Glomus jugulotympanicum paraganglioma

Case contributed by Dr Mohamed Mahmoud Elthokapy

Presentation

Long date progressive dizziness, pulsatile tinnitus. Mixed hearing loss. Reddish lesion bulging into the external auditory canal.

Patient Data

Age: 35 years
Gender: Female

MRI with contrast

MRI

A large right skull base extra-axial infiltrative soft tissue mass lesion is seen implicating the right middle cranial fossa, petrous bone, and cerebellopontine angle showing multiple small flow voids, giving the tumor the characteristic “salt and pepper” appearance with vivid enhancement after post-contrast series, It is seen obliterating the inner and middle ears structures, Bony invasion is also demonstrated, with the mass extending extra-cranial along the right lateral upper cervical aspect. The mastoid antrum is obliterated causing an accumulation of fluid. 

Pathology

Multiple attempts for biopsy were abandoned after significant hemorrhage, despite the preoperative embolization procedure. This delayed plans for possible combined otolaryngology and neurosurgical approach resection, as the expected diagnosis of paraganglioma, was not confirmed.

Patient subsequently had a successful, uncomplicated biopsy confirmed the diagnosis of paraganglioma. Given the patient’s overall high functionality and the likely associated morbidity of surgery, radiation therapy was recommended in attempts at slowing tumor growth and reducing vascularity.

Case Discussion

The most characteristic location of glomus tympanicum tumors is lateral to the cochlear promontory. However, if the tumor is large and involves the hypotympanum, it cannot be distinguished from a glomus tympanicum tumor, and the term glomus jugulotympanicum applies. Our case demonstrates an exaggerated example of this dilemma with intracranial and extracranial locations.

The difficult decision regarding treatment options must be made with careful consideration of the associated morbidities of slow tumor growth and recurrence with conservative management versus the surgical morbidities of aggressive surgical resection. Imaging is arguably the most important component of this decision-making process, with the radiologist contributing significantly to multiple aspects of the management of glomus tumors.

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