Inferior orbital adenoid cystic carcinoma with skull base and cranial nerves infiltration-case report

Cancer research · Head and neck squamous cell carcinoma

This is the second documented case of primary inferior orbital adenoid cystic carcinoma and the first with concurrent skull base and cranial nerve infiltration without lacrimal gland involvement.

Background Primary orbital adenoid cystic carcinoma (ACC) not arising from the lacrimal gland is very rare with few previous case reports either involving the orbital apex, medial orbit or inferior orbit. There is only one previous case report of primary adenoid cystic carcinoma involving the inferior orbit. The aim of this case report was to present a rare instance of primary adenoid cystic carcinoma (ACC) arising in the inferior orbit without lacrimal gland involvement, uniquely associated with skull base and cranial nerve infiltration, and to emphasize the importance of imaging and histopathological evaluation in early diagnosis and management of uncommon orbital tumors. Case presentation Our case is a 65-year-old male presented with progressive ophthalmoplegia. Clinical examination revealed minimal left proptosis. MRI demonstrated an ill-defined inferior orbital mass, extending through the pterygopalatine fossa along the maxillary, vidian, and infraorbital nerves. The lacrimal gland was not involved. CT imaging showed skull base infiltration of the pterygoid process. Biopsy confirmed primary adenoid cystic carcinoma with basaloid and tubular histological patterns. No distant metastasis was identified on systemic staging. The patient received palliative chemoradiotherapy, with subsequent imaging showing partial tumor regression. Conclusion This is the second documented case of primary inferior orbital ACC and the first with concurrent skull base and cranial nerve infiltration without lacrimal gland involvement. Early recognition and accurate imaging by combined MRI with contrast and CT is required for early detection of both perineural spread (PNS) and skull base invasion with consequent early management. Why it matters The report highlights the rare occurrence of inferior orbital adenoid cystic carcinoma infiltrating the skull base and cranial nerves without lacrimal gland involvement, emphasizing the need for early imaging detection and appropriate therapeutic strategies to improve outcomes in aggressive orbital malignancies. Limitations Limitations include a single patient case, lack of quantitative outcome data, and absence of molecular profiling to guide targeted therapy.

The report highlights the rare occurrence of inferior orbital adenoid cystic carcinoma infiltrating the skull base and cranial nerves without lacrimal gland involvement, emphasizing the need for early imaging detection and appropriate therapeutic strategies to improve outcomes in aggressive orbital malignancies.

Evidence level: Klinikai vizsgálat. Formális klinikai vizsgálati eredmény.

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