Download Epibulbar Inflammatory Myofibroblastic Tumor After Haematopoietic

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

lymphocytes, plasma cells,
histiocytes, macrophages,
and foam cells, among
benign appearing spindleshaped 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.