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Journal of Neuro-Oncology 54: 287–299, 2001.

© 2001 Kluwer Academic Publishers. Printed in the Netherlands.

Surgical approaches to pineal region tumors

Kenneth M. Little1 , Allan H. Friedman1 and Takanori Fukushima1,2


1
Division of Neurosurgery, Duke University Medical Center, Durham; 2 Carolina Neuroscience Institute for
Skull Base Surgery, Raleigh, NC, USA

Key words: germ cell, infratentorial, occipital, pineal region tumors, supracerebellar, surgical approaches,
transtentorial, ventriculoscopy

Summary

Direct surgical resection of pineal region tumors has become safer, more effective, and now plays an essential role
in their management. Tissue diagnosis allows for the initiation of appropriate therapies and resection can be curative
or improve the efficacy of adjuvant therapies. Several approaches have been reported. Based on our operative
experience with 57 patients over a 20-year period, we conclude that the Infratentorial Supracerebellar and Parieto-
Occipital Paramedian Transtentorial approaches provide excellent exposure while allowing minimally invasive,
relatively low risk access to the majority of pineal region tumors. Indications, positioning, techniques, advantages,
and disadvantages are discussed. A review of other approaches, pertinent historical remarks, and a discussion of the
role of surgery in the contemporary management of pineal region tumors are presented.

Introduction interest in direct surgical treatment began to return and


the associated major morbidity and mortality has since
The repertoire of surgical approaches to pineal region fallen to less than 7% [1–8,21–26].
tumors has evolved considerably over the past 100 Based on our experience with 57 pineal region tumor
years with an associated decline in operative mortal- resections over a 20-year period (1980–2000), we con-
ity from 100% to less than 4% [1–8]. Victor Horsley clude that the majority of pineal region tumors can be
is credited with the first attempt at pineal tumor resec- approached either from the Infratentorial Supracere-
tion in 1905 through an infratentorial approach, but the bellar approach or the Parieto-Occipital Paramedian
first success was not reported until almost a decade Transtentorial (POTT) approach with excellent expo-
later by Krause through an infratentorial supracerebel- sure and minimal morbidity. Our purpose is to discuss
lar approach [9–11]. Some proposed approaches were our selection of surgical approaches as well as the appli-
more direct, involving incision or block removal of cation of less invasive microsurgical techniques.
parietal and occipital cortex producing homonymous
hemianopsia or hemianesthesia [12,13]. Another stan-
dard approach introduced by Dandy involved division Preoperative considerations
of the posterior corpus callosum leading to a partial
cerebral disconnection syndrome [14,15]. Given the A variety of surgical approaches to pineal region
associated morbidity, operative mortality (as high as tumors have been proposed (Table 1). These
80%), and a lack of evidence demonstrating a therapeu- approaches may be divided into anterior and poste-
tic benefit from resection, many neurosurgeons came to rior; posterior approaches being further divided into
rely on indirect therapy, consisting of CSF shunting and supratentorial and infratentorial (Figure 1). Based on
radiation [16–20]. With the introduction of approaches a review of the literature, the most frequently utilized
designed to minimize neurologic deficits, the advent approaches during the past two decades have been
of the operating microscope and new imaging modal- the Occipital Transtentorial (OTT) and Infratentorial
ities, and the refinement of microsurgical techniques, Supracerebellar (ITSC) [1–5,7,8,24–48].
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Table 1. Summary of important surgical approaches to the pineal region divided into
(1) anterior and (2) posterior with posterior being divided into (a) supratentorial,
(b) infratentorial, and (c) combined.

Approach Reference
Anterior neuroendoscopic
Ventriculofiberscope Fukushima [78,79]
Posterior-supratentorial
Parietooccipital paramedian transfalcine Foerster [61,62], Fukushima [78,79]
Interhemispheric transcallosal Dandy [14]
Interhemispheric retrocallosal Glasauer [63], McComb et al. [64]
Occipital transtentorial Heppner, Poppen [67], Jamieson [68]
Posterior-infratentorial
Infratentorial supracerebellar Krause [48], Stein [49]
Infratentorial paramedian supracerebellar Yasargil [46]
Posterior-combined
Combined supra/infra-tentorial transsinus Sekhar [6,70]

Major or original contributors are indicated.

Figure 1. Summary of pineal region surgical approaches.


Approaches to pineal region tumors may be divided into ante-
rior (1) and posterior (2–4). Posterior approaches may be fur- Figure 2. Sagittal view of the pineal region: Pi = pineal gland;
ther sub-divided into supratentorial (2 & 3) and infratentorial (4). Sp = splenium of corpus callosum; vG = vein of Galen; vR =
Most pineal region tumors can be accessed through the operative basal vein of Rosenthal; ICv = internal cerebral vein; SS = strait
corridors depicted by 2 (Parietooccipital Paramedian Transtento- sinus; C = confluence of sinuses; Te = tentorium cerebelli;
rial with or without Transfalcine extension) or 4 (Infratentorial Fa = Falx cerebri.
Supracerebellar).
Pineal region anatomy
In our series of 57 patients, we primarily utilized
the ITSC and POTT (with or without transfalcine The pineal gland is a diencephalic structure protrud-
extension) approaches. The OTT and Infratentorial ing from the posterior wall of the third ventricle, sitting
Paramedian Supracerebellar approaches have also within the quadrigeminal cistern. The pineal region is
proven effective under certain circumstances [49,50]. defined dorsally by the splenium of the corpus callo-
The most appropriate approach to a particular lesion sum and tela choroidea; ventrally by the quadrigeminal
depends primarily upon (1) the lateral, rostral–caudal, plate; rostrally by the posterior third ventricle; caudally
and anterior–posterior extension of the tumor, (2) the by the vermis of the cerebellum; and laterally by the
tumor’s relation to the vein of Galen venous complex, thalami and the tentorial edges (see Figure 2).
(3) the suspected histopathology and vascularity of The pineal region contains critical venous struc-
the lesion, and (4) the surgeon’s experience with each tures that are often intimately related to pineal region
approach. tumors. They must be surgically negotiated and, with
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few exceptions, not sacrificed [51]. In this region, the outweigh the potential benefits. Furthermore, most
great cerebral vein of Galen is formed by the union of cases of obstructive hydrocephalus will resolve after
the internal cerebral veins, basal veins of Rosenthal, radical resection. In most cases, therefore, we proceed
precentral cerebellar vein, vermian vein, and the inter- with radical resection, trying to avoid permanent CSF
nal occipital veins. The vein of Galen, in turn, drains diversion.
into the strait sinus which runs between dural layers
where the tentorium cerebelli joins the falx cerebri
[51,52]. Identifying the tumor’s position in relation to Surgical approaches
these anatomic structures by MRI is critical in select-
ing the surgical approach. Pertinent relationships are Infratentorial supracerebellar approach
the tumor’s position relative to the vein of Galen com-
plex and its extension laterally, caudally along the History
quadrigeminal plate into the cerebellomesencephalic In 1911, Fedor Krause suggested the infratentorial,
fissure, and rostrally into the third ventricle. supracerebellar approach for tumors in the pineal
region and in 1913 this approach was used with the
Management of hydrocephalus patient in the sitting position for the first success-
ful pineal region tumor resection [48–50]. The ITSC
Approximately 90% of patients with pineal region approach was refined with microsurgical techniques
tumors have hydrocephalus at the time of presenta- and popularized by Stein [24,58].
tion and cerebrospinal fluid (CSF) dynamics should be
stabilized prior to further treatments [26,37,53,54]. In Indications
our experience, however, most cases of hydrocephalus The ITSC approach is best indicated for relatively small
resolve after radical pineal region tumor resection. to medium size tumors that are confined to the midline
Patients presenting with non-acute hydrocephalus with rostral/caudal extension in the sagittal plane. The
are often managed with a ventriculostomy catheter tumor must lie below the vein of Galen complex with-
placed at the time of resection, leaving it in place during out inferior extension into the cerebellomesencephalic
the perioperative period for intensive care patient (ICP) fissure or lateral extension beyond the tentorial edges.
management. Patients who present with acute obstruc-
tive hydrocephalus undergo emergent ventriculostomy Anatomic considerations
or, in some cases, ventriculoperitoneal shunt (VPS) This approach allows for minimal brain retraction
placement. CSF is sent for tumor markers (β-HCG, because of the natural plane between the cerebellum
AFP) and cytology during the first CSF diverting pro- and tentorium. The torcular Heropheli and transverse
cedure. Because there is a risk of peritoneal metastasis sinuses are at risk during opening. A few bridging veins
after VPS placement in patients with malignant germ from the midline superior cerebellar surface to the ten-
cell tumors or pineoblastoma, we avoid VPS placement torium can be safely sacrificed, however, lateral dor-
whenever possible [55–57]. sal cerebellar bridging veins and, in particular, petrosal
Patients who present with hydrocephalus may be veins must be avoided to minimize the risk of postop-
managed with a third-ventriculostomy combined with erative cerebellar venous congestion and swelling. The
an attempted tumor biopsy via ventriculoscopy through vein of Galen system lies dorsally to tumors selected
an anterior burr hole. Though tumor manipulation with- for this approach, interfering less with tumor resection.
out subsequent VPS placement may also increase the
risk of CSF spread, tumor dissemination can some- Positioning
times be avoided by careful ventricular irrigation via Sitting. This position allows for less cerebellar
externalized ventricular drain (EVD) prior to third- retracion, decreased venous pressure, and familiar
ventriculostomy [54]. Though the recent trend in anatomical orientation [26,49,58]. However, it has been
neurosurgery is towards the minimally invasive neu- associated with a higher risk of air embolism, subdu-
roendoscopic third-ventriculostomy, we find that poor ral hematoma, pneumocephalus, and the potential for
long-term efficacy, the uncertain diagnostic yield from decreased brainstem perfusion. Furthermore, the sur-
endoscopic biopsy, the risk of CSF tumor dissem- geon’s arms must remain extended leading to discom-
ination, and the risk of biopsy-related hemorrhage fort and fatigue.
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Concorde. This position, proposed by Kobayashi to be retracted by gravity. The risks of air embolism
et al., is an alternative to the sitting position that and brain stem hypoperfusion are significantly reduced
decreases the risk of air embolism [40]. However, compared to the sitting position.
we have found that the cerebellum must be retracted
against gravity risking contusion. Venous drainage may Dissection
be decreased possibly causing elevated intracranial A linear skin incision is made beginning 3 cm above
pressure. To orient the microscope optimally, further- the inion extending to the level of the first cervical ver-
more, the surgeon must lean sideways at the waist over tebra (Figure 4a). Burr holes are made above the trans-
the patient. This physically demanding posture can lead verse sinus, on either side of the superior sagittal sinus
to unnecessary back strain and distraction. and below the transverse sinus, approximately 5 cm on
either side of the midline. A large bone flap is turned
Reverse transsphenoidal. For the past 20 years, we extending from above the torcular Heropheli and trans-
have been primarily using the reverse transsphenoidal verse sinuses to the foramen magnum. Extending the
position , a variation of the Three-quarter Lateral Prone craniotomy above the torcular will allow for a broader
position (Figure 3). The Mayfield 3-pin headholder is exposure through upward retraction. The dura mater
applied as shown in Figure 3. The left side of the head is is opened with an inverse semicircular incision, based
positioned down for right-handed surgeons, right side at the transverse sinuses extending the full width of
down for left-handers. The shoulders are rotated three- the craniotomy (Figure 4b). Arachnoid adhesions are
quarters prone with the head rotated nearly prone and sharply dissected and, as midline bridging veins are
flexed anteriorly. The torso is elevated 30◦ relative to encountered, they are cauterized allowing the cerebel-
the floor with the left arm over the top of the bed at lum to fall down from the tentorium with gravity. As
90◦ of shoulder abduction and 90◦ of elbow flexion. much as possible, lateral bridging veins need to be
The right arm is supported on an arm-board moder- preserved to facilitate cerebellar venous drainage. A
ately stretched with 45◦ of anterior extension and 45◦ of 2 mm tapered retractor is placed with gentle down-
lateral abduction. This position provides comfortable, ward pressure onto the superior vermis and advanced
efficient surgeon access while allowing the cerebellum

Figure 4. (A) Skin incision and bone flap for the ITSC approach.
The ITSC approach requires a relatively larger suboccipital osteo-
plastic craniotomy. A linear skin incision is made from 3 to 4 cm
above the inion to the level of the C1 vertebral body. Burr holes
should be made above the transverse sinus on either side of the
Figure 3. Right-Side-Up Reverse Trans-sphenoidal position for superior sagittal sinus and below the transverse sinus medial to
the ITSC approach. This position is similar to the Three-quarter the sigmoid sinuses. The craniotomy should extend from the fora-
Lateral Prone position. Critical positioning details include: men magnum to above the transverse sinus in order to allow
(1) Right shoulder rotated three-quarters prone; (2) Head rotated upward retraction of the torcular Heropheli. (B) Dural flap. The
nearly completely prone with anterior neck flexion; (3) Torso up dura mater is opened with an inverse semicircular incision, based
30◦ ; (4) Left arm beyond the top of and below the table with 90◦ at the transverse sinuses extending the full width of the cran-
of shoulder abduction and 90◦ of elbow flexion; (5) Right arm iotomy. Relief cuts may be made to allow for maximum upward
moderately stretched with 45◦ of anterior extension and 45◦ lat- retraction. Upward retraction may be used to further elevate the
eral abduction supported on an arm board. torcular Heropheli and expand the field of view.
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retraction resulting in minimal morbidity, less risk of


cerebellar swelling, and greater surgeon comfort.

Disadvantages
Because of the tentorium cerebelli, the vein of Galen
system, and the inability to look down at the midbrain,
the ITSC exposure is narrow and deep, providing dif-
ficult access to larger tumors and tumors with lateral
or superior–inferior extension. This approach is con-
traindicated when the tumor extends superiorly above
the apex of the straight sinus, inferiorly into the cere-
bellomesencephalic fissure, or laterally beyond the ten-
Figure 5. Deep exposure along the ITSC operative corridor. vG = torial edge. In cases with dominant midline cerebellar
vein of Galen; vR = basal vein of Rosenthal; PC = precentral drainage, ligation of bridging veins still carries the risk
cerebellar vein; Tu = tumor; r = tapered retractor blade; Te = of postoperative cerebellar swelling with obstructive
tentorium cerebelli. hydrocephalus or tonsilar herniation [59].

separating the superior cerebellar surface from the ten-


torium along the midline. Once the thick arachnoid Parietooccipital paramedian transtentorial
membrane is opened widely to expose the precentral approach
cerebellar vein and CSF is aspirated, the retractors may
no longer be necessary. History
The precentral cerebellar vein will be found within In 1921, Dandy proposed the posterior transcallosal
the arachnoid membrane and can be preserved. How- exposure of pineal region tumors in humans [14,60].
ever, in the majority of cases the precentral cerebellar His exposure was associated with significant morbid-
vein can be coagulated and divided with little conse- ity as it involved a large parieto-occipital bone flap,
quence. It is essential, however, to preserve the internal sacrificing bridging veins, retracting the brain against
cerebral veins and basal veins of Rosenthal to avoid tha- gravity, and incising the splenium of the corpus cal-
lamic venous infarction. The tumor will be found ante- losum [15]. In 1929, Foerster described his experi-
rior to the precentral cerebellar vein, inferior to the vein ence in three patients in whom he exposed the pineal
of Galen, and medial to the basal veins of Rosenthal region and quadrigeminal plate through an interhemi-
(Figure 5). spheric approach. His dissection proceeded between
The arachnoid adhesions must be opened widely the falx and the occipital lobe dividing the inferior
to the tentorial edge to gain adequate tumor expo- sagittal sinus and falx cerebri medially and the tento-
sure avoiding damage to critical venous structures. rium posteriorally [62]. In 1970, Glasauer reported a
Ultra-long microinstruments such as those used in variation of the OTT approach that involved a more
the transsphenoidal approach are necessary to perform anterior approach along the mesial border of the occipi-
accurate microsurgical dissection in the pineal region. tal lobe [63]. The advantage over the OTT approach was
After the tumor is exposed, tumor tissue is taken for better access to inferiorly extending tumors while pre-
intraoperative frozen sections and gross-total removal serving the splenium of the corpus callosum. McComb
is performed. et al. described a similar approach; the posterior intra-
hemispheric retrocallosal approach to the third ven-
tricle region [64]. To gain access to the pineal region
Advantages while preserving the splenium, the approach is directed
This approach provides central access to the mid- through the tentorial incisura. To expand the exposure,
line pineal region from below the vein of Galen and they suggest tentorial incision, falx incision, and sple-
its tributaries. Once these venous structures are sep- nial retraction. The POTT described here begins more
arated from the tumor, they no longer significantly anterior than those described by Foerster, Glasauer
obstruct the surgeon’s view of this region. The reversed and McComb et al. but more posterior than Dandy’s
transspenoidal position requires only modest cerebellar approach.
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Indications
We recommend this approach for larger tumors or
tumors extending too far beyond the midline to be
managed by the ITSC approach. It is excellent for
tumors with extension inferiorly into the cerebellomes-
encephalic fissure, superiorly above the apex of the
straight sinus, or laterally beyond the tentorial edge.
Also, with posterior extension of the bone flap, the
microscope angle can be changed and access can be
gained to tumors arising from or extending into the
third ventricle.

Anatomic considerations
Though preserving the superior sagittal sinus is a con-
Figure 6. Positioning for the POTT approach. Critical position-
cern, the primary risk early in the dissection is asso-
ing details include: (1) Operated-side down; (2) Torso in lateral
ciated with negotiating the parasagittal veins. Though decubitus position 30◦ to the floor; (3) head flexed laterally to
the posterior one-thirds of the superior sagittal sinus allow a comfortable microscope viewing angle of 45◦ . An elon-
receives few draining veins, veins are likely to be gated, three-sided skin incision is made over the parieto-occipital
encountered at the anterior limits of the dissection. juncion based just over the midline.
Major draining veins from the occipital lobe often
travel along the mesial superior border of the brain the left side in the dependent orientation. If the tumor
for some distance prior to entering the sagittal sinus. has roughly equal lateral extension, the non-dominant
This allows the surgeon a corridor to the mesial pari- hemisphere is placed in the dependent orientation. The
etal surface. Sacrificing these veins should be avoided. vertex is tilted up until the falx is at a 45˚ viewing angle
The other venous structures to be preserved by the sur- from the surgeon’s perspective. The body is placed in
geon through this approach are the internal cerebral the supine position if the patient is relatively thin with a
veins and internal occipital vein. They are likely to be mobile neck and in the lateral decubitus position when
encountered dorsal to the tumor and should be pre- the patient is relatively large with an immobile neck.
served to avoid venous hypertension and hemorrhage. This orientation allows for decreased intracranial
While preservation of these veins is common wisdom, venous pressure without significantly increasing the
there is a lack of definitive evidence supporting this risk of air embolism. The right cerebral hemisphere
fear [64]. Should the falx need to be incised to broaden falls away from the falx with gravity, thus minimizing
access to the contralateral quadrigeminal cistern, the the need for retraction. This position has the advan-
inferior sagittal sinus may be coagulated and divided, tages of allowing a comfortable position for the sur-
we have encountered no ill effects contributable to its geon, and allowing the operative cerebral hemisphere
sacrifice. The splenium of the corpus callosum will be to be retracted away from the falx cerebri by gravity
encountered in the deep, anterior aspects of the expo- (facilitated by CSF drainage). Also, with the operative
sure. Excessive retraction or incision of this structure corridor oriented to allow a neutral microscope view-
should be avoided to prevent hemialexia [65]. Finally, ing angle of 45◦ , it is convenient to manipulate the
the tentorium may be divided to gain better access microscope over a wide range of angles to maximize
to the caudal quadrigeminal cistern; in doing so care the excellent exposure provided by this approach.
must be taken to preserve the straight and transverse
sinuses. Dissection
A three-sided, elongated skin incision is made cen-
Positioning tered over the parieto-occipital junction with the medial
The 3-pin Mayfield headholder is placed as shown in aspect just across the midline (Figure 6). Burr holes are
Figure 6. The head is oriented with the operative side placed on the dependant side of the midline, just lateral
down and the falx cerebri parallel to the floor in the to the superior sagittal sinus, at the anterior and poste-
anterior–posterior direction. If the tumor is primar- rior aspects of the proposed flap. Additional burr holes
ily eccentric to the left, the patient is positioned with are place at the inferior corners. A large osteoplastic
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bone flap is removed. The dura is opened in a similar Long microinstruments should be used to allow effec-
manner and reflected with its base towards the superior tive microdissection.
sagittal sinus, taking care to preserve the superficial
cortical veins. Advantages
The dependent cerebral hemisphere is dissected The primary advantage of this approach over other pos-
from the falx while taking care to preserve the bridg- terior approaches is that it provides a better view of
ing veins when possible, particularly at the anterior the caudal quadrigeminal cistern for tumors extend-
extent of the exposure. Bridging veins at risk for avul- ing inferiorly. Infratentorial approaches would require
sion are sharply dissected from the arachnoid allowing extensive cerebellar retraction to gain access into
safer mobilization of the hemisphere away from the this region. As with the more posterior supratentorial
falx with gravity. Small veins that must be sacrificed approaches discussed above, the splenium is preserved
are coagulated and divided. After CSF decompression but may be retracted anteriorly to extend the exposure.
through an EVD, lumbar drain, or brian needle and gen- Similar to the posterior interhemispheric approaches,
tle pressure against the mesial aspect of the dependent Dandy’s approach, and Foerster’s approach, the infe-
hemisphere, 2 mm tapered retractors are placed against rior sagittal sinus and falx may be divided to expose
the brain laterally and the falx medially (Figure 7). the contralateral cerebellomesencephalic cistern bet-
After the incisura is reached, greater anterior exposure ter. Furthermore, this approach is less invasive than
may be gained with gentle anterior retraction of the the combined supra/infratentorial transsinus approach
splenium. Access to the cerebellomesencephalic fis- described by Sekhar [6].
sure may be gained by incising the tentorium paral-
lel to the straight sinus. Contralateral access may be Disadvantages
gained by coagulating and dividing the inferior sagittal This approach places the anterior occipital and poste-
sinus and incising the falx. Stay sutures may be placed rior parietal bridging veins at risk. In our experience,
to retract the divided falx and tentorium. The tumor however, we have been able to preserve most or all of
will be visualized beneath the vein of Galen complex. these by opening a large craniotomy flap which allows
Extreme care should be taken to preserve these veins. for small adjustments in the approach. The second dis-
advantage is related to operator experience. The ori-
entation is not as straightforward as with the concorde
position and may require frequent reorientation.

Other approaches

Infratentorial paramedian supracerebellar


approach

A variation on the ITSC approach was described by


Yasargil [46]. In 1990, Van den Berg described this
approach for the resection of pineal region tumors [47].
It is indicated for tumors located below the vein of
Galen complex that project laterally beyond the mar-
gins of the tentorial incisura or inferiorly into the cere-
Figure 7. Deep exposure along the POTT operative corridor. The bellomesencephalic fissures. This approach may be
parietal (Par) and occipital (Oc) lobes are retracted gently down- used when the midline supracerebellar approach will
ward with two tapered retractor blades (r). The tentorium cere- not allow lateral access beyond the tentorial incisura
belli (Te) is incised lateral to the strait sinus (SS) to expose the or deep into the cerebellomesencephalic fissure, where
quadrigeminal cistern. The falx (F) is retracted upward and, if access would lead to excessive cerebellar retraction.
necessary for contralateral exposure, incised after coagulation of At our institution, we have used this approach in
the inferior sagittal sinus (ISS). The tumor (Tu) is visualized after
gentle anterior retraction of the splenium (Sp) beneath the vein
three cases (two pinealoblastomas and one midbrain
(vG) of Galen system. ICv = internal cerebral vein; vR = basal exophytic glioma). In general, we find that the lateral
vein of Rosenthal; IOv = internal occipital vein. and inferior access offered by this approach can be
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better gained through the POTT approach (with or with- operative corridors between the veins of the Galenic
out division of the falx). Though not reported, the risk system.
of cerebellar infarction and swelling is theoretically
greater in the infratentorial paramedian supracerebel- Combined supra/infratentorial transsinus
lar approach compared to the midline approach as approach
anatomic studies suggest that more bridging veins
from the cerebellar surface to the tentorial sinuses Initially described by Sekhar for the resection of a
may have to be sacrificed over the lateral superior pineal region meningioma, this approach has been pro-
cerebellar surfaces [66]. This approach can be help- posed for the resection of giant pineal region tumors
ful when the patient has a steep tentorial apex and a [6,70]. The indications set forward by Ziyal et al. are
midline approach would require excessive cerebellar (1) tumors with a large diameter (>4.5 cm), (2) tumors
compression. that extend well above and below the plane of the ten-
torium, (3) tumors that extend well below the plane of
Occipital transtentorial approach cerebellar retraction, (4) tumors that are very vascular
or that encase important venous structures as they may
The OTT approach was described by Heppner in need to be approached from many sides.
1959 and popularized by Poppen in 1966 [49,67]. The primary benefit of this approach is that it pro-
Because Poppen’s variation was technically difficult vides the combined access of the ITSC and OTT
and revealed a narrow exposure, the modification pro- approaches. The complications of transverse sinus lig-
posed by Jamieson in 1971 has become the preferred ation after intraoperative test occlusion are reportedly
version [49,68]. The OTT approach has been utilized modest but the risk of permanent venous drainage com-
to access pineal region tumors with superior extension promise remains a considerable concern. Many tumors
above the incisura, inferior extension into the cerebel- that meet the criteria outlined above could be resected
lomesencephalic fissure, and lateral extension beyond more safely with greater exposure using the POTT
the medial borders of the tentorium. approach.
Patients are positioned in the concorde position or
in the three-quarters prone position with the dominant Posterior interhemispheric trans/retro-callosal
hemisphere down. Though orientation may be easier
to maintain, the concorde position is not recommended In 1915, Dandy described the posterior transcallosal
due to its association with decreased venous drainage exposure of pineal region tumors in canines and in 1921
and surgeon discomfort [69]. proposed its use in humans [14,60,71]. From that time
Compared to the ITSC approach, the OTT approach until a decade later, he had seven fatalities before he
allows for wider dissection around tumors or vascu- succeeded in resecting a pineal region tumor [15]. His
lar lesions [69]. By incising the tentorium adjacent and exposure involved a large parieto-occipital bone flap,
parallel to the strait sinus, access into the quadrigem- sacrificing bridging veins, retracting the brain against
inal cistern is gained without cerebellar retraction. gravity, and incising the splenium of the corpus cal-
Because of the orientation of the operative corridor, losum. The deep veins were not infrequently sacri-
this approach also allows a better view of the third ficed. Although this approach was associated with a
ventricle. The risk of venous infarction is less, as just mortality rate of up to 90%, it was preferred by most
above the tentorium, there are less likely to be bridging neurosurgeons before the introduction of the operating
veins between the occipital lobe surface and the supe- microscope [24].
rior sagittal sinus. Modern microsurgical techniques have been incor-
In our experience, the benefits gained from the OTT porated to make this approach safer and more versatile
approach are greater with the POTT approach, with- for accessing pineal region tumors [64]. With modi-
out a significant increase in morbidity. Though the fications including tentorial or falcine incision, better
OTT approach provides an excellent view into the quadrigeminal cistern exposure is gained. With gentle
third ventricle, it limits the surgeon’s ability to visu- retraction of the splenium or a posterior transcallosal
alize ipsilateral tumor extension, the distance between incision, access to the posterior third ventricle can be
the surgeon and the pineal region is increased, and gained. McComb et al. provide an excellent discus-
the surgeon is more likely to be limited by narrow sion of the posterior interhemispheric retrocallosal and
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transcallosal approaches in the right lateral decubitus for access to the floor of the third ventricle and one
and three-quarters prone positions [64,72]. more anterior for access to the posterior third ventri-
cle [54,80]. To minimize CSF dissemination, Oi et al.
Anterior approaches suggest irrigation through the coronal burr hole EVD
during and after endoscopic tumor manipulation [54].
Pineal region tumors may be approached anteriorly After this, the floor of the third ventricle is opened. CSF
when there is significant anterior extension into the should again be obtained for cytology from the EVD
third ventricle. These approaches are particularly effec- prior to removing it in the postoperative period. Endo-
tive for tumors arising from within the third ventricle scopic biopsies may be more accurate than stereotactic
with extension into the pineal recess and quadrigemi- biopsies because of the heterogenatity of pineal region
nal cistern. The primary approaches are transcallosal– tumors [80]. It may be possible to obtain a more exten-
transchoroidal and transcortical–subchoroidal. The sive and representative biopsy after direct visualization
pineal region or third ventricle should only be accessed of the tumor’s gross morphology by ventriculoscopy.
through the transcortical route if ventricular dilatation
is present. With the transcallosal approach, the ven-
tricle is entered medially and the choroidal fissure is Discussion
opened above the choroid plexus [73,74]. With this
approach, there is less risk to central venous structures Historical remarks
draining the basal ganglia and internal capsule. With
the transcortical approach, the choroidal fissure should Initial attempts at pineal region tumor resection were
be entered sub-choroidally to avoid excessive lateral frequently met with unsatisfactory results including an
retraction [75]. inability to reach the pineal region, limited resections,
One of the major limitations of this approach is and high morbidity and mortality [9–11]. Despite these
encountered with tumors that extend below the col- frustrations, there were many noteworthy innovations
licular plate. The risks associated with this approach prior to the era of microneurosurgery that contributed
include damage to bridging veins or the superior sagit- greatly to contemporary pineal region surgery.
tal sinus, forniceal damage with memory deficits, and Victor Horsley is credited with attempting the first
damage to centrally draining veins with basal gan- surgical resection of a pineal tumor in 1905 [11]. He
glia or internal capsule hypertension. The transcortical used an infratemporal approach and, after an additional
approach carries the additional risk of postoperative failed attempt in 1909, he recommended a supraten-
seizures. torial approach with splitting of the tentorium [9,10].
Other unsuccessful attempts were reported from 1908
Endoscopic management to 1911 [11].
In 1910 Ludwig Pussep is reported to have been the
The appeal of neuroendoscopy is that it may pro- first to successfully reach the pineal region by a direct
vide a minimally invasive means of both (1) treat- surgical approach via an occipital approach in which
ing hydrocephalus and (2) obtaining tissue diagnosis he split the transverse sinus and tentorium [11]. His
in patients who may have chemo- or radiosensitive success was limited as the child died on the third post-
tumors. Neuroendoscopic procedures initially devel- operative day. Regardless of high morbidity and mor-
oped as an approach to intraventricular lesions, specifi- tality rates, some surgeons persisted and developed less
cally third ventricular [76,77]. Fukushima first reported invasive approaches that were later improved to yield
the use of the flexible ventriculofiberscope for pineal better results.
region tumor biopsies [78,79]. This method is less An important accomplishment occurred in 1913
invasive than with rigid ventriculoscopes because it when Fedor Krause successfully resected a 4 cm pineal
is more likely that both the posterior third ventricle tumor with good results [11]. With the patient in
and the floor of the third ventricle can be accessed for the sitting position, the tumor was resected using an
tumor biopsy and third-ventriculostomy, respectively, infratentorial–supracerebellar approach. The impor-
through a single anterior burr hole. With rigid scopes, tance was that this approach took advantage of a natural
it is often necessary to enter through two burr holes plane between the cerebellum and tentorium without
(the ‘biportal’ approach); one on the coronal suture the need for sacrificing neural structures.
296

In 1915, Dandy described the posterior transcallosal (4) the relief of mass effect and, potentially, obstructive
exposure of pineal region tumors in canines and in 1921 hydrocephalus [53].
proposed its use in humans [14,60,71]. From that time
until a decade later, Dandy had seven fatalities before Tissue diagnosis. Histopathology is the most reliable
he succeeded in resecting a pineal region tumor [15]. prognostic factor and it guides the type and extent
Although this approach was associated with a mortality of adjuvant therapy [83–87]. Germinomas, for exam-
rate of up to 90%, it was preferred by most neurosur- ple, are extremely radiosensitive yet their course is
geons before the introduction of the operating micro- unaffected by resection whereas benign teratomas and
scope [24]. meningiomas are curable with radical resection alone
Van Wagenen in 1931 reported a direct trans- and are poorly responsive to chemotherapy or radi-
temporoparietal cortex, transventricular approach to ation. Pineal region low-grade gliomas, furthermore,
the pineal region [13,81]. The approach relied on dilata- are best treated with radical resection alone whereas
tion of the right lateral ventricle and left patients with patients with high-grade gliomas should be treated with
visual field deficits. In 1937, Horrax reported an occipi- chemotherapy and radiation after resection.
tal and partial temporoparietal lobectomy for the resec-
tion of a pineal tumor [81]. Extensive cortical resection Radical resection versus stereotactic and neuroen-
was carried out to access a huge pineal region tumor. doscopic biopsy. Compared to ventriculoscopic or
These posterior transcortical approaches have been stereotactic biopsy, open radical resection offers
largely abandoned and are not recommended due to several benefits. For non-germinomatous germ cell
the concomitant high morbidity and the availability of tumors, an increase in survival is correlated with the
safer, more effective approaches. extent of resection [87]. Furthermore, cytoreduction
The OTT approach was initially described by enhances the efficacy of adjuvant therapis, especially
Heppner in 1959, popularized by Poppen in 1966, in the case of malignant germ cell tumors [84]. Addi-
and modified to its most preferred form by Jamieson tionally, open resection may relieve aqueductal steno-
in 1971. It is one of the most frequently reported sis and obstructive hydrocephalus.
approaches in recent literature [1–5,27,32]. Mortality rates between open resection and stereo-
The most formidable obstacles to pineal region tactic biopsy are comparable. Regis et al. in a review
approaches at the beginning of the 20th century were of 370 cases of pineal region tumor biopsies, reported
the lack of an operating microscope and a limited an overall mortality of 1.3%, morbidity of 0.8%, and
understanding of the surrounding anatomy, particularly diagnostic yield of 94% [88]. Thus, a sub-optimal diag-
critical deep venous structures that were often sacri- nostic rate with no possible benefit of gross-total resec-
ficed. Prior to microneurosurgery, the high operative tion or cytoreduction are achieved in the context of a
morbidity and mortality led most neurosurgeons to pur- mortality rate comparable to open resection. Stereo-
sue more conservative therapy consisting of CSF shunt- tactic biopsy may not adequately sample tumors of
ing and radiation therapy [16–18,20]. After the advent mixed-germ-cell histopathology, leading to diagnostic
of microneurosurgery, CT, and MRI, interest in direct errors and possibly suboptimal treatment. Furthermore,
surgical treatment began to return [21–26,38]. some of the patients undergoing stereotactic biopsy
may require open resection based on biopsy results,
The role of surgery in the management of placing them at the additional risk associated with a
pineal region tumors second surgery. In our practice, we first perform a ven-
ticuloscopic biopsy if there is concomitant obstruc-
Pineal region tumors are challenging to treat because tive hydrocephalus, allowing for concurrent third-
they consist of a broad spectrum of histopathalogy ventriculostomy. Stereotactic biopsy is performed if
and are intimately related to critical neurovascular the tumor has strong radiographic and tumor marker
structures; the indications for and timing of direct (serum and/or CSF) evidence of germinoma. In addi-
surgical resection remain subjects of debate. Direct sur- tion, we avoid open resection in patients with evidence
gical resection can provide (1) tissue diagnosis with of multifocal or disseminated disease at presentation.
a greater diagnostic yield than stereotactic biopsy,
(2) a cure with benign lesions such as meningioma Timing of surgery. Because of the importance of
and benign teratoma, (3) cytoreduction to improve the obtaining a tissue diagnosis, direct surgical resec-
efficacy of chemotherapy and radiation therapy, and tion, endoscopic biopsy, or stereotactic biopsy are
297

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