Pure interventricular craniopharyngioma: A case report and literature review

Issa Ali Muftah Lahirish; MB Sales; Cassia Prado; Jose Marcus Rotta; Mateus Reghin Neto

doi:10.4103/ljms.ljms_54_21

CASE REPORT

Year : 2021 | Volume : 5 | Issue : 4 | Page : 174-176

Pure interventricular craniopharyngioma: A case report and literature review

Issa Ali Muftah Lahirish¹, MB Sales², Cassia Prado³, Jose Marcus Rotta², Mateus Reghin Neto⁴
¹ Department of Neurosurgery and Microsurgical Anatomy, Beneficiencia Portuguesa Hospital, Institute of Neurological Sciences (ICNE), Sao Paulo, Brazil
² Department of Neurosurgery, Servidor Publico Estadual institute, Sao Paulo, Brazil
³ Department of Pathology, Servidor Publico Estadual institute, Sao Paulo, Brazil
⁴ Department of Neurosurgery and Microsurgical Anatomy, Beneficiencia Portuguesa Hospital, Institute of Neurological Sciences (ICNE); Department of Neurosurgery, Servidor Publico Estadual institute, Sao Paulo, Brazil

Date of Submission	30-Aug-2021
Date of Acceptance	03-Dec-2021
Date of Web Publication	28-Feb-2022

Correspondence Address:
Dr. Issa Ali Muftah Lahirish
Department of Neurosurgery and Microsurgical Anatomy, Beneficiencia Portuguesa Hospital, Sao Paulo
Brazil

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ljms.ljms_54_21

Abstract

Craniopharyngiomas (CPs) of the third ventricle are rare and can be difficult to treat due to their deep location and relation to neurological structures. A 60-year-old woman presented with a history of memory disturbances with progressive course. On examination, she was conscious but disoriented with time and place, visual field showed bilateral superolateral quadrantanopia, and KPS was 90. Brain images revealed a third ventricular mass that bowed the floor of the third ventricle, expanding into the supraoptic recess, compressing the optic chiasm downward and forward. The mass had solid and cystic components at its inferior and periphery. The patient underwent right pretemporal approach with orbital roof being removed, and a translamina terminalis route was used to reach the third ventricle and the tumor was completely removed. Preoperative localization of the ventricular CPs is essential to choose the optimal surgical approach to avoid undesired injuries and improve the surgical outcome.

Keywords: Craniopharyngiomas, lamina terminalis, pretemporal approach, third ventricle

How to cite this article:
Muftah Lahirish IA, Sales M B, Prado C, Rotta JM, Neto MR. Pure interventricular craniopharyngioma: A case report and literature review. Libyan J Med Sci 2021;5:174-6

How to cite this URL:
Muftah Lahirish IA, Sales M B, Prado C, Rotta JM, Neto MR. Pure interventricular craniopharyngioma: A case report and literature review. Libyan J Med Sci [serial online] 2021 [cited 2023 Mar 27];5:174-6. Available from: https://www.ljmsonline.com/text.asp?2021/5/4/174/338631

Introduction

Craniopharyngiomas (CPs) are rare and slow-growing benign tumors and mostly are located in the suprasellar region; pure third ventricular CPs are even rare, ranging from 0.5% to 11% of all CPs, and tend to occur in elder individuals. Due to their deep localization and relationship with hypothalamic structures, these tumors present characteristic surgical and clinical problems. In the current report, we present a 60-year-old woman presented with chronic headache and memory disturbances.

Case Report

Patient history and examinations

A 60-year-old woman presented with a history of memory disturbances with progressive course; she is a known case of diabetes mellitus and hypertension on treatment. On examination, she was conscious but disoriented with time and place, visual field showed bilateral superolateral quadrantanopia, KPS was 90, and neurological examination otherwise was unremarkable.

Nonenhanced computed tomography scan of the head revealed a round and heterogeneous mass within the third ventricle, and calcification at the inferior aspect of the mass was seen. Magnetic resonance imaging (MRI) revealed a third ventricular mass that bowed the floor of the third ventricle, expanding into the supraoptic recess, compressing the optic chiasm downward and forward. The mass had solid and cystic components in its inferior and periphery [Figure 1]a, [Figure 1]b, [Figure 1]c, [Figure 1]d, [Figure 1]e.

Figure 1: Brain images showing a round contour mass with cystic and solid components. (a) T1 noncontrast sagittal magnetic resonance imaging showing hyperintense lesion with cystic hypointense lesion (head arrow), displacing the optic chiasm downward and forward (white arrow), intact pituitary gland (yellow arrow), and suprasellar cistern (red arrow). (b) Coronal T2-weighted image showing hypointense lesion occupying the third ventricle (arrow) with free suprasellar cistern. (c) axial computed tomography scan without contrast showing calcifications at inferior aspect of the tumor (arrow). (d) Axial T1-weighted image without contrast showing hyperintense lesion inside the third ventricle. (e) Axial FLAIR magnetic resonance imaging showing edema surrounding the third ventricular mass (arrow)

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Surgery

The patient underwent right pretemporal approach as described by Tedeschi et al.^[1] with orbital roof being removed. Transylvian dissection was performed and skull base cisterns were released; then, the translamina terminalis route was used to reach the third ventricle and the tumor was carefully dissected from the hypothalami and completely resected [Figure 2]a, [Figure 2]b, [Figure 2]c, [Figure 2]d. The postoperative MRI showed gross total resection of the tumor [Figure 3]a and [Figure 3]b. The histopathological examination revealed adamantinomatous WHO grade I CP [Figure 4]a, [Figure 4]b, [Figure 4]c, [Figure 4]d.

Figure 2: Intraoperative images: (a) After Sylvian fissure dissection – 1 = Supraclinoidal segment of carotid artery; 2 = Anterior cerebral artery (A1); 3 = Optic chiasm; 4 = Lamina terminalis. (b) The lamina terminalis has been opened and the cyst has been evacuated. (c) Tumor dissection from the hypothalami and floor of the third ventricle. (d) After the tumor has been grossly resected with empty third ventricle and intact third ventricular floor, black arrow indicating mammillary body

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Figure 3: Postoperative magnetic resonance imaging. (a) sagittal T1-weighted image with contrast showing gross total resection of the third ventricular mass with normally enhanced pituitary gland. (b) axial T1-weighted image with contrast showing gross total resection of the third ventricular mass

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Figure 4: Histopathology slides of adamantinomatous craniopharyngioma WHO grade I. (a) Microscope view of the infiltrative tumor tongues (H and E). (b) 1 - gliosis; 2 - superficial layer of palisading epithelium; 3 - stellate reticulum; 4 - nuclear whorls and “wet keratin”; 5 - calcification. (c) 1 - palisading nuclei; 2 - squamous whorls. (d) 1 - xanthogranulomatous react with foamy macrophages; 2 - intralobular cystic degenerations; 3 - “wet keratin”

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Discussion

The incidence of third ventricular CPs has been infrequently reported, ranging from 0.5% to 11% of all CPs.^[2],[3],[4] The first case of pure third ventricular CP was described in 1953 by Dobos et al.,^[5] and since then, there are few cases described in the literature.^[3] CPs originate from the epithelial remnants of Rathke's pouch; the pial membrane will serve as a barrier preventing Rathke's pouch cells from coming into direct contact with the brain vesicle (the precursor of the infundibulum and the third ventricular floor). Without the protection of a mature pial membrane, Rathke's pouch cells may end up implanting on the neuroectoderm of the developing cerebral vesicle. It will be pure third ventricular CP if these cells develop into a tumor.^[4],[6]

Suprasellar craniopharyngiomas have a high prevalence of visual and endocrine disturbances (between 70 and 90% of cases), and less prevalence of psychiatric symptoms (less than 15%). In contrast, Third ventricular CPs have a low prevalence of endocrine (27%) and visual (28%) disturbances and a higher prevalence of psychiatric abnormalities (40%) and memory dysfunction (33%). These differences must be related to the different location of the tumor, which is located above the suprasellar area and involves the third ventricle floor, including the mammillary bodies, and the hypothalamus. Third ventricular CPs are usually diagnosed in old patients that may due to slow growing of tumor and its position inside the third ventricle that delays invasion of neurological structures that lead to delay onset of symptoms.^{[2],[3],[4],[7]}

Pascual et al.^[7] were able to distinguish among four theoretical topographical relationships between the tumor and the third ventricle floor; (1) suprasellar tumor pushing the intact third ventricle floor upward; (2) suprasellar mass breaking through the third ventricle floor and invading the third ventricle cavity; (3) intraventricular mass within the third ventricle cavity and floor; and (4) intraventricular mass completely located within the third ventricle cavity and with the intact floor lying below its inferior surface.

Prieto et al.^[8] have suggested that five major CP topographies can be defined according to the degree of hypothalamic distortion caused by the tumor: sellar-suprasellar, pseudointraventricular, secondary intraventricular, not strictly intraventricular, and strictly intraventricular. Seven key radiologic variables identified on preoperative MRI allowed a correct overall prediction of CP topography in 86% of cases: (1) third ventricle occupation, (2) pituitary stalk distortion, (3) relative level of the hypothalamus in relation to the tumor, (4) chiasmatic cistern occupation, (5) mammillary body angle, (6) type of chiasm distortion, and (7) tumor shape.

Behari et al.^[2] reported six cases of purely intraventricular CPs. In all cases, the diagnosis was confirmed preoperatively by an MRI examination. A total of four patients presented cystic lesions and two presented solid lesions. All of the patients presented with intracranial hypertension and papilledema. All of the patients were submitted to surgery, by different approaches, and two patients received radiotherapy for residual lesions. Hung et al.^[9] reported five cases of purely third ventricular CPs, including two cases of mixed cystic and solid tumors and three cases of pure solid masses. All of them were submitted to surgery; four patients underwent frontal transcortical transventricular approach and one patient underwent translamina terminalis approach.

Differential diagnosis of primary tumors arising inside the third ventricle includes colloid cyst, germinoma, lymphoma, choroid plexus papilloma, and glioma.^{[2],[3],[10],[11]}

There are two histopathological types of CPs, adamantinomatous and papillary. The former is found predominantly in children; papillary CPs are seen exclusively in adults. This type predominantly contains a solid component but may also contain cystic components, and calcifications are rare but can occur. In the current case, the radiological images showed solid and cystic lesion with calcifications, and although not common in this age group, the histopathological result was adamantinomatous WHO grade I CP.

The surgical approaches to interventricular and suprasellar CPs are different; for this reason, the preoperative accurate localization of the lesion is essential to establish the surgical plan and missing the correct localization can lead to an inadequate surgical access.^[2],[3],[7] The most common approaches for interventricular CPs are the frontal transcortical, transcallosal, or translaminar terminalis approaches.^[9] The selection of optimal surgical approach and extend of resection of interventricular CPs are challenging to a surgeon. There has been no agreement as to which of the surgical approaches is best for this type of surgery. However, Pascual et al.^[7] found in their retrospective review that the translamina terminalis approach, although providing a lower rate of total removal, resulted in no mortality.

Conclusion

Third ventricular CPs are rare, and preoperative localization is essential to choose the optimal surgical approach to avoid undesired injuries and improve the surgical outcome.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

1.	Tedeschi H, de Oliveira EP, Wen HT, Rhoton AL Jr. Perspectives on the approaches to lesions in and around the cavernous sinus. Oper Tech Neurosurg 2001;4:82-107.
2.	Behari S, Banerji D, Mishra A, Sharma S, Sharma S, Chhabra DK, et al. Intrinsic third ventricular craniopharyngiomas: Report on six cases and a review of the literature. Surg Neurol 2003;60:245-52.
3.	Da Cunha M, Pletz AL. Strictly intraventricular craniopharyngioma: Case report and literature review. Arq Bras Neurocir 2018;37:113-8.
4.	Diniz LV, Junior LA, Rodrigues LP, Vieira Veloso JC, Yamashita S. Interventricular craniopharyngioma a case report. J Neurol Stroke 2018;8:185-8.
5.	Dobos EI, Freed CG, Ashe SM. An intrinsic tumor of the third ventricle. J Neuropathol Exp Neurol 1953;12:232-43.
6.	Bao Y, Pan J, Qi ST, Lu YT, Peng JX. Origin of craniopharyngiomas: Implications for growth pattern, clinical characteristics, and outcomes of tumor recurrence. J Neurosurg 2016;125:24-32.
7.	Pascual JM, González-Llanos F, Barrios L, Roda JM. Intraventricular craniopharyngiomas: Topographical classification and surgical approach selection based on an extensive overview. Acta Neurochir (Wien) 2004;146:785-802.
8.	Prieto R, Pascual JM, Barrios L. Topographic diagnosis of craniopharyngiomas: The accuracy of MRI findings observed on conventional T1 and T2 images. AJNR Am J Neuroradiol 2017;38:2073-80.
9.	Hung ND, Ngan VK, Duc NM. Intrinsic third ventricular papillary craniopharyngioma: A report of five cases and literature review. Int Med Case Rep J 2021;14:83-7.
10.	Iwasaki K, Kondo A, Takahashi JB, Yamanobe K. Intraventricular craniopharyngioma: Report of two cases and review of the literature. Surg Neurol 1992;38:294-301.
11.	Tayari N, Etemadifar M, Hekmatnia A, Mahzouni P, Maghzi AH, Rouzbahani R. Intrinsic third ventricular craniopharyngioma: A case report. Int J Prev Med 2011;2:178-85.