- Department of Neurosurgery, University of Texas Health Science Center, San Antonio, Texas, USA
- Department of Radiology, University of Texas Health Science Center, San Antonio, Texas, USA
Byron C. Branch
Department of Neurosurgery, University of Texas Health Science Center, San Antonio, Texas, USA
DOI:10.4103/2152-7806.148547Copyright: © 2015 Branch BC. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
How to cite this article: Branch BC, Tantiwongkosi B, Altmeyer W, Bartanusz V. Posterior fossa giant tumefactive perivascular spaces: 8-year follow-up in an adolescent. Surg Neurol Int 05-Jan-2015;6:2
How to cite this URL: Branch BC, Tantiwongkosi B, Altmeyer W, Bartanusz V. Posterior fossa giant tumefactive perivascular spaces: 8-year follow-up in an adolescent. Surg Neurol Int 05-Jan-2015;6:2. Available from: http://sni.wpengine.com/surgicalint_articles/posterior-fossa-giant-tumefactive-perivascular-spaces-8-year-follow-up-in-an-adolescent/
Background:Cystic masses in the posterior fossa are ominous appearing lesions with broad differential diagnosis. Giant tumefactive perivascular spaces (GTPS) are rarely occurring pathological findings in the posterior fossa with unclear etiology and ill-defined long-term prognosis.
Case Description:We present a case of a 15-year-old male diagnosed with posterior fossa GTPS. The patient remained asymptomatic during the 8-year follow-up after diagnosis with the serial magnetic resonance imaging (MRI) showing no change in the size and morphology of the lesion.
Conclusion:This case supports prior literature on supratentorial GTPS suggesting that the natural history of GTPS is mostly benign. Identification of GTPS in the posterior fossa could prevent the patient from unnecessary surgery or other aggressive treatment modalities.
Keywords: Cerebral cyst, giant tumefactive perivascular spaces, posterior fossa, prognosis
Posterior fossa cystic lesions can appear quite ominous when discovered on a patient's imaging workup, they may present a challenging differential diagnosis, and tend to be associated with a dismal prognosis. The identification and study of cerebral cysts has been advanced over the past few decades with the introduction of magnetic resonance imaging (MRI) into clinical practice. Presently the natural history and prognosis for many types of intracranial cystic lesions can be known based largely on imaging alone with the goal to identify those pathologies that need rapid medical or surgical intervention. In the same way it is extremely important to recognize benign pathological findings to prevent unnecessary interventions.
Posterior fossa giant tumefactive perivascular spaces (GTPS) are one such discovery, which has been divulged during the MRI era, but its natural history and pathogenesis remains uncertain.[
The patient was addressed to our institution for second opinion. His medical history started 8 years ago at his age of 15 when he was hospitalized with a 3-day history of dizziness, gait instability, nausea, and headaches. On clinical examination at that time, he had mild diplopia on left lateral gaze and convergence, incapacity of upward vertical gaze, mild right upper and lower extremity weakness (M4), right sided hypoesthesia, mild gait instability, but no cerebellar signs in the extremities.
The MRI of the brain revealed a well-defined lobulated cystic lesion in the right middle cerebral peduncle close to the forth ventricle with mild mass effect to the cerebellar vermis [
Magnetic resonance imaging (MRI) of the brain performed at the time of symptom onset 8 years ago demonstrated a well-defined lobulated cystic lesion in the right middle cerebral peduncle close to the forth ventricle with mild mass effect to the cerebellar vermis. The lesion contained multiple thin septations (black arrow in C) and followed cerebrospinal fluid (CSF) signal in all pulse sequences (a) axial T1-weighted image, (b) axial postcontrast T1-weighted image, (c) axial T2-weighted image, (d) diffusion-weighted, (e) apparent diffusion coefficient). There is no definite enhancement or diffusion restriction. Mild edema is noted in the right middle cerebellar peduncle (white arrow in C). Single voxel proton MR spectroscopy (f) showed unremarkable concentration of N-acetylaspartate (NAA), choline (Cho), Creatine (Cr) with normal Cho/Cr ratio and Cho/NAA ratio
During his hospitalization the patient underwent a craniotomy with biopsy of the cyst. Via the midline posterior fossa craniectomy and supracerebellar-infratentorial approach, the right ponto-mesencephalic region was identified. With the help of image guidance a cystic cavity containing xantochromic fluid was entered about 3–4 mm below the surface. Following biopsy of the cyst wall, the closure and immediate postoperative course were uneventful. The surgical pathology of the cyst revealed nontumoral edematous parenchyma without an epithelial lining containing signs of old hemorrhage and reactive astrocytes with glial satellitosis. The histologic reports were inconclusive and a nondefinitive diagnosis of pilocytic astrocytoma was given based on the history, imaging, location, and histology.
In the postoperative period, the patient completely recovered from upper gaze palsy and double vision, as well as his left hemiparesis and hemihypoesthesia resolved. He was followed on 2-year intervals and remained neurologically intact at the most recent 8-year follow-up. Serial MRI imaging revealed no change in the size and shape of the lesion (
GTPS typically appear as multiloculated cystic lesions located unilaterally within the thalamomesencephalic region and white matter of the cerebral hemispheres and less commonly within the cerebellar white matter, as they are thought to develop from the perivascular spaces of the perforating arteries in these areas.[
While the exact etiology of GTPS remains elusive, several hypotheses have surfaced over the past decades. The first theory argues that GTPS are distensions of perivascular spaces, likely caused by a disorder of the permeability of the arterial wall. A permeable arteriole situated in the center of the cavity, as evidenced by the histologic presence of severe lesions of segmental necrotizing angeitis, may facilitate dilation of the Virchow–Robin space.[
We should note that GTPS appear to be a separate process, although not unrelated, from dilated perivascular spaces or cerebral lacunes (also termed ‘unidentified bright objects’ on MRI T2-weighted sequences) associated with aging and cerebrovascular disease.[
As our knowledge of GTPS increases, it is becoming evident that while these lesions can appear quite ominous, they hold a relatively benign prognosis. The difficultly herein for clinicians becomes ensuring that an accurate diagnosis is made, and that more malignant pathologies are not confused with GTPS and vica-versa. Within the differential diagnosis of posterior fossa cystic lesions we should consider: Parasitosis, abscesses, cystic glial and astrocytic tumors, craniopharyngioma, hemangioblastoma, dermoid and epidermoid cyst, arachnoid and Rathkes cleft cysts, porencephalic cysts, encephalomalacia, subependymal cysts, neuroepithelial cysts, metabolic deposition disorders.[
An important insight into the pathology of GTPS is its benign nature. Our patient remained symptom free with a large cerebellar cystic lesion, which did not change in size over an 8-year follow-up period. No other report of GTPS located in the posterior fossa has documented a follow up period longer than 1 year. One report in the literature documents GTPS located in the frontal lobe, which remained stable in size and asymptomatic over a 17-year follow up.[
GTPS should be included into the differential diagnosis of cystic masses in the posterior fossa. Although differentiating GTPS from other benign pathologies may be challenging, it is important to keep this benign pathological entity on the forefront. As documented by our case, posterior fossa GTPS has a long-term favorable prognosis and its identification would prevent from unnecessary surgery or other more aggressive treatment modalities.
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