scholarly journals Extracranial invasion of a recurrent, transformed anaplastic pleomorphic xanthoastrocytoma: a case report

2018 ◽  
Vol 45 (S1) ◽  
pp. S2-S3
Author(s):  
K.D. Langdon ◽  
D. Krivosheya ◽  
M.O. Hebb ◽  
B. Wehrli ◽  
L.C. Ang
Keyword(s):  
Posterior Fossa ◽  
Jugular Foramen ◽  
Jugular Bulb ◽  
Neck Mass ◽  
Pleomorphic Xanthoastrocytoma ◽  
Braf V600e ◽  
Rare Tumour ◽  
Who Grade ◽  
Original Tumour ◽  
The Right

Pleomorphic xanthoastrocytoma (PXA) is a rare tumour comprising <1% of all primary central nervous system tumours and the majority (~98%) occur supratentorially. We report on a 40-year-old female with a past medical history of a rare posterior fossa/cerebellar PXA who presented with a right-sided neck mass, decreased shoulder power and longstanding right tongue deviation with right-sided hemi-atrophy. The patient had prior tumour debulking. Recent MRI demonstrated an enhancing posterior fossa mass extending to the skull base at the jugular foramen and another mass in the upper neck along the jugular bulb with displacement and encasement of the right common carotid artery down to C5. Resection of the neck mass reveals an anaplastic PXA. The tumour has close approximation with adjacent peripheral nerves and is positive in 2 lymph nodes. Comparison with the original tumour molecular and immunohistochemical profiles reveals a conserved BRAF V600E mutation but the transformed malignant glioma now expresses dot-like EMA positivity and ATRX is completely lost (mutated). Transformation of a PXA (WHO Grade II) into an anaplastic PXA (WHO Grade III) has been well documented, but extracranial extension is extraordinarily rare. We report herein the first documented case of a posterior fossa PXA that underwent malignant transformation and extracranial invasion to the parapharyngeal space.

Variations in the Structure of the Jugular Foramen of Human Skull

2013 ◽  
Vol 10 (2) ◽  
pp. 45-49 ◽  
Author(s):  
SM Akram Hossain ◽  
SM Moshadeq Hossain ◽  
Fakhrul Amin Mohammad Hasanul Banna
Keyword(s):  
Internal Jugular Vein ◽  
Jugular Vein ◽  
Jugular Foramen ◽  
Jugular Bulb ◽  
Cross Sectional ◽  
Medical College ◽  
Geographical Variations ◽  
Pass Through ◽  
The Right ◽  
Study Type

Context: The jugular foramen is one of the most fascinating foramen present at the base of the skull attracting the imagination of many Anatomists worldwide as many important structures pass through it, and amongst them the intriguing structure is the internal jugular vein. The shape and size of the jugular foramen is related to the size of the internal jugular vein and the presence or absence of a prominent superior bulb. As most of the textbooks of Anatomy describe that the right jugular foramen is usually larger than the left jugular foramen. Henceforth the present study was undertaken in 55 skulls from the dept. of Anatomy. Measurements were taken with the help of sliding vernier caliper. Study type: Cross-sectional descriptive type. Place and period of study: Department of Anatomy, Rajshahi Medical College, Rajshahi and Pabna Medical College, Pabna from April 2010 to June 2011. Materials and Methods: Total fifty five (55) human adult skulls were collected from the Anatomy department of Rajshahi Medical College, Rajshahi and Pabna Medical College, Pabna at different times of the study period. The study was conducted to observe variations in the structure of the jugular foramen of the human’s skull. Result: Out of 55 skulls (110 foramina) studied, the presence of dome indicating the presence of jugular bulb was found bilaterally in 100% of cases. 58.18% of cases showed that the size of right foramina were larger than the left foramina whereas 20% of cases showed that right foramina were equal to the left and in 21.82% of cases the left foramina were larger than the right side foramina. An important observation in the present study was the presence of either complete or partial septation in the jugular foramen. Conclusion: The findings of the study reveals that there are some differences among some parameters. The variations are might be due to the geographical variations of the skeletons. It needs further study with larger sample size from different geographical areas of Bangladesh. DOI: http://dx.doi.org/10.3329/bja.v10i2.17281 Bangladesh Journal of Anatomy, July 2012, Vol. 10 No. 2 pp 45-49

scholarly journals Imaging and Histopathologic Nuances of Epithelioid Glioblastoma

2018 ◽  
Vol 2018 ◽  
pp. 1-5 ◽  
Author(s):  
Brian H. Le ◽  
Richard A. Close
Keyword(s):  
Mass Effect ◽  
Clonic Seizure ◽  
Cystic Mass ◽  
Braf V600e ◽  
World Health ◽  
Significant Past Medical History ◽  
Who Grade ◽  
Epithelioid Glioblastoma ◽  
Health Organization ◽  
The Right

A 27-year-old male without significant past medical history presented following collapse resulting from a syncopal episode at work. There was an episode of vomiting, and one of tonic-clonic seizure activity, which was spontaneously resolved after approximately one minute. His neurologic exam was nonfocal, with full strength in the bilateral upper and lower extremities, and no sensory deficits were elicited. MRI studies demonstrated a 4.7 cm rim-enhancing cystic mass in the right temporal-parietal region, with resultant mass effect and edema. At surgery, intraoperative pathologic consultation favoured a primary glial neoplasm. Subsequent complete histologic examination on permanent sections confirmed the presence of glioblastoma, with a morphologic pattern and immunohistochemical profile most consistent with epithelioid glioblastoma (WHO grade IV). Epithelioid glioblastoma is a rare, especially aggressive variant of IDH-wildtype glioblastoma, recognized in the 2016 World Health Organization classification. Approximately 50% of such tumors harbour the BRAF V600E mutation, which has also been observed in some melanomas where selective inhibitors have demonstrated a therapeutic role. The especially aggressive behaviour and poor clinical outcome typically observed for this variant of glioblastoma demonstrate the importance of emerging areas relevant to neurooncology, specifically those of proteomic characterization and therapeutic nanomedicine.

The microsurgical anatomy of the jugular foramen

1995 ◽  
Vol 83 (5) ◽  
pp. 903-909 ◽  
Author(s):  
S. Adetokunboh Ayeni ◽  
Kenji Ohata ◽  
Kiyoaki Tanaka ◽  
Akira Hakuba
Keyword(s):  
Infratemporal Fossa ◽  
Cranial Nerves ◽  
Jugular Foramen ◽  
Jugular Bulb ◽  
Microsurgical Anatomy ◽  
Content Type ◽  
Suboccipital Craniectomy ◽  
The Right ◽  
Posterior Meningeal Artery ◽  
Distal Sigmoid

✓ The microsurgical anatomy of the jugular foramen was studied in 10 fixed cadavers, each cadaver consisting of the whole head and neck. Five of the cadavers were injected with latex. The jugular foraminal region was exposed using the infratemporal fossa type A approach of Fisch and Pillsbury in five cadavers (10 sides) and the combined cervical dissection—mastoidectomy—suboccipital craniectomy approach in five cadavers (10 sides). The right foramen was larger than the left in seven cases (70%), equal in two cases (20%), and smaller in one case (10%). The dura covering the intracranial portal of the foramen had two perforations, a smaller anteromedial perforation through which passed the ninth cranial nerve (CN IX), and a larger posterolateral perforation, through which passed the 10th and 11th cranial nerves (CNs X and XI) and the distal sigmoid sinus. The perforations were separated by a fibrous septum in 16 specimens (80%). After exiting the posterior fossa, CNs IX, X, and XI all lay anteromedial to the superior jugular bulb (SJB) within the jugular foramen. The inferior petrosal sinus (IPS) entered the foramen between CNs IX and X in most cases; however, in 10% of our cases it entered the foramen between CNs X and XI, and in 10% it entered the foramen caudal to CN XI. The IPS terminated in the SJB in 90% of our cases; in 40%, the IPS termination consisted of multiple channels draining into both the SJB and internal jugular vein. This study shows that the arrangement of the neurovascular structures within the jugular foramen does not conform to the hitherto widely accepted notion of discrete compartmentalization into an anteromedial pars nervosa containing CN IX and the IPS and a posterolateral pars venosa containing the SJB, CNs X and XI, and the posterior meningeal artery.

scholarly journals Extended Anterolateral Infralabyrinthine Transjugular Approach for Microsurgical Resection of Giant Glomus Vagale Tumor: Operative Video and Technical Nuances

2020 ◽  
Author(s):  
James K. Liu ◽  
Kevin Zhao ◽  
Soly Baredes ◽  
Robert W. Jyung
Keyword(s):  
Skull Base ◽  
Cranial Nerves ◽  
Jugular Foramen ◽  
Neck Mass ◽  
Preoperative Embolization ◽  
Rare Type ◽  
Lower Cranial Nerve ◽  
Microsurgical Resection ◽  
The Right ◽  
Lower Cranial Nerves

AbstractGlomus vagale tumor is a paraganglioma of the vagus nerve. It is a rare type of benign tumor that occupies the head and neck and skull base regions. Patients often present with lower cranial nerve dysfunctions such as difficulty swallowing, tongue weakness, and hoarseness. Surgical treatment can be complex and difficult due to its high vascularity, frequent involvement of lower cranial nerves, and surrounding critical vascular structures. In this operative video, we demonstrate an extended anterolateral infralabyrinthine transjugular approach for microsurgical resection of a giant glomus vagale tumor in a 53-year-old male who presented with an enlarging neck mass, difficulty swallowing, right tongue weakness, and hoarseness. Imaging revealed a giant glomus vagale tumor in the right parapharyngeal space extending into the jugular foramen with occlusion of the internal jugular vein. After preoperative embolization, the patient underwent a near-total resection of the tumor with a small microscopic residual at the pars nervosa. In summary, the extended anterolateral infralabyrinthine transjugular approach is a useful strategy for removal of giant glomus vagale tumors extending into the skull base. The surgical technique and nuances are described in a step-by-step fashion in this illustrative operative video.The link to the video can be found at: https://youtu.be/L0EosQK95LE.

scholarly journals MODL-11. COMPARISON OF HUMAN & MURINE PA/PXA CHARACTERISTICS

2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii413-iii413
Author(s):  
Alexander C Sommerkamp ◽  
Pengbo Sun ◽  
Annika K Wefers ◽  
Britta Ismer ◽  
Kathrin Schramm ◽  
...  
Keyword(s):  
Immune Cell ◽  
Pleomorphic Xanthoastrocytoma ◽  
Braf V600e ◽  
Cell Infiltration ◽  
Low Grade ◽  
Immune Cell Infiltration ◽  
Preclinical Research ◽  
Who Grade ◽  
Pediatric Tumor ◽  
Tumor Types

Abstract Pediatric low-grade gliomas (pLGGs) are the most common brain tumors in children. Despite recent advances in the molecular characterization of this heterogeneous set of tumors, the separation of specific tumor types is still not fully established. Pilocytic astrocytoma (PA; WHO grade I) and pleomorphic xanthoastrocytoma (PXA; WHO grade II) are two pLGG types that can be difficult to distinguish based on histology alone. Even though their clinical course is different, they are often grouped as ‘pLGG’ in clinical trials (and therefore treated similarly). Based on a cohort of 89 human pediatric tumor samples, we show that PAs and PXAs have clearly distinct methylation and transcriptome profiles. The difference in gene expression is mainly caused by cell cycle- and development-associated genes, suggesting a key difference in the regulatory circuits involved in tumor growth. In addition to BRAF V600E, we found NTRK fusions and a previously unknown EGFR:BRAF fusion as mutually exclusive driving events in PXAs. Both tumor types show marked signs of immune cell infiltration, but with significant qualitative differences, which might represent therapeutic vulnerabilities. To pave the way for further research on PA and PXA, we developed corresponding mouse models using the virus-based RCAS system, which allows introduction of an oncogenic driver into immunocompetent mice for molecular and preclinical research. The murine tumors do not only histologically resemble their human counterparts but also show a similar growth behavior. Expression analysis revealed that the murine PXAs have a stronger gene signature of proliferation and immune cell infiltration compared to PAs.

scholarly journals Successful Endovascular Therapy of a Penetrating Zone III Jugular Bulb Injury

2012 ◽  
Vol 18 (2) ◽  
pp. 195-199 ◽  
Author(s):  
K. Yamanaka ◽  
A. Yamamoto ◽  
K. Ishida ◽  
J. Matsuzaki ◽  
T. Ozaki ◽  
...  
Keyword(s):  
Coil Embolization ◽  
Air Embolism ◽  
Optimal Strategies ◽  
Venous Occlusion ◽  
Jugular Foramen ◽  
Jugular Bulb ◽  
Venous Bleeding ◽  
Endovascular Approach ◽  
The Right ◽  
Enhanced Computed Tomography

Penetrating venous injuries via Zone III of the neck extended over jugular bulb are rare. The optimal strategies for these venous injuries are currently unknown because many of the vital structures in this region are poorly accessible to the surgeon and therefore it is difficult to control bleeding. A 76-year-old man got drunk and fell down onto a paper door. The wooden framework of the paper door was broken and got stuck deep in the right side of his neck. Enhanced computed tomography showed the wood stick had penetrated through the right jugular foramen and injured the jugular bulb. We successfully performed right sigmoid and jugular vein occlusion via an endovascular approach using Guglielmi detachable coils at first and then to draw out the wood stick in order to avoid venous bleeding. To our best knowledge, these venous injuries have reported in only four cases. Only one case was performed by endovascular approach using n-butyl cyanoacrylate (NBCA). Coil embolization is much better than NBCA in the light of reducing complications due to adhesion to the inserted wood stick and embolization of unintended vessels. Venous occlusion using coil embolization is the best way to treat a penetrating jugular bulb injury via zone III because of reducing the hemorrhage and air embolism.

P14.74 Grade II and III pleomorfic xanthoastrocitoma: a monocentric, retrospective analysis

2021 ◽  
Vol 23 (Supplement_2) ◽  
pp. ii52-ii52
Author(s):  
P Gaviani ◽  
G Simonetti ◽  
M Farinotti ◽  
V Redaelli ◽  
A Botturi ◽  
...  
Keyword(s):  
Braf Mutation ◽  
Low Frequency ◽  
Pleomorphic Xanthoastrocytoma ◽  
Adult Patients ◽  
Braf V600e ◽  
Treatment Modalities ◽  
Molecular Characteristics ◽  
Who Grade ◽  
Genetic Features ◽  
Grade Ii

Abstract BACKGROUND Pleomorphic xanthoastrocytoma (PXA) represent an astrocytic neoplasm that is typically well circumscribed and can have a relatively favorable prognosis. Tumor progression to anaplastic PXA (WHO grade III), however, is associated with a more aggressive biologic behavior and worse prognosis. The factors that drive anaplastic progression are largely unknown. The activating BRAF mutation is a genetic alteration that could be present in both PXA and anaplastic PXA; Identification of the mutation can be important as BRAF V600E mutant protein could be targeted therapeutically by small molecule inhibitors of both RAF and MEK. The low frequency of these tumors has fostered heterogeneity of therapeutic approaches, particularly in adult patients. We performed a monocentric, retrospective study on adult patients with a PXA diagnosis treated by our Institution, in order to search for markers of diagnosis and prognosis. MATERIAL AND METHODS All consecutive patients older than 18 years with a histological diagnosis of a primary grade II and III pleomorphic xanthoastrocytoma operated and treated by our Institution from 2011 to 2021 were consecutively and retrospectively included in the study. All pathological diagnoses and PXA cases were reviewed according to the WHO classification. Histological and molecular characteristics, including BRAF mutation status, as well as clinical data, treatment modalities, and patient outcome were analyzed and described. RESULTS 21 patients were included, 10 males and 11 females, with a median age of 40 years old (range 18–66). In 10 patients the histology showed an anaplastic PXA and in 11 patients a grade II tumor. Genetic features, including IDH status were available to all patients and BRAF mutation study was available in 8/10 anaplastic PXA. All patients underwent main surgery. Anaplastic features were associated with minimal PFS, whereas BRAF mutation tended to be associated with a better PFS. CONCLUSION Anaplastic features and BRAF mutation can lead to identify subgroups with distinct prognosis. These results should be validated in a larger and prospective study.

Microsurgical Anatomy of the Jugular Process as an Anatomical Landmark to Access the Jugular Foramen: A Cadaveric and Radiological Study

2018 ◽  
Vol 16 (4) ◽  
pp. 486-495 ◽  
Author(s):  
Noritaka Komune ◽  
Satoshi Matsuo ◽  
Koichi Miki ◽  
Ken Matsushima ◽  
Yojiro Akagi ◽  
...  
Keyword(s):  
Jugular Foramen ◽  
Jugular Bulb ◽  
Morphological Features ◽  
Cadaveric Study ◽  
Surgical Approaches ◽  
Microsurgical Anatomy ◽  
Close Relationship ◽  
Superior Surface ◽  
Posterior Condylar ◽  
The Right

Abstract BACKGROUND The jugular process forms the posteroinferior surface of the jugular foramen and is an important structure for surgical approaches to the foramen. However, its morphological features have not been well described in modern texts. OBJECTIVE To elucidate the microsurgical anatomy of the jugular process and examine its morphological features. METHODS Five adult cadaveric specimens were dissected in a cadaveric study, and computed tomography data from 31 heads (62 sides) were examined using OsiriX (Pixmeo SARL, Bernex, Switzerland) to elucidate the morphological features of the jugular process. RESULTS The cadaveric study showed that it has a close relationship with the sigmoid sinus, jugular bulb, rectus capitis lateralis, lateral atlanto-occipital ligament, and lateral and posterior condylar veins. The radiographic study showed that 9/62 sigmoid sinuses protruded inferiorly into the jugular process and that in 5/62 sides, this process was pneumatized. At the entry of the jugular foramen, if the temporal bone has a bulb-type jugular bulb, and if surgery concerns the right side of the head, the superior surface of the jugular process is more likely to be steep. CONCLUSION The jugular process forms the posteroinferior border of the jugular foramen. Resection of the jugular process is a critical step for opening the jugular foramen from the posterior and lateral aspects. Understanding the morphological features of the jugular process, and preoperative and radiographical examination of this process thus help skull base surgeons to access the jugular foramen.

scholarly journals Undifferentiated Nasopharyngeal Carcinoma Presenting with Shoulder Mass

2020 ◽  
Vol 2020 ◽  
pp. 1-3
Author(s):  
Cosphiadi Irawan ◽  
Rahmat Cahyanur ◽  
Reyhan Eddy Yunus
Keyword(s):  
Palliative Chemotherapy ◽  
Nasopharyngeal Cancer ◽  
Bone Tumour ◽  
Undifferentiated Carcinoma ◽  
Neck Mass ◽  
Definitive Treatment ◽  
Unknown Primary ◽  
Common Cancer ◽  
Stage Ivb ◽  
The Right

Nasopharyngeal cancer (NPC) is the most common cancer among head and neck cancer that usually presented with unilateral neck mass. Unusual symptoms of NPC can lead us to diagnosis misleading and delayed definitive treatment. We present a case of NPC with bone metastasis in the shoulder. A 33-year-old female presented with right shoulder mass caused by undifferentiated carcinoma of unknown primary, based on biopsy of shoulder mass. After four months, she was complaining painless neck swelling, headache, and hearing impairment in the left ear. Bone MRI showed malignant bone tumour in the right humerus. Neck CT scan showed mass in the nasopharyngeal and bilateral lymphadenopathy. Biopsy in nasopharyngeal revealed undifferentiated carcinoma of nasopharyngeal cancer (WHO-3 type A). The patient was diagnosed as NPC stage IVb and thus was treated with palliative chemotherapy. After three cycles of cisplatin docetaxel, patient condition improved.

scholarly journals Role of Extradural Clinoidectomy and Optic Unroofing in Resection of an Anterior Clinoidal Meningioma with Encasement of the Internal Carotid Artery and Its Branches

2021 ◽  
Author(s):  
Sima Sayyahmelli ◽  
Zhaoliang Sun ◽  
Emel Avci ◽  
Mustafa K. Başkaya
Keyword(s):  
Carotid Artery ◽  
Internal Carotid Artery ◽  
Skull Base ◽  
Vision Loss ◽  
Internal Carotid ◽  
Extent Of Resection ◽  
World Health ◽  
Who Grade ◽  
The Right ◽  
Supraclinoid Internal Carotid Artery

AbstractAnterior clinoidal meningiomas (ACMs) remain a major neurosurgical challenge. The skull base techniques, including extradural clinoidectomy and optic unroofing performed at the early stage of surgery, provide advantages for improving the extent of resection, and thereby enhancing overall outcome, and particularly visual function. Additionally, when the anterior clinoidal meningiomas encase neurovascular structures, particularly the supraclinoid internal carotid artery and its branches, this further increases morbidity and decreases the extent of resection. Although it might be possible to remove the tumor from the artery wall despite complete encasement or narrowing, the decision of whether the tumor can be safely separated from the arterial wall ultimately must be made intraoperatively.The patient is a 75-year-old woman with right-sided progressive vision loss. In the neurological examination, she only had light perception in the right eye without any visual acuity or peripheral loss in the left eye. MRI showed a homogeneously enhancing right-sided anterior clinoidal mass with encasing and narrowing of the supraclinoid internal carotid artery (ICA). Computed tomography (CT) angiography showed a mild narrowing of the right supraclinoid ICA with associated a 360-degree encasement. The decision was made to proceed using a pterional approach with extradural anterior clinoidectomy and optic unroofing. The surgery and postoperative course were uneventful. MRI confirmed gross total resection (Figs. 1 and 2). The histopathology was a meningothelial meningioma, World Health Organization (WHO) grade I. The patient continues to do well without any recurrence and has shown improved vision at 15-month follow-up.This video demonstrates important steps of the microsurgical skull base techniques for resection of these challenging tumors.The link to the video can be found at https://youtu.be/vt3o1c2o8Z0

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