Posterior fossa giant adenoid cystic carcinoma with skull base invasion mimicking glomus jugulare: A case report and review of literature

Abstract

The author describes a rare case of giant adenoid cystic carcinoma (ACC) mimicking large paraganglioma with lower cranial nerve palsy. A 60-year-old female presented with a progressive increase in postauricular swelling with unilateral hearing loss, facial deviation, difficulty in swallowing, and hoarseness of voice. MRI brain showed highly vascular infiltrating and osteolytic mass suggestive of large glomus jugulare versus sarcoma. It was completely engulfing the jugular foramen and lower cranial nerves with bony erosion of the jugular foramen and occipital condyle. The whole mastoid was filled with the tumor. On digital subtraction angiography the majority of blood supply was from the occipital branch of the external carotid artery and vertebral artery. The patient underwent percutaneous embolization followed by external carotid ligation and resection of the mass. The postoperative course was uneventful. Histopathology was suggestive of mixed ACCs. The patient received radiotherapy. After 1 year of follow up no recurrence or distant metastasis was noted.

Keywords: Giant adenoid cystic carcinoma; embolization; glomus jugulare; lower cranial nerve palsy.

Figure 1.

(a) Patient showing large retroauricular mass with (arrow mark) black dot suggestive of puncture site for glue, (b) CT head Axial view showing large herniating mass with the destruction of, jugular foramen, mastoid, and lateral suboccipital region, (c) T1 Sequence MRI (Axial) showing large hypointense mass occupying both middle and posterior cranial fossa abutting internal carotid artery, (d) T 2 sequence (Axial) showing large hyperintense mass with multiple flow voids (Arrow mark showing internal carotid artery), (e) T1 contrast sequence (Axial) showing large homogenously enhancing mass at retromastoid region extending anteromedially up to ventral brain stem (arrow mark), (f) T1 contrast sequence (coronal) showing large homogenously enhancing mass at retromastoid region (arrow mark) with preserved plane from the brain stem.

Figure 2.

(a) Left ECA injection oblique view, showing hypervascular tumor with large intense tumor blush and multiple enlarged sinusoidal spaces within the tumor core, (b) LVA injection, AP view showing the core of the lesion which is made up of large communicating sinusoidal spaces, (c) LAT view, fluorospot image, direct needle puncture done under roadmap with an attempt to access the deep-seated sinusoidal spaces first, (d & e) Glue cast, Occipital artery angiogram, Lat view, showing no further filling and LVA angiogram, AP view, after Second percutaneous injection of nBCA, shows no further filling from LVA injection, and <10% filling from occipital artery injection (oval); the LVA is no more filling on the occipital artery injection as the muscular collaterals have been retrogradely filled with glue (thin arrow), (f) Final check angiogram ECA injection AP view, showing near total devascularization with a small area of fine blush in the anteromedial portion of the tumor.

Figure 3.

(a) Post-op NCCT head showing near total resection of mass both at intracranial and extracranial region (Arrow mark), (b) Post-op image of the patient after 2 months showing healthy scar mark (arrow mark) and overlying skin.

Figure 4.

(a) Histopathology suggested mixed adenoid cystic carcinoma with both solid and cribriform components, but solid components are predominant (H&E, 100X), (b) IHC demonstrated GFAP negative tumor cells (100X), (c) S100 positive on IHC showing ductal component (100X), (d) p63 positive on IHC showing myoepithelial component (100X).

Figure 5.

(a) Image of patient’s postauricular region showing radiation-induced cutaneous reaction (Pigmentation [yellow arrow] and ulceration[green arrow]).