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