gms | German Medical Science

Objective: The inflammatory pseudotumor is a rare entity of unknown etiology which may occur in several areas of the body. Some authors suggest that it is reactive in nature while others suggest it is a true soft tissue neoplasm with multifocality and a recurrence potential. Its occurrence however in the infraorbital canal is a rare condition which can lead to erosion of the skull base and involvement of the cranial nerves in the posterior part of the cavernous sinus. Most of these cases result rapidly in a Tolosa-Hunt syndrome with oculomotor symptoms, headache and cranial polyneuropathy. We report the case of an inflammatory pseudotumor initially confined to the infraorbital canal with a slow growth pattern but for conal widening in a 67-year-old woman with a four-year history of progressive swelling under her right eye and discuss the treatment options.

Method: Cranial MRI with contrast medium was performed. The tumor was macroscopically totally resected via a sublabial transmaxillary approach. En-bloc resection was not possible. PCR and immunhistochemistry were performed.

Results: A homogenous contrast-enhancement in the right infraorbital canal was seen on imaging. Surgery revealed a cone-shaped capsulated tumor within a widened infraorbital canal, which had displaced and split the infraorbital nerves fibers, was found. The capsule was well-preserved; the tumor was completely resected and revealed the classical features an inflammatory pseudotumor with fibrous tissues, lympho-plasmacellular infiltration, blood vessel and perivascular nerve fascicles. Moreover positive CD3, CD20, CD138, Kappa, Lambda, BCl2 and MIB1 markers. There was a mixed cellular inflammation predominately T-lymphocytes with follicular architecture and polyclonal lymphoid proliferation. Postoperatively the patient had solely hyperesthesia in the right infraorbital region. Follow-up MRI 3 and 6 months after surgery did not reveal any recurrence. The patient remained stable on 1-year follow-up after treatment.

Conclusions: Proposed treatment of pseudotumors is steroid therapy. However recurrence is a common problem on discontinuation of steroid therapy. When confined to a particular region treatment can be achieved through a complete surgical resection with satisfactory results as was in our case.