Extragonadal Germ Cell Tumors: Types, Causes, and Risk Factors

Germ cell tumors are typically associated with the reproductive organs, but they can also develop outside of these areas. These extragonadal germ cell tumors can occur in various parts of the body, and their causes and risk factors may differ from those of their gonadal counterparts. Learn more about this rare type of cancer and its potential treatments.

Extragonadal germ cell tumor is defined as the malignant formation of tumors by the gonadal cells outside the primary reproductive organs. It usually refers to the abnormal proliferation of the cells meant to form sperm (in testicles) or eggs (in ovaries) anywhere in the body except the gonads. These tumors generally grow in the pineal gland (in the brain), in the mediastinum (area between the lungs), or in the retroperitoneum (back wall of the abdomen). Based on their malignancy, these tumors are categorized into:

• Teratomas are extremely rare, benign germ cell tumors often seen in the pelvis and abdomen. Teratomas are large and more common than malignant germ cell tumors.
• Malignant germ cell tumors – Cancerous germ cell tumors come under this class. They are further subdivided into:
• Non-seminomas – These are large, lobulated, and heterogenous tumors predominantly present in the yolk sac. The most common sites of this tumor metastasis are the lungs, lymph nodes, brain, and bones.
• Seminomas – These are bulky lobulated homogenous tumors that rarely undergo metastasis. Although local invasion is uncommon, it may sometimes spread to the lymph nodes. Read more about The Latest Research on Germ Cell Tumors and Prognosis

Causes

The exact etiology behind extragonadal germ cell tumors remains unknown. Still, some risk factors that contribute to the development of this tumor have been identified. These include:

• · Klinefelter syndrome is associated with an increased risk of germ cell tumors in the chest.
• · Being male
• · Being 20 years of age or older
• · Smoking
• · Alcohol consumption

Symptoms

Extragonadal germ cell tumor symptoms are seen in children, adolescents, and young adults. The main symptoms include:

• · Fever
• · Constipation
• · Headache
• · Fatigue
• · Respiratory distress
• · Chest pain
• · Unsteady gait
• · Trouble in seeing or moving the eyes
• · Cough
• · Trouble urinating Read about Prostate Cancer: What are the Symptoms and Treatment?

Symptoms and diagnosis of extragonadal germ cell tumors

The symptoms of extragonadal germ cell tumors can vary depending on the location of the tumor. Common symptoms include pain, swelling, and a mass or lump in the affected area. Diagnosis typically involves imaging tests such as CT scans or MRIs, as well as biopsy to confirm the presence of cancerous cells. It is important to seek medical attention if you experience any concerning symptoms or have a family history of germ cell tumors.

Screening tests

The diagnosis begins with a general physical examination enabling the doctor to understand the symptoms better. The physician recommends additional screening tests for extragonadal germ cell tumor if a malignancy is suspected. These include:

Treatment of Extragonadal Germ Cell Tumors

The following approaches treat extragonadal germ cell tumors:

• Chemotherapy – This multimodality treatment is used in patients to shrink tumors and kill metastasized tumor cells. The commonly employed chemotherapeutic drug is the BEP, a combination of cisplatin, etoposide, and bleomycin.
• Surgery – Surgery is done to remove benign tumors whenever possible altogether. In large tumors, chemotherapy or radiation therapy is preferred to shrink the tumors. Once this is completed, surgical resection is recommended to remove the chemotherapy-resistant disease and reduce tumor remission.
• Radiation therapy – High-powered beams such as X-rays or protons are used to kill the cancer cells. It is used for treating progressive or recurrent extragonadal germ cell tumors.