Böbrek hücreli karsinoma: bir pediyatrik olgu sunumu

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Introduction
Renal cell carcinoma (RCC) which constitutes 90%
to 95% of renal tumors in adulthood is very rare among
children (5.9% of pediatric kidney tumors) (1).
Approximately 350 patients have been reported in case
reports and small series. Pediatric RCC may be quite
different from adult type in terms of clinical presentation, behavior, and coexistence with genetic abnormalities and neuroblastoma (2). Pediatric RCC usually presents with the symptoms of primary tumor as opposed
to the incidental diagnosis of adult RCC in almost 50%
of the cases. We hereby report a 12-year-old patient with
RCC who was diagnosed upon referral for a blunt trauma to the kidney.
Case Report
A 12-year-old girl was brought to the Emergency
Department of Pediatrics with the complaints of a large
swelling and pain in her upper left quadrant following a
blunt trauma three days prior to the referral. On physical examination, a mass was palpated, which almost
entirely invaded the upper left quadrant and left flank.
WBC count, blood biochemistry and urine analysis
were found to be normal while hematocrit and hemoglobin values were 27.7% and 9.9 gr/dL, respectively.
Abdominal ultrasound revealed a 15-cm thick-walled
mass that originated from lower pole of the left kidney
* Department of Urology, Gülhane Military Medical Academy
Haydarpaþa Training Hospital, Ýstanbul
** Department of Pathology, Gülhane Military Medical Academy
Haydarpaþa Training Hospital, Ýstanbul
Reprint request: Dr. Cüneyt Adayener, Department of Urology,
Gulhane Military Medical Academy Haydarpaþa Training Hospital,
Üsküdar-34668, Ýstanbul
E-mail: cuneytadayener@yahoo.com
Date submitted: February 21, 2007
Accepted: July 31, 2007
OLGU SUNUMU/CASE REPORT Gülhane Týp Dergisi 2008; 50: 134-136
© Gülhane Askeri Týp Akademisi 2008
Summary
A 12-year-old girl was admitted to the Department of
Pediatrics with the complaints of a large swelling and pain
in her upper left quadrant following a blunt trauma three
days prior to the referral. Computed tomography revealed
a mass around the left kidney compatible with a large
hematoma, and open surgical exploration was performed.
Left nephrectomy was carried out since the frozen section
specimen obtained from the wall of the mass that was full
of hematoma was suspicious for malignancy. Result of the
histopathological examination was clear cell carcinoma limited to the kidney. Our case, who developed a renal cell
carcinoma at a young age, might have been diagnosed too
late if the trauma had not happened. Literally, "a blunt
trauma to her kidney might have saved her life."
Key words: Pediatric renal carcinoma, trauma
Özet
Böbrek hücreli karsinoma: bir pediyatrik olgu sunumu
On iki yaþýnda kýz hasta, karnýna aldýðý künt travmadan 3
gün sonra, sol üst kadranda aðrý ve büyük bir þiþlik yakýnmasý ile çocuk hastalýklarý kliniðine getirildi. Çekilen tomografisinde sol böbrek çevresinde geniþ hematom ile uyumlu
kitle tespit edilen hasta explore edildi. Ýçi hematom ile dolu
olan kitle duvarýndan alýnan örneðin “frozen section”
incelemesi malignite þüpheli gelmesi üzerine hastaya sol
nefrektomi uygulandý. Histopatolojik inceleme sonucu
böbreðe sýnýrlý berrak hücreli karsinom olarak belirlendi.
Genç yaþta böbrek hücreli karsinomu olan olgumuzun tanýsý,
eðer travma olmasaydý çok geç konabilirdi. Daha doðru bir
deyiþle "böbreðine aldýðý künt travma olgumuzun hayatýný
kurtarmýþ olabilir."
Anahtar kelimeler: Pediyatrik böbrek karsinomu, travmaCilt 50 · Sayý 2 · Gülhane TD Pediatric renal cell carcinoma · 135
and lied towards umbilicus, and that contained internal
septations. The wall of the mass showed contrast uptake
and its contrast content was calculated to be 26 HU on
abdominal computed tomography (CT), which was
consistent with hematoma (Figure 1).
Open surgical exploration revealed that the mass
contained a hematoma, and a nephrectomy was carried
out since the frozen section of the wall of the mass was
suspicious for malignancy. Eventually, clear cell carcinoma limited to the kidney was diagnosed on histopathological investigation (Figure 2). The patient was evaluated in terms of Von-Hippel-Lindau disease in the
Department of Pediatrics postoperatively. Her abdominal and cranial CT, retinal examination and other laboratory findings were normal. The patient is disease-free
in terms of her primary pathology at the 10th postoperative year now.
Discussion
RCC is extremely rare among pediatric population.
Only 0.3% to 1.3% of all cases are younger than 15 years. The mean age for pediatric RCC is around 9 years
as opposed to the relatively earlier age for Wilms' tumor
(3). Due to its rareness among children, there is no distinct criteria pertaining to the diagnosis, treatment and
follow-up of pediatric disease. Therefore, characteristics of adult RCC have been considered in this regard.
Two recent studies reported that around 45% of the
cases were at stage I at the time of diagnosis while previous studies reported advanced disease at presentation
(4,5). Increase in the rate of early diagnosis may be
explained by the increase in the availability of advanced
imaging modalities.
The most common sign of pediatric RCC is abdominal mass (21.9% to 64%) and hematuria (29.2% to
50%) (5,6). Abdominal mass is palpable in around 20%
of the cases (5). The mass in our case belonged to the
hematoma from the injured kidney with tumor, and the
tumor had manifested after a blunt trauma. Kidneys
with tumors have been shown to be more vulnerable to
trauma than normal kidneys. Tumors were found incidentally in 4 of the 675 cases who underwent surgical
exploration after renal trauma (7). This is a higher rate
than the incidence of the disease in normal population,
which is 8.7/100 000 new patients per annum (8).
Papillary histology has previously been reported to
be more frequent among children (8). However, the
ratio of clear cell carcinoma were 45% and 58.5% in two
recent pediatric series (4,5).
The prognosis in children has been reported to be
no different than adults, and patient age, dimensions of
the tumor, histologic type and vascular invasion were
important criteria for prognosis (3,9). However, a more
recent retrospective study suggested contradictory findings, and it was suggested that the stage of the tumor
and completeness of the excision were the most important prognostic factors (10). Estimated 20-year event
free survival rates for stage I and II cases were 89%,
while the ratio decreased to 18% and 22.6% in stages 3
and 4 patients, respectively (5).
It is a pity that many patients remain undiagnosed
until their masses become palpable since there are usually no symptoms during the early period of the disease.
Our case, who developed a RCC at a very young age,
might have presented after it was too late if the trauma
had not happened. Literally, "a blunt trauma to her kidney might have saved her life.