Keywords
Nephroblastoma - pediatrics - sonography - varicocele - Wilms’ tumor
Introduction
Wilms’ tumor (WT), also known as Nephroblastoma, is the most common malignant abdominal
neoplasm in children.[1] It usually presents with abdominal mass or pain. Hematuria, hypertension, urinary
tract infection, and other gastrointestinal (GI) symptoms are less common presenting
complaints.[2] Furthermore, presentation with varicocele in children is extremely rare – a thorough
and exhaustive search yielded only three previously reported cases in English language
literature.[3],[4],[5] In addition, those previously reported cases were in much older children than the
index case (two to four-and-a-half years old).[3],[4],[5]
Case Report
An 11-month old male infant presented to the Pediatric Emergency Unit on account of
painful, left hemiscrotal swelling noticed by the mother 4 days prior to presentation.
There was no history of hematuria, crying on micturition, abdominal swelling, abdominal
pain, or other GI symptoms. Past medical, family, and social histories were insignificant.
Scrotal examination revealed normal bilateral testicular descent. Multiple, large,
visible vessels were seen distending the left hemiscrotum (Grade III). The right hemiscrotum
was normal. Abdominal examination revealed left flank/left upper quadrant fullness
causing asymmetry of the abdomen. There was also a large, nontender, ballotable left
abdominal mass. No evidence of ascites. Both iris were present on ocular examination
and the tongue appeared normal in size. (These were examined to exclude any association
with Beckwith–Wiedemann syndrome.) An assessment of left-sided varicoceles secondary
to a left renal mass was made.
Abdominal ultrasonography (USG) revealed a large, oval, well-circumscribed, solid
mass occupying the middle and lower poles of the left kidney. The mass measured approximately
9.2 × 7.3 × 8.2 cm (L × AP × T). The mass was heterogeneous in appearance and contained
an amorphous focus of increased echogenicity (possibly due to fat and/or calcium deposition).
No cystic or necrotic areas were seen within it.
The adjacent normal renal parenchyma appears to claw around the mass, suggesting renal
origin of the mass. The mass showed moderate vascularization on Doppler sonography.
The right kidney was sonographically normal. There were no metastatic deposits in
the liver, no sonographic evidence of ascites, no enlarged para-aortic, or periportal
lymph nodes. The inferior vena cava was patent and compressible, with adequate intraluminal
color filling. The renal veins were not visualized. Other abdominal organs were within
normal limits.
Scrotal sonography showed numerous, dilated, tortuous/serpiginous, hypoechoic, tubular
structures (>2 mm in diameter) demonstrating florid vascularity on color Doppler insonation
within the left hemiscrotum [Figure 1]A and [Figure 1]B. Both testes and epididymides and the right hemiscrotum were sonographically normal.
No abnormal fluid collection within the scrotal sacs.
Figure 1 (A and B): (A) Gray-scale sonogram of the left scrotum showing multiple, dilated, tortuous/serpiginous,
hypoechoic, tubular structures (downward arrows); the left testis with its mediastinum
is sonographically normal (upward arrows). (B) Duplex sonogram of the left scrotum
showing florid vascularity of the hypoechoic tubular structures on color Doppler insonation
(downward arrows)
On computerized tomography (CT) of the abdomen, the left renal mass was heterogeneous
in appearance measuring approximately 9 × 8× 8 cm [Figure 2]A. It showed minimal contrast enhancement with a claw-like configuration of the adjacent
renal tissue, which suggests the mass is of renal origin [Figure 2]B. The left renal vein was patent but appeared markedly compressed by the mass [Figure 2]C. The bowel loops were displaced contralaterally by the mass. No metastatic deposits
in the liver. No ascites. Chest radiograph was normal.
Figure 2 (A-C): (A-C) Plain, arterial, and venous phases of axial abdominal CT showing a heterogeneous
mass with minimal contrast enhancement on the left side of the abdomen (arrow) compressing
the left renal vein (arrowhead)
At surgery, the mass was confined to the middle and lower poles of the left kidney
(stage I). The left renal vein was severely compressed by the mass. The patient underwent
a left nephrectomy. Histopathologic analysis confirmed the mass to be WT. Vincristine
and dactinomycin were used for adjuvant chemotherapy postop.
Discussion
The worldwide prevalence of WT is 1 case per 10,000 live births.[1] WT is relatively more common in blacks than in whites and is rare in East Asians.[1] The peak incidence occurs between the second and fourth years of life; it is very
rare during first year of life.[1] The index case was diagnosed in an 11-month-old male infant.
Varicoceles are abnormal dilatation, elongation, and tortuosity of the pampiniform
plexus (and to a lesser extent, dilatation of the smaller cremasteric plexus) secondary
to retrograde flow into internal spermatic vein.[6] They can be idiopathic/primary/congenital or secondary.[6] Primary/idiopathic/congenital varicoceles are due toincompetent or absent valve
at level of left renal vein or inferior vena cava on right side [6] and usually do not become clinically obvious until puberty.[3] Secondary varicoceles develop due to compression of the left renal vein by a tumor,
aberrant renal artery, obstructed renal vein, hydronephrosis, and cirrhosis.[6] Secondary varicoceles do not empty on recumbent position (nondecompressible varicocele).[6]
The prevalence of idiopathic/primary/congenital varicoceles is about 0.2% in boys
from birth to 6 years of age.[7] Therefore, the presence of varicocele in a boy aged <6 years old should trigger
a careful search for a secondary cause. Since varicoceles are almost never seen in
children <9 years old,[8] when seen in this age group, a WT is a strong possibility,[8] just as it turned out in the index case. Consequently, it is apposite to perform
renal and/or retroperitoneal scan in children with varicoceles on scrotal sonography.[9]
The prognosis and survival rate of WT depends on pathologic pattern, age at the time
of diagnosis, and extent of disease.[1] Four-year relapse-free survival is 91% for stage I, 88% for stage II, 79% for stage
III, and 78–84% for stage IV.[1] The index patient had stage I disease at presentation, and is still alive and relapse-free
3 years after diagnosis.
In conclusion, radiologists, pediatricians, and pediatric surgeons should be aware
that testicular varicocele is a rare presentation of WT in children.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms.
In the form the patient(s) has/have given his/her/their consent for his/her/their
images and other clinical information to be reported in the journal. The patients
understand that their names and initials will not be published and due efforts will
be made to conceal their identity, but anonymity cannot be guaranteed.
