Pediatric Case Report
Inflammatory Myofibroblastic
Tumor of the Bladder in a 10-Year-old Girl
Evren Süer, Ömer Gülpınar, Murat Mermerkaya, Berk Burgu, Pinar Celepli,
Ays ¸e Sertçelik, and Tarkan Soygür
Inflammatory myofibroblastic tumors (IMTs) of the bladder are rarely encountered bladder tumors during the pediatric
age. The unknown malignant potential of these tumors causes controversy for their treatment and follow-up. We report
a 10-year-old girl who was referred to our clinic with dysuria and enuresis. The clinicopathological evaluation was
compatible with IMT and a bladder preserving approach was used. There was no recurrence in the first year of follow-up
examinations. UROLOGY 80: 1138 –1140, 2012. © 2012 Elsevier Inc.
B
enign and malignant bladder tumors are rarely
seen in childhood and can interfere with each
other. Due to the difference of treatment strate-
gies for these tumors, distinguishing 2 tumors is of critical
importance. Inflammatory myofibroblastic tumors (IMTs)
are a rare spindle tumor which is seen in every site of the
body, including the bladder.
1
An absolute definition for
these tumors’ malignant potential is not possible for now
and the excision of these tumors with bladder preservation
is the most preferred approach in the literature.
2
Herein, we
report about a case of a 10-year-old girl with an IMT of the
bladder who was successfully treated by a bladder preserving
approach.
CASE REPORT
A 10-year-old girl with a 2-month history of dysuria and
enuresis was referred to our department for evaluation.
Previous urinalysis and urine culture tests did not dem-
onstrate any pathologic finding. Physical examination
demonstrated a midline, suprapubic mass with irregular
contours that was palpable. An ultrasound scan showed a
solid mass, mostly extravesical with an intraluminal com-
ponent, which is positioned at the anterolateral surface of
the bladder. The upper urinary tract was normal. For a
suspected sarcoma, magnetic resonance imaging of the
abdominopelvic region and a thorax computerized to-
mography was performed for a complete diagnostic and
staging workup.
The magnetic resonance imaging revealed an ex-
travesical mass measuring 46 40 mm that protruded
through the bladder lumen. We did not detect further
pathologic findings during the diagnostic workup.
When biopsy and transurethral resection was discussed
with the family, they preferred transurethral resection.
Cystoscopy demonstrated a tumor at the dome of the
bladder. Transurethral resection of this bladder mass was
performed and specimens were obtained for pathologic
evaluation. The histology demonstrated cystitis cystica
and papillary epithelial hyperplasia. Because of this un-
satisfactory report, an ultrasound-guided biopsy of the
suprapubic mass was executed. Spindle mesenchymal
cells with mixed inflammatory cell components were seen
in the histopathological evaluation.
Immunohistochemical staining showed spindle cells
staining with anaplastic lymphoma kinase 1 (ALK-1),
smooth muscle actin (SMA), desmin, calponin, and ker-
atin. Myogenin staining was negative. These findings
were consistent with IMT.
Tumor excision with preservation of the bladder was
the accepted approach for this patient because of the
negative histopathological outcomes to suggest a rhabdo-
myosarcoma (Fig. 1). A pfannenstiel incision was per-
formed. The mass, which was situated at the dome of the
bladder and the adherent peritoneal and bladder tissue,
was excised. The bladder was closed and a urethral cath-
eter was placed for the drainage of the bladder. On gross
examination, the mass was 3 3 2.7 cm, with no
capsule, not easily distinguished from the surrounding
parenchyma, and light brown. There was no evidence of
necrosis or hemorrhage. The histologic appearance of the
lesion showed a tumor consisting of plump or spindled
myofibroblasts in an edematous myxoid background with
many blood vessels and an infiltrate of chronic inflam-
matory cells (lymphocytes, plasma cells).
Immunohistochemically, the tumor cells exhibited
strong cytoplasmic positivity for SMA, vimentin, and
ALK-1 (Fig. 2) and negativity for WT-1, desmin, S100,
CD68, and EMA. The early postoperative period was
uneventful and previous urinary symptoms recovered. In
Financial Disclosure: The authors declare that they have no relevant financial
interests.
From the Department of Urology, University of Ankara, I
˙
bn-i Sina Hospital,
Sihhiye, Ankara, Turkey; and Department of Pathology, University of Ankara, I
˙
bn-i
Sina Hospital, Sihhiye, Ankara, Turkey
Reprint requests: Murat Mermerkaya, M.D., Department of Urology, Ankara Uni-
versity, I
˙
bn-i Sina Hospital, 06110 Sıhhiye, Ankara, Turkey. E-mail: muratmer@
gmail.com
Submitted: June 13, 2012, accepted (with revisions): July 16, 2012
1138 © 2012 Elsevier Inc. 0090-4295/12/$36.00
All Rights Reserved http://dx.doi.org/10.1016/j.urology.2012.07.023