Pediatric Germ Cell Tumors

Germ cell tumors develop from cells that normally form eggs or sperm. They can be benign or malignant and occur in the ovaries, testes, or outside the reproductive organs — such as at the base of the spine, in the chest or in the brain.

Types of germ cell tumors

• Teratomas — mature (usually benign) or immature
• Germinomas — called dysgerminoma in the ovary and seminoma in the testis
• Yolk sac tumors — the most common malignant type in young children
• Embryonal carcinoma and choriocarcinoma — rarer types
• Mixed germ cell tumors

Extragonadal germ cell tumors

Tumors outside the ovaries and testes — for example sacrococcygeal teratoma in newborns, mediastinal (chest) tumors in teenagers, and intracranial germ cell tumors.

Causes and risk factors

• Undescended testes
• Some genetic conditions, such as Klinefelter syndrome and disorders of sex development
• Most cases have no known cause

Symptoms

• A lump or swelling in the testis, abdomen or lower back
• Abdominal pain (especially with ovarian twisting)
• Constipation or difficulty passing urine
• Cough or breathlessness (chest tumors)
• Early puberty, headaches or vision changes (brain tumors)

Prognosis

Most children with malignant germ cell tumors are cured with surgery and platinum-based chemotherapy.

Tumor markers in the blood, imaging and pathology together confirm the diagnosis and stage.

Tumor markers

Alpha-fetoprotein (AFP) and beta-hCG are measured in the blood; they help confirm the tumor type and are followed to monitor treatment.

Imaging

• Ultrasound of the abdomen, pelvis or testes
• CT or MRI of the primary site and the chest
• MRI of the brain and spine for intracranial tumors

Surgery and biopsy

The tumor is often removed at diagnosis and examined by a pathologist; in some situations a biopsy comes first.

Staging

Staging describes whether the tumor was completely removed, whether tumor markers return to normal, and whether there is spread to lymph nodes, lungs or other organs.

Treatment depends on the tumor type, site and stage.

Surgery

Complete removal while preserving fertility whenever possible. Some low-stage tumors need surgery only, followed by close monitoring of tumor markers.

Chemotherapy

Platinum-based combinations (such as cisplatin or carboplatin with etoposide and bleomycin) for malignant tumors that are more advanced or come back.

Radiation therapy

Mainly for intracranial germinomas, which are very sensitive to radiation.

High-dose chemotherapy with stem cell transplant

An option for relapsed tumors.

Treatment side effects and follow-up care

Regular tumor marker tests and scans, and checks on hearing, kidney function and fertility.

Our pediatric oncologists and surgeons work together to treat germ cell tumors.