PB0242 - Epistaxis in a patient with Glanzmann Thrombasthenia : about a Strasbourg case revealing an endonasal tumor

Background: Epistaxis is a common and often neglected clinical situation in the case of Glanzmann Thrombasthenia (GT).

Aims: Before a resurgence of posterior epistaxis, treated by injection of activated eptacog alfa (rFVIIa) and tranexamic acid, a vascularized purple tumor of the right middle cornet is diagnosed at nasoscopy. MRI confirms the presence of a hypervascularized polypoid formation whose characteristics are compatible with an agioma.

Methods: Antiplatelet antibodies are negative in MAIPA assay, knowing that the last platelet transfusion was 3 years ago. Management begins with an embolization of the sphenopalatine artery, followed by a right unilateral ethmoidectomy.
The protocol consists on transfusion of 3 platelet concentrates and infusion of 3.4mg/kg of rFVIIa for 4 days. Perioperative bleeding is estimated at 600 ml. The follow-up was simple.
The anatomic pathology is in favor of glomangiopericytoma. A second MRI made 2 months after the operation shows complete excision. Bi-monthly follow-up is established to diagnose a possible early recurrence.

Results: Sinonasal glomangiopericytoma is a rare tumor arising from the periyctes surrounding capillaries and accounts for less than 0.5% of all sinonasal tumors. It falls within the category of borderline and low-malignant-potential soft tissue tumors of the nose and paranasal sinuses, requiring long-term monitoring.

Conclusion(s): This rare case of association illustrates the need for multidisciplinary management and makes us aware that in case of frequent recurrence, the exploration of an epistaxis must be based on a nasal endoscopy and imaging of the facial mass even in GT patients, just as the presence of recurrent rectorragia or melenate should lead to gastrointestinal endoscopic exploration.