial myxoma
although care but important and its possibility
of must be kept as one of differentials in all
cases, who present with destructive skull -
base lesions. The primary aim of the treatment
is gross total excision, as en-block resection
may not be always possible in cases with
extensive intracranial extension. Further these
cases need close monitoring with regular
follow-up and screening cranial MRI to pick
up early recurrences and provide tailored
made appropriate treatment to each cases.
Correspondence
Guru Dutta Satyarthee, Associate Professor,
Department of neurosurgery, All India Institute of
Medical Sciences
Email: drguruduttaaims @gmail.com
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