Papillary Craniopharyngioma —
Mitsuhiro
TADA,
Case
of the Third Ventricle
Report—
Toshimitsu AIDA, Mitsuyuki KOIWA*, Yoshimi Takeshi KASHIWABA* and Hiroshi ABE
CHONO*,
Department of Neurosurgery, Hokkaido University School of Medicine, Sapporo; *Kashiwaba
Neurosurgical
Hospital
, Sapporo
Abstract A papillary clinical
craniopharyngioma
presentation
papilloma ventricle,
and
in the
Key words:
approach
papillary resonance
without
neurological
craniopharyngioma,
third
Craniopharyngiomas are epithelial neoplasms occur ring in the sellar/suprasellar region and comprise 2-3% of all intracranial neoplasms. Occurrence elsewhere in the intracranial compartment is very rare. There are two histological subtypes: the classic adamantinomatous craniopharyngioma, and the less common squamous cell craniopharyngioma, often referred to as papillary craniopharyngioma due to the prominent papillae formation sometimes pre sent.') Here, we describe a patient with papillary craniopharyngioma localized in the third ventricle manifesting with a peculiar clinical presentation and a neuroradiological appearance mimicking a choroid plexus papilloma of the third ventricle. Case
Report
male visited
our outpatient
clinic com
plaining of a dull headache persisting for 2 months. Neurological examination disclosed no abnor malities except slightly blurred margin of the optic discs. Visual acuity and fields were normal. Precon Received Author's
April present
ventricle
occurred
appearance
in a 45-year-old resembled
male.
a choroid
The
plexus
or endocrinological ventricle,
sequelae.
surgery,
computed
tomography,
imaging
Introduction
A 45-year-old
third
the neuroradiological
of the third ventricle. The tumor originated from the right anterolateral wall of the third forming a discrete mass with prominent papillae formation. The tumor was totally removed
by a transcallosal
magnetic
localized
was unusual
2, 1992;
Accepted
address:
M. Tada, M.D., Switzerland.
June
trast computed tomographic (CT) scans revealed a round isodense mass in the third ventricle, contain ing no calcification or cyst, which was homogeneous ly enhanced postcontrast. Magnetic resonance (MR) imaging showed a low-intensity mass with a fine honeycomb structure on the T,-weighted image, which was iso or high intensity on the T2-weighted image, and homogeneously enhanced by gadolin ium-diethylenetriaminepenta-acetic acid (Gd-DTPA) (Fig. 1). Angiography showed the mass to be avas cular. Laboratory tests found no endocrinological abnormality. The tumor was removed on June 26, 1991, by an in terhemispheric transcallosal approach; approaching the tumor through a transforaminal and sub choroidal route. The tumor, confined to the third ventricle, was an ivory-colored discrete mass with a cauliflower-like papillary surface (Fig. 2). The tumor was removed totally. No cyst, motor oil-like fluid, or calcification were found. The tumor was attached to the right anterolateral wall of the third ventricle in ferior to where vasculature entered the tumor. The floor of the third ventricle was completely intact (Fig. 3). He developed
transient
diabetes
insipidus
for
11, 1992
Service
de Neurochirurgie,
Centre
Hospitalier
Universitaire
Vaudois,
Lausanne,
Fig. 3 Postoperative postcontrast axial CT scan (left) and T,-weighted coronal MR image (right). Note that the floor of the third ventricle is in tact.
Microscopic examination of the tumor specimen disclosed anastomosing cords of squamous epithelium embedded in loose connective tissue. Pro Fig. 1
Preoperative MR images. A, B: T, (A: TR 600 msec, TE 15 msec) and T2-weighted (B: TR 3000 msec, TE 90 msec) axial images without contrast enhancement; C, D: Gd-DTPA enhanced coronal (C) and sagittal (D) im ages. Note that the pituitary stalk is intact.
gressive loosening of the parenchyma in the center resulted in separation of adjacent masses of cells, forming a papillary structure (Fig. 4 upper). This papillation was prominent in the tumor periphery (Fig. 4 lower). The histological diagnosis was papillary craniopharyngioma (papillary squamous epithelioma). Discussion
Fig.
2
Operative
view
of
the
foramen
transcallosal
approach.
The
the foramen,
has a papillary
several days postoperatively, but weeks later without neurological docrinological abnormalities.
of
tumor,
Monro; blocking
structure.
was discharged 3 symptoms or en
Suprasellar craniopharyngioma frequently extends into the third ventricle, but localized growth in the third ventricle is relatively rare. Review of the 29 reported cases of this lesion 2,1,1,1-11,11-21) disclosed some interesting features. Craniopharyngiomas of the third ventricle occur predominantly in males (the male/female ratio is approximately 2:1) in contrast to the equal sex distribution of intra/suprasellar le sions. Patients tend to be older, mean age 48 years. Headache (80%) and papilledema (60%) are the main symptoms; visual field loss (24%) and en docrinological abnormality (12%) are less frequently seen. The neuroradiological appearance mimics a choroid plexus papilloma. CT demonstrates isoden sity and homogeneous enhancement. Cyst formation is less frequent (36%), but calcification is usually ab sent. MR imaging demonstrates the tumor as low or isointensity on T,-weighted image and iso or high in tensity on T2-weighted image, with homogeneous and striking Gd-DTPA enhancement.
anterolateral wall in the present case, allowing total removal without causing permanent hypothalamic deficits. In contrast, adamantinomatous cranio pharyngiomas are usually characterized by exten sive adhesions to the adjacent brain, often with finger-like projections into the parenchyma, making total removal quite difficult without causing hypothalamic damage. 1,3,16) The tumor in our case was successfully removed through the transcallosal transforaminal and sub choroidal approach. Of the nine well-documented cases of total removal, four including our case used the transcallosal approach, 21,23)four the transcor tical-transventricular, 13,23,24) and one the trans-lamina terminalis,27>indicating the superiority of approaches from above to basal approaches. The transcallosal approach achieves wide exposure of the tumor, all stages of removal are under visual control, and im portant arterial branches supplying the diencephalon can be preserved more effectively. 1,1,23) References 1) Fig. 4
Photomicrograph of the tumor specimen, show ing anastomosing trabeculae of a well-differen tiated squamous epithelium and progressive detachment in the center of the squamous cell cords (upper). The periphery of the tumor shows a prominent papillary structure (lower). HE stain, x 100.
The gross appearance is a discrete mass, sometimes with cauliflower-like papillation. Papillary structures, as in this case, are formed by disintegration of the tumor parenchyma due to pro gressive loosening in the center.") This structure is therefore a result of degenerative processes in squamous cell type craniopharyngioma. However, only seven of 24 previously reported cases had such a gross appearance. 11,13,15,19,20,22,24) Craniopharyngioma of the third ventricle may originate in the ectopic epithelial nest of the infun dibulum"W or tuber cinereum.19,20) In our case, however, the firm attachment through which vas culature entered the tumor from the adjacent brain occurred only at the right anterolateral wall of the third ventricle. Other authors have reported such sparing of the third ventricle floor. 13,24,26) This may in dicate that some distant straying of an epithelial nest has occurred. There was no firm adhesion between the tumor and the surrounding third ventricle wall except at the
2)
3)
4)
5)
Apuzzo MLJ, Zee C-S, Breeze RE: Anterior and mid-third ventricular lesions: A surgical overview, in Apuzzo MLJ (ed): Surgery of the Third Ventricle. Baltimore, Williams & Wilkins, 1987, pp 495-542 Asari S, Sakurai M, Suzuki K, Hamasaki M, Sadamoto K: Craniopharyngioma in the third ventri cle. Neurol Med Chir (Tokyo) 20: 1039-1047, 1980 (in Japanese) Baskin DS, Wilson CB: Surgical management of craniopharyngiomas. A review of 74 cases. J Neurosurg 65: 22-27, 1986 Bollati A, Giunta F, Lenzi A, Marini G: Third ventri cle intrinsic craniopharyngioma. J Neurosurg Sci 17: 316-317, 1973 Brodsky MC, Hoyt WF, Barnwell SL, Wilson CB: In trachiasmatic craniopharyngioma: A rare cause of chiasmal thickening. Case report. J Neurosurg 68: 300-302, 1988
6)
Burger PC, Scheithauer BW, Vogel FS: Surgical Pathology of the Nervous System and its Coverings, ed 3. New York, Churchill Livingstone, 1991, pp 536-546
7)
Carmel PW: Tumours in the third ventricle. Acta Neurochir (Wien) 75: 136-193, 1985 Cashion EL, Young JM: Craniopharyngioma in the third ventricle. J Tenn Med Ass 55: 156-160, 1962 Cashion EL, Young JM: Intraventricular cranio
8) 9)
10) 11)
pharyngioma: Report of two cases. J Neurosurg 34: 84-87, 1971 Chin HW: Adult intraventricular craniopharyn gioma. Strahlentherapie 159: 214-216, 1983 Dobos EI, Freed CG, Ashe SMP: An intrinsic tumor of the third ventricle. J Neuropath Exp Neurol 12: 232-243, 1953
12)
13)
14)
Ferrara M, Bizzozero L, D'angero V, Fiumara E: In traventricular craniopharyngioma. Clinical and surgical considerations. JNeurosurg Sci 33: 161-164, 1989 Fukushima T, Hirakawa K, Kimura M, Tomonaga M: Intraventricular craniopharyngioma: Its characteristics in magnetic resonance imaging and successful total removal. Surg Neurol 33: 22-27, 1990 Giangaspero F: Suprasellar papillary squamous epithelioma (“papillary craniopharyngioma”).Am J Surg Pathol 8: 57-64, 1984
15) Goldstein SJ, Wilson DD, Young AB, Guidry GJ: Craniopharyngioma intrinsic to the third ventricle. Surg Neurol 20: 249-253, 1983 16)
Hoffman HJ, De Silva M, Humphreys RP, Drake JM, Smith ML, Blaser SI: Aggressive surgical management of craniopharyngiomas in children. J Neurosurg 76: 47-52, 1992
17) Ikezaki K, Fujii K, Kishikawa T: Magnetic resonance imaging of an intraventricular craniopharyngioma. Neuroradiology 32: 247-249, 1990 18)
King TT: Removal of intraventricular craniopharyn giomas through lamina terminalis. Acta Neurochir (Wien) 45: 277-286, 1979
19) Kubota T, Fujii H, Ikeda K, Ito H, Yamamoto S, Nakanishi I: A case of intraventricular cranio pharyngioma with subarachnoid hemorrhage. No Shinkei Geka 8: 495-501, 1980 (in Japanese) 20)
Kunishio K: Craniopharyngioma in the third ventri cle: Necropsy findings and histogenesis. J Neurol Neurosurg Psychiatry 50: 1053-1056, 1987
21)
Lanzieri CF, Sacher M, Som PM: CT changes in the septum pellucidum associated with intraventricular craniopharyngiomas. J Comput Assist Tomogr 9: 507-510, 1985
22)
Linden CN: Intrinsic third ventricle craniopharyn gioma: CT and MR findings. J Comput Assist Tomogr 13: 362-363, 1989 Long DM, Chou SN: Transcallosal removal of craniopharyngiomas within the third ventricle. J Neurosurg 39: 563-567, 1973 Matthews FD: Intraventricular craniopharyngioma. AJNR 4: 984-985, 1983 Namba S, Tsuboi M: Craniopharyngioma in the third ventricle. No To Shinkei 29: 865-869, 1977 (in Japanese) Rush JL, Kusske JA, De Feo DR, Pribram HW: In traventricular craniopharyngioma. Neurology (Min neap) 25: 1094-1096, 1975 Urasaki E, Fukumura A, Itho Y, Itoyama Y, Yamada M, Ushio Y, Yokota A, Wada S: Craniopharyngioma wholly located in the third ventri cle. No Shinkei Geka 16: 1399-1404, 1988 (in Japanese)
23)
24) 25)
26)
27)
Address of
reprint
requests
Neurosurgery,
Medicine, Japan.
North-15,
to: M. Tada, Hokkaido West-7,
M.D.,
University Kita-ku,
Department School Sapporo
of 060,
Mitsuhiro
TADA,
Case
of the Third Ventricle
Report—
Toshimitsu AIDA, Mitsuyuki KOIWA*, Yoshimi Takeshi KASHIWABA* and Hiroshi ABE
CHONO*,
Department of Neurosurgery, Hokkaido University School of Medicine, Sapporo; *Kashiwaba
Neurosurgical
Hospital
, Sapporo
Abstract A papillary clinical
craniopharyngioma
presentation
papilloma ventricle,
and
in the
Key words:
approach
papillary resonance
without
neurological
craniopharyngioma,
third
Craniopharyngiomas are epithelial neoplasms occur ring in the sellar/suprasellar region and comprise 2-3% of all intracranial neoplasms. Occurrence elsewhere in the intracranial compartment is very rare. There are two histological subtypes: the classic adamantinomatous craniopharyngioma, and the less common squamous cell craniopharyngioma, often referred to as papillary craniopharyngioma due to the prominent papillae formation sometimes pre sent.') Here, we describe a patient with papillary craniopharyngioma localized in the third ventricle manifesting with a peculiar clinical presentation and a neuroradiological appearance mimicking a choroid plexus papilloma of the third ventricle. Case
Report
male visited
our outpatient
clinic com
plaining of a dull headache persisting for 2 months. Neurological examination disclosed no abnor malities except slightly blurred margin of the optic discs. Visual acuity and fields were normal. Precon Received Author's
April present
ventricle
occurred
appearance
in a 45-year-old resembled
male.
a choroid
The
plexus
or endocrinological ventricle,
sequelae.
surgery,
computed
tomography,
imaging
Introduction
A 45-year-old
third
the neuroradiological
of the third ventricle. The tumor originated from the right anterolateral wall of the third forming a discrete mass with prominent papillae formation. The tumor was totally removed
by a transcallosal
magnetic
localized
was unusual
2, 1992;
Accepted
address:
M. Tada, M.D., Switzerland.
June
trast computed tomographic (CT) scans revealed a round isodense mass in the third ventricle, contain ing no calcification or cyst, which was homogeneous ly enhanced postcontrast. Magnetic resonance (MR) imaging showed a low-intensity mass with a fine honeycomb structure on the T,-weighted image, which was iso or high intensity on the T2-weighted image, and homogeneously enhanced by gadolin ium-diethylenetriaminepenta-acetic acid (Gd-DTPA) (Fig. 1). Angiography showed the mass to be avas cular. Laboratory tests found no endocrinological abnormality. The tumor was removed on June 26, 1991, by an in terhemispheric transcallosal approach; approaching the tumor through a transforaminal and sub choroidal route. The tumor, confined to the third ventricle, was an ivory-colored discrete mass with a cauliflower-like papillary surface (Fig. 2). The tumor was removed totally. No cyst, motor oil-like fluid, or calcification were found. The tumor was attached to the right anterolateral wall of the third ventricle in ferior to where vasculature entered the tumor. The floor of the third ventricle was completely intact (Fig. 3). He developed
transient
diabetes
insipidus
for
11, 1992
Service
de Neurochirurgie,
Centre
Hospitalier
Universitaire
Vaudois,
Lausanne,
Fig. 3 Postoperative postcontrast axial CT scan (left) and T,-weighted coronal MR image (right). Note that the floor of the third ventricle is in tact.
Microscopic examination of the tumor specimen disclosed anastomosing cords of squamous epithelium embedded in loose connective tissue. Pro Fig. 1
Preoperative MR images. A, B: T, (A: TR 600 msec, TE 15 msec) and T2-weighted (B: TR 3000 msec, TE 90 msec) axial images without contrast enhancement; C, D: Gd-DTPA enhanced coronal (C) and sagittal (D) im ages. Note that the pituitary stalk is intact.
gressive loosening of the parenchyma in the center resulted in separation of adjacent masses of cells, forming a papillary structure (Fig. 4 upper). This papillation was prominent in the tumor periphery (Fig. 4 lower). The histological diagnosis was papillary craniopharyngioma (papillary squamous epithelioma). Discussion
Fig.
2
Operative
view
of
the
foramen
transcallosal
approach.
The
the foramen,
has a papillary
several days postoperatively, but weeks later without neurological docrinological abnormalities.
of
tumor,
Monro; blocking
structure.
was discharged 3 symptoms or en
Suprasellar craniopharyngioma frequently extends into the third ventricle, but localized growth in the third ventricle is relatively rare. Review of the 29 reported cases of this lesion 2,1,1,1-11,11-21) disclosed some interesting features. Craniopharyngiomas of the third ventricle occur predominantly in males (the male/female ratio is approximately 2:1) in contrast to the equal sex distribution of intra/suprasellar le sions. Patients tend to be older, mean age 48 years. Headache (80%) and papilledema (60%) are the main symptoms; visual field loss (24%) and en docrinological abnormality (12%) are less frequently seen. The neuroradiological appearance mimics a choroid plexus papilloma. CT demonstrates isoden sity and homogeneous enhancement. Cyst formation is less frequent (36%), but calcification is usually ab sent. MR imaging demonstrates the tumor as low or isointensity on T,-weighted image and iso or high in tensity on T2-weighted image, with homogeneous and striking Gd-DTPA enhancement.
anterolateral wall in the present case, allowing total removal without causing permanent hypothalamic deficits. In contrast, adamantinomatous cranio pharyngiomas are usually characterized by exten sive adhesions to the adjacent brain, often with finger-like projections into the parenchyma, making total removal quite difficult without causing hypothalamic damage. 1,3,16) The tumor in our case was successfully removed through the transcallosal transforaminal and sub choroidal approach. Of the nine well-documented cases of total removal, four including our case used the transcallosal approach, 21,23)four the transcor tical-transventricular, 13,23,24) and one the trans-lamina terminalis,27>indicating the superiority of approaches from above to basal approaches. The transcallosal approach achieves wide exposure of the tumor, all stages of removal are under visual control, and im portant arterial branches supplying the diencephalon can be preserved more effectively. 1,1,23) References 1) Fig. 4
Photomicrograph of the tumor specimen, show ing anastomosing trabeculae of a well-differen tiated squamous epithelium and progressive detachment in the center of the squamous cell cords (upper). The periphery of the tumor shows a prominent papillary structure (lower). HE stain, x 100.
The gross appearance is a discrete mass, sometimes with cauliflower-like papillation. Papillary structures, as in this case, are formed by disintegration of the tumor parenchyma due to pro gressive loosening in the center.") This structure is therefore a result of degenerative processes in squamous cell type craniopharyngioma. However, only seven of 24 previously reported cases had such a gross appearance. 11,13,15,19,20,22,24) Craniopharyngioma of the third ventricle may originate in the ectopic epithelial nest of the infun dibulum"W or tuber cinereum.19,20) In our case, however, the firm attachment through which vas culature entered the tumor from the adjacent brain occurred only at the right anterolateral wall of the third ventricle. Other authors have reported such sparing of the third ventricle floor. 13,24,26) This may in dicate that some distant straying of an epithelial nest has occurred. There was no firm adhesion between the tumor and the surrounding third ventricle wall except at the
2)
3)
4)
5)
Apuzzo MLJ, Zee C-S, Breeze RE: Anterior and mid-third ventricular lesions: A surgical overview, in Apuzzo MLJ (ed): Surgery of the Third Ventricle. Baltimore, Williams & Wilkins, 1987, pp 495-542 Asari S, Sakurai M, Suzuki K, Hamasaki M, Sadamoto K: Craniopharyngioma in the third ventri cle. Neurol Med Chir (Tokyo) 20: 1039-1047, 1980 (in Japanese) Baskin DS, Wilson CB: Surgical management of craniopharyngiomas. A review of 74 cases. J Neurosurg 65: 22-27, 1986 Bollati A, Giunta F, Lenzi A, Marini G: Third ventri cle intrinsic craniopharyngioma. J Neurosurg Sci 17: 316-317, 1973 Brodsky MC, Hoyt WF, Barnwell SL, Wilson CB: In trachiasmatic craniopharyngioma: A rare cause of chiasmal thickening. Case report. J Neurosurg 68: 300-302, 1988
6)
Burger PC, Scheithauer BW, Vogel FS: Surgical Pathology of the Nervous System and its Coverings, ed 3. New York, Churchill Livingstone, 1991, pp 536-546
7)
Carmel PW: Tumours in the third ventricle. Acta Neurochir (Wien) 75: 136-193, 1985 Cashion EL, Young JM: Craniopharyngioma in the third ventricle. J Tenn Med Ass 55: 156-160, 1962 Cashion EL, Young JM: Intraventricular cranio
8) 9)
10) 11)
pharyngioma: Report of two cases. J Neurosurg 34: 84-87, 1971 Chin HW: Adult intraventricular craniopharyn gioma. Strahlentherapie 159: 214-216, 1983 Dobos EI, Freed CG, Ashe SMP: An intrinsic tumor of the third ventricle. J Neuropath Exp Neurol 12: 232-243, 1953
12)
13)
14)
Ferrara M, Bizzozero L, D'angero V, Fiumara E: In traventricular craniopharyngioma. Clinical and surgical considerations. JNeurosurg Sci 33: 161-164, 1989 Fukushima T, Hirakawa K, Kimura M, Tomonaga M: Intraventricular craniopharyngioma: Its characteristics in magnetic resonance imaging and successful total removal. Surg Neurol 33: 22-27, 1990 Giangaspero F: Suprasellar papillary squamous epithelioma (“papillary craniopharyngioma”).Am J Surg Pathol 8: 57-64, 1984
15) Goldstein SJ, Wilson DD, Young AB, Guidry GJ: Craniopharyngioma intrinsic to the third ventricle. Surg Neurol 20: 249-253, 1983 16)
Hoffman HJ, De Silva M, Humphreys RP, Drake JM, Smith ML, Blaser SI: Aggressive surgical management of craniopharyngiomas in children. J Neurosurg 76: 47-52, 1992
17) Ikezaki K, Fujii K, Kishikawa T: Magnetic resonance imaging of an intraventricular craniopharyngioma. Neuroradiology 32: 247-249, 1990 18)
King TT: Removal of intraventricular craniopharyn giomas through lamina terminalis. Acta Neurochir (Wien) 45: 277-286, 1979
19) Kubota T, Fujii H, Ikeda K, Ito H, Yamamoto S, Nakanishi I: A case of intraventricular cranio pharyngioma with subarachnoid hemorrhage. No Shinkei Geka 8: 495-501, 1980 (in Japanese) 20)
Kunishio K: Craniopharyngioma in the third ventri cle: Necropsy findings and histogenesis. J Neurol Neurosurg Psychiatry 50: 1053-1056, 1987
21)
Lanzieri CF, Sacher M, Som PM: CT changes in the septum pellucidum associated with intraventricular craniopharyngiomas. J Comput Assist Tomogr 9: 507-510, 1985
22)
Linden CN: Intrinsic third ventricle craniopharyn gioma: CT and MR findings. J Comput Assist Tomogr 13: 362-363, 1989 Long DM, Chou SN: Transcallosal removal of craniopharyngiomas within the third ventricle. J Neurosurg 39: 563-567, 1973 Matthews FD: Intraventricular craniopharyngioma. AJNR 4: 984-985, 1983 Namba S, Tsuboi M: Craniopharyngioma in the third ventricle. No To Shinkei 29: 865-869, 1977 (in Japanese) Rush JL, Kusske JA, De Feo DR, Pribram HW: In traventricular craniopharyngioma. Neurology (Min neap) 25: 1094-1096, 1975 Urasaki E, Fukumura A, Itho Y, Itoyama Y, Yamada M, Ushio Y, Yokota A, Wada S: Craniopharyngioma wholly located in the third ventri cle. No Shinkei Geka 16: 1399-1404, 1988 (in Japanese)
23)
24) 25)
26)
27)
Address of
reprint
requests
Neurosurgery,
Medicine, Japan.
North-15,
to: M. Tada, Hokkaido West-7,
M.D.,
University Kita-ku,
Department School Sapporo
of 060,
