J Neurooncol (2016) 130:351–366
DOI 10.1007/s11060-016-2138-5
TOPIC REVIEW
Microsurgical resection of pineal region tumors
Adam M. Sonabend1 • Stephen Bowden1 • Jeffrey N. Bruce1
Received: 9 February 2016 / Accepted: 2 May 2016 / Published online: 19 May 2016
Ó Springer Science+Business Media New York 2016
Abstract The extensive variety of possible histologic
subtypes makes it imperative to establish a tissue diagnosis
in patients with pineal region tumors. Management deci-
sions regarding adjuvant therapy, prognosis, and follow-up
strategies vary with the histologic diagnosis. Specialized
surgical and stereotactic techniques have evolved to pro-
vide the neurosurgeon with an array of safe and effective
options for obtaining a tissue diagnosis. Advanced micro-
surgical techniques combined with improved preoperative
management and postoperative critical care methods have
made aggressive surgical resection a mainstay of man-
agement. Aggressive surgical resection has resulted in
excellent long-term prognoses for nearly all patients with
benign tumors and a large percentage of patients with
malignant tumors. However, pineal region surgery remains
fraught with potential pitfalls, and these favorable results
are dependent on an advanced level of surgical expertise.
Keywords Pineal region tumor
Supracerebellar-
infratentorial approach
Occipital-transtentorial approach

Transcallosal-interhemispheric approach
Microsurgery

Operative technique
Electronic supplementary material The online version of this
article (doi:10.1007/s11060-016-2138-5) contains supplementary
material, which is available to authorized users.
& Jeffrey N. Bruce
jnb2@cumc.columbia.edu
1
Department of Neurological Surgery, Columbia University
Medical Center, New York, NY, USA
Background
The role of surgical resection in the treatment of pineal
region tumors has grown markedly over the last few dec-
ades due to enlightened appreciation of the value of
accurate tissue diagnosis combined with improved micro-
surgical techniques. The pineal region is among the most
complex areas of the brain with regard to pathology,
necessitating a histologic diagnosis for optimal treatment
planning. The spectrum of distinct tumor types that may
arise in this location are divided into four categories,
including germ cell tumors, pineal parenchymal cell
tumors, glial cell tumors, as well as a wide variety of
miscellaneous tumors and cysts [1]. All types exist on a
continuum from benign to malignant and mixed tumors of
more than one cell type may also occur [2–4]. In addition
to neoplastic disease, vascular lesions can also occur,
including cavernous malformations, arteriovenous malfor-
mations, and vein of Galen malformations [5, 6].
Research in pineal region tumors has been historically
limited due to a relatively low incidence [only 1.2 % of all
Central Nervous System (CNS) tumors] [7], the diversity
of pathology challenging the characterization of outcome
for patients by histological type, and differences in man-
agement paradigms. Conservative non-surgical manage-
ment with empiric radiation was historically favored
because of the highly specialized surgical demands in the
pre-microsurgical era and exquisite radiosensitivity of
some tumor types [8–13]. Unfortunately, this strategy of
‘‘blind radiation’’ led to unnecessary and potentially
harmful radiation exposure in a large percentage of patients
with benign or radiation-resistant tumors [14–16].
Management of pineal region tumors now incorporates
surgical resection in most cases. Evidence suggests resec-
tion is associated with better outcomes. Further, the greater
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sophistication and diversification in treatment strategies Management

necessitates tumor tissue for proper tailoring of adjuvant
therapy, determining prognosis, and establishing a follow-
up plan [1, 17–27].
The first attempts at resecting pineal region lesions
were attempted by Horsley, Brummer and Schloffer [28,
29], but were associated with considerable morbidity in
the absence of microsurgical techniques. Outcomes soon
improved, with Oppenheim and Krause [30] performing
the first successful removal of a tumor from the pineal
region in 1913. In 1921, Dandy [31] outlined a transcal-
losal approach to pineal tumors after developing the
technique in dogs. The technical repertoire continued to
expand when, in 1926, Krause [32] reported modest
success with an infratentorial approach. Stein [25] suc-
cessfully modified Krause’s infratentorial approach and
incorporated microsurgical techniques, ushering in the
modern era of pineal surgery in 1971. Additional supra-
tentorial approaches have since been refined and added to
the surgical repertoire [1, 33–38]. The largest series of
pineal region surgery published over the last 15 years
have demonstrated that resection of these lesions is pos-
sible, with morbidity and mortality rates ranging between
1–20 and 0–10 %, respectively, and rates of gross total
resection between 49 and 91 % (Table 1) [39–41].
Several adaptable approaches are available to patients with
pineal region lesions to confirm diagnosis, manage asso-
ciated symptoms such as hydrocephalus or mass effect, and
facilitate cytoreduction. These management strategies
should be tailored to the clinical presentation, with special
attention to associated clinical conditions such as hydro-
cephalus, elevated tumor markers, metastatic disease,
advanced age, and general medical status of the patient
(Fig. 1).
Ruling out pineal cyst
It is important to rule out the presence of a pineal cyst, par-
ticularly for pineal region lesions found incidentally, as cysts
have been found in 1.0–4.0 % of patients undergoing brain
MRI [42]. Pineal cysts may mimic pilocytic astrocytomas
radiographically, though tumors can be distinguished clini-
cally by their progressive and symptomatic nature. Cysts are
generally\2 cm in greatest dimension [42], with a contrast-
enhancing rim representing compressed pineal gland tissue.
They are benign, normal variants of the gland which are
generally asymptomatic and do not require treatment unless
they are causing aqueductal obstruction.

Table 1 Literature overview of microsurgical series for pineal region tumors in the last 20 years (modified with permission from [39])
Authors Year n Approach Age Pathology GTR Mortality Major Permanent
group (%) (%) morbidity (%) minor
morbidity (%)

Bruce and Stein [71] 1995 160 ITSC A and P All 45 4 3 19

TCIH
OTT
Chandy and Damaraju [112] 1998 48 ITSC A and P ‘‘Benign 55 0 NA NA
OTT lesions’’
Kang et al. [113] 1998 16 OTT A and P All 37.5 0 0 19
ITSC
TCIH
Shin et al. [114] 1998 21 OTT A and P All 54.5 0 0 5
Konovalov and Pitskhelauri [4] 2003 201 OTT A and P All 58 10 NA [20
ITSC
Hernesniemi et al. [40] 2008 119 ITSC A and P All 88 0 1 4.9
OTT
Bruce [39] 2011 128 ITSC A and P All 49 2 1 NA
TCIH
OTT
Qi et al. [41] 2014 143 OTT A and P All 91.6 0.7 3.5 5.6
TCIH transcallosal interhemispheric, ITSC infratentorial supracerebellar, OTT occipital transtentorial, TT transcortical transventricular, NA not
available, A Adult, P pediatric

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Fig. 1 A treatment algorithm
for the approach to the patient
with a pineal region lesion,
including consideration of the
presence of hydrocephalus and
germ cell markers
(Abbreviations: XRT/Chemo =
external radiotherapy and
chemotherapy, STR = subtotal
resection, GTR = gross total
resection)
Management of hydrocephalus
Obstructive hydrocephalus, present in most patients on
presentation, can be managed in several ways. Symp-
tomatic patients are best managed with a stereotactic-gui-
ded endoscopic third ventriculostomy to gradually reduce
intracranial pressure and resolve symptoms prior to tumor
resection [43]. While consideration may be given to ven-
triculoperitoneal shunting, endoscopic third ventricu-
lostomy is preferable as it eliminates potential
complications such as infection, overshunting, and peri-
toneal seeding of malignant cells. Mildly symptomatic
patients in whom gross total resection is anticipated will
benefit from a ventricular drain placed at the time of sur-
gery [20, 44]. Mild asymptomatic hydrocephalus fre-
quently resolves without the CSF diversion following
resection of a pineal mass, as the third ventricle is com-
municated with the quadrigeminal cistern or aqueductal
compression is relieved.
Tumor markers and non-operative diagnosis
The presence of a-fetoprotein or b-human chorionic
gonadotropin, measureable in either serum or CSF, is
353
pathognomonic for malignant germ cell elements and
precludes the need for surgical resection [45–47]. CSF
levels are more sensitive than serum levels in detection [2,
45, 47]. a-Fetoprotein indicates the presence of fetal yolk
sac elements and is associated with endodermal sinus
tumors, embryonal cell carcinomas, or immature teratomas
[45, 47–52], whereas b-human chorionic gonadotropin,
produced by trophoblastic elements, indicates the presence
of choriocarcinomas, embryonal cell carcinomas, or ger-
minomas [45, 47–49, 51, 53–56]. Though the presence of
germ cell markers is confirmatory for a malignant germ cell
tumor, the absence of germ cell markers should be inter-
preted cautiously because it does not rule out the presence
of a germinoma or embryonal cell carcinoma. Similarly, if
a-fetoprotein is elevated in the presence of a germinoma, it
is likely that an embryonal cell carcinoma or endodermal
sinus tumor is present as part of a mixed tumor [57].
The clinical utility of these tumor markers to the neu-
rosurgeon is twofold. They are useful in establishing a
baseline post-operatively to monitor treatment response or
detect recurrence. More importantly, they reliably indicate
the presence of a germ cell tumor, allowing those patients
to forego surgery entirely in favor of radiation and
chemotherapy for these particularly sensitive tumor types.
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Surgery
Anatomical considerations
The pineal gland is an encapsulated structure that resides in
a deep position near the geometric center of the brain. The
pineal gland is essentially an extra-axial structure, a feature
that facilitates the establishment of a surgical plane
between adjacent structures during resection. The pineal
gland is bordered by the posterior commissure ventrally,
the habenular commissure dorsally, and the corpus callo-
sum superiorly (Fig. 2) [58]. The velum interpositum,
which incorporates the internal cerebral veins and choroid
plexus, lies in close proximity to the gland. The basal veins
of Rosenthal join the internal cerebral veins to form the
vein of Galen before draining into the straight sinus. The
orientation of the deep venous system relative to the gland
is variable and has consequences for surgical planning
(Fig. 3). The more common superior and dorsal orientation
is most conducive to the infratentorial supracerebellar
approach. The less common inferior and ventral location is
more conducive to a supratentorial approach. The gland
receives its blood supply from branches of the medial and
lateral choroidal arteries through anastomoses to the peri-
callosal, posterior cerebral, superior cerebellar, and
quadrigeminal arteries [46, 59].
Most masses in the pineal region originate infratentori-
ally and expand into the posterior third ventricle. With
further progression, they may extend into the thalamus or
posteriorly over the dorsal surface of the quadrigeminal
plate. Malignant tumors in this region, particularly when
they are of glial origin, can invade the midbrain and tha-
lamus, ultimately determining the tumor’s resectability.
Resection versus biopsy
Given the pathologic diversity found in this region, a tissue
diagnosis is necessary to optimize a treatment plan
including choice of adjuvant therapy, postoperative
workup, and estimating prognosis [4, 44, 60–63]. CSF
cytology and radiographic characteristics may provide
insight into these decisions, but are not sufficiently sensi-
tive to supplant tissue diagnosis [64–69]. The only excep-
tion to the rule of tissue diagnosis is the presence of
malignant germ cell markers which obviate the need for
chemotherapy and radiation therapy without histologic
confirmation [44, 69, 70].
Tissue diagnosis can be achieved by either biopsy or
open surgical resection. Careful consideration of the
patient’s clinical presentation, the tumor’s radiographic
characteristics, and the surgeon’s experience with each
method should be considered, as neither is universally
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applicable. There are, however, some scenarios in which
one method is strongly preferred. Patients with known
primary systemic tumors, multiple lesions, or other
comorbidities that increase surgical risk are good candi-
dates for stereotactic biopsy [44, 45]. Radiographic evi-
dence of brainstem invasion might favor stereotactic
biopsy, but the degree of invasion seen radiographically is
unreliable and may not reflect a dissectible tumor capsule
at surgery. Open resection has the advantage of facilitating
the collection of larger amounts of tissue. Extensive tissue
sampling is particularly desirable for pineal region lesions
given their frequent heterogeneity and mixed cell popula-
tions, as histologic differences may be subtle even to
experienced neuropathologists. The clinical benefit that
comes with reduction of the tumor burden is another
advantage of open resection. Resection is usually complete
and curative for the third of tumors that are benign
(Table 2) [44, 61, 71]. The clinical advantage gained by
debulking is less apparent with malignant tumors; however,
anecdotal evidence favors more radical resection when
possible in order to improve the response to adjuvant
therapy [34, 44, 71–73]. Patients with mild hydrocephalus
gain a particular benefit with open surgery, as total resec-
tion may preclude the need for shunting [20, 44].
Two options are available if the decision to pursue
biopsy is made. Stereotactic biopsy offers the advantages
of relative ease of performance, minimal anesthesia, and
minimal risk of complications [19, 23, 74–76]. There
remains a risk of hemorrhage via several mechanisms,
including bleeding in highly vascular tumors, damage to
the deep venous system, and bleeding into the ventricle
[20, 44, 75, 77]. However, several large series have vali-
dated the relative safety of this technique despite these
lesions’ hazardous location [19, 74]. Neuroendoscopic
biopsy has become an alternative to stereotactic biopsy
recently, taking advantage of flexible endoscopes to
simultaneously perform a third ventriculostomy and biopsy
through the same burr hole. This approach carries similar
bleeding risks and, perhaps more significantly, is also
subject to inadvertent sampling bias.
Craniotomy techniques
Several variations exist for approaches to the pineal region,
generally categorized as either supratentorial or infraten-
torial [38, 44, 61]. Supratentorial approaches include the
transcallosal interhemispheric, occipital transtentorial, and
the less common transcortical transventricular [34, 44, 78,
79]. The infratentorial approach most commonly employed
is the infratentorial supracerebellar [25, 80]. The choice
between these depends mainly on the surgeon’s experience
and comfort, as many of these approaches are
J Neurooncol (2016) 130:351–366 355

Fig. 2 Sagittal (a) and dorsal (b) drawings of pineal region anatomy (modified with permission from Diane Abeloff; from [90])

interchangeable. There are several caveats: large tumors
that extend above the tentorium or laterally to the trigone
of the lateral ventricle are generally most amenable to the
field of view from a supratentorial approach [44]. These
greater exposures come at the cost of working around the
convergence of the vein of Galen and internal cerebral
veins, where they can interfere with tumor removal.
Most pineal tumors lie infratentorially and in the mid-
line, giving the infratentorial supracerebellar approach
several natural advantages [44, 80]. One key benefit comes

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with the sitting position, whereby gravity allows both the

cerebellum and the tumor to drop downward, naturally
enlarging the exposure while minimizing the need for
retraction. Further, this facilitates dissection of the tumor
off the deep venous system and velum interpositum, which
is often the most technically challenging portion of tumor
dissection. Second, this approach allows for a midline
trajectory. Lastly, the deep venous system lies superior to
the mass, thus shielding it through the majority of the
dissection. While offering many advantages, including the
possibility of removal of lesions extending anteriorly into
the third ventricle, the infratentorial supracerebellar
approach is less optimal for lesions with significant
supratentorial or lateral extension.

Patient positioning

Numerous patient positions can be employed, including the

sitting, lateral, and prone positions, with each having its
respective advantages and disadvantages (Fig. 4).

Sitting position

The sitting position is the preferred set-up for the

infratentorial supracerebellar approach (Fig. 4a) [44, 78].
Gravity minimizes the pooling of blood in the operative
field and facilitates dissection of the tumor from the deep
venous system. The risks inherent to this position include
air embolism, pneumocephalus, and subdural hematoma
associated with cortical collapse. These can be anticipated
and managed with proper precautions [44, 81]. End tidal
CO2 is a sensitive indicator of air emboli. Precordial
Doppler monitoring is another method of detecting these
Fig. 3 Magnetic resonance images demonstrating the variable rela-
tionship between the deep venous system and pineal region tumors.
small amounts of air entering the venous system and should
a The deep venous system is superior and dorsal to the tumor. This is be used. A central venous catheter can be used to remove
the most common configuration and is more conducive to the entrapped air.
infratentorial supracerebellar approach. b The deep venous system is The patient is brought to a sitting position from supine
inferior and ventral. This is less common but more conducive to a
supratentorial approach (from [115])
by manipulating the table while an assistant stabilizes the
patient’s head. A reverse table capable of being lowered
close to the floor is ideal. The head is flexed such that the
Table 2 Extent of resection for 181 Consecutive Pineal Region tentorium is approximately parallel to the floor. The
Surgeries at the New York Neurological Institute (1990–2014) (rep-
patient’s legs should be elevated to assist venous return.
rinted with permission from [39])
The surgeon should ensure at least two fingerbreadths of
Biopsy Subtotal Radical subtotal space between the patient’s chin and sternum to avoid
resection resection/gross total
resection airway compromise and impairment of venous return. A
three-pin vise-type head holder or skull clamp immobilizes
Benign 2 5 70 the head. A Greenberg self-retaining retractor or analogous
Malignant 12 29 63 system is attached to assist with cerebellar retraction and
Total 14 (6 %) 34 (19 %) 133 (73 %) hold cottonoids. The microscope should be balanced in a
Radical subtotal resection = no visible tumor at surgery or on post- position to allow direct horizontal view of the operative
operative MRI but tumor was not well encapsulated field.

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Fig. 4 Patient positioning in preparation for pineal region tumor surgery. a Sitting position, b three-quarter prone position, c prone position,
d concorde position (a–c from [79]; d from [116])
Lateral and three-quarter prone positions
The lateral decubitus position is generally used with the
patient lying on their right side, such that the dependent,
nondominant right hemisphere is relaxed down away from
the falx [82]. For most approaches, the head should be
positioned approximately 30° above horizontal in the
midsagittal plane. In contrast, the occipital transtentorial
approach requires that the head should be positioned with
the patient’s nose rotated 30° towards the floor.
A more accommodating variant of the lateral position is
the three-quarter prone position (Fig. 4b) [83]. The legs are
flexed and pressure points are minimized with pillows, an
axillary roll under the patient’s right axilla, and a roll under
the left thorax. A three-pin Mayfield head-holder supports
the head, which is slightly extended and rotated to the left
at a 45° oblique angle, again such that the nondominant
hemisphere is dependent. The patient is secured with straps
so that the table can be rotated during the operation to
improve exposure when necessary. The legs and feet are
357
elevated to facilitate venous return. This position better
suits the more posterior approaches, such as the occipital
transtentorial, rather than the more vertical transcallosal
approach, as the nondominant hemisphere is largely
retracted by gravity and the horizontal operative plane
reduces surgeon fatigue.
Prone position
The prone position is a simple and safe option for supra-
tentorial approaches and comes with several advantages
(Fig. 4c) [44, 80]. First, it is generally a comfortable posi-
tion for the surgeon’s hands, though the elevated operative
field makes it difficult to sit. Second, it is particularly
useful for two surgeons to work together, as it allows for
placement of an operative microscope with a bridge setup
and thus simultaneous binocular vision. Rotating the head
15° away from the craniotomy side yields a common
variation known as the Concorde position (Fig. 4d) [84].
The prone position is less practical for the infratentorial
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supracerebellar approach due to the steep angle of the
tentorium, but nonetheless remains a viable option for the
surgeon pursuing a supratentorial route to resection.
Approaches
Infratentorial supracerebellar approach
The infratentorial supracerebellar approach (Fig. 5, Video 1)
is usually performed with the patient in the sitting position
[44, 61, 80]. If necessary, a ventricular drain can be placed
in the trigone of the lateral ventricle through a Frazier burr
hole. Exposure is begun with a midline incision extending
from just above the torcula and external occipital protuber-
ance down to the level of the C4 spinous process. The cut is
brought through the nuchal ligament of the suboccipital
musculature. The craniotomy is centered just below the
torcular and must be extensive enough to provide room for
the surgical instruments and adequate light from the oper-
ating microscope. Craniotomy with replacement of the bone
flap reduces the incidence of postoperative aseptic menin-
gitis, fluid collections, and discomfort, and is thus preferred
over craniectomy. Slots are drilled over the sagittal sinus,
above the torcula, and over the lateral sinuses bilaterally. A
final slot is made 1 or 2 cm above the foramen magnum in
the midline. The slots are connected with a craniotome,
allowing the bone flap to be elevated. Care must be taken to
remove sufficient bone above the transverse sinus to ensure
a clear view along the tentorium. Bone edges are waxed and
all venous bleeding is controlled to avoid air emboli.
A curvilinear dural opening is done between the lateral
aspects of the craniotomy with a concavity directed supe-
riorly. The dural flap is reflected upward and anchored with
slight tension. Excess retraction obstructs the sinuses and
should be avoided. The inferior dura acts to support the
cerebellar hemispheres. If the posterior fossa appears tight,
fluid can be removed through a ventricular drain or by
opening the cisterna magna.
Opening of the infratentorial corridor is followed by
cautery and careful division of the arachnoidal adhesions
and midline bridging veins between the dorsal surface of
the cerebellum and the underside of the tentorium and
straight sinus using microsurgical techniques. The presence
of extensive collateral circulation minimizes complications
from venous sacrifice; however, to minimize risk, it is
desirable to spare any veins found laterally [85]. Cauter-
izing the bridging veins and dividing them midway can
minimize bleeding from the sinus. Following division of
these attachments, the cerebellum drops away from the
tentorium, providing an excellent corridor with minimal
brain retraction. The exposed dorsal surface of the cere-
bellum can be protected with padding such as Telfa, and a
brain retractor can be temporarily used to bring the
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Fig. 5 Infratentorial supracerebellar approach. a Sagittal diagram c
showing a the initial trajectory in line with the vein of Galen and b the
adjustment downward in line with the central axis of the tumor after
opening the arachnoid over the quadrigeminal plate. b Operative
photograph showing the dorsal surface of the tumor after opening of
the arachnoid and division of the precentral cerebellar vein.
c Diagram of the exposure seen in b. d Operative photograph with
a view into the third ventricle after excision of the tumor. e Diagram of
the tumor bed seen in d (from [116])
cerebellum more inferiorly and posteriorly. Deeper adhe-
sions and bridging veins can be divided when they become
visible near the anterior vermis with further cerebellar
retraction. With the retractor in place, the opalescent
arachnoid covering the pineal region can be seen.
Because of the range of angles and depths encountered
along the operative trajectory, a microscope with a variable
objective is preferred to facilitate the use of long instru-
ments. A freestanding armrest assists the surgeon and
prevents fatigue.
The arachnoid overlying the quadrigeminal plate is
sharply opened. This is generally an avascular plane
requiring minimal cautery. The precentral cerebellar vein,
identified as it courses from the anterior vermis to the vein
of Galen, should be dissected, cauterized, and divided.
Although this vein can be taken without substantial risk,
the sacrifice of any other veins of the deep venous system is
not advisable. The retractor is then adjusted to visualize the
inferior portion of the tumor. The trajectory of the micro-
scope is re-oriented to be in line with the central axis of the
tumor, away from the initial plane parallel to the tentorium,
where it would otherwise lead to direct encounter with the
vein of Galen (Fig. 5a). The retractor can often be removed
following removal of CSF once the quadrigeminal cistern
is widely opened.
Once the posterior surface of the tumor is exposed, the
central portion is cauterized and opened with a long-han-
dled knife or bayonet scissors (Fig. 5b, c). Specimens can
be taken from within the capsule and sent for frozen
intraoperative pathology consultation for diagnosis. The
accuracy of frozen tissue diagnosis is low due to hetero-
geneity and mixed cell tumors, however, and this should be
taken into consideration during intraoperative decision
making. Following initial sampling, the tumor is internally
debulked using a variety of instruments including suction,
cautery, tumor forceps, or an ultrasonic aspiration device if
necessary. Most tumors are soft and are amenable to
debulking with a large-bore Japanese-style suction with
variable control. As the tumor is decompressed, the capsule
can be separated more easily from the surrounding thala-
mus and midbrain. Most of the vessels along the wall of the
capsule are choroidal vessels and need not be preserved.
Capsule dissection continues until the third ventricle is
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encountered. The tumor is then carefully dissected inferi-
orly off the brainstem. This is often the most difficult
portion of the tumor dissection but can be facilitated by
retracting the tumor superiorly and dissecting it bluntly off
the brainstem under direct vision. Last, the superior aspect
of the tumor is removed after separating the attachments
along the velum interpositum and the deep venous system.
These can be carefully cauterized and sharply dissected,
although injury to the deep venous system can be difficult
to control and must be avoided.
The degree of tumor invasion dictates the intraoperative
decision regarding the extent of resection. Direct brainstem
invasion is the extreme example in limiting resection, and
the extent pursued is determined by the surgeon’s judg-
ment. Analyses of retrospective series have found an
association between extent of resection and survival for
pineal cell tumors including pineoblastoma, but other
smaller studies have failed to confirm this association [86–
88]. Our experience has shown that gross total resection
also decreases the risk for postoperative hemorrhage and
thus, should be pursued in the absence of contraindications.
Following complete tumor resection, the surgeon should
have a comprehensive view into the third ventricle
(Fig. 5d, e). Flexible mirrors are used to examine the
inferior portion of the tumor bed in order to verify the
extent of resection and to avoid leaving any blood clots.
Careful attention must be given to hemostasis [44, 81] with
direct but careful cautery preferred over the use of hemo-
static agents which can float into the ventricle and cause
CSF obstruction. If absolutely necessary, long strips of
Surgical draped over the surface of the cerebellum and
covering the tumor bed can provide hemostasis with small
risk of migration and subsequent obstruction.
Once hemostasis is obtained the dura is closed in as
watertight a manner as possible. The bone flap is plated
into place to reduce postoperative pain and inflammation.
The patient should be extubated with a reasonable degree
of head elevation to avoid shifting the decompressed brain
within the cranial vault.
Transcallosal interhemispheric approach
The transcallosal interhemispheric approach between the
falx and hemisphere of the brain involves a corridor along
the parieto-occipital junction. Dandy’s early work recog-
nized the importance of the deep venous system and the
cortical bridging veins between the hemisphere and the
sinus. Any of the aforementioned patient positions can be
used for this approach, although the prone or sitting posi-
tion is generally preferred.
A linear incision is used on the scalp. Subsequent
positioning of the bone flap is variable and depends on
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where the tumor resides in the third ventricle [79, 89, 90];
however, a wide craniotomy, provides flexibility in deter-
mining the corridor and avoiding bridging veins. The
craniotomy is generally centered over the vertex to avoid
manipulation of the occipital lobe. Burr holes are made
over the sagittal sinus, both anteriorly and posteriorly, and
a craniotome is used to turn a generous craniotomy. The
craniotomy should extend 1–2 cm to the left of the sagittal
sinus. Bleeding encountered from the sagittal sinus can be
controlled with hemostatic agents.
The dura is opened in a U-shaped fashion and reflected
medially toward the sagittal sinus. The bridging veins are
inspected, and an approach is chosen that will sacrifice the
minimum number of veins. Sufficient exposure can rarely
be achieved without sacrifice of at least one bridging vein,
although sacrifice of more than one should be avoided if
possible. Because these tumors are deeply seated, even a
small opening provides a wide angle of deep exposure. The
exposed hemisphere is covered with Bicol or Telfa and a
retractor system such as the Greenberg retractor is used to
frame the opening. Two retractors are placed to draw the
parietal lobe back in a gentle arc. These retractors can be
used along the falx, which may be divided inferiorly to
provide further retraction. This is generally a nonvascular
corridor and contains few adhesions between the falx and
the cingulate gyrus.
The corpus callosum is identified with the operating
microscope by its stark white appearance. The pericallosal
arteries are identified as a paired structure running over the
corpus callosum. These arteries can be retracted either
together to one side or with separate retractors to each side.
The opening into the corpus callosum, centered over the
maximal bulge of the tumor, is generally about 2 cm (see
Fig. 6a). Whereas posterior openings in the splenium have
been performed routinely without reported deficits, we
have generally avoided this approach to minimize the risk
of a disconnection syndrome. The corpus callosum is
generally thin and is opened by gentle suction and cautery.
The lateral extent of this opening is a balance between
exposing the tumor and avoiding damage to the pericallosal
arteries. If necessary, the tentorium and falx can be divided
to provide additional exposure (Fig. 6b).
Once through the corpus callosum, the dorsal surface of
the tumor can be seen, and early identification of the veins
of the deep venous system is necessary to prevent injury.
The importance of the deep venous system and the degree
of venous collaterals is a topic of anecdotal speculation, as
consequences of venous sacrifice in the posterior fossa are
not well-reported in the literature. Even so, there is some
evidence that one vein can be sacrificed safely in several
instances, but interruption of two would likely greatly
increase the risk of a devastating infarction [91]. Once the
tumor is exposed, it is debulked and then dissected as
J Neurooncol (2016) 130:351–366
Fig. 6 Transcallosal interhemispheric approach. a Overview of the corpus callosum opening. b Magnified view of the opening into the corpus
callosum. The falx and tentorium may be divided for further exposure (a from [79]; b from [117])
described previously. Leaving a ventricular drain in place
for 1 or 2 days is optional.
Occipital transtentorial approach
The occipital transtentorial approach is a variation of the
supratentorial approaches (Fig. 7, Video 2) [35, 92]. A
three-quarter prone or lateral position is generally pre-
ferred. This approach to the pineal region uses an oblique
trajectory for lesions that are essentially midline and may
therefore be disorienting to surgeons who are not familiar
with it. However, division of the tentorium provides
excellent exposure of the quadrigeminal plate, thus making
it particularly advantageous for tumors that extend
inferiorly.
A linear scalp incision is used. A burr hole is placed in
the midline over the sagittal sinus just above the torcula,
with a second burr hole 6–10 cm above this. A craniotome
is used to turn a generous craniotomy extending 1–2 cm to
the left of midline. Alternatively, it is feasible to place the
burr holes adjacent to the sagittal sinus without crossing the
midline. If this option is chosen, it is important to get as
close as possible to the sagittal sinus to avoid a bony
overhang that limits the operative view.
Retraction of the nondominant occipital lobe is aided by
gravity, owing to the three-quarter prone position, and
further facilitated by the lack of bridging veins near the
occipital pole. Mannitol and ventricular drainage are useful
for relaxing the brain and minimizing the risk for hemi-
anopia from excessive occipital lobe retraction. Additional
brain relaxation is obtained by reverse Trendelenburg bed
positioning. A brain retractor is initially placed to gently
361
retract the ipsilateral hemisphere. It can be removed as the
operation progresses and CSF is gradually drained.
Under the operating microscope, the straight sinus is
identified so that the tentorium can be divided adjacent to it
(Fig. 7a). Intraoperative Doppler ultrasound can be useful
for localizing the straight sinus, and avoiding it during the
tentorial opening. A retractor can be placed over the falx
for exposure. The falx can be divided to facilitate further
retraction if necessary. At this point, the arachnoid over-
lying the tumor and the quadrigeminal cisterns can be seen
(Fig. 7b). Tumor removal proceeds as described earlier
while taking care to avoid injury to the deep venous sys-
tem. Closure and hemostasis proceed similarly to methods
described above.
Transcortical transventricular approach
The transcortical transventricular approach uses a trajec-
tory through the right lateral ventricle via a transcortical
incision [78]. This approach is rarely preferred because the
exposure is limited and the need for a cortical incision is
undesirable. Obviously, an entry point should be chosen in
noneloquent cortex. Stereotactic guidance is often useful
with this approach and may be desirable for a tumor that
extends into the lateral ventricle.
Postoperative care
High potency corticosteroids should be maintained for the
first few days, then tapered with improvement of the
patient’s condition [81, 89]. Additionally, seizure
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362
Fig. 7 Occipital transtentorial approach. a Dividing the tentorium and falx can increase exposure of the pineal region, b tumor exposure after
dividing the tentorium, c operative photo of the diagram in b. d Operative photo after tumor removal (from [117])
prophylaxis is recommended during early recovery for
supratentorial approaches, but is not necessary long-term.
Lethargy and mild cognitive impairment are common,
making it difficult to evaluate neurological status in the
immediate postoperative period, particularly in patients
with extensive subdural air as a result of the sitting posi-
tion. In the first 72 h post-operatively, careful and frequent
neurological examinations are necessary. Any changes
should be investigated with appropriate radiographic
imaging to rule out the possibility of hydrocephalus,
hemorrhage, or residual air. Shunt malfunction caused by
air, blood, or operative debris is a frequent problem arising
during this timeframe. This is particularly worrisome
because deterioration and major morbidity can occur
rapidly. Early mobilization and ambulation, including
physical therapy and rehabilitation consults, improve
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J Neurooncol (2016) 130:351–366
outcomes. A drain placed at the time of surgery should be
removed or converted to a shunt in these first 72 h to
minimize infection risk. Lastly, MRI with gadolinium
contrast should be performed within 48 h to determine the
extent of resection and guide future management decisions
[93].
Complications
Patients frequently experience ocular symptoms in the
postoperative period, most notably impaired extraocular
movements, including limited upgaze and convergence
[37, 71, 81], and pupillary dysfunction, such as difficulty
focusing. These problems are often transient, though they
can persist for several months. A residual mild limitation of
J Neurooncol (2016) 130:351–366
upgaze is not unusual but bears little clinical significance.
Otherwise, permanent impairment is rare. As with most
neurological deficits, their persistence and magnitude are
proportional to the degree to which they were present
preoperatively [81, 94]. Similarly, ataxia is frequently
present but resolves within a few days after surgery.
More severe morbidity is rare but can be a sequela of
overzealous brainstem manipulation. This can lead to cog-
nitive impairment or, in its extreme form, to akinetic mutism.
Complications are more common in previously irradiated
patients, patients with invasive tumors, and those who were
progressively symptomatic preoperatively [81, 94].
One of the most devastating complications is hemor-
rhage into an incompletely resected tumor bed. Patients
with highly vascular, invasive tumors such as malignant
pineal parenchymal tumors are at greatest risk [19, 81, 95].
Small hemorrhages can be managed conservatively, but a
large hemorrhage may require immediate evacuation. Such
decisions must consider the possibility of obstructive
hydrocephalus. Another potential vascular complication is
venous infarct, which can extend into the midbrain with
devastating consequences. This rare and unpre-
dictable complication is thought to be the result of venous
insufficiency in a small subset of patients who cannot tol-
erate the sacrifice of bridging veins in the cerebellum.
There are several approach-specific complications to
consider as well. Brain retraction and sacrifice of bridging
veins in supratentorial approaches may lead to hemiparesis
[81, 96]. In particular, retraction of the parietal lobe may
cause sensory or stereognostic deficits on the opposite side
[89]. Occipital lobe retraction during the transtentorial
approach can cause visual field defects [34, 89, 97].
Although disconnection syndromes have been reported
with corpus callosum incisions, this has been rare in our
experience, even when the splenium is divided [79, 89].
Complications related to the sitting position include
subdural hematoma, hygroma, and ventricular collapse [61,
71, 81]. These conditions are also commonly self-limited.
Air embolism is rarely a problem but can be anticipated
intraoperatively by a drop in end-tidal carbon dioxide
levels or with Doppler monitoring.
Overall, pineal tumor patients are generally young and
have relatively few medical problems. Consequently, the
incidence of medical complications such as cardiac or
respiratory problems is low.
Surgical outcomes
Surgery in the pineal region is among the most arduous of
microsurgical challenges and good outcomes rely on the
expertise of individual surgeons. Outcomes have improved
dramatically with the advent of modern microsurgical
363
techniques. Large case series in the last 20 years report
operative mortality rates between 0 and 4 % and permanent
morbidity in 0–5.6 % of patients (Table 1) [39–41]. Long-
term outcomes remain largely reliant on tumor histology
and responsiveness to adjuvant therapy. Outcomes are
particularly variable with malignant tumors, but complete
surgical removal, excellent long-term follow-up, and
probable cure can be expected for benign tumors [44, 45,
61, 98–100].
Postoperative work-up
Imaging for follow-up planning should include the afore-
mentioned brain MRI with gadolinium contrast within 72 h
of surgery as well as a spinal MRI to look for spinal
seeding in patients with pineal cell tumors, malignant germ
cell tumors, and ependymomas [1, 44, 101, 102]. Spinal
imaging may be difficult to interpret early in the postop-
erative period because blood clots or operative debris may
mimic spinal metastasis. If the images are equivocal, serial
images should be obtained before instituting spinal irradi-
ation. CSF cytology should be evaluated by lumbar punc-
ture postoperatively, although this has not been particularly
helpful in predicting the metastatic potential of these
tumors [11, 44, 51, 103–105]. Overall, the incidence of
spinal seeding is low, and prophylactic spinal irradiation is
not recommended unless there is clear radiographic evi-
dence of metastasis [20, 44, 48, 106–110]. The one pos-
sible exception is in patients with highly malignant
pineoblastomas [44, 71, 111]. In addition to these imaging
studies, tumor markers, when present preoperatively,
should be measured in the postoperative period to serve as
a baseline for detecting early recurrence or for monitoring
response to treatment.
Conclusion
The extensive variety of possible histologic subtypes
makes it imperative to establish a tissue diagnosis in
patients with pineal region tumors. Management decisions
regarding adjuvant therapy, prognosis, and follow-up
strategies vary with the histologic diagnosis. Specialized
surgical and stereotactic techniques have evolved to pro-
vide the neurosurgeon with an array of safe and effective
options for obtaining a tissue diagnosis.
Advanced microsurgical techniques combined with
improved preoperative management and postoperative
critical care methods have made aggressive surgical
resection a mainstay of management. Aggressive surgical
resection has resulted in excellent long-term prognoses for
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nearly all patients with benign tumors and a large per-
centage of patients with malignant tumors.
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