Elusive Neck Carcinoma Simulating Idiopathic Intracranial Hypertension Due to Jugular Venous Outflow Obstruction - Case Report

ABSTRACT

Few cases have been reported of extra-cranial tumours in the neck causing intracranial hypertension due to jugular vein compression and consequent outflow obstruction. We present a case of a patient presenting with transient vision loss due to intracranial hypertension of unidentifiable cause on initial imaging workup. Upon further evaluation, the patient was found to have a neck tumour compressing the right jugular vein with stenosis of the ipsilateral transverse sinus – both contributing to his intracranial hypertension. Atypical patients presenting with symptoms concerning for intracranial hypertension may benefit from imaging below the level of the head to evaluate for extra-cranial causes.

Introduction

Idiopathic intracranial hypertension (IIH) typically presents with headache and visual changes and is defined by increased intracranial pressure without an identifiable radiological cause.^1,2 IIH classically presents in obese females of child-bearing age. Initial imaging workup for IIH should include magnetic resonance (MR) imaging of the brain and computed tomography (CT) or MR venography. Stenosis or thrombosis of the dural venous sinuses can simulate IIH, and venography is diagnostic for these conditions. We present a case of a patient presenting with transient vision loss due to intracranial hypertension of unidentifiable cause on initial imaging workup. Upon further evaluation the patient was found to have a neck tumour compressing the right jugular vein with stenosis of the ipsilateral transverse sinus.

Case presentation

A 41-year-old male presented with 2 month history of transient vision loss upon standing in his left eye and frequent headaches. Funduscopic examination revealed asymmetrical papilloedema with substantial haemorrhagic oedema in the left eye. He had a normal body mass index (BMI) and he did not regularly follow with a primary care physician. Due to concern for intracranial hypertension MR imaging of the brain and MR venography of the head were requested. The brain imaging found mild distention of the optic nerve sheaths and no other abnormalities. The venography revealed right transverse sinus stenosis that was not noted until the imaging was re-examined due to significantly worsened stenosis on repeat imaging (Figure 1a,b). The opening pressure on lumbar puncture was elevated at 48 cm of cerebrospinal fluid (CSF) with normal cytology. He was started on acetazolamide for symptomatic treatment.

Figure 1.

(a) Magnetic resonance venography (MRV) (sagittal view) of the head at our patient’s initial presentation demonstrating mild hypoplasia of the right transverse sinus (white arrow). (b) Repeat MRV of the head 23 months later showing worsening of the right transverse sinus stenosis with possible occlusion (white arrow).

At a return visit, 2 days later, he reported worsened positional vision loss with left eye floaters and pulsatile tinnitus. Over the following week he progressed to similar visual symptoms in his right eye and new neck pain radiating up to the occiput. MR imaging of the cervical spine was reported as showing no abnormalities. Due to the rapid symptom progression a non-programmable lumbo-peritoneal shunt was sited, which completely resolved his visual symptoms and headache.

However, his neck pain worsened in the month following shunt placement and a mass appeared behind his right ear. CT scanning of the neck revealed an ill-defined mass measuring 3.6 × 2.2 x 3.1 cm in the right sternocleidomastoid with partial occlusion of the jugular vein (Figure 2a,b). The previous MR imaging was reviewed revealing the mass was present but had not been appreciated on the initial read (Figure 2a). Biopsy of the mass revealed metastatic p16 positive squamous cell carcinoma with the primary tumour suspected to have arisen from the tonsils.

Figure 2.

(a) Axial T2-weighted magnetic resonance imaging of the cervical spine without contrast showing the tumour on the right side at the level of the cervical spine (white arrow). (b) Coronal computed tomography scan of the neck with contrast 4 months after presentation showing progression of the tumour (white arrow).

He completed a course of chemotherapy and radiation but returned 6 months after shunt placement with recurrent transient positional visual loss, pulsatile tinnitus, headaches, and right facial paraesthesia. Funduscopic examination revealed moderate-severe papilloedema bilaterally. He was restarted on acetazolamide and evaluated by neurosurgery with shunt series x-rays, which revealed an intact shunt device. A physical examination of the shunt revealed functional pumping capabilities and the shunt was at the appropriate flow setting. The symptoms were controlled on acetazolamide for the next 16 months until they abruptly returned with fever, shortness of breath, and a left afferent pupillary defect 23 months after the initial presentation. Repeat MR imaging and venography revealed progressive right transverse sinus stenosis with possible occlusion, likely causing his worsening intracranial pressure (Figure 1b).

Unfortunately, his clinical condition rapidly declined and imaging revealed bilateral pulmonary nodules suggestive of metastatic disease. He underwent palliative treatment and died 2 months later.

Discussion

The pathophysiology of IIH is largely unknown, however theories include CSF hyper-secretion, CSF outflow obstruction, and venous sinus stenosis. Several cases have been reported of patients with neoplastic processes within the cranium including metastatic renal cell carcinoma, prostate carcinoma, Ewing sarcoma, plasmacytoma, and cholesteatoma that were presumed to be due to IIH on the initial workup, but were found to be due to neoplastic processes causing sinus compression on further investigation.^3–6 Ten cases have been reported of jugular bulb paragangliomas or postoperative glomus jugulare tumour resection causing jugular venous outflow obstruction.^7–12 These patients presented similarly to ours but with imaging revealing either unilateral or bilateral causes of intracranial hypertension, unlike our patient whose extra-cranial cause of intracranial hypertension was initially not appreciated on imaging. Only a few cases have been reported of tumours outside of the head causing intracranial hypertension through jugular venous outflow obstruction, including one case of a child with a hypoplastic transverse sinus with a retropharyngeal rhabdomyosarcoma compressing his primary outflow tract on the contralateral side.^13–15

Stenosis of the transverse or other venous sinuses has been found in over 90% of patients with IIH and is commonly bilateral.^16,17 Venous sinus stenosis can be a result of intracranial hypertension or extrinsic compression from tumours. It is unclear if increased intracranial pressure causes stenosis, or if stenosis leads to intracranial hypertension; one theory proposes a positive feedback loop of intracranial hypertension results from compressive stenosis in vulnerable portions of sinuses, which worsens CSF reabsorption.¹⁸ Alternately, one study found that unilateral congenital transverse sinus hypoplasia is seen in up to 33% the general population, which causes narrowed sinuses that mimic stenosis.¹⁹ Our patient had pre-existing right transverse sinus narrowing, either from a hypoplastic sinus or intracranial hypertension from the tumour that caused stenosis of the sinuses, which worsened on repeat imaging over 2 years (Figure 1a,b). The patient’s primary jugular venous outflow tract on the right side was compressed by the tumour. Figure 3a shows a patent right jugular vein at the patient’s initial imaging; whereas Figure 3b shows compression of the right jugular vein several months later due to tumour growth. Alternately, there could have been stenosis of the jugular vein that was not captured on the initial MR venography due to the more rostral location of the tumour. The combination of both compression of the right jugular venous outflow tract and worsening of the right transverse sinus stenosis contributed to the patient’s intracranial hypertension.

Figure 3.

(a) Axial T1-weighted magnetic resonance venography at presentation showing a patent jugular vein (white arrow). (b) Axial computed tomography scan of the neck with contrast 4 months after presentation showing compression of the equivalent region of the jugular vein (white arrow).

Conclusion

Initial workup for patients with intracranial hypertension typically includes MR imaging of the brain followed by MR venography and lumbar puncture with CSF analysis. Subtle findings on MR imaging may be identified in IIH including globe flattening and optic nerve head protrusion.²⁰ Patients with atypical presentations, such as males with a normal BMI as in our case, with no identifiable cause on initial workup may need to be considered for imaging below the level of the head. Our patient did initially have an MR imaging of the cervical spine in which the lesion was not appreciated. Further evaluation may also include additional CSF studies including repeat cytology to assist in diagnosing intracranial pathology, such as neoplastic meningitis. Physicians should have a high index of suspicion for patients with an atypical presentation of IIH because delayed treatment may have serious consequences including permanent vision loss in up to one-fourth of patients.²¹ Treatment of identifiable tumours in the head or neck with medical or surgical management may provide symptomatic relief or prevent progression of serious complications from intracranial hypertension.

Funding Statement

The authors reported there is no funding associated with the work featured in this article.

Disclosure statement

No potential conflict of interest was reported by the authors.

Patient consent

The patient consented to publication of the case and accompanying images in writing.

Previous presentation

The details were presented as a poster at the Women in Ophthalmology Summer Symposium on August 21-23, 2020, which was a virtual meeting.