Semmelweis University Budapest, Hungary Department of Ophthamology Head: Prof. Zoltán Zsolt Nagy Epibulbar Inflammatory Myofibroblastic Tumor After Haematopoietic Stem Cell Transplantation: Case Report Ágnes Füst 1, É Szalai 1, J Tóth 1,2, L Ocskay 3, B Csákány 1, ZZ Nagy 1 1. Department of Ophtalmology, Semmelweis University 2. 2nd Department of Pathology, Semmelweis University 3. Department of Ophthalmology, United St. István and St. László Hospital The authors have no financial interests to disclose WCC 2015, San Diego

Introduction: Inflammatory myofibroblastic tumor = Inflammatory pseudotumor Relatively uncommon tumor-like mass Has a predilection for children and adolescents, although it can arise as late as the eighth decade of life Usual anatomical locations: abdominopelvic region, lung, retroperitoneum, etc.

Purpose To report a case of an epibulbar inflammatory myofibroblastic tumor which presented after haematopoietic stem cell transplantation

Report of the case 13-year-old male patient 2009: diagnosed with X-linked adrenoleukodystrophy 2011: three allogenic hematopoietic stem cell transplantations to stop demyelination. The third was successful. After the third transplantation, before engraftment: HSV infection: massive mucositis, cheilitis, inflammation of right eyelids and conjunctivitis Treatment: intravenous acyclovir and foscarnet The conjunctival inflammation persisted for weeks Mild conjunctival scarring

Report of the case Presented in March 2013 with a slowly growing subconjunctival mass on the right eye BCVA: 1.0 both eyes An excisional biopsy was carried out, and the specimen was sent for histopathological examination. The postoperative period was uneventful No recurrence until now

The subconjunctival mass The right eye showed also mild conjunctival scarring, mostly near the lower fornix, and there was neovascularization on the lower third of the cornea

Ultrasound biomicroscopy homogenous low internal reflectivity with no invasion of the conjunctiva and the underlying sclera

Diagnosis: inflammatory myofibroblastic tumor lymphocytes, plasma cells, histiocytes, macrophages, and foam cells, among benign appearing spindle-shaped stroma cells spindle cells were 100% smooth muscle actin positive Diagnosis: inflammatory myofibroblastic tumor

Conclusions Inflammatory myofibroblastic tumor following hematopoietic stem cell transplantation has rarely been reported. Only few cases exist in the literature, where the affected organs were liver, kidney, esophagus, brain, lung and bladder. Fangusaro: Bone Marrow Transplant. 2004, Tsutsumi: Bone Marrow Transplant 2005, Ogura: Bone Marrow Transplant 2004, Bahat: Bone Marrow Transplant 2007, Priebe-Richter: Eur J Haematol 2005, Sastre-Garau: Pathol. 2002. The conjunctival inflammatory myofibroblastic tumor from any reason is a rare entity. Favini: Pediatr Blood Cancer. 2010, Goto: Jpn J Ophthalmol.2004. To our knowledge we describe a case of previously unreported epibulbar inflammatory myofibroblastic tumor occurring in association with hematopoietic stem cell transplantation.

Conclusions For inflammatory myofibroblastic tumors developing after hematopoietic stem cell transplantation immunosuppression, chemotherapy, irradiation, chronic inflammation, herpes simplex and Epstein-Barr virus infection and graft versus host disease are the factors most likely associated. Fangusaro: Bone Marrow Transplant. 2004, Mergan: J Pediatr Surg 2005 In our case, the inflammatory myofibroblastic tumor might be connected to the herpes simplex virus infection and the consecutive chronic ocular surface disease.