The Profuse Bleeding Potential of Orbital Epithelioid Hemangioendotheliomas.

Epithelioid hemangioendothelioma is a rare vascular tumor originating from vascular endothelial or pre-endothelial cells. We present the case of a 4-month-old male with a rapidly enlarging left zygomatico-orbital tumor causing mass effect on the eye globe. Examination revealed a large, nontender, solid lesion. CT angiography showed no major feeder or intralesional vessels. Complete surgical excision was performed, which was complicated by life-threatening intraoperative bleeding and successfully controlled with electrocautery. Microscopically, tumor cells exhibited varying morphologies. Immunohistochemistry confirmed the diagnosis of epithelioid hemangioendothelioma (positive for CD31 and CD34, negative for CK AE1/AE3). We also highlight 2 similar case reports with life-threatening bleeding complications. Surgeons should be aware of this condition and optimize surgical preparation, including blood products, to manage potential bleeding complications.

Primary alveolar rhabdomyosarcoma of the brain: a case report.

BACKGROUND: Primary brain rhabdomyosarcoma is a rare primary brain malignancy with few case reports. The vast majority of cases of primary brain rhabdomyosarcoma occur in pediatric patients, and immunohistochemistry can distinguish it from embryonal subtypes; however, few cases of primary brain rhabdomyosarcoma in adults have been reported in the literature. CASE PRESENTATION: We report the case of a 26-year-old White male patient who was found to have primary brain alveolar rhabdomyosarcoma after developing headaches for several months. A brain MRI revealed a mixed cystic and solid tumor along the vermis of the cerebellum. The patient underwent a gross total surgical resection, which confirmed the diagnosis of alveolar rhabdomyosarcoma. Further staging workup for another primary focus or disseminated disease yielded negative results, confirming the diagnosis of primary alveolar rhabdomyosarcoma of the brain. CONCLUSION: The standard of care for managing this rare type of brain tumor involves surgery with adjuvant chemoradiotherapy. Further studies should be conducted for a better diagnostic and therapeutic understanding.

PTEN R130Q Papillary Tumor of the Pineal Region (PTPR) with Chromosome 10 Loss Successfully Treated with Everolimus: A Case Report.

Papillary tumors of the pineal region (PTPR) can be observed among adults with poor prognosis and high recurrence rates. Standards of therapy involve total surgical excision along with radiation therapy, with no promising prospects for primary adjuvant chemotherapy, as long-term treatment options have not been explored. Chromosome 10 loss is characteristic of PTPR, and PTEN gene alterations are frequently encountered in a wide range of human cancers and may be treated with mTORC1 inhibitors such as everolimus. In parallel, there are no reports of treating PTPR with everolimus alone as a monopharmacotherapy. We report the case of a patient diagnosed with PTPR (grade III) characterized by a PTEN R130Q alteration with chromosome 10 loss that was treated with everolimus pharmacotherapy alone, resulting in an asymptomatic course and tumor regression, a rare yet notable phenomenon not described in the literature so far with potential to alter the management approach to patients with PTPR.

Frontal Sinus Morphometry in Relation to Surgically Relevant Landmarks in the Middle East Population: Can We Globalize?

BACKGROUND: The frontal bone is frequently approached during neurosurgical procedures. Feared complications of such surgeries include cerebrospinal fluid leak, among others, and frequently result from a breach of the frontal sinus. For this reason, the sinus should be avoided when possible. The supraorbital notch (SON) is a reliable and easily identifiable surgical landmark and its relation to the frontal sinus has been previously studied. However, the frontal sinus shows significant variability in size and shape between populations. METHODS: In the present study, we investigate the frontal sinus dimension and its relation to the SON in the Middle Eastern population. RESULTS: The analysis of a set of computed tomography scans reveals a significant variation in size between genders, and we subsequently provide neurosurgeons in the region with population-targeted, gender-specific risk maps. CONCLUSIONS: We finally conclude that a 2-cm margin rostral and lateral to the SON is safest.

The effect of war stress on multiple sclerosis exacerbations and radiological disease activity.

OBJECTIVE: The relationship between stressful life events and multiple sclerosis (MS) exacerbations or radiological disease activity is at best controversial. The aim of this study is to examine the relationship between exposure to war-related events incurred during the July 2006 Israeli-Lebanese war and clinical relapses and MRI disease activity among Lebanese MS patients. METHODS: We studied a group of 216 patients with clinically definite relapsing remitting MS (RRMS), on whom clinical data was available for the war period and for the preceding and following year(s). The number of relapses was determined during the war period and during similar periods over a 3-year span. All patients with brain MRI during the war period had their scans reviewed for evidence of disease activity as defined by the presence of gadolinium enhancing (Gd+) lesions. A group of patients with brain MRI performed outside the war period was used for comparison. RESULTS: The total number of relapses during the war period (n=23) was significantly higher than during non-war periods (mean=8.4, SD=0.86) (p=0.006). Of the 18 patients with brain MRI during the war, 5/7 with relapses and 1/11 without relapses had Gd+ lesions (p=0.013). More patients had Gd+ lesions during the war period (33%) compared to controls (13%) (p=0.075). INTERPRETATION: Our study shows that exposure to war-related events is likely to lead to an increase in both clinical relapses and MRI disease activity in patients with MS.

Silent sinus syndrome: an acquired condition.

We present the case of a 30-year-old man with silent sinus syndrome. A CT scan obtained 10 years earlier showed completely normal maxillary sinuses. This case illustrates the acquired nature of this disorder.

[Adjustable shunt system for the treatment of intracranial cerebrospinal fluid collections. Analysis of a series of 50 cases]. / Intérêt de la valve à pression d'ouverture réglable dans le traitement de l'hydrocephalie et des kystes arachnoïdiens. Analyse d'une série de 50 cas.

OBJECTIVE: The objective of this work is to evaluate the results of a draining valve of the cerebrospinal fluid, whose opening pressure is transcutaneously adjustable, in the treatment of intracranial collections. MATERIAL AND METHODS: A series of 50 operated patients is retrospectively reviewed. Forty-four patients are treated for hydrocephalus of variable causes and six patients for arachnoidal cysts. Offering eight opening pressures, the adjustable valve Sophy was used in all these patients. The mean follow-up is of 48 months. RESULTS: Nineteen patients (38%) needed an adjustment of their valve's opening pressure. This adjustment was achieved to higher levels in seven patients presenting with overdrainage and to lower levels in twelve patients presenting with underdrainage. In 4 patients a subdural collection was observed and treated. Six patients underwent a second surgery for infection or obstruction. Taking all etiologies into consideration, and after the adjustment of the valve's opening pressure, 40 patients had a positive clinical result (obvious amelioration of the symptomatology), ten patients did not improve. CONCLUSION: The valve Sophy provides a simple solution for the patients in whom it is difficult to choose an adapted opening pressure, it particularly allows to avoid a reintervention when this is rendered necessary by a hypo or a hyper drainage programmable valve.