Cystic-Appearing Intracranial Neoplastic Lesions
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Pictorial Essay Cystic-appearing Intracranial neoplastic lesions Pablo Sartori1, Nicolás Sgarbi2 1 Diagnóstico Mediter, Ciudad Autónoma de Buenos Aires, Argentina. 2 Clinical Radiology Department, Hospital de Clínicas de Montevideo, Montevideo, Uruguay. Abstract Cystic and cyst-appearing intracranial lesions are common findings of brain neoplastic tumors. This paper intends to re- viewed the origin of this lesion, radiologic appearance and differential diagnosis as guidance for the treating physician. This paper will review the cystic lesions of neoplastic etiology that most frequently occur in the brain. Keywords Brain, cysts, neoplasms, tomography, magnetic resonance imaging Introduction There is a more aggressive variant known as pilomyxoid as- trocytoma.6 A cystic lesion can be defined as an epithelium-lined cavity On computed tomography (CT), these tumors appear as iso- that contains fluid or semisolid material.1-3 to hypodense solid/cystic expansive masses; calcifications are Expansive lesions may be entirely cystic or have cavitary areas rare, and there is enhancement of the solid component after and are sub-classified into intra-or extra-axial lesions depend- intravenous (IV) contrast administration.4,6,7 ing on their location. On MRI, the cystic component appears isointense on T1- and The most common intracranial expansive cystic lesions are T2-weighted images compared with cerebrospinal fluid (CSF) detailed in Table 1. The purpose of this article is to illustrate the wide spectrum Table 1 of intracranial cystic lesions of neoplastic origin and describe their morphologic characteristics. Location Examples Pilocytic astrocytoma Intra-axial tumors4,5 Hemangioblastoma Central neurocytoma Differential diagnoses are mentioned in Table 2. Ependymoma 1) Pilocytic astrocytoma Medulloblastoma This is the most common glial tumor in children and young Intra-axial Ganglioglioma adults, usually located near the midline. Two-thirds of lesions occur in the cerebellum, in the optic chiasma, around the Pleomorphic xanthoastrocytoma third ventricle and in the hypothalamic-pituitary region. In Dysembryoplastic embryonal tumor adults, one-half of tumors are supratentorial.4,6,7 Cerebellar astrocytomas have been reported to be associated Glioblastoma multiforme with neurofibromatosis type I, Ollier’s disease and Turcot and Metastasis PHACE syndromes.4,6-8 Signs and symptoms depend on location. In the posterior Meningioma fossa tumors, there is a mass effect with signs of raised intra- Schwannoma cranial pressure.6 Extra-axial Pituitary macroadenoma These tumors appear as well-circumscribed solid/cystic mass- es of round or oval shape, with a solid mural nodule, occa- Craniopharyngioma 6,7 sionally with a necrotic center. Source: References 4, 5. Rev. Argent. Radiol. 2021;85:11-20 11 Cystic-appearing Intracranial neoplastic lesions signal intensity, and hyperintense on FLAIR images, and it is 2) Hemangioblastoma associated with a mural nodule that is usually isointense on This is a rare vascular tumor, generally associated with von T1-weighted sequences and hyperintense on T2-weighted / Hippel-Lindau disease, although these tumors may be seen FLAIR images, generally enhancing after the administration sporadically.4,8 Hemangioblastomas occur in young patients in of gadolinium (Fig. 1).4-7 the cerebellum or, less commonly, in the spinal cord.4,8 Both the cystic and solid components may enhance after On CT, these tumor appear as cystic/solid posterior fossa mass- gadolinium administration.6,7 es, with enhancement of the solid portion after IV contrast a b c d Fig. 1 Brain MRI. (A) Axial T1-weighted, (B) coronal T2-weighted, (C) axial FLAIR and (D) gadolinium-enhanced axial T1- weighted images. Seven-year-old male patient with headaches and dizziness. The images show an expansive, predominantly cystic lesion on the right cerebellar hemisphere (star) with an eccentric solid mural nodule (arrows). The lesion produces mass effect with midline shift to the left, surrounded by a mild vasogenic edema. The solid portion shows irregular enhancement after gadolinium administration. Considering patient’s age and clinical presentation, the lesion is consistent with pilocytic as- trocytoma (confirmed by biopsy). Table 2: Differential diagnoses of cystic-appearing intracranial neoplastic lesions. Tumors with cystic components Differential diagnoses Ependymoma and medulloblastoma (children in the posterior fossa) Metastases or hemangioblas- Pilocytic astrocytoma toma (adults) Hemangioblastoma Pilocytic astrocytoma Oligodendroglioma, Pilocytic astrocytoma, subependymal giant cell astrocytoma, ependymoma, Central neurocytoma meningiomas Pituitary adenoma, hypothalamic or optic pathway glioma, Rathke cleft cyst, epidermoid cyst, throm- Craniopharyngioma bosed aneurysms Supratentorial intraparenchymal: Pilocytic astrocytoma, Pleomorphic xanthoastrocytoma, primitive Ependymoma neuroectodermal tumor, ganglioglioma/gangliocytoma and oligodendroglioma Medulloblastoma Pilocytic astrocytoma, atypical teratoid/rhabdoid tumor and ependymoma Ganglioglioma Ependymoma Pleomorphic xanthoastrocytoma Meningioma, glioblastoma, DNET, oligodendroglioma, metastasis Dysembryoplastic neuroepithelial tumor (DNET) Gangliocytoma, Pilocytic xanthoastrocytoma Glioblastoma Metastasis, abscesses Metastasis Abscesses, glioblastoma, astrocytoma, Pleomorphic xanthoastrocytoma Meningiomas Schwannomas Schwannomas Meningiomas Pituitary macroadenoma / Craniopharyngioma Craniopharyngioma /pituitary macroadenoma 12 Rev. Argent. Radiol. 2021;85:11-20 Pablo Sartori et al. a b c d Fig. 2 Brain MRI. (A) Axial T1-weighted, (B) T2-weighted images, (C) Axial and (D) coronal T1-weighted images with gado- linium. Images show a cystic expansive lesion on the left cerebellar hemisphere (star), with internal septa and a peripheral mural nodule. The solid portion strongly enhances after gadolinium administration (arrows). The diagnosis of Hemangioblastoma was confirmed by pathology. a b c d Fig. 3 Brain MRI. (A) Axial T1-weighted image, (B) axial T2-weighted image (C) coronal T2-weighted image and (D) gadolinium- enhanced axial T1-weighted image. A solid expansive lesion originates near the foramen of Monro, containing multiple cysts (arrows) and causing midline shift and left supratentorial obstructive hydrocephalus, heterogeneously enhancing after gado- linium administration. Central neurocytoma was diagnosed by biopsy. administration.8 On MRI, these tumors appear as iso- to hy- Central neurocytomas present with symptoms of increased in- pointense lesions on T1-weighted sequences and hyperintense tracranial pressure secondary to obstructive hydrocephalus.9-11 on T2-weighted sequences, with a strongly enhancing solid These tumors appear as heterogeneous, well-demarcated, mural nodule (Fig. 2).8 lobulated lesions with a solid/cystic component, with an at- On T2-weighted sequences, signal voids occur because of the tachment to the septum pellucidum.4,9-11 high vascularity of these tumors.8 Calcifications are rare and Central neurocytomas appear heterogeneous on CT; on MRI, the presence of spontaneous hyperintensity on T1-weighted they have a “bubbly” appearance due to the presence of sequences is suggestive of recent bleeding.8 cysts. These tumors appear isointense on T1-weighted se- Digital angiography is a diagnostic tool that may be consid- quences, iso- to hyperintense on T2-weighted sequences, ered, given the high vascularity of these tumors. with associated calcifications in 50% of cases (Fig. 3).4,9,11 3) Central neurocytoma 4) Ependymoma This is a benign tumor, formerly known as “ependymoma of the These tumors may be either supratentorial (40%) or in- foramen of Monro” or “intraventricular oligodendroglioma”.9 fratentorial (60%) in location. The former may affect the Typically intraventricular, located in the lateral ventricles close brain parenchyma or ventricular cavities, being more com- to the foramen of Monro, although it may have other loca- mon in young patients. Those arising in the posterior fossa tions (gray nuclei, fourth ventricle, intramedullary location, occur in children.12,13 etc.), this tumor usually affects young adults and has a favor- Symptoms vary and depend on tumor location (supra/infraten- able prognosis.4,9-11 torial), with possible manifestations of increased intracranial Rev. Argent. Radiol. 2021;85:11-20 13 Cystic-appearing Intracranial neoplastic lesions pressure occurring most frequently with infratentorial tumors creased intracranial pressure secondary to obstructive hydro- due to their intraventricular location. Patients with supratento- cephalus being common.14 rial Ependymoma may experience headache, seizures or neu- These tumors are often associated with Turcot syndrome type rologic deficit.12,13 2, Gorlin-Goltz syndrome, Li Fraumeni syndrome, Rubinstein- These tumors appear as masses with cystic areas and chunky Taybi syndrome, Fanconi anemia and Nijmegen syndrome.14 calcification, and may occasionally bleed.12 Medulloblastomas are frequently located at the poste- Supratentorial ependymomas are larger in size (owing to rior fossa midline, involving the vermis, extending into the their typically intra-parenchymal location) and tend to con- fourth ventricle, with leptomeningeal dissemination; there- tain a cystic component, while infratentorial ependymomas fore a complete evaluation of the neuroaxis should be per- commonly have an intraventricular location and therefore are formed.14,15 smaller in size.13 In adults and older children, these tumors may be located