Endodermal sinus tumor or yolk sac tumor refers to a rare type of cancer growing in the cells responsible for sperm and egg production (germ cells). Endodermal sinus tumors are present most commonly in the testicle or ovary. But, these sinus tumors may also grow in other areas of the body like the brain, abdomen, and chest. Endodermal sinus tumors tend to grow and spread to other body parts if the condition is not treated well. These kinds of tumors are the most common malignant tumors in children of age under 3. Due to this, it is known as infantile embryonal carcinoma. The prognosis is good in this age group and in contrast to the pure form typical of infants, endodermal sinus tumors are often found and combined with other kinds of germ cell tumors like embryonal carcinoma and teratoma. The pure teratoma is benign; however, the endodermal sinus tumor is malignant.
Endodermal Sinus Tumor Symptoms
The ovarian endodermal sinus tumors represent about one-quarter of germ cell tumors and approximately 2 to 3% of the overall ovarian cancers. However, in contrast to the ovarian endodermal sinus tumors, the testicular form is uncommon with 1% of childhood tumors. The early stages of the yolk sac tumor or endodermal sinus tumor exhibit no particular signs and symptoms. However, with the development of the tumor, a set of symptoms occur that may include vomiting, nausea, fatigue, cough, moderate to severe headache, weight loss, night sweats, fever, shortness of breath, or changes in bowel habits or function.
However, the signs and symptoms for the location-specific endodermal sinus tumor may include vision problems, chest pain, testicular mass, and swelling or mass growth in the abdomen. The other symptoms of this tumor may involve swelling in the hydrocephalus or brain region. It may also involve; the swelling of the buttocks, shoulder, arm, and testicles.
Endodermal Sinus Tumor Causes
The causes of germ cell tumors are not always known and doctors have no idea regarding some medical conditions that can cause children to develop the endodermal sinus tumor or germ cell tumor. The causes of an endodermal sinus tumor or yolk sac tumor include birth defects involving the urinary tract, spine, genitals, and central nervous system. It may also involve genetic conditions that can lead to extra or missing sex chromosomes.
Boys with testes that stay up inside the pelvis region are susceptible to developing endodermal sinus tumors. Anyone with ovaries is susceptible to ovarian germ cell tumors. However, women under thirty are at an increased risk of developing germ cell tumors. The doctors may check for biomarkers in the blood and cerebrospinal fluid to diagnose the presence of germ cell tumors. However, a biopsy will still be necessary to aid in the diagnosis of an endodermal sinus tumor.
Endodermal Sinus Tumor Prognosis
Forty-seven patients with yolk sac tumors were retrospectively studied. Twenty-two patients had pure endodermal sinus tumors and twenty-five had germ cell tumors with yolk sac as a component of this disease. The five-year survival rate in stages I, II, III, and IV was 95%, 75%,30%, and 25% respectively.
Tumor-reductive surgery and staging affected the prognosis of this disease. When ascites are minimal, tumor-reductive surgery is advisable. Chemotherapy without cisplatin is inferior to cisplatin-based chemotherapy. However, there was no effect of p53 on chemosensitivity in yolk sac tumors of the ovary.
Patients with stage I disease had a more favorable prognosis than stage III and IV disease. Regimens of chemotherapy that included cisplatin showed better results than those without cisplatin. Prognostic differences in cases were significant in which the size of the tumor was less than 2 cm in diameter and cases where ascites were less than 100 ml or absent.
Endodermal Sinus Tumor Treatment
Most of the treatments of an endodermal sinus tumor involve a combination of chemotherapy and surgery. However, the first-line treatment for an endodermal sinus tumor is the surgical treatment to remove the tumor. The surgery is followed; by chemotherapy that uses powerful medicines to kill the cancer cells or stop the cancerous or tumor cells from growing and multiplying in number.
Medications like etoposide, cisplatin, and bleomycin are also prescribed. Before the advent of modern chemotherapy, the endodermal sinus tumor was highly lethal. However, the prognosis of this tumor has improved a lot since then. This kind of tumor is both rare and fatal. However, if treated promptly with surgery and chemotherapy, the fatal outcomes are exceedingly rare. This type of tumor can also appear in the vagina, abdomen, liver, brain, or uterus. The endodermal sinus tumor is rare in both sexes and is the most common form of testicular cancer.