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Table 21.5 Stage summary for rhabdomyosarcoma
Stage Site Size Node status Metastasis
1 Favorable site Any N0 or N1 M0
2 Unfavorable site Tumor ≤5 cm N0 M0
3 Unfavorable site Tumor ≤5 cm N1 M0
3 Unfavorable site Tumor >5 cm N0 or N1 M0
4 Any Any Any M1
N0, no positive nodes; N1, nodes positive; M0, no metastatic disease; M1, metastatic disease.
Stratication and Staging
e staging, grouping, and risk stratication of
RMS relies on a complex algorithm based on key
information on the patient’s history and tumor
biology. e basics are summarized as follows:
●
Stage: Preoperative determination based on
tumor size, location, and clinical node and
metastatic status. Of note, bladder/prostate
RMS can never be stage 1 because of its unfavorable location (Table 21.5).
●
Group: Postoperative classication
based on completeness of surgical resection. e assigned group is not static and
can be altered prior to the initiation of
chemotherapy.
●
Risk group: is is based on a combination of
stage, group, patient age, histology, and fusion
status. Risk groups are routinely revised and
updated and are used to guide decisions on
therapy, to stratify patients in clinical trials, and to counsel parents and patients on
prognosis.
Bladder/Prostate
Rhabdomyosarcoma
Initial evaluation
e evaluation for a new or suspected bladder/
prostate RMS is based on the medical history
and physical examination followed by a renal and
bladder ultrasound (Figure 21.6A). Information
on the age of the child, presence or absence of
voiding problems, including gross hematuria or
symptoms of outlet obstruction and a possible history of bladder augmentation may help to identify
risk factors for less common bladder masses. Basic
laboratory biochemical and hematological studies
should include a comprehensive metabolic panel,
complete blood count, and coagulation panel.
Early management of bladder/prostate RMS
involves complex decision-making which should
be the responsibility of a multi-disciplinary team.
ese patients will then require complete staging
with CT chest, CT/MRI of the abdomen and pelvis, positron emission tomography (PET) CT, and
bone marrow biopsies (Figure 21.6B, C).
Treatment
●
Surgery
●
Chemotherapy
●
Radiation
Historically bladder/prostate RMS was initially
managed with radical cystoprostatectomy but
this approach is now used in less than 10% of
cases. e initial diagnosis is most commonly
conrmed by endoscopic biopsy. is is then
followed by an assessment of the feasibility of an
organ-sparing surgical resection. Since most BP
RMS are not amenable to complete resection at
the time of diagnosis, initial treatment consists
of chemotherapy – typically comprising vincristine, actinomycin, and cyclophosphamide
(VAC). Numerous alternative chemotherapeutic agents have been evaluated but none have
so far been shown to oer superior survival to
VAC – which is approximately 80%. Radiation
therapy is used primarily for local disease control in cases where the primary tumor is not
completely removed. e concept of “pre-treatment re-excision” refers to cases where aer
the initial biopsy, the tumor is re-excised before
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Figure 21.6 (A) Bladder ultrasound obtained during the initial evaluation of a suspected pelvic
mass revealing a large, heterogeneous mass near the base of the bladder. (B) Sagittal T2 weighted
magnetic resonance imaging of a patient with a large bladder/prostate rhabdomyosarcoma, which
has cranially displaced the Foley catheter. (C) Positron emission tomography (PET) computed
tomography of the same patient demonstrating PET-avid retroperitoneal lymphadenopathy.
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initiating chemotherapy. A number of regimens
incorporating dierent timing of radiation, chemotherapy, and surgery have been described. It
is important to note that residual tumor seen on
follow-up imaging aer chemoradiation therapy
may not be malignant tissue but may consist
of cells, which have dierentiated into mature
rhabdomyoblasts which do not require additional treatment.
Paratesticular Rhabdomyosarcoma
Treatment
●
Primary surgery
●
Chemotherapy
●
Radiation
Paratesticular RMS, unlike bladder/prostate
RMS, is designated as a favorable site and should
therefore be initially managed by radical surgery
whenever possible. is includes an ipsilateral
radical inguinal orchiectomy with high ligation
(at the internal inguinal ring) of the spermatic
cord (Figure 21.7A, B). Upon conrmation of the
diagnosis, patients with paratesticular RMS are
treated with combination VAC chemotherapy.
e decision to perform ipsilateral retroperitoneal lymph node dissection (RPLND) is based on
the patient’s age and imaging ndings. In children under 10 years of age this is limited to those
with suspicious lymph nodes on staging crosssectional imaging. Regardless of the presence or
absence of lymphadenopathy, ipsilateral retroperitoneal lymph node dissection should be performed in all patients aged 10 and over because of
the much higher incidence of occult lymph node
involvement in this age range. Radiation therapy
is reserved for patients with locally advanced disease, those with conrmed lymph node involvement and those whose orchidectomy had been
performed by a transcrotal approach
Vaginal/Uterine
●
Chemotherapy
●
Radiation
●
Surgery
Figure 21.7 (A) Paratesticular rhabdomyosar-
coma presenting as a solid scrotal swelling.
As in adults, exploration and orchidectomy
should be performed via an inguinal approach
and not by a scrotal incision. (B) Specimen
delivered through the inguinal incision prior to
removal. Penrose drain and clamps proximal
to the mass achieve vascular control of the
spermatic cord.
Gynecological RMSs are also classied as a favorable site and are usually managed with primary
chemotherapy followed by radiation. Surgical
intervention is typically limited to the initial
diagnostic biopsy, which is then followed by complete staging. Chemotherapy alone is curative in
less than 50% of patients but with the addition of
radiation therapy, the 5-year survival increases to
over 80%. Brachytherapy oers similar survival
outcomes to external beam radiation therapy but
with fewer short and long-term radiation side
eects.
Pre-pubertal primary testicular tumors / Initial Evaluation 295
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PRE-PUBERTAL PRIMARY
TESTICULAR TUMORS
Introduction
Primary testicular tumors comprise approximately 1–2% of all solid tumors in prepubertal
children. Although they aect 1 in 100,000 male
children, up to 75% of these tumors have little or
no malignant potential. e peak age of diagnosis is around 2–4 years of age with a second peak
in young adults. Common risk factors include; a
previous history of undescended testis, a family
history of testicular cancer, and a co-existing disorder of sex development (DSD) particularly one
that involves the Y- chromosome.
Initial Evaluation
Key points of the history and physical examination include whether the mass is symptomatic (a
painless mass is of greater concern), and pubertal
status. Scrotal ultrasound is the initial imaging
of choice (Figure 21.8). Careful attention should
be made to the appearance of the lesion on ultrasound, its location within or adjacent to the testis
(i.e. whether it is a paratesticular mass or a primary testicular mass), and the appearances of
contralateral testis. Multifocal tumors are more
indicative of malignancy.
Laboratory evaluation should always include
the standard serum tumor markers (STM) for testicular tumors, including alpha-fetoprotein (AFP),
beta-human chorionic gonadotropin (β-hCG),
and lactate dehydrogenase (LDH). However, AFP
may not be a reliable tumor market in the rst
8 months of life because of a physiological elevation of AFP occurring in this age range. If there is
suspicion that the lesion may represent a stromaltype tumor, additional hormonal evaluation is
indicated, including serum testosterone, estradiol,
and inhibin levels. e dierential diagnosis for a
newly discovered testicular mass is summarized
in Table 21.6.
Because of the relatively low incidence of met-
astatic disease associated with testicular tumors
in children, further evaluation with CT should
be used selectively. In asymptomatic children
Figure 21.8 Testicular ultrasound demonstrating a heterogeneous intratesticular mass suspicious
for a primary testicular malignancy.
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Table 21.6 Differential diagnosis for a newly
discovered testicular mass
Tumor Associations
Germ cell tumor Teratoma and yolk-sac
most common
Stromal tumors Precocious puberty
Epidermoid cyst Concentric rings on
ultrasound
Metastatic lesion Lymphoma
Gonadoblastoma Malignant precursor and
DSD
DSD, difference in sex development.
presenting with a primary testicular tumor it
can be deferred until aer surgery has been performed and the results of the nal pathology
evaluation are available. However, a preoperative CT scan to assess the total tumor burden is
indicated in children with marked elevation of
standard tumor markers or clinical evidence of
metastatic disease.
Staging
e staging employed by the children’s oncology
group diers from the one used for staging testicular tumors in adults (Table 21.7). is reects
the fact that the majority of prepubertal tumors
are benign and are conned to the testis. Aer
puberty, however, the staging is the same as for
adults with testicular tumors.
Treatment
●
Surgery
●
Chemotherapy
●
Radiation
Table 21.7 Children’s oncology group pediatric
testicular tumor staging
Stage Description
I Limited to testis, completely resected
No evidence of disease beyond testis
STM normal
II Microscopic residual disease
Elevated STM
Tumor rupture or scrotal biopsy prior
to orchiectomy
III Retroperitoneal lymph node
involvement
IV Distant metastasis
a
Lymph nodes >4 cm meet imaging criteria, lymph
nodes 2–4 cm need pathologic conrmation (i.e.
biopsy), and lymph nodes 1–2 cm have unclear signicance and may require additional evaluation
prior to completing staging.
a
or neurological involvement. An inguinal incision
should always be used for surgical exploration of
a primary testicular tumor in a pre-pubertal boy
because of the possible need to proceed to radical orchiectomy. If the diagnosis is uncertain and
preoperative tumor markers are normal, particularly in patients with small (<2 cm), unifocal
tumors, partial orchiectomy with intraoperative
frozen section is a reasonable approach. In these
cases, the use of intraoperative ultrasound can
be helpful in locating the margins of the tumor
prior to excision. If the frozen section raises suspicion of malignancy the surgeon can proceed
to perform a radical orchiectomy, whereas if the
pathology is more consistent with a benign process, the aected testis can be spared. e possible
requirement for further treatment (surgery, chemotherapy, or radiation therapy) is determined by
the pathology and tumor characteristics.
Germ Cell Tumors
e initial treatment of testicular tumors in children usually consists of either radical or partial
orchiectomy. Preoperative chemotherapy is limited to children in whom a delay in starting chemotherapy could be life threatening because they
have a large burden of disea se with pul monary and/
Teratoma
Teratomas are the most common benign testicular tumor in prepubertal children. ey consist of
a combination of the three germ-cell layers: endoderm, ectoderm, and mesoderm. Epidermoid
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cysts are considered part of this spectrum of
tumors but contain only a single germ-cell layer
and have the classic appearance of concentric
hyperechoic rings on ultrasound. Teratomas
are not usually accompanied by elevated serum
tumor markers. e denitive treatment is orchiectomy (either radical or partial) performed via
an inguinal incision.
Yolk-Sac tumor
Yolk-sac tumors (YST) are the most common
malignant testicular tumors in children. ey are
almost universally associated with elevated levels
of AFP and are characterized by the histologic
nding of Schiller-Duval bodies on microscopy.
Ninety percent of YSTs present as Stage I disease
and are managed by radical orchiectomy followed by monitoring serum AFP levels and chest
and abdominal imaging at increasing intervals.
Patients who present with stage II–IV disease
(or stage I patients who experience a recurrence
aer orchiectomy) are treated with bleomycin,
etoposide, and cisplatin chemotherapy. In addition, patients with retroperitoneal lymph node
involvement should undergo formal radical
lymph node dissection.
Stromal Tumors
Leydig and Sertoli cell tumors
Leydig cell tumors (LCT) are the most common
stromal tumor in childhood and are oen associated with elevations in serum testosterone, which
can lead to precocious puberty. Histologically
they are dened by the presence of Reinke crystals, which are pathognomonic for LCTs. In prepubertal patients these tumors are universally
benign and complete surgical excision is considered curative. Sertoli cell tumors (SCT), are the
second most common testicular stromal tumor
and may be may be associated with gynecomastia in the postpubertal age range. ey are benign
tumors with no reported cases of metastasis in
children. A variant of Sertoli cell tumor can occur
in patients with Peutz-Jeghers syndrome. Because
they are benign tumors, Leydig and Sertoli cell
tumors in children can be managed initially by
testicular sparing surgery (partial orchiectomy)
with intraoperative frozen section.
Juvenile granulosa cell tumors
Juvenile granulosa cell tumors (JGCT) are almost
exclusively seen in the rst year of life and can
even be present at birth. ese tumors are hormonally inactive, benign tumors that are oen
associated with Y-chromosomal abnormalities.
In addition to surgical excision, a karyotype
should be performed to rule out a concomitant
genetic disorder.
KEY POINTS
●
Molecular proling is likely to play a
key role in risk-stratication and the
design of targeted therapy for children
with renal tumors and genitourinary
rhabdomyosarcoma.
●
e overall survival of children with
genitourinary malignancies is improving dramatically as a result of the
introduction of multimodal treatment
(surgery, chemotherapy, and radiation therapy). e current challenge
is to reduce the burden of treatment –
related morbidity without compromising survival rates.
●
Prepubertal testicular tumors are
not usually as aggressive as testicular
tumors in adults. Most cases can be
managed with surgery and observation
alone.
FURTHER READING
Saltzman, A.F., Cost, N.G., 2018. Childhood kid-
ney tumors. American Urological Association
Update Series 37, 187–195.
Husmann, D.A., 2019. Cancer screening in the
pediatric cancer patient: a focus on genitourinary malignancies, and why does a urologist need to know about this? Journal of
Pediatric Urology 15, 5–11.
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Gooskens SL, Houwing ME, Vujanic GM, Dome
JS, Diertens T, Coulomb l’Herminé A, et al,
2017. Congenital mesoblastic nephroma
50 years after its recognition: a narrative
review. Pediatric Blood & Cancer 64, e26437.
Malempati, S., Hawkins, D.S., 2012.
Rhabdomyosarcoma: review of the children’s
oncology group (cog) soft-tissue sarcoma
committee experience and rationale for current COG studies. Pediatric Blood & Cancer
59, 5 –10.
Ross, J.H., Kay, R., 2004. Prepubertal testis
tumors. Rev Urol 6, 11–18.
Pediatric Genitourinary Trauma
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DAVID J CHALMERS
Topics covered
22
General evaluation
Renal trauma
Ureteric injuries
INTRODUCTION
Trauma is the leading cause of mortality in children
and young people. Management principles generally
mirror those of adult trauma, which has the benet
of more evidence-based guidelines. However, there
are some key dierences. Renal injury is most frequently caused by blunt force – oen in conjunction
with multisystem organ trauma. With some important exceptions, all but the most severe renal injuries
are generally managed non-operatively. Ureteral
injuries can occur from penetrating trauma or iatrogenic injury during surgery in the retroperitoneum or pelvis. Bladder and urethral injuries are
usually associated with other severe injuries involving pelvic fractures and carry the greatest potential
risk of long-term morbidity. Isolated external genital trauma, depending on the history, should always
trigger suspicion of possible sexual abuse. While
pediatric urologists rarely have prime responsibility
for the evaluation and treatment of pediatric trauma
patients, they are relied upon heavily as consultants.
Bladder injuries
Urethral injuries
Injuries to the external genitalia
GENERAL EVALUATION
History
Evaluation begins by obtaining a comprehensive
history from the patient, family or consulting
clinician, followed by a physical examination if
possible. Information should be sought on the
mechanism of injury, associated injuries and
any signicant comorbidities. It is important to
enquire about the presence of hematuria and to
look for evidence of abdominal or ank tenderness, rib fractures, and contusions or abrasions to
the abdomen, pelvis, or ank. Penetrating injuries
to the torso, multisystem trauma and signicant
hematuria always warrant further investigation.
It is important to note, however, that genitourinary injuries associated with multisystem trauma
are not always accompanied by hematuria. For
this reason, the absence of hematuria should not
preclude further investigation if other indications
are present.
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Physical Examination
Hemodynamic parameters are not reliable indicators of acute shock in children. Tachycardia
may be a response to pain or anxiety and the
cardiovascular system in children is capable
of compensating more eectively for signicant blood loss through vasoconstriction and
increased cardiac output. Signs of acute blood
loss, anemia, and/or hypovolemia are indicators
of advanced hemodynamic instability and even
if the other vital signs are reassuring the clinician should nevertheless proceed with further
evaluation and decisions on management. e
physical examination should focus on the abdomen and genitalia. A ank mass or ecchymosis
suggests perinephric hematoma – which may
also be associated with urinary extravasation.
Ecchymosis of the perineum in a buttery pattern is suggestive of blood tracking within Colles
Fasica while scrotal or labial hematoma can
result from genital trauma or pelvic injury. e
presence of blood at the urethral meatus or urinary retention should raise suspicion of urethral
injury or disruption.
Figure 22.1 Grade 4 renal injury in a kidney
with previously undiagnosed ureteropelvic
junction obstruction. This occurred following
a fall from playground monkey bars. There
were no other injuries and the injury failed
to improve with conservative management.
Ultimately, a nephrectomy was performed.
RENAL TRAUMA
e kidney is the commonest site of injury in the
genitourinary system. e majority of renal injuries result from blunt trauma and approximately
80% are accompanied by injuries to other organs.
Males outnumber females by almost 2:1, with the
commonest causes being motor vehicle collisions,
trac accidents and falls. Contact sports are an
uncommon cause of serious renal injury in this
age group.
It is thought that the kidney is more susceptible to blunt trauma in children than adults
because it is proportionately larger and is less
well protected by the ribs. Children also have less
retroperitoneal fat and less musculature to buer
and protect the kidneys. Horseshoe kidneys and
hydronephrotic kidneys are at particularly high
risk of injury even in the absence of associated
organ injuries (Figure 22.1).
Evaluation and Investigations
Because hemodynamic signs are a less reliable
guide to the severity of injury in children, greater
reliance must be placed on the clinical history,
physical examination, and the presence of associated injuries.
e indications for radiographic evaluation
include;
●
Blunt trauma resulting from signicant deceleration, such as a high-velocity motor vehicle
collision or fall
●
Blunt trauma leading to other signicant
multi-organ injuries
●
Penetrating injury to the abdomen or ank
●
Gross macroscopic hematuria or signicant
microscopic hematuria
Haematuria is a much less reliable guide to
the severity of renal trauma than in adults and
>50% of children who have sustained a renal
injury may not have hematuria at the time of
presentation.
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Ultrasound is a reasonable rst-line investigation if the child is clinically stable and the history and ndings on examination do not arouse
concern. However, the accuracy of ultrasonography in detecting grade III renal lacerations is
only 60% and is even lower in the detection of
low grade renal injuries. Any evidence of renal
injury detected by ultrasonography should then
prompt a computerized tomography ( CT ) scan
for a more detailed anatomical and functional
assessment.
Contrast-enhanced CT with delayed imaging
is the cornerstone of modern staging of blunt renal
injuries. e A merican Associat ion for t he Su rgery of
Trauma (AAST) organ injury scale for renal trauma
(Figure 22.2) has been widely adopted to characterize renal injuries and guide their management.
Grade I injuries are dened by decreased
uptake of contrast material or subcapsular
hematoma. ey are essentially renal contusions
and account for 80% of renal trauma cases.
Grade II and III injuries are tears in the renal
parenchyma. Tears <1 cm are considered grade
II and >1 cm as grade III. ey are classied as
minor if they are limited to the renal parenchyma
and do not extend into the collecting system.
Grade II and grade III injuries may be associated
with extensive perinephric hematomas, but not
urinary extravasation.
Grade IV injuries are lacerations, which also
involve the collecting system. Grade IV injury
may also consist of damage to the hilar vessels –
typically as a consequence of rapid deceleration
causing shearing damage to the vessel wall, intramural haemorrhage, vascular occlusion, and clot
formation. In such cases, renal perfusion in the
injured kidney may be compromised even if the
parenchyma remains intact.
Figure 22.2 American Association for the Surgery of Trauma (AAST) renal trauma classication.
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