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

Semmelweis UniversityBudapest, Hungary

Epibulbar Inflammatory Myofibroblastic Tumor After Haematopoietic Stem Cell

Transplantation: Case Report

Department of OphthamologyHead: Prof. Zoltán Zsolt Nagy

WCC 2015, San Diego

Ágnes Füst 1, É Szalai 1, J Tóth 1,2, L Ocskay 3, B Csákány 1, ZZ Nagy 1

= 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.

Introduction:Inflammatory myofibroblastic

tumor

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

Purpose

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

Report of the case

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

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

Ultrasound biomicroscopy

lymphocytes, plasma cells, histiocytes, macrophages, and foam cells, among benign appearing spindle-shaped stroma cells

Diagnosis: inflammatory myofibroblastic tumor

spindle cells were 100% smooth muscle actin positive

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.

Conclusions