Histopathological, Demographic, and Clinical Signatures of Medulla Oblongata Germ Cell Tumors: A Case Report With the Review of Literature.

The medulla oblongata is one of the rarest sites of occurrence for germ cell tumors (GCTs) of the central nervous system. As there is scant data regarding epidemiology, clinical presentations, optimal intervention, and long-term prognosis, we aimed to delineate the features of this rare entity by presenting our representative case and performing a quantitative review of the literature. A 24-year-old woman presented to our department with vertigo and swallowing difficulties. Magnetic resonance imaging revealed a homogenously enhanced exophytic lesion arising from the medulla oblongata and extending to the fourth ventricle. Surgical resection was performed and a histological diagnosis of pure germinoma was made. The patient underwent chemotherapy and whole-ventricular irradiation. No recurrence has been experienced for 4 months after the surgery. According to the literature, the prognosis of GCTs at the medulla oblongata seems no worse than those at typical sites. Striking features including occurrence at an older age, female preponderance, and a predominance of germinoma were noteworthy. The pattern of local recurrence suggests extensive radiation coverage is not a prerequisite. Special attention is needed for cardiac and respiratory functions as the main factors eliciting mortality.

Alteration of major venous drainage routes in a patient with achondroplasia after ventriculoperitoneal shunt placement.

Achondroplasia is the most common form of dwarfism, caused by a mutation in fibroblast growth factor receptor 3 gene, leading to multiple pathological conditions. Herein, we present a case of an infant with achondroplasia associated with hydrocephalus and severe foramen magnum stenosis. Computed tomography (CT) venography showed prominent suboccipital emissary veins comprising major venous drainage routes and hypoplastic transverse sinuses, which increased the risk of foramen magnum decompression. The infant was initially treated with ventriculo-peritoneal (VP) shunt. After 8 months, CT venography showed regression of suboccipital emissary veins and more prominent transverse sinuses. Subsequently, foramen magnum decompression was safely performed without sacrificing major venous drainage routes.

Atretic cephalocele associated with sinus pericranii: a single-center analysis.

PURPOSE: The purpose of this report is to investigate the association of atretic cephalocele (AC) with sinus pericranii (SP) in order to improve the management of AC. METHODS: Pediatric patients with AC who underwent repair surgeries were retrospectively analyzed. Anomalies including SP were evaluated using computed tomography angiography and venography (CTA/V) as well as magnetic resonance imaging. RESULTS: Fourteen patients were identified, comprising of 5 males and 9 females. The AC was located interparietally in 8 cases and occipitally in 6 cases. Seven cases (50%) were found to be associated with SP, all of which did not involve major intracranial venous circulation. Five out of 8 parietal ACs (63%) were accompanied by SP, while 2 out of 6 occipital ACs (33%) exhibited SP. All of the SP associated with AC only contributed to a small portion of the venous outflow, and the repair surgeries were successfully performed without excessive bleeding. CONCLUSION: More than half of the ACs were associated with SP, with a higher prevalence in the parietal region compared to the occipital region. For the appropriate management of AC, preoperative investigation of SP using CT venography is considered important.

Brain Metastasis Mimicking Glioma on Imaging Appearance During Tyrosine Kinase Inhibitor Administration: A Case Series and Literature Review.

OBJECTIVE: Preoperative imaging diagnosis is critical to planning treatment strategies; however, it is occasionally challenging and sometimes misleading. The effects of molecularly targeted therapies on imaging appearances remain uncharted. We investigated the imaging characteristics of brain metastasis during tyrosine kinase inhibitor (TKI) administration. METHODS: We analyzed the 12 cases of brain metastasis from lung cancer in our institute, including a case of a 49-year-old woman under gefitinib. Additionally, we reviewed the cases of brain metastasis from lung cancer with gefitinib treatment in the literature. RESULTS: A woman during five-year gefitinib treatment for postoperative recurrence of lung adenocarcinoma was found to have a cerebellar tumoral lesion incidentally on magnetic resonance imaging (MRI). This lesion did not harbor any peritumoral edema, along with appearing hypometabolic on fluorodeoxyglucose (FDG) positron emission tomography (PET). This appearance was inconsistent with a typical metastatic appearance, and high-grade glioma was instead highly suspected, leading to a decision to proceed to gross total tumor resection. The pathological diagnosis, however, was brain metastasis from lung cancer. The other 11 cases without TKI treatment showed peritumoral edema on MRI and higher accumulation of FDG on PET. The two cases of brain metastasis with gefitinib in the literature showed no peritumoral edema on MRI. CONCLUSION: TKIs like gefitinib can affect tumor biology, leading to a loss of typical imaging findings such as peritumoral brain edema and hyper-metabolism. As preoperative imaging diagnosis guides us in surgical planning, including biopsy or resection, ongoing treatment information should be fully integrated into imaging interpretation.

Stereotactic radiosurgery for recurrent skull base polymorphous adenocarcinoma invading the cavernous sinus: illustrative case.

BAKGROUND: Polymorphous adenocarcinomas (PACs) are rare tumors arising from the salivary glands. Radical resection and postoperative radiotherapy are the mainstays of treatment. However, complete tumor resection is not always achievable when the tumor invades the skull base. Stereotactic radiosurgery (SRS) could be a less invasive alternative for treating skull base PACs. OBSERVATIONS: A 70-year-old male with a history of surgery for a right palatine PAC presented with right visual impairment, diplopia, and ptosis. Imaging studies revealed tumor recurrence invading the right cavernous sinus (CS). SRS using a gamma knife was performed for this recurrence, prescribing a marginal dose of 18 Gy at a 50% isodose line. Five months after SRS, his symptoms were relieved, and the tumor was well-controlled for 55 months without any adverse events. LESSONS: To the best of the authors' knowledge, this is the world's first case of recurrent skull base PAC invading the CS that was successfully treated with salvage SRS. Thus, SRS may be an applicable treatment option for skull base PACs.

Tyrosine Kinase Inhibitor-associated Cerebral Arterial Occlusive Disease Treated with High-flow Bypass Surgery: A Case Report.

Nilotinib, one of the tyrosine kinase inhibitors, has been used to treat chronic myeloid leukemia (CML) and Philadelphia chromosome-positive acute lymphoblastic leukemia (Ph+ ALL). Nilotinib-associated cerebral arterial occlusive disease, which is treated with medicine with/without bypass surgery or stenting, has been sporadically reported to occur. The mechanism of the nilotinib-associated cerebral disease has not been clarified and is still controversial. Here we present the case of a 39-year-old woman with Ph+ ALL treated with nilotinib, which led to symptomatic intracranial arterial stenosis. We performed high-flow bypass surgery and observed the arterial stenotic change in the stenotic portion intraoperatively, whose findings strongly supported the theory of atherosclerosis and seemed to be irreversible.

Carotid Endarterectomy.

Stroke is the second leading cause of death worldwide. One of the main causes of stroke is carotid artery stenosis. Stenosis with atherosclerosis in the carotid artery can cause stroke by hemodynamic ischemia or artery to artery embolism. A most common surgical intervention for carotid artery stenosis is carotid endarterectomy (CEA). Many studies on CEA have been reported and suggested medical indications. For symptomatic carotid stenosis, generally, CEA may be indicated for patients with more than 50% stenosis and is especially beneficial in men, patients aged 75 years or older, and patients who underwent surgery within 2 weeks of their last symptoms. For asymptomatic carotid stenosis, CEA may be indicated for those with more than 60% stenosis, though each guideline has different suggestions in detail. In order to evaluate the indication for CEA in each case, it is important to assess risks for CEA carefully including anatomical factors and comorbidities, and to elaborate each strategy for each operation based on preoperative imaging studies including carotid ultrasonography, magnetic resonance imaging and angiography. In surgery there are many tips on operative position, procedure, shunt usage and monitoring to perform a safe and smooth operation. Now that carotid artery stenting has been rapidly developed, better understanding for CEA is required to treat carotid artery stenosis adequately. This chapter must be a good help to understand CEA well.

Large Cell Neuroendocrine Carcinoma in the Sinonasal Cavity with Large Intracranial Extension Treated with Endonasal Endoscopic and Transcranial Combined Surgery: A Case Report.

Large cell neuroendocrine carcinoma (LCNEC) is a rare malignant tumor that typically arises in the lungs. It is especially rare in the sinonasal cavity, and treatment has not been established. In this study, we present the case of a 56-year-old woman with a large sinonasal LCNEC that extended into her brain. We performed endonasal endoscopic and transcranial combined surgery followed by chemoradiation therapy. The combined surgery enabled us to approach and remove the extensive tumor from two different directions at one time less invasively. We have achieved good tumor control for 18 months so far.

Phospholipase C ß3 is Required for Climbing Fiber Synapse Elimination in Aldolase C-positive Compartments of the Developing Mouse Cerebellum.

In the cerebellum of neonatal mice, multiple climbing fibers (CFs) form excitatory synapses on each Purkinje cell (PC). Only one CF is strengthened in each PC from postnatal day 3 (P3) to P7, whereas the other weaker CFs are eliminated progressively from ∼P7 to ∼P11 (early phase of CF elimination) and from ∼P12 to ∼P17 (late phase of CF elimination). Type 1 metabotropic glutamate receptor (mGluR1) triggers a canonical pathway in PCs for the late phase of CF elimination. Among downstream signaling molecules of mGluR1, phospholipase C ß3 (PLCß3) and ß4 (PLCß4) are expressed complementarily in PCs of aldolase C (Aldoc)-positive (+) and Aldoc-negative (-) cerebellar compartments, respectively. PLCß4 is reported to mediate the late phase of CF elimination in the anterior half of the cerebellar vermis which corresponds to the Aldoc (-) region. However, roles of PLCß3 and Aldoc in CF synapse elimination are unknown. Here, we investigated CF innervation of PCs in Aldoc-tdTomato knock-in mice that underwent lentivirus-mediated knockdown (KD) of PLCß3 in PCs during postnatal development. By recording CF-mediated excitatory postsynaptic currents from PCs and immunostaining CF synaptic terminals, we found that significantly higher percentage of PCs with PLCß3-KD remained multiply innervated by CFs in Aldoc (+) compartments after P12, which was accompanied by impaired elimination of somatic CF synapses and reduced dendritic CF translocation. In contrast, deletion of Aldoc had no effect on CF synapse elimination. These results suggest that PLCß3 is required for the late phase of CF elimination in Aldoc (+) PCs.