Pediatrics - Testicular Tumors In Children & Adolescents

Front 1

Are mixed germ cell tumors the most common testicular tumor type in all age groups?

Back 1

*No, in pre-pubertal males virtually all of the germ cell tumors are of one tissue type.

Front 2

Are teratomas more or less worrisome in pre-pubertal males compared to adolescents or adults?

Back 2

*Even less worrisome.

Front 3

Are most stromal testicular tumors (i.e Leydig cell or Sertoli Cell) benign or malignant?

Back 3

*Benign, although occasionally especially in older patients they may behave in a malignant fashion.

Front 4

What is the most common germ cell tumor in children?

Back 4

*Teratoma, and remember they are almost universally benign in children as opposed to the malignant behavior that they can manifest in adults.

Front 5

What is the most common malignant tumor of the testicle in children?

Back 5

*That is yolk sac tumor. again teratome is the most common germ cell tumor but if behaves almost universally in a benign fashion.

Front 6

What percentage of testicular tumors in pre-pubertal patients are benign?

Back 6

*75%

Front 7

What is the risk of developing testicular cancer in a man that had an undescended testicle?

Back 7

More recent studies have found that the risk of
testicular cancer in a man with a history of UDT is closer to 5-
fold that of the general population, which translates into a lifetime
risk for these men of 1% to 2%.

Front 8

Can early orchiopexy ameliorate the risk of testicular cancer?

Back 8

Whether early orchiopexy can
ameliorate that risk is unclear, although a recent study suggests that it may.

Petterson A, Richiardi L, Nordenskjold A et al: Age at surgery
for undescended testis and risk of testicular cancer. N Engl J Med
2007; 356: 1835.

Front 9

What risk does CIS pose for future development of a germ cell tumor?

Back 9

CIS generally portends a 50% risk of germ cell tumor development
at 5 years. Because of the increased risk of these findings, some
authors have suggested performing a testis biopsy in young men with
a history of cryptorchidism to look for CIS, particularly in those with
other risk factors such as subfertility or microlithiasis.

Front 10

Is testicular microlithiasis a risk factor for testicular cancer?

Back 10

Data are insufficient to quantify the risk, if any, of
testicular cancer in boys or men incidentally found to have TM.
Studies suggest that TM may be most significant when it occurs in conjunction with other risk factors for testicular cancer.

*In a study of subfertile (a known risk factor for testicular cancer) men 20% with TM had CIS on biopsy vs 0.5% without TM.22 In another study of men with a history of orchiectomy for testicular cancer 78% with TM in the remaining testicle had CIS vs only 11% without TM.

Front 11

Do all patients with disorders of sexual development have an increased risk of testicular cancer?

Back 11

However unique to patients with sexual development
disorders is the risk of tumor formation in dysgenetic or streak gonads, which seems to exist almost exclusively in patients with Y
chromatin.

Front 12

You are seeing a patient in your clinic with Turner syndrome and the mother asks you if there is any risk that her son might be at increased risk for testicular cancer?

Back 12

Patients with traditional Turner syndrome and a 45 XO
karyotype are at no significant risk for gonadal tumors. However patients
with Turner syndrome and any portion of a Y chromosome in
their karyotype have an approximately 12% risk of tumor development,
despite the fact that the gonads are histologically indistinguishable
from those of patients with traditional Turner syndrome.

Similarly the gonads of patients with pure gonadal dysgenesis and Y chromatin as well as the streak gonad of those with mixed gonadal dysgenesis have a 15% to 30% risk of tumor development.

Front 13

A patient with mixed gonadal dysgenesis presents to your office. She has a history of a karyotype showing 45X/46XY. Is there any concern in this patient?

If she were to have a malignancy in a streak gonad what would it most likely be?

Back 13

The tumors arising in the setting of gonadal dysgenesis are usually gonadoblastomas.

While gonadoblastomas are benign, they are prone to malignant degeneration and overt malignant behavior is
seen in 10% of cases.

Although malignancy usually occurs after puberty, there have been reports in children as well. The dysgenetic gonads should be removed early in life in patients with at risk karyotypes.

Front 14

For patients with gonadoblastoma what is the most likely histology?

Back 14

Dysgerminoma (seminoma when it occurs in a testis) is
the most common histological malignancy.

Front 15

What is the presentation for the majority of testicular tumors?

Back 15

The majority of testis tumors present as a palpable testicular mass.

Front 16

A 4 year old boy presents to your clinic with a hydrocele. The history and physical examination are not consistent with a hernia as the fluid doesn't reduce and the history is negative for changes in size of the fluid collection. You think that this is most likely a hydrocele. Would you do any further studies before performing a hydrocelectomy in this patient?

Back 16

A minority of patients may present with a hydrocele, and
boys presenting with hydroceles that obscure physical examination of
the testis should undergo scrotal ultrasound

Front 17

You are asked to consult on a 1 month old child with a testicular mass and an elevated AFP. The requesting physician states that this is definantly a malignant yolk sac tumor and should be removed emergently, what is your response?

Back 17

Since yolk sac tumors do
not elaborate HCG, AFP is the only important tumor marker in
prepubertal patients (elaborated by 90% of yolk sac tumors in children).

An important caveat in the use of AFP for these patients
is that serum AFP is normally high in infancy and does not decrease to normal adult levels until nearly age 1 year.

Therefore, while an elevated AFP in a child older than 1 year with a testis tumor
almost always reflects the presence of a yolk sac tumor, an elevated level in infants can occur in the setting of a benign tumor.

Front 18

Why is there merit to the argument of partial orchiectomy in pre-pubertal children with testicular masses?

What scenario precludes a testis sparing option in this patient population?

Back 18

Tumor
excision without orchiectomy is particularly attractive in prepubertal
patients because most tumors are benign in this population.
An elevated AFP level in a child older than 1 year virtually
always reflects the presence of a yolk sac tumor and precludes a
testis sparing approach. However, in all infants, older children
with a normal AFP and boys presenting with androgenization the
likelihood of a benign tumor is considerable.

Front 19

How is teh testis sparing approach performed?

Back 19

For a testis sparing approach, the testis is delivered into the inguinal
incision (the cord having been occluded with a non-crushing clamp or
vessel loop), the field is draped off with towels and the tunica vaginalis
is opened. The tumor is excised or enucleated and sent for frozen
section. If a benign histology is confirmed, the testicular defect is
closed with absorbable suture and the testis is returned to the scrotum.
If a malignancy is detected or the frozen section is non-diagnostic,
orchiectomy is performed. Reports of small series suggest that this
approach is safe and effective for preserving testicular tissue.

Front 20

What is the role for RPLND for testicular cancer in children?

Back 20

*Retroperitoneal surgery has
been reserved for biopsy of radiographically equivocal nodes or for resection of a persistent retroperitoneal mass following chemotherapy.

Finally, the morbidity of RPLND is likely to be greater in children
than in adolescents and adults. Children are particularly prone to bowel
obstruction following laparotomy, and the feasibility of a nerve sparing
approach to RPLND for testis cancer in children is questionable with
no long-term functional data available.

Front 21

What is the appropriate management for stage one yolk sac tumors in children?

Back 21

In all of these
studies stage 1 tumors were managed with orchiectomy followed by
surveillance. Surveillance includes frequent physical examinations,
radiographic evaluation of the chest and retroperitoneum, and
serum tumor marker measurement.

Front 22

How were children with metastatic testicular cancer, locally advanced disease, or persistantly elevated tumor markers after orchiectomy with yolk sac tumor treated in most trials and what is the surivavl rates?

Back 22

Metastatic disease was treated
with 2 to 4 courses of multiagent platinum-based chemotherapy.
Patients who presented with locally advanced disease, metastases
or persistently elevated serum tumor markers were similarly
treated with multiagent platinum-based chemotherapy. The survival
of all patients treated with this approach is nearly 100%.

Front 23

How is teratoma treated in children?

Back 23

Teratomas are universally benign in prepubertal patients,
while clinical metastases are seen in up to 60% of adults.39-41
Because of its benign nature, prepubertal teratoma can be managed with tumor excision alone.

Front 24

What is an epidermoid cyst?

Back 24

Epidermoid cysts probably represent a monodermal variant of teratoma.
Epidermoid cysts are composed entirely of keratin producing
squamous epithelium, and are universally benign in children and
adults. They may be treated by tumor enucleation with no oncological
evaluation or follow-up.

Front 25

How should you treat the post-chemo mass in children with testicular cancer if the tumor markers have normalized?

Back 25

Some patients treated with chemotherapy for metastatic disease
will have a residual retroperitoneal mass following therapy. If
tumor markers have normalized, these masses should generally be
resected. Although 40% to 50% will contain only necrotic tissue
and fibrosis, 10% to 20% will have persistent malignancy and 40%
to 45% will have mature teratoma in the mass.