Craniopharyngioma: A comprehensive review of the clinical presentation, radiological findings, management, and future Perspective

Abstract

Craniopharyngioma (CP) is a rare intracranial tumor arising from the epithelial remnants of Rathke's pouch, most frequently originating in the sellar/parasellar region. Histologically, CP is a benign low-grade tumor (WHO grade 1) with two distinct phenotypes: adamantinomatous CP (ACP) and papillary CP (PCP). Craniopharyngioma constitutes 1-3% of all primary intracranial tumors in adults and 5-10 % of intracranial tumors in children. The annual incidence ranges from 0.13 to 2 per 100,000 population per year with no gender predilection. Due to its unique anatomical locations, the most frequently reported clinical manifestations are headache, visual impairment, nausea/vomiting, and endocrine deficiencies resulting in sexual dysfunction in adults and growth failure in children. Growth hormone deficiency is the most predominant endocrinological disturbance associated with craniopharyngioma. Computed tomography (CT) is gold standard to detect calcifications in CP tissue (found in 90 % of these tumors). Magnetic Resonance Imaging (MRI) further characterizes craniopharyngiomas and helps to narrow down the differential diagnoses. In almost all craniopharyngioma cases, surgery is indicated to: establish the diagnosis, relieve mass-related symptoms, and remove as much tumor as is safely possible. Recent neurosurgical technical advances, including innovative surgical approaches, detailed radiotherapy protocols, targeted therapy, replacement of lost hormonal functions and quality of life all have the potential to improve the outcome of patients with craniopharyngioma. In this article, we present extensive literature on craniopharyngioma clinical presentation, radiological findings, management, and future prospective. The present article helps to identify further research areas that set the basis for the management of such a complex tumor.

Keywords: Craniopharyngioma; Endocrine; Hormone; Review; Tumor.

Fig. 1

A 16-year-old female with short stature, bi-temporal hemianopia, and pan-hypopituitarism. (A–C) Axial, coronal, and sagittal brain CT without contrast. (D–F) Axial, coronal, and sagittal brain MRI with contrast. (A–B) The images demonstrate a well-defined sellar/suprasellar hypodense lesion with areas of hyperdensities. It is oval-shaped and measures approximately 5.1 x 3.1 × 1.7 cm along its maximum dimensions. It is associated with peripheral calcification. The lesion is obstructing the third ventricle and causing subsequent supratentorial hydrocephalus, most evident on the lateral ventricles. (D-F) The lesion has peripheral solid component and central cystic component. The solid component demonstrates heterogenous avid enhancement post Gadolinium administration. The mass is compressing and displacing the optic nerves and optic chiasm.

Fig. 2

A 14-year-old female with headache, visual disturbance, and new-onset syncope. (A–C) Axial, coronal, and sagittal brain CT without contrast. (D–F) Axial, coronal, and sagittal brain MRI with contrast. (A–C) The images demonstrate an extra-axial sellar/suprasellar heterogenous hyperdense lesion. It is oval-shaped and measures approximately 4.1 X 4.5 × 5.2 cm along its maximum dimensions. It is showing areas of dense calcifications within the lesion. The lesion is compressing the hypothalamus and third ventricle causing obstructive supratentorial hydrocephalous. (D-F) The lesion demonstrates homogenous avid enhancement post Gadolinium administration.

Fig. 3

A 36-year-old female with headache, short-term memory loss, and new-onset blurry vision. (A–C) Axial, coronal, and sagittal pre-operative brain MRI with contrast. (D–F) Axial, coronal, and sagittal post-operative brain MRI with contrast. (A–C) The images demonstrate an extra-axial sellar/suprasellar heterogeneously enhanced lesion. Multiple non-enhancing cystic changes as well as foci of calcifications are noted. The lesion is oval-shaped and measures approximately 3.0 X 3.7 × 4.2 cm along its maximum dimensions. The pituitary stalk is not visualized. The mass is significantly elevating the hypothalamus and the third ventricle. It is filling the interpeduncular fossa, splaying the cerebral peduncles and compressing the midbrain. However, there is no significant brainstem edema. The optic chiasm is also compressed and displaced superiorly. The vessels of the circle of Willis vessels are displaced rather than encased (D-F) Post-operative images demonstrating a complete resection of the lesion. Post-surgical changes, in the form of pneumocephalus and bifrontal subdural collections, are noted.

Fig. 4

(A–B) Adamantinomatous craniopharyngioma at low power (A) surrounded by reactive brain parenchyma. At intermediate power (B) the palisading basal cells (green arrow) is recognized. (C–F) Papillary craniopharyngioma at low power (C–D) displaying cystic and papillary structures. The lining is comprised of a well-differentiated squamous epithelium as observed at intermediate power (E–F).