Hindawi Publishing Corporation
Case Reports in Urology
Volume 2013, Article ID 675059, 4 pages
http://dx.doi.org/10.1155/2013/675059
Case Report
Inflammatory Myofibroblastic Bladder Tumor in a Patient with
Wolf-Hirschhorn Syndrome
Antonio Marte,
1
Paolo Indolfi,
2
Carmine Ficociello,
2
Daniela Russo,
3
Matilde Oreste,
2
Gaetano Bottigliero,
2
Giovanna Gualdiero,
2
Ciro Barone,
2
Elena Vigliar,
3
Cristiana Indolfi,
2
and Fiorina Casale
2
1
Pediatric Surgery, Second University of Naples, Largo Madonna delle Grazie, 80138 Naples, Italy
2
Pediatric Oncology Service, Pediatric Department, Second University of Naples, Italy
3
Pathology Department, Federico II University of Naples, Italy
Correspondence should be addressed to Antonio Marte; antonio.marte@unina2.it
Received 12 June 2013; Accepted 10 July 2013
Academic Editors: S.-S. Chen, A. Goel, and M. Sheikh
Copyright © 2013 Antonio Marte et al. his is an open access article distributed under the Creative Commons Attribution License,
which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Inlammatory myoibroblastic tumor (IMT) is a rare neoplasm described in several tissues and organs including genitourinary
system, lung, head, and neck. he etiology of IMT is contentious, and whether it is a postinlammatory process or a true neoplasm
remains controversial. To our knowledge, we report the irst reported case of IMT of urinary bladder in a pediatric patient with
Wolf-Hirschhorn (WHS). We also review the literature about patients with associated neoplasia.
1. Introduction
IMT is a rare neoplasm usually seen in children and adoles-
cents, mostly occurring between 2–16 years of age. Females
are afected slightly more commonly than males. It is also
known as cellular inlammatory pseudotumor, plasma cell
granuloma, and inlammatory ibrosarcoma and is composed
of spindle cells with associated inlammatory cells iniltrate
[1]. his type of tumor has been described in several organs
and anatomical sites including genitourinary system where
the tumor usually originates in the bladder, but it has also
been reported in the kidney, urethra, prostate, ureter, and rete
testis [2]. he etiology of IMT, its behavior, and its cell of
origin remain matters of debate [1]. Originally considered a
lesion with a benign clinical course, it is now clear that IMT
can have an aggressive behavior and, occasionally, an unfa-
vorable prognosis [3]. For this reason, it is important to difer-
entiate this lesion from sarcoma for therapeutic management,
and this can be diicult both clinically and histologically [3].
To gain more knowledge about this rare tumor, we reported
a case of IMT of the urinary bladder in a girl with WHS.
2. Case Report
A previously healthy 8-year-old female, with WHS, was
admitted to our clinic in February 2012 for a persistent
abdominal pain and macroscopic hematuria. Abdominal
ultrasound revealed a multilobated tumor in the bladder
adhering to the let bladder wall. A computerized tomography
(CT) scan of abdomen conirmed these indings, and a
solid mass (approximate size 5 × 4.5 cm) iniltrating the
dome and the let bladder wall not extending to perivesical
tissues nor lymph node enlargement was revealed. Due
to ultrasonographic (Figure 1) and CT scan features (large
base, poor vascularization, multilobated appearance, and
size >4 cm), the patient underwent cystoscopic multiple
biopsies. Histopathology revealed a spindle cell lesion with
mixed inlammatory cells in the background. According to
histopathology and immunohistochemical characteristics, a
provisional diagnosis of IMT was made. Because of the size
of the tumor, a complete transurethral resection was not
technically possible and a chemotherapy or radiotherapy was
not justiied in the absence of histological malignant tissue,