Choroid Plexus Carcinoma in Adults: Literature Review and First Report of a Location into the Third Ventricle.

Choroid plexus carcinoma (CPC) is a rare intraventricular neoplasm originating from choroid plexus. CPC is the most aggressive choroid plexus tumor. Almost all the CPCs are detected in children, and the preferred location is the lateral ventricle. We reviewed the literature to evaluate the state of the art concerning the management of CPC in adults and report the first case described of the extremely rare localization into the third ventricle. A 38-year-old woman presented a medical history of Parinaud syndrome and occasional facial weakness. Brain magnetic resonance imaging revealed a mass lesion in the pineal region and posterior part of the third ventricle with obstructive hydrocephalus. She underwent subtotal resection through a supracerebellar infratentorial approach. After the histopathological diagnosis of CPC, the patient underwent a second surgery with gross total resection and adjuvant radiotherapy. CPC in adults, given its extreme rarity, does not have a standardized treatment. Gross total resection should be the first step of the treatment: however, according to the literature, gross total resection is achieved only in 40-75% of cases in CPC as opposed to 95% in choroid plexus papilloma, mainly due to the difficulty in managing a highly vascularized tumor in such a deep location. Chemotherapy has not an established role and adjuvant treatment is based on radiotherapy. In the case described hereby the gross total resection associated with surgical treatment of hydrocephalus and adjuvant radiotherapy has achieved a good clinical and radiological outcome.

Quantitative analysis of cerebrospinal fluid dynamics at phase contrast cine-MRI: predictivity of neurosurgical "Shunt" responsiveness in patients with idiopathic normal pressure hydrocephalus.

BACKGROUND: Aqueductal stroke volume (ACSV) measured by phase-contrast cine (PCC)-MRI has been proposed with controversy as a tool for the selection of patients with normal pressure hydrocephalus (NPH) as candidates for shunt-surgery. The aim of this study was to assess if PCC-MRI scan measurements of ACSV could select properly these patients. METHODS: We retrospectively reviewed charts and MRI of 38 shunted patients (72.16±6.16 years). ACSV measurements were performed 7-30 days before shunt and at the first and sixth months after surgery. Normally distributed variables were compared in the two groups (improved/unimproved) by t-test for baseline values and with repeated measures analysis of variance. RESULTS: Twenty-six patients (68,4%) improved after VPS (mean time of symptom onset was 8.15±7.19 months). Mean preoperative ACSV value was 271.85±143.03, which decreased by 21.6% (mean 213±125.14) at the first month and 40.3% sixth months after VPS (mean 162.15±91.5). Twelve patients (31.6%) did not improve (mean time of symptom onset was 29±5.62 months). Mean preoperative ACSV value was 79.83±31.24, decreased to 8.7% (mean 72.83±28.66) at first month after VPS. 21.2% (mean 62.83±31.12) after six months. We found statistical difference between preoperative ACSV of improved and unimproved patients (P<0.01), onset time of symptoms (P<0.01) and the changes in ACSV after one and six months in both groups (P<0.001). CONCLUSIONS: ACSV is useful to stratify patients with NPH after surgery (improved /not improved) suggesting to proceed with serial ACSV measurements before deciding treatment.

Osteopontin in the Cerebrospinal Fluid of Patients with Severe Aneurysmal Subarachnoid Hemorrhage.

Aneurysmal subarachnoid hemorrhage (SAH) is associated with high morbidity and mortality. In SAH patients, plasma osteopontin (OPN) has been shown to independently predict poor outcome. The aim of the study is to investigate, in a selected population with severe SAH, OPN time course in cerebrospinal fluid (CSF) and plasma during the first week after aneurism rupture, and OPN prognostic value. We included 44 patients with the following criteria: (1) age 18 and 80 years, (2) diagnosis of SAH from cerebral aneurysm rupture, (3) insertion of external ventricular drain. Plasma and CSF were sampled at day 1, 4, and 8. OPN levels, in CSF and plasma, displayed a weak correlation on day 1 and were higher, in CSF, in all time points. Only in poor prognosis patients, OPN levels in CSF significantly increased at day 4 and day 8. Plasma OPN at day 1 and 4 was predictor of poor outcome. In conclusion, plasma and CSF OPN displays a weak correlation, on day 1. The higher levels of OPN found in the CSF compared to plasma, suggest OPN production within the CNS after SAH. Furthermore, plasma OPN, at day 1 and 4, seems to be an independent predictor of poor outcome.

Posttraumatic Middle Meningeal Artery Pseudoaneurysm: Case Report and Review of the Literature.

BACKGROUND: Intracranial pseudoaneurysm is a rare entity, with few cases described in the literature, and is mostly associated with a history of traumatic brain injury. Traumatic aneurysms comprise <1% of all intracranial aneurysms. In particular, middle meningeal artery (MMA) aneurysms are uncommon and usually caused by a skull fracture in the temporal region. About 40 traumatic MMA aneurysms are reported in the literature, and only 28 nontraumatic aneurysms are reported, usually related to high-flow conditions. The behavior of these aneurysms is largely unknown: both spontaneous resolution and aneurysm growth, leading to subsequent rupture, have been reported. Surgical and endovascular management are feasible for MMA aneurysms; however, the criterion standard treatment is not defined. CASE DESCRIPTION: We report the case of a traumatic pseudoaneurysm of the right MMA treated with an endovascular approach and provide a review of the literature. CONCLUSIONS: Aneurysms of the MMA are a rare entity that must be taken into account in the setting of a traumatic brain injury or predisposing factors. The diagnosis and aggressive treatment are mandatory, preventing the devastating consequences of their rupture. Endovascular and surgical techniques are well defined and available, even though there is not a demonstrated superiority in any of them.

Rare Case of Dumbbell-Shaped Spinal Cavernous Hemangioma and Literature Review.

BACKGROUND: Spinal epidural cavernous hemangiomas are rare vascular malformations. Exceptionally, they present with dumbbell-shaped morphology. When they happen, it's mandatory to include their pathology in the differential diagnosis because of their similarity to schwannomas. CASE DESCRIPTION: We report the case of a 72-year-old woman with a dumbbell-shaped thoracic epidural cavernous hemangioma. A literature review of diagnostic features and current treatment options are also discussed. CONCLUSIONS: Surgery is safe and effective in both improving patient condition and preventing acute hemorrhage that can worsen the outcome, causing neurologic and potentially irreversible deficits. The favorable result we obtained in our patient suggests that surgery should be evaluated as the first option, even in patients with large epidural cavernous hemangiomas.

Translaminar Approach for Treatment of Hidden Zone Foraminal Lumbar Disc Herniations: Surgical Technique and Preoperative Selection of Patients with Long-Term Follow-Up.

BACKGROUND: Lumbar disc herniation is a common degenerative disease of the lumbar spine with a prevalence of 1%-3% in some population studies. In 10% of patients, there is a fragment migrated cranially in Macnab's "hidden zone." In selected cases, this fragment can be removed with a translaminar approach that was described in 1998. We provide a detailed description of the technical advantages and pitfalls of the translaminar approach in a consecutive series of 32 patients treated at our institution. METHODS: Patients were divided preoperatively and postoperatively into 5 classes based on the Oswestry Disability Index (ODI): class 1, ODI 0%-20% (minimal disability); class 2, 20%-40% (moderate disability); class 3, 40%-60% (severe disability); 60%-80% (crippled); 80%-100% (bedridden or disabling symptoms). RESULTS: Four (12.5%) patients were upgraded 1 ODI class after the operation; 6 (18.7%) patients were upgraded 2 classes, 8 (25%) patients were upgraded 3 classes, and 11 (34.4%) patients were upgraded 4 classes. In 3 (9.4%) patients, ODI class did not change after the operation. After surgery, 7 (21.9%) patients developed mild low back pain. Mean follow-up was 25 months. CONCLUSIONS: When performed by dedicated spinal neurosurgeons, the translaminar approach is safe and effective in patients with long-term follow-up. Most patients showed an improvement in ODI. Major pitfalls were related to surgical selection and the narrow working space.

Computed tomography perfusion and computed tomography angiography in vasospasm after subarachnoid hemorrhage.

BACKGROUND: The aim of the study was to evaluate the effectiveness and reliability of a combined computed tomography angiography (CTA) and computed tomography perfusion (CTP) approach in the diagnosis of cerebral vasospasm after subarachnoid hemorrhage. METHODS: Nineteen patients with clinical signs of arterial vasospasm and positive transcranial Doppler (TCD) were enrolled and underwent CTP. Mean time transit (MTT), cerebral blood flow (CBF) and cerebral blood volume (CBV) values of 20 standardized ROI (regions of interest) were analyzed, and CTA used to measure the gauge of 26 arterial ramifications. CTA measurements were compared with those taken upon hospitalization. Of the 19 patients, 11 were scheduled for digital subtraction angiography (DSA), performed less than 12 hours after execution of the CTA-CTP protocol. The results were compared with findings of DSA and/or clinical follow-up and CT or TCD. RESULTS: Computed tomography angiography diagnosis of vasospasm was confirmed in all cases (100% sensitivity and 100% specificity), while CTP yielded 3 false negatives (70% sensitivity and 100% specificity). All patients sent for endovascular treatment had received diagnostic confirmation of vasospasm by angiography. CTP thresholds proved reliable in both diagnosis and indicating treatment. CONCLUSIONS: Combined one-shot CT angiography and CT perfusion represents a valid alternative to DSA in the diagnosis and management of cerebral vasospasm.

Orbital meningeal melanocytoma: Histological, immunohistochemical and molecular characterization of a case and review of the literature.

AIMS: We provide morphological, immunohistochemical and molecular characterization of the 3rd "intermediate-grade" orbital meningeal melanocytoma, testing for the first time Vysis Melanoma FISH Probe Kit. We reviewed the literature in order to discuss the main differential diagnoses and to provide a better molecular description of these unusual tumors of difficult diagnosis and controversial management. METHODS: Histochemical stains (Haematoxylin and Eosin, Perls, reticulin), immunohistochemistry (HMB45, p16, Melan-A, S100, EMA, Ki67, CD68), polymerase chain reaction amplification and sequence analysis (BRAF, exon 15; NRAS exons 2 and 3; c-KIT, exons 11, 13, 17, 18; GNAQ, exons 4 and 5; GNA11, exons 4 and 5) and fluorescent in situ hybridization (RREB1, 6p25; MYB, 6q23; CCND1, 11q13; CEP 6, 6p11.1-q11.1) were performed on paraffin-embedded, formalin-fixed material. RESULTS: Histological diagnosis of "intermediate-grade" melanocytoma was supported by zonal necrosis and increased Ki67-index (12%). Immunophenotype: HMB45+(strong, >75%), Melan-A+(strong, >75%), p16+(∼20%), S100 -/+ (<5%), EMA -/+ (<5%), CD68 - (positive histiocytes). No gene mutations nor copy-number alterations were identified. The patient was asymptomatic and disease-free 3 years after total surgical excision. CONCLUSIONS: Adequate sampling and accurate immunohistochemical characterization are important for a correct diagnosis. Molecular analysis could provide important additional information (especially for "intermediate-grade" tumors), but further data are needed.

Primary choroid plexus papilloma of the sacral nerve roots.

The authors describe a unique case of a choroid plexus papilloma of the sacral nerve roots. This 60-year-old woman was admitted to the hospital because of a 1-year history of sacral pain, rectal and urinary bladder retention, and paradoxical episodic incontinence. Physical examination revealed sensory abnormalities in the S-2 dermatomes and poor rectal and bladder sphincter contractions. Contrast-enhanced spinal MR imaging showed a well-circumscribed, ovoid, homogeneously enhancing mass at the S1-2 level suggesting a diagnosis of ependymoma or schwannoma, and surgery allowed the identification and complete removal of a soft gray mass intimately adhering to the sacral nerve roots. Histological examination revealed a tumor consisting of papillary structures lined by a single layer of columnar cells, with an immunophenotype that satisfied the diagnostic criteria of choroid plexus papilloma. After diagnosis, contrast-enhanced brain MR imaging excluded the presence of a primary choroid plexus papilloma in the cerebral ventricles, thus ruling out a drop metastasis along the CSF pathways. A review of the literature did not reveal any similar cases of choroid plexus papilloma, and so the authors also discuss the inclusion of primary or metastatic papillary tumors in this unusual location as part of the differential diagnosis.