Intraventricular Craniopharyngiomas—Overcoming Their Relative Inaccessibility: Institutional Experience With a Review of Literature

Abstract

Introduction: Craniopharyngiomas constitute 2–4% of intracranial neoplasms. Intraventricular craniopharyngiomas (IVCrs) are the rarely encountered varieties of these lesions.Objective: The objective of the study was to study the special features in clinical presentation, imaging, management, and surgical outcome of IVCrs.Materials and Methods: This retrospective analysis included the combined experience from two tertiary care institutions. Medical records of histopathologically proven cases of IVCrs from January 1994 to June 2021 were assessed, and images were analyzed based on the criteria by Migliore et al. for inclusion of solely intraventricular lesion with the third ventricular ependyma demarcating it from the suprasellar cistern.Results: Among the 25 patients included (mean age: 35.4 years), the most common presentation included headache (n = 21, 84%), vomiting and other features of raised ICP (n = 18, 72%), visual complaints (n = 12, 48%), and endocrinopathies (n = 11, 44%). Fifteen had predominantly cystic tumors, two were purely solid, and eight were of mixed consistency. Primary open microsurgical procedures were performed in 18 (72%) patients, of which four (16%) were endoscope-assisted. Seven (28%) underwent a purely endoscopic procedure. One underwent a staged surgery with endoscopic cyst fenestration and intracystic interferon (IFN)-alpha therapy, followed by microsurgical excision. Complete excision was achieved in 10 patients, near-total in nine, and partial excision in six. Four patients underwent a ventriculoperitoneal shunt (one before the definitive procedure). At a median follow-up of 36 months (range:11–147 months), five patients developed a recurrence, and one had a stable small residue. This patient and two others with small cystic recurrences were observed. One patient was managed with radiotherapy alone. Another underwent re-surgery after a trial of radiotherapy, and the last patient developed a local recurrence, which was managed with radiotherapy; he then later developed an intraparenchymal recurrence, which was operated.Conclusion: Purely IVCrs present with raised intracranial pressure, and visual disturbances are less common. Their deep-seated location and limited surgical field-of-view makes minimally invasive endoscopic-assisted surgery most suitable for their excision. The thin-walled cystic lesions may be occasionally adherent to the ependymal wall in close vicinity to the thalamus–hypothalamus complex, making complete excision difficult. Their responsiveness to radiotherapy, often leads to a gratifying long-term outcome.

Craniopharyngioma and the Third Ventricle: This Inescapable Topographical Relationship

“…More recently, we were able to compile and analyze comprehensively the cohort of CPs with a verified strictly 3V topography (n=245), as well as the historical cohort of well-described papillary CPs published in the medical literature (n= 350) ( 30 , 31 ). Although the strictly 3V topography has remained controversial throughout history, some authors considering it an exceptional, ectopic location ( 32 ), while others even argue over its validity ( 33 ), the surgical series by Depoujarny ( 18 ), Cao ( 20 ), and Zhou ( 21 ) contribute to verify this particularly challenging location, confirming the anatomical integrity of the 3VF found in numerous strictly 3V CPs in prior studies (see Table 1 ) ( 34 – 49 ). The optimal surgical view of the brain undersurface obtained through the EEA unequivocally show the ballooned and stretched infundibulum wrapping around the lower pole of these lesions, which stay hidden within the 3V chamber ( 13 , 14 , 40 , 46 ).…”

“…Even more importantly, a 3V-centered scheme also helps to predict the extension and strength of the CP-hypothalamic attachment, which largely determines the risk of radical removal ( 12 , 26 , 27 ). The works by Deopujarny et al., and Cao et al., whose surgical series encompass more than 800 CPs, have verified to a large extent our topographical concepts ( 18 , 20 ). The attention that should be given to the hypothalamic symptoms caused by 3V CPs and the types of CP-hypothalamus attachment associated with this topography is the main message we wish to emphasize from these studies published in Frontiers in Oncology .…”

“…Frontiers in Oncology’s research topic, “ Advances in craniopharyngiomas: from physiology to clinical management ” gathers a series of papers specifically focused on the clinical assessment and surgical treatment of the subgroup of intrinsic or strictly 3V CPs ( 18 – 21 ). The studies by Deopujary et al.…”

“…and Zhao et al. direct their attention on the physiological and neuropsychological disturbances derived from the hypothalamic injury caused by resecting intra-3V CPs ( 18 , 19 ). The feasibility of combining the extended endonasal endoscopically assisted approach (EEEA) with a trans-lamina terminalis (TLT) access to successfully remove strictly 3V CPs is the major objective of Cao et al.…”

“…In 5 out of 6 strict CP cases in the series by Cao (83%) and 6 out of 9 in Zhou’s paper (66%), the lesions corresponded to the papillary type, percentages that fit well with the 82% rate of papillary lesions found in our systematic review ( 20 , 21 , 30 ). Depoujarny observed preoperatively symptoms related to hypothalamus dysfunction in 60% of their 3V CP patients overall, the most prominent being memory loss (25%), increased sleepiness (20%) and abnormal uninhibited behaviors, including hyperphagia (36%) ( 18 ). These figures also match with the rate of mental alterations in strictly 3V CPs (59%) and papillary CPs (50%) identified in our reviews ( 30 , 31 ).…”