Extra-axial Posterior Fossa Research Articles

Emotional Lability as a Symptom of Extra-axial Posterior Fossa Tumors: a Case-Control Review of Neuroanatomy and Patient-Reported Quality of Life.

Introduction Emotional lability (EL), the uncontrollable and unmotivated expression of emotion, is a rare and distressing symptom of brainstem compression. In published case reports, EL from an extra-axial posterior fossa tumor was alleviated by tumor resection. The primary aim herein was to radiographically establish the degree of compression from mass lesions onto brainstem structures. Secondarily, we compared changes in patient-reported quality of life (QOL) pre- and postoperatively. Methods A retrospective review of posterior fossa tumors treated between 2002 and 2018 at Vancouver General Hospital revealed 11 patients with confirmed EL. Each case was matched to three controls. A lateral brainstem compression scale characterized mass effect at the level of the medulla, pons, and midbrain in preoperative axial T2-weighted fluid-attenuated inversion recovery magnetic resonance imaging (FLAIR MRI) scans. Compression and clinical variables were compared between patient groups. Short Form-36 version 1 health surveys were retrospectively obtained from patient charts to compare pre- versus postoperative changes in survey scores between EL and control patients. Results EL symptoms ceased postoperatively for all EL patients. EL tumors exert greater compression onto the pons ( p = 0.03) and EL patients more commonly have cerebellar findings preoperatively ( p = 0.003). Patients with EL-causing tumors experienced greater improvement postoperatively in "Health Change" ( p = 0.05), which was maintained over time. Conclusion Findings suggest that compression onto the pons inhibits control over involuntary, stereotyped expression of emotion. This adds to evidence that EL may be attributed to cerebellum deafferentation from cortical and limbic structures through the basis pontis, leading to impaired modulation of emotional response. QOL results augment benefits of offering patients EL-alleviating tumor resection surgery.

Symptoms: Sudden Hearing Loss and Vertigo

Symptoms: Sudden Hearing Loss and Vertigo

Primary Occipital Ewing’s Sarcoma with Subsequent Spinal Seeding

Ewing's sarcoma is a primary bone cancer that mainly affects the long bones. This malignancy is particularly common in pediatric patients. Primary cranial involvement accounts for 1% of cases, with occipital involvement considered extremely rare. In this case study, primary occipital Ewing's sarcoma with a posterior fossa mass and subsequent relapse resulting in spinal seeding is reported. A 3-year-old patient presented with a 1-year history of left-sided headaches, localized over the occipital bone with progressive torticollis. Computed tomography (CT) imaging showed a mass in the left posterior fossa compressing the brainstem. The patient then underwent surgical excision followed by adjuvant chemoradiation therapy. Two years later, the patient presented with severe lower back pain and urinary incontinence. Whole-spine magnetic resonance imaging (MRI) showed cerebrospinal fluid (CSF) seeding from the L5 to the S4 vertebrae. Primary cranial Ewing's sarcoma is considered in the differential diagnosis of children with extra-axial posterior fossa mass associated with destructive permeative bone lesions. Although primary cranial Ewing's sarcoma typically has good prognosis, our patient developed metastasis in the lower spine. Therefore, with CNS Ewing's sarcoma, screening of the entire neural axis should be taken into consideration for early detection of CSF seeding metastasis in order to decrease the associated morbidity and mortality.

Giant extra-axial posterior fossa tuberculoma in a three-year-old child

Giant extra-axial posterior fossa tuberculoma in a three-year-old child

SUNCT Syndrome: diagnosis and treatment.

Short-lasting Unilateral Neuralgiform headache attacks with Conjunctival injection and Tearing (SUNCT) is a syndrome predominant in males, with a mean age of onset around 50 years. The attacks are strictly unilateral, generally with the pain persistently confined to the ocular/periocular area. Most attacks are moderate to severe in intensity and burning, stabbing or electrical in character. The mean duration of paroxysms is 1 minute, with a usual range of 10 to 120 seconds (total range 5 to 250 seconds). Prominent, ipsilateral conjunctival injection and lacrimation regularly accompany the attacks. Nasal stuffiness/rhinorrhoea are frequently noted. In addition, there is subclinical forehead sweating. During attacks, there is increased intraocular pressure on the symptomatic side and swelling of the eyelids. No changes in pupil diameter have been observed. Attacks can be triggered mostly from trigeminally innervated areas, but also from the extratrigeminal territory. There are also spontaneous attacks. An irregular temporal pattern is the rule, with symptomatic periods alternating with remissions in an unpredictable fashion. During active periods, the frequency of attacks may vary from <1 attack/day to >30 attacks/hour. The attacks predominate during the daytime, nocturnal attacks being seldom reported. A SUNCT-like picture has been described in some patients with either intra-axial or extra-axial posterior fossa lesions, mostly vascular disturbances/ malformations. In the vast majority of patients, however, aetiology and pathogenesis are unknown. In SUNCT syndrome, there is a lack of persistent, convincingly beneficial effect of drugs or anaesthetic blockades that are generally effective in cluster headache, chronic paroxysmal hemicrania, trigeminal neuralgia, idiopathic stabbing headache ('jabs and jolts syndrome'), and other headaches more faintly resembling SUNCT syndrome. Single reports have claimed that carbamazepine, lamotrigine, gabapentin, corticosteroids or surgical procedures may be of help. However, caution is recommended when assessing any therapy in a disorder such as SUNCT syndrome, in which the rather chaotic and unpredictable temporal pattern makes the assessment of any drug/therapeutic effect per se a particularly difficult matter.

Dural Cavernous Haemangioma with Bony Infiltration: Case Reports

We report the findings in a 5-year-old boy with headache, intermittent vomiting and blurred vision who was found to have an enhancing, dural based extra-axial posterior fossa mass. This was proven histologically to be a dural cavernous haemangioma with bony infiltration. These uncommon lesions may closely resemble meningioma on computed tomography (CT), magnetic resonance imaging (MRI) and angiography in terms of location, enhancement, bony changes and signal characteristics [1,2]. To our knowledge this is the first report of a dural cavernous haemangioma with bony infiltration as seen on CT and only the second in the literature [3].

Multiple Arteriovenous Malformations Located in the Cerebellum, Posterior Fossa, Spinal Cord, Dura, and Scalp with Associated Port-Wine Stain and Supratentorial Venous Anomaly

Multiple arteriovenous malformations involving the left cerebellum and extra-axial posterior fossa with associated calcified cyst, spinal cord, dura, and scalp were present in a 7-year-old girl, who became symptomatic after a cerebellar hemorrhage. A large port-wine stain was noted on the right temporoparietal scalp, consistent with the scalp arteriovenous malformation area, as demonstrated by angiogram. In addition to the multiple arteriovenous malformations, nonfilling of the superior sagittal sinus and tortuous, irregular cortical veins were recognized.

Anterior pontomesencephalic vein and basilar artery in exophytic brainstem gilioma.

The angiographic differentiation of an intrinsic exophytic brainstem glioma from an extra-axial posterior fossa tumor is not always easy. The location of the basilar artery alone may not be significant. Displacement of the anterior pontomesencephalic vein anterior to the basilar artery appears to be a reliable indicator of an intrinsic exophytic brainstem glioma.

Computed tomography in the diagnosis of extra-axial posterior fossa masses.

Extra-axial posterior fossa masses can be diagnosed reliably by computed tomography (CT) in most cases. Acoustic and trigeminal neurinomas, meningiomas, cholesteatomas, and other extra-axial masses can usually be distinguished from intra-axial masses by asymmetric widening of the basal subarachnoid spaces, bone destruction, continuity of the tumor mass with the tentorium or bone, and more sharply defined margins. Multiple-cut study of the posterior fossa improved visualization of the fourth ventricle and basal cisterns. Interpretation of cisternal changes in association with changes in the fourth ventricle and abnormal attenuation coefficients permits accurate diagnosis of posterior fossa masses.

Lateral recesses of the fourth ventricle.

One hundred-fifty pneumoenecephalographic studies, during which serial anteroposterior tomography was performed, were reviewed to determine the appearance of the lateral recesses of the fourth ventricle in both normal and pathological states. Displacement of the lateral recesses will aid in differentiating between intra- and extra-axial posterior fossa tumors. In prepontomedullary tumors, a “trumpet-like” enlargement of the lateral recess orifice is seen. When cerebellar degeneration is present, change in size, shape, and position of the posterosuperior and lateral recesses may indicate fourth ventricular enlargement not otherwise suspected.