Adenoid cystic carcinoma of jugular foramen

Adenoid cystic carcinoma is a slowly growing malignant tumor with high local recurrence, perineural and vascular invasion. This tumor might arise from the glands of upper respiratory tract and oral cavity (eg, salivary or serous or mucous). Here we report the case of a 65-year-old woman who was referred to our unit for left retro-auricular radiating pain with trigger points and frontal headache since 6 months. There was no involvement of cranial nerves. Imaging screening using MRI, Positron emission tomography with 2-[fluorine-18] fluoro-2-deoxy-D-glucose, Gallium-68 DOTA-Phe1-Tyr3-Octreotide (68Ga DOTATOC) Positron emission tomography-CT suggested a suspicion of schwannoma or paraganglioma of the jugular foramen. However, the CT-guided biopsy revealed presence of adenoid cystic carcinoma. These warrants performing mandatory histological analysis combined with imaging screening suspicion of schwannoma or paraganglioma.