scholarly journals Neuroendocrine Carcinoma of the Jugular Foramen

2008 ◽  
Vol 87 (2) ◽  
pp. 86-91 ◽  
Author(s):  
John P. Leonetti ◽  
Mobeen A. Shirazi ◽  
Sam Marzo ◽  
Douglas Anderson

We describe what might have been the first reported case of a neuroendocrine carcinoma of the jugular foramen. A 50-year-old woman presented with progressive left-sided sensorineural hearing loss, vertigo, pulsatile tinnitus, headaches, and ataxia. Magnetic resonance imaging revealed a 4-cm left-sided jugular foramen tumor. The patient underwent near-total resection of the tumor. Despite lower cranial nerve preservation, postoperative paralysis of cranial nerves IX and X occurred, and vocal fold medialization was performed 5 days later. The final pathologic diagnosis was neuroendocrine carcinoma. The patient was treated with concurrent chemotherapy and intensity-modulated radiation therapy. This article will discuss the pathologic features and the management of jugular foramen tumors, along with the differential diagnosis of these rare tumors.

Neurosurgery ◽  
2007 ◽  
Vol 61 (1) ◽  
pp. 76-81 ◽  
Author(s):  
Juan J. Martin ◽  
Douglas Kondziolka ◽  
John C. Flickinger ◽  
David Mathieu ◽  
Ajay Niranjan ◽  
...  

Abstract OBJECTIVE Jugular foramen region schwannomas are rare intracranial tumors that usually present with multiple lower cranial nerve deficits. For some patients, complete surgical resection is possible but may be associated with significant morbidity. Stereotactic radiosurgery is a minimally invasive alternative or adjunct to microsurgery for such tumors. We reviewed our clinical and imaging outcomes after patients underwent gamma knife radiosurgery for management of jugular foramen schwannomas. METHODS Thirty-four patients with 35 tumors (one patient had bilateral tumors) underwent radiosurgery between May 1990 and December 2005. Twenty-two patients had previous microsurgical resection and all patients experienced various cranial neuropathies. A median of six isocenters were used. Median marginal and maximum doses were 14 and 28 Gy, respectively. RESULTS None of the patients were lost to evaluation and the mean duration of follow-up was 83 months. Tumors regressed in 17 patients, remained stable in 16, and progressed in two. Five- and 10-year actuarial control rates were 97 and 94%, respectively. Preexisting cranial neuropathies improved in 20% and remained stable in 77% after radiosurgery. One patient worsened. The function of all previous intact nerves was preserved after radiosurgery. CONCLUSION Stereotactic radiosurgery proved to be a safe and effective management for newly diagnosed or residual jugular foramen schwannomas. Long-term tumor control rates and stability or improvement in cranial nerve function was confirmed.


Neurosurgery ◽  
2012 ◽  
Vol 72 (1) ◽  
pp. 42-46 ◽  
Author(s):  
Cara L. Sedney ◽  
Yoichi Nonaka ◽  
Ketan R. Bulsara ◽  
Takanori Fukushima

Abstract BACKGROUND: Jugular foramen schwannomas are uncommon and surgically challenging lesions. OBJECTIVE: To determine the importance of surgical technique on morbidity and recurrence of jugular foramen schwannomas. METHODS: A retrospective review and case-control analysis of a single-senior-surgeon series of 81 patients with surgically treated jugular foramen schwannomas was performed, focusing on operative technique. Patients undergoing an aggressive, total tumor resection (series 1) were compared with those undergoing more conservative resection focusing on preserving the pars nervosa (series 2). RESULTS: There was a statistically significant (P = .04) decrease in permanent deficits of the cranial nerve 9/10 complex with a conservative technique. Recurrence was seen in 3 patients (5.7%) in series 1 and in 3 patients (10.7%) in series 2 (P = .36). Recurrence was treated with reoperation in 1 patient, radiation in 1 patient, and observation in the others. CONCLUSION: Although radical gross total resection is desirable, it is not optimal for cranial nerve preservation in patients with jugular foramen schwannomas. A more conservative approach resulted in a statistically significant decrease in lower cranial nerve deficits. There was a nonstatistically significant trend toward increasing recurrence, which may be treated with multiple modality therapy in the modern era.


2021 ◽  
Author(s):  
Cristina Toledo-Gotor ◽  
Nerea Gorría ◽  
Miren Oscoz ◽  
Katia Llano ◽  
Pablo la Fuente Rodríguez-de ◽  
...  

Abstract Background Multiple lower cranial nerve palsies have been attributed to occipital condyle fractures in older children and adults, but no clinical details of other possible mechanisms have been described in infants. Case Report A 33-month-old boy suffered blunt head trauma. A bilateral skull base fracture was diagnosed, with favorable outcome during the first days after trauma. On the sixth day, the patient began to refuse drinking and developed hoarseness. Physical examination and additional investigations revealed paralysis of left VII, IX, X, and XI cranial nerves. A follow-up computed tomography (CT) scan disclosed a left petrous bone fracture involving the lateral margin of the jugular foramen, and a cranial magnetic resonance imaging (MRI) study showed a left cerebellar tonsil contusion. He improved after methylprednisolone was started. Three months later, he was asymptomatic, although mild weakness and atrophy of the left sternocleidomastoid and trapezius muscles remained 1 year later. Discussion A posttraumatic “jugular foramen syndrome” is rare in children, but it has been reported shortly after occipital condyle fracture, affecting mainly IX, X, and XI cranial nerves. In this toddler, delayed symptoms appeared with unilateral involvement. While an occipital fracture was ruled out, neuroimaging findings suggest the hypothesis of a focal contusion as a consequence of a coup-contrecoup injury. Conclusion This exceptional case highlights the importance of gathering physical examination, anatomical correlation, and neuroimaging to yield a diagnosis.


2012 ◽  
Vol 18 (2) ◽  
pp. 183-192
Author(s):  
Forhad H Chowdhury ◽  
Mohammod R Haque ◽  
Mahmudul Hasan

Introduction: Jugular foramen tumor is a rare tumor. Jugular foramen schwannoma is rarer. This type of tumor may present in combination of different cranial nerve palsies in the form of different syndromes or may also be diagnosed incidentally. Management of such tumor is not straight forward.Case reports: Two young male presented with headache, vomiting, gait instability, right sided hearing loss. Clinically they had different cranial nerves dysfunction. Imaging showed jugular foramen tumor extending from posterior fossa to almost common carotid bifurcation at neck in both cases. Near total microsurgical excisions of the tumor were done through retrosigmoid suboccipital plus transmastoid high cervical approach with facial nerve mobilization in one case and without mobilization in other case. In case 1 combination of lower cranial nerve palsies is unique with inclusion of VIII nerve and that does not belong to any of the jugular foramen syndromes (i.e. Vernet, Collet- Sicard, Villaret, Tapia, Schmidt, and Jackson). Here IX, X, XI, XII and VIII cranial palsies was present (i.e.Collet-Sicard syndrome plus VIII nerve syndrome!). In the second case there was IX & X dysfunction with VIII dysfunction. We also went through the short review of the literature here DOI: http://dx.doi.org/10.3329/bjo.v18i2.12015 Bangladesh J Otorhinolaryngol 2012; 18(2): 183-192


1984 ◽  
Vol 60 (5) ◽  
pp. 1045-1053 ◽  
Author(s):  
Andrew H. Kaye ◽  
Joseph F. Hahn ◽  
Sam E. Kinney ◽  
Russell W. Hardy ◽  
Janet W. Bay

✓ Thirteen patients with schwannomas of the jugular foramen were operated on at the Cleveland Clinic between 1974 and 1983. The authors' experience in managing these rare tumors is presented. Three major growth patterns of jugular foramen schwannoma were seen, and it is postulated that the position of the tumor depends on its point of origin from the nerves as they pass through the pars nervosa of the jugular foramen. The more distal lesions will expand inferiorly out of the base of the skull, and the more proximal lesions will enlarge into the posterior fossa. Tumors in the mid region will tend to expand primarily into bone. The schwannoma was primarily intracranial in six patients. In five patients the tumor expanded the bone at the base of the skull, with only a small intracranial component, and in two patients the tumor was primarily extracranial, with a small extension into the bone or posterior fossa. The presentation of the patients varied according to the tumor growth pattern. Deafness, vertigo, and ataxia were present in all patients with a major intracranial component, and in most of these there were only minimal deficits of the jugular foramen nerves. By contrast, lower cranial nerve involvement, including hoarseness and weakness of the trapezius and sternocleidomastoid muscles, occurred in patients in whom the tumor was primarily within the bone or extracranial. Three of the five patients with the major component of the schwannoma within the bone also had deafness. Symptomatic history was longest in those with tumor mainly involving the bone at the base of the skull, and shortest in patients with entirely extracranial tumor. Surgical resection was accomplished with a joint neurosurgical-otological approach, usually combining a posterior fossa exploration with either a translabyrinthine transcochlear or infralabyrinthine procedure. The exact nature of the operation depended upon the presence of intracranial tumor and on the extent of bone or extracranial involvement. Total excision was performed in all cases. There was no operative mortality, and surgery resulted in loss of function of the ninth, 10th, and 11th cranial nerves in most patients. The major postoperative morbidity consisted of swallowing difficulties and sputum aspiration.


Author(s):  
Gregg MacLean ◽  
Alan Guberman ◽  
Antonio Giulivi

ABSTRACT:Dysarthria, dysphagia and repeated aspiration in a 54-year-old woman diagnosed and treated for myasthenia gravis 7 years earlier were initially thought to represent a late exacerbation of myasthenia. A cervical mass invading the jugular foramen and causing multiple lower cranial nerve palsies was biopsied and found to represent invasive ectopic thymoma.


Author(s):  
James K. Liu ◽  
Kevin Zhao ◽  
Soly Baredes ◽  
Robert W. Jyung

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.


Author(s):  
Pamela Shaw ◽  
David Hilton-Jones

Disorders affecting the lower cranial nerves – V (trigeminal), VII (facial), IX (glossopharyngeal), X (vagus), XI (accessory) and XII (hypoglossal) – are discussed in the first part of this chapter. The clinical neuroanatomy of each nerve is described in detail, as are disorders – often in the form of lesions – for each nerve.Trigeminal nerve function may be affected by supranuclear, nuclear, or peripheral lesions. Because of the wide anatomical distribution of the components of the trigeminal nerve, complete interruption of both the motor and sensory parts is rarely observed in practice. However, partial involvement of the trigeminal nerve, particularly the sensory component, is relatively common, the main symptoms being numbness and pain. Reactivation of herpes zoster in the trigeminal nerve (shingles) can cause pain and a rash. Trigeminal neuralgia and sensory neuropathy are also discussed.Other disorders of the lower cranial nerves include Bell’s palsy, hemifacial spasm and glossopharyngeal neuralgia. Cavernous sinus, Tolosa–Hunt syndrome, jugular foramen syndrome and polyneuritis cranialis are caused by the involvement of more than one lower cranial nerve.Difficulty in swallowing, or dysphagia, is a common neurological problem and the most important consequences include aspiration and malnutrition (Wiles 1991). The process of swallowing is a complex neuromuscular activity, which allows the safe transport of material from the mouth to the stomach for digestion, without compromising the airway. It involves the synergistic action of at least 32 pairs of muscles and depends on the integrity of sensory and motor pathways of several cranial nerves; V, VII, IX, X, and XII. In neurological practice dysphagia is most often seen in association with other, obvious, neurological problems. Apart from in oculopharyngeal muscular dystrophy, it is relatively rare as a sole presenting symptom although occasionally this is seen in motor neurone disease, myasthenia gravis, and inclusion body myositis. Conversely, in general medical practice, there are many mechanical or structural disorders which may have dysphagia as the presenting feature. In some of the disorders, notably motor neurone disease, both upper and lower motor neurone dysfunction may contribute to the dysphagia. Once dysphagia has been identified as a real or potential problem, the patient should undergo expert evaluation by a clinician and a speech therapist, prior to any attempt at feeding. Videofluoroscopy may be required. If there is any doubt it is best to achieve adequate nutrition through the use of a fine-bore nasogastric tube and to periodically reassess swallowing. Anticholinergic drugs may be helpful to reduce problems with excess saliva and drooling that occur in patients with neurological dysphagia, and a portable suction apparatus may be helpful. Difficulty in clearing secretions from the throat may be helped by the administration of a mucolytic agent such as carbocisteine or provision of a cough assist device.


2020 ◽  
Vol 12 (3) ◽  
pp. 136-139
Author(s):  
Eric Tamrazian ◽  
Bijal Mehta

The jugular foramen and the hypoglossal canal are both apertures located at the base of the skull. Multiple lower cranial nerve palsies tend to occur with injuries to these structures. The pattern of injuries tend to correlate with the combination of nerves damaged. Case Report: A 28-year-old male was involved in an AVP injury while crossing the highway. Exam showed a GCS of 15 AAOx3, with dysphagia, tongue deviation to the right, uvula deviation to the left and a depressed palate. Initial imaging showed B/L frontal traumatic Sub-Arachnoid Hemorrhages (tSAH), Left Frontal Epidural Hematoma and a Basilar Skull Fracture. On second look by a trained Neuroradiologist c At 3 month follow up, patient’s tongue normalized to midline and his dysphagia resolved. Discussion: Collette-Sicard syndrome is a rare condition/syndrome characterized by unilateral palsy of CN: IX, X, XII. This condition has been rarely described as a consequence of blunt head trauma. In most cases, the condition is self-limiting with patients regaining most to all of their neurological functions within 6 months. Nerve traction injuries and soft tissue edema compressing the cranial nerves are the leading two hypothesis. In conclusion, injuries with focal neurological deficits which were not apparent on initial imaging should be reviewed by relevant experts with concomitant knowledge of the patient’s history.


2011 ◽  
Vol 3 (1) ◽  
pp. 15-23 ◽  
Author(s):  
C Rayappa

ABSTRACT Jugular foramen tumors are rare cranial base lesions that present diagnostic and management difficulties. Paragangliomas were the most frequent lesions, followed by schwannomas and meningiomas. These tumors have characteristic radiological features. Radical resection of these tumors with preservation of the lower cranial nerves is the treatment of choice. Despite the advances in skull base surgery, new postoperative lower cranial nerve deficits still represent a challenge.


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