Extracranial Germ Cell Tumor: Causes and Treatment

Extracranial germ cell tumor (EGCT) refers to the abnormal growth of germ cells in the gonads and other areas of the body except for the brain. The majorly affected regions are the testes, ovaries, tissues, and lymphatic and circulatory systems. This tumor can be benign or malignant and is common in adolescents. Read Extragonadal Germ Cell Tumors: Types, Causes, and Risk Factors

Malignant childhood EGCTs are classified as gonadal tumors and extragonadal tumors. The former mainly affects the testes (in males) and the ovaries (in females). Extragonadal germ cell tumors are frequently seen along the midline of the body. This includes the sacrum, coccyx, mediastinum, back of the abdomen, and neck. EGCTs are grouped into three classes, namely:

Teratomas are a type of germ cell tumor comprising tissues like hair, muscle, and bones. Depending on the cellular morphology, they are subdivided into –
Mature teratomas – The cells resemble normal cells but may release hormones or enzymes that manifest the signs of EGCT. It usually occurs in the testes or ovaries at the start of puberty.
Immature teratomas – In this type, abnormal cellular morphology is seen. It occurs in areas other than the gonads and can metastasize to other body parts.
Malignant germ cell tumors are malignant and can be found anywhere in the body. They are collectively called seminomatous germ cell tumors when they affect the testes, ovaries, or mediastinum. In contrast, when such tumors are seen in the pineal gland, abdomen, or other body parts, they are called nonseminomatous germ cell tumors.
Mixed germ cell tumors comprise at least two types of malignant germ cell tumors. They can form in the ovaries, testes, or other body parts. Read more The Latest Research on Germ Cell Tumors and Prognosis

Causes of Extracranial Germ Cell Tumor

The exact etiology behind EGCT remains unknown, but some risk factors that contribute to the development of EGCT have been identified. These include:

· Genetic syndromes like Klinefelter syndrome, Swyer syndrome, and Turner syndrome
· Having an undescended testicle
· Having gonadal dysgenesis
· Having cryptorchidism
· Smoking
· Alcohol consumption

Symptoms of EGCT

The symptoms of EGCT are seen in children, adolescents, and young adults. The main symptoms include:

Fever
· Constipation
· Abnormal bleeding in the vagina
· No menstrual periods in females
· Lump in the testes in males
· Lumps along the midline of the body, including the coccyx, neck, and abdomen
· Respiratory distress
· Chest pain
· Cough
· Trouble urinating
· Early puberty

Screening tests

The diagnosis begins with a general physical examination enabling the doctor to understand the symptoms better. If a malignancy is suspected, the physician recommends additional screening tests for EGCT. These include:

Serum tumor marker test: A blood sample is tested to measure the levels of certain substances called tumor markers—the presence of tumor markers like alpha-fetoprotein and beta-human chorionic gonadotropin help to detect EGCT.
Imaging tests: Imaging tests like X-ray, MRI, and CT scans are done to produce images of the area where the tumor is suspected. This help to determine the location of the tumor and identify its extent of growth. They also assist the physician in identifying if the tumor has spread to other body parts.
Biopsy: In this procedure, a small sample of the abnormal tissue from the tumor is removed and examined by a pathologist. This helps to distinguish the type of tumor cells present in the malignancy.
Bone scan: A small amount of radioactive material is injected intravenously, and the entire body is scanned. This material gets deposited in bones with cancer, making it easier to identify whether there are any rapidly dividing cells in the bones.

Treatment of Extracranial Germ Cell Tumor

The following approaches treat EGCT:

Chemotherapy – This multimodality treatment is used in patients to shrink tumors and kill metastasized tumor cells. The commonly employed chemotherapeutic drug is BEP, a combination of cisplatin, etoposide, and bleomycin. High-dose chemotherapy with stem cell rescue is considered a treatment option for recurrent EGCT.
Surgery – Surgery is done to remove the tumors whenever possible altogether. If the tumors are large, chemotherapy is initially utilized to shrink the tumor. Resection, Radical inguinal orchiectomy, and unilateral salpingo-oophorectomy are the main surgical procedures used to treat EGCT.
Radiation therapy – High-powered beams such as X-rays or protons kill cancer cells. It is used for treating progressive or recurrent EGCTs.