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

•This can be detected by the patient, a parent, or by a physician on routine physical examination.

•Occasionally the patients present with a hydrocele.

•About 10–25 % of patients with a malignant tumors present with a hydrocele.

•Therefore, if a child presents with a hydrocele that clearly obscures physical examination of the testis, an ultrasound should be done to exclude an associated tumor.

•Rarely, patients may present with testicular pain.

•Signs related to metastatic disease are uncommon in children as the most common sites – the retroperitoneum and lungs – rarely result in physical findings.

•The most common presenting symptom in a patient with seminoma is a painless testicular mass. Other symptoms can include testicular pain (45 %) or heaviness.

•A history of previous testicular trauma is common. The trauma typically draws the patient’s attention to the mass and not a cause.

•Seminoma that has spread to retroperitoneal lymph nodes can cause back pain or abdominal discomfort.

•Widely disseminated metastatic disease to lungs, liver, bone, or brain is rare but may produce systemic symptoms.

•A history of cryptorchidism or other genitourinary anomalies can be elicited in some patients.

•Once a testis tumor is suspected, a thorough physical examination is undertaken.

•Physical examination usually reveals a painless scrotal swelling with a hard mass or associated hydrocele.

•Some hormonally active tumors may appear in association with precocious puberty or gynecomastia.

•A hard mass may be palpable on physical examination. However, normal physical findings are not sufficient to exclude a tumor.

•In most cases the general examination will be normal.

•But occasionally signs of androgenization or other physical findings will be present suggesting a particular tumor type or advanced disease.

27.6Staging

•In the Children’s Oncology Group staging system:

•Stage I:

–Patients with locally confined disease, negative radiographic studies, and the expected decline in tumor markers post-operatively.

•Stage II:

–Patients with microscopically positive margins in the scrotum or spermatic cord and/ or with persistently elevated tumor markers after orchiectomy. Patients who underwent transcrotal biopsy prior to orchiectomy at a separate setting are also considered stage 2.

•Stage III:

–Patients with retroperitoneal lymphadenopathy.

•Stage IV:

–Patients with distant metastases (most commonly in the lungs).

•Adolescents with germ cell tumors are generally staged as adults utilizing the TNM system of the American Joint Committee on Cancer and the International Union Against Cancer.

The Children’s Oncology Group Staging System

•Stage I: Patients with locally confined disease, negative radiographic studies, and the expected decline in tumor markers post-operatively.

•Stage II:

–Patients with microscopically positive margins in the scrotum or spermatic cord and/or with persistently elevated tumor markers after orchiectomy.

–Patients who underwent transcrotal biopsy prior to orchiectomy at a separate setting.

•Stage III: Patients with retroperitoneal lymphadenopathy.

•Stage IV: Patients with distant metastases (most commonly in the lungs)

•Testicular seminoma is staged according to the American Joint Committee on Cancer (AJCC) 2010 staging guidelines.

•This is a TNM staging system comprising separate categorizations for the primary tumor, regional lymph nodes, distant metastases, and serum tumor markers.

•These four categories are used to determine the stage of the patient’s disease.

•Modern treatment decisions are based, in part, on the subdivisions of this staging system.

•Primary tumor staging

–Tis – Intratubular germ cell neoplasia (carcinoma in situ)

–T1 – Tumor limited to testis/epididymis without vascular or lymphatic invasion; tumor may invade into the tunica albuginea but not the tunica vaginalis

–T2 – Tumor limited to testis/epididymis with vascular or lymphatic invasion or tumor extending through tunica albuginea with involvement of the tunica vaginalis

–T3 – Tumor invading spermatic cord with or without vascular/lymphatic invasion

–T4 – Tumor invading scrotum with or without vascular/lymphatic invasion

The Intergroup Staging System for

Testicular Germ Cell Tumors

•Stage I: Tumor limited to the testis and completely resected (85 % of children <4 years present with stage I disease, whereas only 35 % of adults with testicular tumors present with stage I)

•Stage II: Tumor removed by transscrotal orchiectomy, involvement of scrotum or spermatic cord, persistently elevated tumor markers.

•Stage III: Retroperitoneal lymph node involvement (≤2 cm, no visceral or

extra-abdominal involvement)

•Stage IV: Distant metastases

27.6.1 Regional Lymph Node Staging

•N0 – No regional lymph node metastases

•N1 – Metastasis with lymph node(s) 2 cm or less in greatest dimension or multiple lymph nodes, none more than 2 cm in greatest dimension

•N2 – Metastasis with lymph node(s) larger than 2 cm but not larger than 5 cm in greatest dimension, or multiple lymph nodes, any one mass larger than 2 cm, but not more than 5 cm, in greatest dimension

•N3 – Metastasis with lymph node(s) larger than 5 cm in greatest dimension

•Distant metastatic staging

–M0 – No distant metastases

–M1a – Nonregional nodal or pulmonary metastasis

–M1b – Distant metastases other than M1a

•Serum tumor marker staging

–S0 – Marker studies within normal limits

–S1 – LDH level less than 1.5 times the reference range, beta-hCG level less than 5000 mIU/mL, and AFP level less than 1000 ng/mL

–S2 – LDH level 1.5–10 times the reference range, beta-hCG level 5000–50,000 mIU/ mL, or AFP level 1000–10,000 ng/mL

–S3 – LDH level more than ten times the reference range, beta-hCG level more than 50,000 mIU/mL, or AFP level more than 10,000 ng/mL

Children’s Oncology Group Staging System for Yolk Sac Tumors

•Stage I:

–Limited to testis

–Completely resected by high inguinal orchiectomy

–Tumor markers negative

–Unknown tumor markers at diagnosis

–Need negative ipsilateral retroperitoneal lymph node biopsy if >2 cm on CT

•Stage II:

–Microscopic residual disease

–Tumor markers remain elevated

–Tumor rupture or scrotal biopsy prior to complete orchiectomy

•Stage III:

–Retroperitoneal lymph node involve987`ment

–(>4 cm on CT)

–RPLN <4 cm, but >2 cm need biopsy

•Stage IV: Distant Metastasis