1. 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

2. 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.

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

4. 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

5. 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

6. 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

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

8. 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

9. 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
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.

10. 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.