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https://www.readbyqxmd.com/read/28515451/hoxa7-hoxa9-and-hoxa10-are-differentially-expressed-in-clival-and-sacral-chordomas
#1
Daniela Jäger, Thomas F E Barth, Silke Brüderlein, Angelika Scheuerle, Beate Rinner, Adrian von Witzleben, André Lechel, Patrick Meyer, Regine Mayer-Steinacker, Alexandra von Baer, Markus Schultheiss, Christian R Wirtz, Peter Möller, Kevin Mellert
Chordomas are rare tumours of the bone arising along the spine from clivus to sacrum. We compared three chordoma cell lines of the clivus region including the newly established clivus chordoma cell line, U-CH14, with nine chordoma cell lines originating from sacral primaries by morphology, on genomic and expression levels and with patient samples from our chordoma tissue bank. Clinically, chordomas of the clivus were generally smaller in size at presentation and patients with sacral chordomas had more metastases and more often recurrent disease...
May 17, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28498375/neuro-ophthalmologic-manifestations-of-cholangiocarcinoma-a-case-series
#2
G Rico, S V Smith, Y Siddiqui, A Whyte, D Gombos, A G Lee
PurposeTo describe the neuro-ophthalmologic findings of cholangiocarcinoma.MethodsWe report a retrospective chart review of cholangiocarcinoma patients presenting at two tertiary care centers in the Texas Medical Center.ResultsFive patients with neuro-ophthalmologic symptoms related to cholangiocarcinoma were identified. One patient presented with diplopia due to metastasis to the left medial rectus muscle, two patients had metastasis to the occipital lobe resulting in homonymous hemianopsia, one patient had involvement of the clivus resulting in sixth nerve palsy, and one presented with a hypercoagulable state-related stroke causing a homonymous hemianopsia and visual hallucinations...
May 12, 2017: Eye
https://www.readbyqxmd.com/read/28494780/metastatic-non-muscle-invasive-bladder-cancer-with-meningeal-carcinomatosis-case-report-of-an-unexpected-response
#3
Diego Teyssonneau, Amaury Daste, Vincent Dousset, Jean-Luc Hoepffner, Alain Ravaud, Marine Gross-Goupil
BACKGROUND: Non-muscle invasive bladder cancer (NMIBC) is usually treated with local therapy including transurethral resection of the bladder tumor and intravesical therapy depending on the stage of the tumor. NMIBC is a rarely a metastatic diseases with lymph node invasion in less of 10%. In the other hand meningeal carcinomatosis is a rare location for metastases with extremely poor outcomes. We described a case report of a patient presenting a metastatic disease to bones and meninges, several years after the treatment of NMIBC, which had been in complete response (CR) for 4 years after chemotherapy treatment...
May 11, 2017: BMC Cancer
https://www.readbyqxmd.com/read/28484567/clival-defect-causing-primary-spontaneous-rhinorrhea
#4
Vivek Tandon, Kanwaljeet Garg, Ashish Suri, Ajay Garg
Primary cerebrospinal fluid (CSF) rhinorrhea due to a defect in clivus is an extremely rare entity, till date only four such cases are reported in the world literature. We present a case of 55-year-old female who presented with primary spontaneous CSF rhinorrhea, for which endonasal surgery was performed about 9 years back. Patient developed recurrent CSF leak with a history of meningitis. Repeat imaging showed clival defect causing CSF leak. Transsphenoidal repair was performed under neuronavigation. In this paper, we discuss the possible mechanism of fistula formation and literature is reviewed...
April 2017: Asian Journal of Neurosurgery
https://www.readbyqxmd.com/read/28474478/incomplete-cavernous-sinus-syndrome-as-the-initial-manifestation-of-a-previously-undetected-metastatic-prostate-adenocarcinoma
#5
María Machío Castelló, Diego Escobar Montatixe, Carlos Cenjor Español, José Miguel Villacampa Aubá, Julia Montoya Bordón, Rafael Carias Calix, Rafael Sáez Pinel
BACKGROUND: Cavernous sinus syndrome presents as unilateral ophthalmoplegia associated with sympathetic denervation, pain, paresthesias, and V1 and V2 distribution numbness. The etiology may be vascular, inflammatory, infectious, and, less commonly, neoplastic (metastatic). METHODS: We report a patient with incomplete cavernous sinus syndrome as the initial manifestation of previously undetected metastatic prostate adenocarcinoma. RESULTS: A 59-year-old man presented with a 2-month history of left hemicranial headaches with ptosis and binocular diplopia...
May 5, 2017: Head & Neck
https://www.readbyqxmd.com/read/28445472/unsuspected-osteochondroma-like-outgrowths-in-the-cranial-base-of-hereditary-multiple-exostoses-patients-and-modeling-and-treatment-with-a-bmp-antagonist-in-mice
#6
Sayantani Sinha, Christina Mundy, Till Bechtold, Federica Sgariglia, Mazen M Ibrahim, Paul C Billings, Kristen Carroll, Eiki Koyama, Kevin B Jones, Maurizio Pacifici
Hereditary Multiple Exostoses (HME) is a rare pediatric disorder caused by loss-of-function mutations in the genes encoding the heparan sulfate (HS)-synthesizing enzymes EXT1 or EXT2. HME is characterized by formation of cartilaginous outgrowths-called osteochondromas- next to the growth plates of many axial and appendicular skeletal elements. Surprisingly, it is not known whether such tumors also form in endochondral elements of the craniofacial skeleton. Here, we carried out a retrospective analysis of cervical spine MRI and CT scans from 50 consecutive HME patients that included cranial skeletal images...
April 2017: PLoS Genetics
https://www.readbyqxmd.com/read/28430034/um-chor1-establishment-and-characterization-of-the-first-validated-clival-chordoma-cell-line
#7
John Henry Owen, Christine M Komarck, Anthony C Wang, Waleed M Abuzeid, Richard F Keep, Erin L McKean, Stephen Sullivan, Xing Fan, Mark E P Prince
OBJECTIVE Chordomas are rare malignant tumors thought to arise from remnants of the notochord. They can be located anywhere along the axial skeleton but are most commonly found in the clival and sacrococcygeal regions, where the notochord regresses during fetal development. Chordomas are resistant to many current therapies, leaving surgery as the primary method of treatment. Cancer cell lines have been useful for developing new cancer treatments in a laboratory setting that can then be transferred to the clinic, but there are only 4 validated chordoma cell lines available...
April 21, 2017: Journal of Neurosurgery
https://www.readbyqxmd.com/read/28411008/cone-beam-computed-tomography-incidental-findings-of-the-cervical-spine-and-clivus-retrospective-analysis-and-review-of-the-literature
#8
Noura A Alsufyani
OBJECTIVE: The aim of this study was to analyze and describe incidental findings in the cervical spine (C-spine) and the clivus encountered in cone beam computed tomography (CBCT) imaging. The wide range of possible anatomic variants and pathoses is discussed in the context of the medical and dental literature to clarify their radiographic appearance and clinical implications as a guide for the oral and maxillofacial radiologist. STUDY DESIGN: A retrospective analysis of radiographic reports was conducted based on CBCT images from 2 oral and maxillofacial imaging centers...
March 19, 2017: Oral Surgery, Oral Medicine, Oral Pathology and Oral Radiology
https://www.readbyqxmd.com/read/28410953/spontaneous-cerebrospinal-fluid-fistula-in-the-clivus
#9
A Codina Aroca, J R Gras Cabrerizo, M De Juan Delago, H Massegur Solench
INTRODUCTION: Spontaneous cerebrospinal fluid (CSF) fistulas are infrequent and only 10 cases in the literature have been located in the clivus. We describe two new cases of CSF fistulas in this site and review the literature. CASE REPORT: The first patient was a 52-year-old woman referred to our centre for intermittent rhinorrhea that had been diagnosed after an episode of meningitis. The second case was a 69-year-old man who was visited for rhinorrhea of one-year duration; he also developed meningitis during the preoperative study...
April 11, 2017: European Annals of Otorhinolaryngology, Head and Neck Diseases
https://www.readbyqxmd.com/read/28409727/clival-chordomas-considerations-after-16-years-of-endoscopic-endonasal-surgery
#10
Matteo Zoli, Laura Milanese, Rocco Bonfatti, Marco Faustini-Fustini, Gianluca Marucci, Giovanni Tallini, Corrado Zenesini, Carmelo Sturiale, Giorgio Frank, Ernesto Pasquini, Diego Mazzatenta
OBJECTIVE In the past decade, the role of the endoscopic endonasal approach (EEA) has relevantly evolved for skull base tumors. In this study, the authors review their surgical experience with using an EEA in the treatment of clival chordomas, which are deep and infiltrative skull base lesions, and they highlight the advantages and limitations of this ventral approach. METHODS All consecutive cases of chordoma treated with an EEA between 1998 and 2015 at a single institution are included in this study. Preoperative assessment consisted of neuroimaging (MRI and CT with angiography sequences) and endocrinological, neurological, and ophthalmological evaluations, which were repeated 3 months after surgery and annually thereafter...
April 14, 2017: Journal of Neurosurgery
https://www.readbyqxmd.com/read/28402528/macrovascular-decompression-of-the-brainstem-and-cranial-nerves-evolution-of-an-anteromedial-vertebrobasilar-artery-transposition-technique
#11
Omar Choudhri, Ian D Connolly, Michael T Lawton
BACKGROUND: Tortuous and dolichoectatic vertebrobasilar arteries can impinge on the brainstem and cranial nerves to cause compression syndromes. Transposition techniques are often required to decompress the brainstem with dolichoectatic pathology. We describe our evolution of an anteromedial transposition technique and its efficacy in decompressing the brainstem and relieving symptoms. OBJECTIVE: To present the anteromedial vertebrobasilar artery transposition technique for macrovascular decompression of the brainstem and cranial nerves...
April 11, 2017: Neurosurgery
https://www.readbyqxmd.com/read/28362929/metastatic-liposarcoma-of-the-skull-base-a-case-report-and-review-of-literature
#12
Nirmeen Zagzoog, Greta Ra, Alex Koziarz, John Provias, Doron Sommer, Saleh A Almenawer, Kesava Reddy
BACKGROUND AND IMPORTANCE: Myxoid liposarcoma is not an uncommon form of sarcoma. However, it usually affects the lower extremity long bones. Scapular involvement is extremely rare, as is a metastasis to the parasellar region. We present a case of liposarcoma of the skull base originating in the scapular region and metastasizing to the sellar and parasellar regions and provide a review of the pertinent literature. CLINICAL PRESENTATION: A 43-year-old female patient diagnosed with left scapular myxoid liposarcoma was treated with surgical resection...
April 1, 2017: Neurosurgery
https://www.readbyqxmd.com/read/28361020/role-of-dynamic-computed-tomography-scans-in-patients-with-congenital-craniovertebral-junction-malformations
#13
Otávio Turolo da Silva, Enrico Ghizoni, Helder Tedeschi, Andrei Fernandes Joaquim
AIM: To evaluate the role of dynamic computed tomography (CT) scan imaging in diagnosing craniovertebral junction (CVJ) instability in patients with congenital CVJ malformations. METHODS: Patients with symptomatic congenital CVJ malformations who underwent posterior fossa decompression and had a preoperative dynamic CT scan in flexion and extended position were included in this study. Measurements of the following craniometrical parameters were taken in flexed and extended neck position: Atlanto-dental interval (ADI), distance of the odontoid tip to the Chamberlain's line, and the clivus-canal angle (CCA)...
March 18, 2017: World Journal of Orthopedics
https://www.readbyqxmd.com/read/28321374/ectopic-pituitary-adenomas-presenting-as-sphenoid-or-clival-lesions-case-series-and-management-recommendations
#14
Bobby A Tajudeen, Edward C Kuan, Nithin D Adappa, Joseph K Han, Rakesh K Chandra, James N Palmer, David W Kennedy, Marilene B Wang, Jeffrey D Suh
Background An ectopic pituitary adenoma presenting as a clival or sphenoid mass is a rare clinical occurrence that may mislead the clinician and result in unnecessary interventions or potential medicolegal consequences. Here, we present one of the largest multi-institutional case series and review the literature with an emphasis on radiological findings and critical preoperative workup. Methods Retrospective chart review. Results Nine patients were identified with ectopic pituitary adenomas of the sphenoid or clivus...
April 2017: Journal of Neurological Surgery. Part B, Skull Base
https://www.readbyqxmd.com/read/28317556/skull-5-from-dmanisi-descriptive-anatomy-comparative-studies-and-evolutionary-significance
#15
G Philip Rightmire, Marcia S Ponce de León, David Lordkipanidze, Ann Margvelashvili, Christoph P E Zollikofer
A fifth hominin skull (cranium D4500 and mandible D2600) from Dmanisi is massively constructed, with a large face and a very small brain. Traits documented for the first time in a basal member of the Homo clade include the uniquely low ratio of endocranial volume to basicranial width, reduced vertex height, angular vault profile, smooth nasal sill coupled with a long and sloping maxillary clivus, elongated palate, and tall mandibular corpus. The convex clivus and receding symphysis of skull 5 produce a muzzle-like form similar to that of Australopithecus afarensis...
March 2017: Journal of Human Evolution
https://www.readbyqxmd.com/read/28283722/fanconi-anemia-correlating-central-nervous-system-malformations-and-genetic-complementation-groups
#16
Benjamin A Johnson-Tesch, Rakhee S Gawande, Lei Zhang, Margaret L MacMillan, David R Nascene
BACKGROUND: Congenital central nervous system abnormalities in children with Fanconi anemia are poorly characterized, especially with regard to specific genetic complementation groups. OBJECTIVE: To characterize the impact of genetic complementation groups on central nervous system anatomy. MATERIALS AND METHODS: Through chart review we identified 36 patients with Fanconi anemia with available brain MRIs at the University of Minnesota (average age, 11...
March 10, 2017: Pediatric Radiology
https://www.readbyqxmd.com/read/28251542/sellar-and-clival-plasmacytomas-case-series-of-5-patients-with-systematic-review-of-65-published-cases
#17
REVIEW
Jane Lee, Edwin Kulubya, Barry D Pressman, Adam Mamelak, Serguei Bannykh, Gabriel Zada, Odelia Cooper
PURPOSE: Parasellar plasmacytomas are rare tumors localized to the sellar region arising from plasma cells. Knowledge of clinical, imaging, surgical, and pathological characteristics is limited to single case reports. METHODS: A retrospective analysis of five primary cases was conducted, followed by systematic review of English language articles using PubMed in accordance with PRISMA guidelines. RESULTS: Five primary case patients include four men and one woman, ages 60-77, followed up to 3 years...
June 2017: Pituitary
https://www.readbyqxmd.com/read/28242132/abducens-nerve-palsy-due-to-inferior-petrosal-sinus-thrombosis
#18
Shivam Om Mittal, Junaid Siddiqui, Bashar Katirji
Isolated unilateral abducens nerve palsy is usually due to ischemia, trauma or neoplasm. Dorello's canal is the space between the petrous apex and superolateral portion of the clivus, bound superiorly by Gruber's ligament. The abducens nerve travels with inferior petrosal sinus (IPS) though the Dorello's canal before entering the cavernous sinus. A 31-year-old man presented with neck pain, and binocular horizontal diplopia, worse looking towards left and at distance. He had a history of intravenous drug abuse but no history of hypertension or diabetes...
February 24, 2017: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
https://www.readbyqxmd.com/read/28239593/ectopic-esthesioneuroblastoma-of-the-sphenoclivus-a-rare-entity
#19
Farah Dayana Zahedi, Balwant Singh Gendeh, Salina Husain, Ramesh Kumar, Thean Yean Kew
Esthesioneuroblastoma is a rare malignant neoplasm of oflactory neuroepithelium and usually located at the olfactory cleft at superior nasal cavity. Ectopic localization of esthesioneuroblastoma is even rarer and usually posed with a diagnostic dilemma and delay in the diagnosis and management, We report a rare case of ectopic esthesioneuroblastoma of the sphenoclivus with the presentation of intermittent unilateral epistaxis, intermittent intractable headache without anosmia. Nasal endoscopy findings showed a pulsatile mass at the anterior face of the sphenoid sinus with extension posteriorly towards the clivus region and occupying the floor of the sphenoid sinus...
March 2017: Indian Journal of Otolaryngology and Head and Neck Surgery
https://www.readbyqxmd.com/read/28236049/analysis-of-the-venous-channel-within-the-clivus-using-multidetector-computed-tomography-digital-subtraction-venography
#20
Katsuhiro Mizutani, Masahiro Toda, Jun Kurasawa, Takenori Akiyama, Hirokazu Fujiwara, Masahiro Jinzaki, Kazunari Yoshida
PURPOSE: Although neuroradiologists and skull base neurosurgeons are aware of the existence of veins within the clivus, such vessels have seldom been described in the literature. The aim of the present study is to elucidate the detailed venous structure of the clivus. METHODS: Computed tomography digital subtraction venography (CT-DSV) images of 50 unruptured aneurysm cases were examined retrospectively. RESULTS: Eighteen emissary veins were identified in 14 (28...
February 24, 2017: Neuroradiology
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