Ovarian Germ Cell Cancer Stage IV

Stage IV ovarian germ cell cancer is the most advanced form of this rare cancer, where the disease has spread from the ovaries to distant organs like the liver or lungs. Though this represents a serious diagnosis, treatment options including surgery and chemotherapy can help manage the disease and improve outcomes.

Table of contents

• What is ovarian germ cell cancer stage IV?
• Associated anatomy
• Signs and symptoms
• Diagnosis
• Treatment options
• Outlook and survival

What is ovarian germ cell cancer stage IV?

Ovarian germ cell tumors develop from reproductive cells called germ cells inside the ovaries. These are the cells that eventually mature into eggs. When these cells undergo abnormal changes and form a cancerous mass, the result is a malignant ovarian germ cell tumor^[5].

Stage IV is the most advanced stage of ovarian germ cell cancer. At this stage, the cancer has spread to distant parts of the body, such as the liver or lungs. Stage IV is divided into two groups: Stage IVa means the cancer has caused fluid buildup in the lining of the lungs, called a malignant pleural effusion. Stage IVb means the cancer has spread to the inside of the liver or spleen, lymph nodes outside the abdomen, or other organs such as the lungs^[4].

Ovarian germ cell tumors are rare, accounting for only 2% to 3% of all ovarian cancers. They most commonly affect teenage girls and young women, usually occurring in people between 10 and 30 years old. Unlike other types of ovarian cancer, these tumors most often affect just one ovary^[2][5].

The most common type of malignant ovarian germ cell tumor is called dysgerminoma. Other types include immature teratoma, yolk sac tumors (also called endodermal sinus tumors), mixed germ cell tumors, and rarer forms such as embryonal carcinoma, choriocarcinoma, and polyembryoma^[2][7].

• Ovaries
• Fallopian tubes
• Uterus
• Pelvis

Signs and symptoms

Ovarian germ cell tumors can be hard to diagnose early because there are often no symptoms in the early stages. However, by the time the cancer reaches stage IV, symptoms are more likely to appear^[5][6].

Common symptoms to watch for include a swollen abdomen without weight gain in other parts of the body. This swelling may be accompanied by abdominal pain, discomfort, or tenderness that can start suddenly and be severe. Some people experience a feeling of fullness or bloating in their belly^[2][6].

Other symptoms include bleeding from the vagina after menopause (when menstrual periods have stopped), or irregular vaginal bleeding. Changes in bowel habits such as diarrhea or constipation may occur. Some people notice changes in their eating habits, like loss of appetite, or experience nausea and vomiting^[2][6].

These tumors can sometimes release hormones that cause changes in the body, including symptoms that resemble early pregnancy such as fatigue, nausea, or breast tenderness. In rare cases, they may cause signs of early puberty^[2].

Diagnosis

To diagnose ovarian germ cell cancer and determine its stage, doctors use several tests that examine the ovaries, pelvic area, blood, and ovarian tissue^[5][6].

The diagnostic process typically begins with your doctor asking about your personal and family health history and performing a physical exam. A pelvic exam is an important part of the evaluation. During this exam, a doctor inserts a tool called a speculum into the vagina to look at the vagina and cervix for signs of disease. The doctor also inserts gloved fingers into the vagina while pressing on the lower abdomen with the other hand to feel the size, shape, and position of the uterus and ovaries. A finger may also be inserted into the rectum to feel for lumps or abnormal areas^[6].

Blood tests are crucial for diagnosis because germ cell tumors often produce chemicals or hormones called tumor markers that doctors can measure in the blood. These tests help identify the presence and type of tumor^[7].

Imaging tests play a key role in diagnosing and staging the cancer. An ultrasound uses sound waves to create pictures of the inside of the abdomen. A CT scan (computed tomography scan) creates detailed three-dimensional images of the body. These scans help doctors see where the cancer has spread. A chest X-ray may be performed to check for cancer spread to the lungs. An MRI scan (magnetic resonance imaging) may also be used to get detailed images of the body’s soft tissues^[7].

Often, the exact stage of cancer cannot be confirmed until after surgery, when doctors can directly examine how far the cancer has spread^[4].

Treatment options

The main treatments for stage IV ovarian germ cell cancer include surgery and chemotherapy. In some cases, targeted cancer drugs or radiotherapy may also be used^[4][6].

Surgery is typically performed by a specialist surgeon called a gynaecological oncologist. During the operation, the surgeon aims to remove as much of the cancer as possible. This approach is called cytoreductive surgery or debulking surgery. The surgeon will usually remove both ovaries, the fallopian tubes, and the womb (including the cervix). They will also check where the cancer has spread in the pelvis and whether it has reached the lymph nodes^[4][7].

However, because many patients with germ cell tumors are young and may want to have children in the future, doctors try to preserve fertility when possible. In some cases, particularly when the cancer affects only one ovary and hasn’t spread extensively, the surgeon may be able to remove only the affected ovary and leave the other one intact^[7].

Chemotherapy is a critical part of treatment for stage IV ovarian germ cell cancer. You might receive chemotherapy after surgery (called adjuvant chemotherapy), or you might receive chemotherapy both before and after surgery (called neoadjuvant chemotherapy with interval cytoreductive surgery). In some cases, chemotherapy may be delivered directly into the abdomen during surgery, a treatment called hyperthermic intraperitoneal chemotherapy or HIPEC^[4].

The most commonly used chemotherapy combination for ovarian germ cell tumors includes bleomycin, etoposide, and cisplatin, often referred to as BEP. Another combination used includes cisplatin, vinblastine, and bleomycin. Studies have shown that patients with completely resected tumors who received three cycles of BEP had excellent results, with 89 out of 93 patients being disease-free^[14].

Some people may also receive treatment with a targeted cancer drug, depending on their individual situation. These drugs may be given with chemotherapy, on their own, or after chemotherapy^[4].

If surgery is not possible because the cancer has spread too widely or if you are not well enough for surgery, you may receive chemotherapy alone to shrink the cancer and slow its growth. Other treatments may be given to help relieve symptoms, such as treatment for fluid in the abdomen (called ascites) or for a blocked bowel. Radiotherapy may also be used to relieve symptoms like pain^[4].

Outlook and survival

The outlook for people with stage IV ovarian germ cell cancer depends on several individual factors, including age, overall health, how well the cancer responds to treatment, and what treatment options are available^[18].

Survival rates compare the chances that someone with a certain type and stage of cancer will survive for five years compared to the general population. For people initially diagnosed with stage IV germ cell tumors of the ovary, the five-year relative survival rate is 71%. This means that people with stage IV ovarian germ cell tumors are 71% as likely to live at least five years as someone without that cancer^[18].

It’s important to note that these survival rates are based on information about people diagnosed between 2012 and 2018, and survival rates often improve over time as better treatments become available. The rates may change when newer data becomes available^[18].

The use of combination chemotherapy after initial surgery has dramatically improved the outlook for many women with these tumors. Even patients with advanced or incompletely removed dysgerminoma can be rendered disease-free following chemotherapy with BEP or other platinum-based combinations. One series found that 97% of patients with germ cell tumors achieved sustained remission after treatment with BEP^[14].

While having stage IV ovarian germ cell cancer is serious, it doesn’t necessarily mean giving up on treatment. Treatment can often help you feel better and possibly live longer. While it’s not common, it’s possible in some cases to cure ovarian cancer even in its advanced stages. Some 20% of those with late-stage ovarian cancer survive more than 12 years after treatment and are considered cured in medical terms^[18].

Your doctor can provide an estimate based on your individual situation of what to expect. This personalized information can help you and your family make informed decisions about your care and plan for the future^[18].