Jugulotympanic paraganglioma: A cause of sequential cranial nerve fallout and obstructive hydrocephalus

Abstract

A 46-year-old male presented with a longstanding history of a sequential cranial nerve fallout. Following extensive imaging which included contrast enhanced computed tomography, computed tomography angiography, and diagnostic digital subtraction angiography, a diagnosis of jugulotympanic paraganglioma was made. The tumour was found to be locally invasive, encapsulating major neurovascular structures and ultimately inaccessible. Furthermore, the clinical presentation was complicated by acute obstructive hydrocephalus secondary to tumour mass effect. Given the rarity of the condition and unusually extensive presentation, neurosurgery alongside a multidisciplinary team collaborated to formulate a management plan aimed at maximizing functional outcome and minimizing intraoperative morbidity. A decision was made to perform palliative surgical debulking procedure followed by multidisciplinary care and rehabilitation. The patient demised in the care of his family prior to any further intervention.

Main article text

Background

Jugulotympanic paraganglioma found within and around the jugular foramen of the skull is considered a generally benign neoplasm that falls under the category of head and neck paragangliomas.(1) Developing from neural crest derivatives, paragangliomas can be divided into two types: sympathetic and parasympathetic, with jugulotympanic paraganglioma being of the parasympathetic type. Initially described in 1945 by Rosenwasser, this well differentiated, and highly vascular tumour is still known to be rare.(2) There is an estimated annual incidence of 0.07 cases per 100 000 people per year.(2) Given its rarity, research regarding glomus jugulotympanic paragangliomas remains critical in promoting awareness that allows for early diagnosis as well as to better establish appropriate and standardised treatment guidelines.

Although metastasis of the lesion is exceedingly uncommon, local invasion can be aggressive with a long-standing history of progressive cranial nerve fallout and auditory symptoms due to compression and mass effect. The authors report a case of jugulotympanic paraganglioma in a 46-year-old male presenting to the Emergency Department with a chronic history of neurological symptoms and worsening cranial nerve deficits.

The patient reported progressive hearing loss that was more pronounced in his left ear compared to his right, a declining sense of taste and smell, and deteriorating eyesight which had progressed to bilateral blindness over 2 weeks. He also complained of dysphagia and worsening neck pain.

Physical examination displayed left sided facial paralysis, ptosis and exophthalmos of the left eye with associated neck stiffness and sialorrhea. Neurological examination revealed multiple left sided cranial nerve palsies including; cranial nerves VI, VII, VIII, IX, X, and XI. Additionally, the patient had a left hemiparesis.

Contrast-enhanced computerized tomography (CT) scan of the brain reported the presence of an extra axial mass with its epicenter at the left jugular foramen, measuring 5.9 cm × 5.2 cm × 7.2 cm in size. The lesion was noted as being avidly enhancing with lobulated margins and intralesional calcifications. Permeative destruction of surrounding bone and left cavernous sinus invasion was seen, as well as encasement of the contents of the left temporal bone foramina. Mass effect resulting in complete attenuation of basal cisterns and partial attenuation of the fourth ventricle was reported, with subsequent development of upstream hydrocephalus and transependymal seepage. These radiological findings suggested a locally aggressive left jugular foramen tumour complicated by acute obstructive hydrocephalus, with the primary diagnosis of a jugulotympanic paraganglioma. The most common differential diagnoses include jugular schwannoma, neurofibroma, meningioma and metastatic disease.(3)

CT angiogram performed showed a hypervascular, locally aggressive mass within the left jugular foramen, with complete encasement of the left internal carotid artery in keeping with a jugulotympanic paraganglioma. Further investigation with magnetic resonance imaging was advised. The patient was subsequently transferred to the neurosurgical department so that definitive interdisciplinary and multi-specialty management could be implemented. Given the extensive spread of the tumour and involvement of vital cranial structures, complete surgical excision was deemed not possible, and options involving surgical debulking were discussed with the patient and his family. Unfortunately, the patient demised before any further imaging or surgical intervention could be conducted.

Figure 1:

CT Brain illustrating the marked hydrocephalus.

Figure 2:

CT Brain showing the extent of the tumour (red arrow) arising from the jugular foramen invading the posterior fossa, causing local bony destruction, with associated tumour calcifications (yellow arrow heads).

Figure 3:

CT Angiography Brain illustrating the tumour mass (asterisk) with lobulated margins (yellow arrowheads) encasing the left internal carotid artery (red arrow) with the right internal carotid artery shown by the white arrow.

Discussion

First described by Rosenwasser in 1945 as “a pathologic curiosity”, Jugulotympanic paraganglioma is a benign, slow-growing paraganglioma originating in the jugular fossa of the temporal bone.(2) The incidence is reportedly 1 in 1.3 million of the population, making it exceedingly rare.(1)

Jugulotympanic paraganglioma arises from embryonic neural crest cells. It has a strong female predominance and most commonly presents in the 5th decade of life. 80% are sporadic, with the remainder having an autosomal dominant hereditary component. Hereditary tumours tend to present earlier and with a higher rate of multicentricity.(4) Current literature estimates 3%–10% of jugulotympanic paragangliomas to be malignant and 1-7% functional (catecholamine, dopamine or noradrenaline secreting).(4) Jugulotympanic paraganglioma typically derives blood supply from the ascending pharyngeal, the external carotid or vertebral arteries.(3)

The most common presenting symptoms are hearing loss, pulsatile tinnitus and neurological fallout of cranial nerves VII, IX, X, XI. Signs include a pulsating mass typically described as ‘the rising sun’ posterior to the tympanic membrane, associated conductive hearing loss, otorrhoea, mastoid bruit and ‘Jugular foramen syndrome,’ which is an isolated paresis of CN IX-X.(5) A hormonally active tumour should be suspected when features such as palpitations, hypertension and hot flushes are present.

Thorough imaging is quintessential to management planning of jugulotympanic paraganglioma. X-ray features include mastoid sclerosis, widening of the jugular foramen and erosion of the petrous temporal bone.(1) The gold standard, however, is a combination of CT and MRI of the brain and skull. CT may show a moth-eaten appearance, typical of bony erosion. CT Angiography is useful to depict the feeding vessels for the purposes of embolisation. MRI further demonstrates tumour vascularity, neural extension and multicentricity.(5)

The two main classifications are displayed in the tables below. Both the classifications use size of tumour and anatomical location as differentiating factors in clinical staging.

Table 1:

Fisch Classification

Class	Description
A	Limited to middle ear
B	Limited to middle ear or mastoid, without involvement of the infralabyrinthine space of the temporal bone
C	Involving the infralabyrinthine and apical space of the temporal bone with extension into the apex
D1	Intracranial extension 2cm in diameter
D2	Intracranial extension >2cm in diameter

Table 2:

Glasscock-Jackson Classification

Grade	Description
I	Involving jugular bulb, middle ear and mastoid
II	Extending under internal auditory canal ± intracranial extension
III	Extending into petrous apex ± intracranial extension
IV	Extending beyond petrous apex into clivus or infratemporal fossa  intracranial extension

The management of jugulotympanic paraganglioma remains a clinical dilemma. The current literature recommends an individualised, multidisciplinary approach to each case. Preoperative embolisation and surgical resection, external beam radiotherapy and stereotactic radiosurgery have been used, either in isolation, or in combination to manage jugulotympanic paraganglioma. Observation and monitoring with serial brain MRI is plausible given the reported growth rate of approximately 1mm annually and that 65% of tumours ‘remained stable and sometimes regressed in size’ on long term follow up.(6)

Given the enduring course of disease, long-term, and possibly lifelong, follow up and rehabilitation is necessary.

Conclusion

Jugulotympanic paraganglioma is a rare paraganglioma of the skull base. Although typically benign, the tumour has a high propensity to be locally invasive and often presents as a collection of auditory symptoms and cranial nerve palsies. Given the proximity of major neurovascular structures, there is no recommended guideline for management. Patients are managed on a case-by-case basis. Multidisciplinary team involvement and long-term follow up are common practice in all scenarios.

References

1. Glomus Tumor (Glomus Jugulare, Glomus Tympanicum, Glomus Vagale). Encyclopedia of otolaryngology, head and neck surgery. Berlin: Springer; 2013: 1029–1029. doi.org/10.1007/978-3-642-23499-6_100430.

2. H. Rosenwasser Glomus jugulare tumors: I. Historical background. Arch Otolaryngol Head Neck Surg. 1968; 88(1): 1–40. doi:10.1001/archotol.1968.00770010005003.

3. R Fernández-de Thomas, O. De Jesus Glomus Jugulare [Internet]. Available from: https://www.statpearls.com/ [cited 3.07.23].

4. R Ramina, JJ Maniglia, YB Fernandes, J. Paschoal Jugular foramen tumors: diagnosis and treatment. Skull Base. 2005; 15(S 2). doi: 10.1055/s-2005-916577.

5. R Rafique, M Afzal, H Sarwar, 2017. Glomus jugulare tumor: a case report and literature review. Pak Postgrad Med J. 2018; 28(3):104–106. doi: 10.51642/ppmj.v28i3.79.

6. S Shyam Kumar, A. Thakar Complex glomus jugulare tumor: management issues. Indian J Otolaryngol Head Neck Surg. 2013; 65(S3):676–681. doi:10.1007/s12070-011-0399-z.