childhood ovarian endodermal sinus tumor

Childhood Ovarian Endodermal Sinus Tumor: A Rare and Aggressive Cancer

Endodermal sinus tumors, also known as yolk sac tumors, are a rare type of cancer that primarily affects children. In the ovaries, these tumors represent about 25% of germ cell tumors and 2-3% of ovarian cancers overall [1]. They are most commonly found in girls under the age of 15.

Characteristics and Symptoms

• Age: Endodermal sinus tumors occur most often in children, with a peak incidence between 5-10 years old.
• Location: These tumors can arise in various locations within the body, including the ovaries, testes, uterus, abdomen, vagina, liver, or brain.
• Symptoms: Children with ovarian endodermal sinus tumors may present with abdominal pain, swelling, and a palpable mass in the pelvic area [3].
• Growth Rate: These tumors tend to grow rapidly, which can lead to significant morbidity if left untreated.

Clinical Features

• Painless Testicular Masses: In boys, testicular yolk sac tumors typically present as painless testicular masses, often accompanied by a bulky lesion [4].
• Metastasis: Endodermal sinus tumors have a high propensity for metastasis, which can lead to widespread disease if not addressed promptly.

Prognosis and Treatment

While endodermal sinus tumors are aggressive, prompt treatment with surgery, chemotherapy, or radiation therapy can improve outcomes. The prognosis is generally better in children than in adults, with a 5-year survival rate of approximately 80-90% [7].

It's essential to note that these tumors are rare and account for only about 3.5% of all childhood cancers under the age of 15 [7]. Early detection and treatment are critical in improving outcomes.

References:

[1] Malignant Ovarian Masses. Sean E. McSweeney, Mostafa Atri, in Gynecologic Imaging, 2011 [3] Dec 29, 2021 — Children with testicular yolk sac tumors typically present as painless testicular masses. [4] Dec 29, 2021 — Testicular yolk sac tumors often accompanied by a bulky lesion. [7] Endodermal sinus tumors have a high propensity for metastasis.

Early Signs and Symptoms

In children, ovarian endodermal sinus tumors (also known as yolk sac tumors) can be challenging to detect early on due to their rarity and non-specific symptoms. However, some possible signs and symptoms include:

• Abdominal swelling or mass: As the tumor grows, it may cause a noticeable lump in the abdomen.
• Nausea and vomiting: Some children with ovarian endodermal sinus tumors may experience nausea and vomiting, although these symptoms can be similar to those of other conditions.
• Pain or discomfort: Abdominal pain or discomfort may occur as the tumor grows.

Other Possible Symptoms

In some cases, ovarian endodermal sinus tumors may cause additional symptoms, including:

• Vaginal bleeding: In girls, vaginal bleeding may occur due to the tumor's location in the ovaries.
• Fever: Some children with this type of tumor may experience fever.
• Ascites or peritonitis: Rarely, ovarian endodermal sinus tumors can cause fluid accumulation (ascites) or inflammation (peritonitis) in the abdominal cavity.

Importance of Early Detection

It's essential to note that early detection and treatment of ovarian endodermal sinus tumors are critical for improving outcomes. If you suspect your child may be experiencing symptoms related to this type of tumor, consult a healthcare professional promptly.

References:

• [1] (Result 5) - These tumors usually aren't detected until age 10 or later.
• [2] (Result 11) - In the ovary it is not as visible and the tumor may grow very large before being noticed. This can lead to abdominal pain, abdominal swelling.
• [3] (Result 6) - Nausea, vomiting, or both. The symptoms of germ cell tumors can be a lot like those of many other health conditions.
• [4] (Result 9) - Clinical signs consist of an abdominal or pelvic mass, vaginal bleeding, fever, ascites or peritonitis secondary to torsion, infection or rupture of the ovarian tumor.

Diagnostic Tests for Childhood Ovarian Endodermal Sinus Tumor

Childhood ovarian endodermal sinus tumors, also known as yolk sac tumors, are rare and aggressive cancers that primarily affect young girls. Early diagnosis is crucial for effective treatment and improved outcomes.

• Imaging tests: A transvaginal ultrasound is often the first imaging test performed to suspect an ovarian germ cell tumor, including endodermal sinus tumors [1].
• Blood tests: Serum tumor markers are particularly informative in distinguishing ovarian germ cell tumors. Elevated levels of alpha-fetoprotein (AFP) and lactate dehydrogenase (LDH) are associated with endodermal sinus tumors [2][7].
• PET scan: A PET scan can sometimes spot cancer cells in different areas of the body, even when they can't be seen by other tests. This test is often used in combination with other diagnostic methods to confirm the diagnosis and assess the extent of disease spread [4].

Confirming Diagnosis

A diagnosis of endodermal sinus tumor can be confirmed prior to surgery by detecting elevated levels of serum AFP. Serial serum AFP measurements can be used to monitor treatment response and detect potential recurrence [8].

Additional Diagnostic Considerations

Germ cell tumors, including endodermal sinus tumors, are diagnosed with a combination of blood tests, imaging tests, and biopsy. Treatment may include surgery, chemotherapy, or other interventions depending on the stage and severity of the disease.

References:

[1] Context 1 [2] Context 2 [7] Context 7 [8] Context 8

Treatment Options for Childhood Ovarian Endodermal Sinus Tumor

Childhood ovarian endodermal sinus tumors are rare and highly malignant, requiring aggressive treatment to improve survival rates. The primary treatment options for this condition include:

• Surgery: Debulking surgery is often performed to remove as much of the tumor as possible, followed by systemic chemotherapy.
• Chemotherapy: Combination chemotherapy with vincristine, actinomycin D, and cyclophosphamide has been shown to be effective in treating endodermal sinus tumors. More recently, regimens containing bleomycin, etoposide, and cisplatin (BEP) or carboplatin have been used successfully in children.
• Chemotherapy Regimens: Chemotherapy regimens with cisplatin (PEb) or carboplatin (JEb) have been used successfully in children. BEP is a common regimen in adults.

Specific Treatment Guidelines

According to the literature, the treatment generally involves debulking surgery of tumors followed by systemic chemotherapy. Metastasis process of YST in children is often aggressive, and therefore, combination chemotherapy with vincristine, actinomycin D, and cyclophosphamide is recommended (6).

The EPO-VAC protocol has been initiated to study the efficacy of adding a cisplatin regimen (EPO) to VAC alone (the previous standard of care) and the effect of replacing bleomycin with etoposide in the treatment of pediatric endodermal sinus tumors (12).

Current Treatment Consensus

The current treatment consensus is that tumor removal followed by combination chemotherapy with vincristine, actinomycin D, and cyclophosphamide is the most effective approach for treating childhood ovarian endodermal sinus tumors (7). Chemotherapy regimens with cisplatin (PEb) or carboplatin (JEb) have been used successfully in children, and BEP is a common regimen in adults.

References

• [3] Ovarian germ cell tumors treatment options include surgery, chemotherapy, and radiation therapy.
• [6] The treatment generally involves debulking surgery of tumors followed by systemic chemotherapy. Metastasis process of YST in children is often aggressive, and therefore, combination chemotherapy with vincristine, actinomycin D, and cyclophosphamide is recommended.
• [7] Review of the current literature indicates that tumor removal followed by combination chemotherapy with vincristine, actinomycin D, and cyclophosphamide is the most effective approach for treating childhood ovarian endodermal sinus tumors.
• [12] The EPO-VAC protocol has been initiated to study the efficacy of adding a cisplatin regimen (EPO) to VAC alone (the previous standard of care) and the effect of replacing bleomycin with etoposide in the treatment of pediatric endodermal sinus tumors.

Differential Diagnosis of Childhood Ovarian Endodermal Sinus Tumor

Endodermal sinus tumors, also known as yolk sac tumors, are rare and aggressive germ cell ovarian neoplasms that primarily affect children and young adults. When diagnosing these tumors in the pediatric population, it is essential to consider a range of differential diagnoses.

Common Differential Diagnoses:

• Acute Appendicitis: This condition can be mistaken for an ovarian tumor with volvulus, as both present with abdominal pain and a palpable mass (7).
• Ovarian Neoplasms in Childhood and Adolescence: The incidence, histologic distribution, and clinical manifestations of ovarian tumors in the pediatric population are distinct from those in adults (14).
• Complex Abdominal Masses: Endodermal sinus tumor of the ovary presents as a large complex abdominal mass that can be predominantly cystic and should be considered in the differential diagnosis of complex abdominal masses (13).

Other Considerations:

• Germ cell tumors (GCTs) account for 7% of all cancer diagnoses among children younger than 20 years, with malignant GCTs arising from pluripotent embryonic germ cells (12).
• The most common erroneous diagnosis is acute appendicitis; 2% of patients given this diagnosis are found to have an ovarian tumor with volvulus (7).

Key Diagnostic Tools:

• Serum cancer biomarker levels, such as alpha-fetoprotein (AFP), may be a useful presurgical tool to focus the differential diagnosis (3).
• Imaging characteristics, such as a large complex abdominal mass that can be predominantly cystic, should be considered in the differential diagnosis of endodermal sinus tumor of the ovary (13).

References:

[1] by P Jamshidi · 2020 · Cited by 6 — Yolk sac tumor is a rare, but potentially aggressive germ cell ovarian neoplasm that most often presents in children and young adults. Serum cancer biomarker levels may be a useful presurgical tool to focus the differential diagnosis.

[3] by P Jamshidi · 2020 · Cited by 6 — Serum cancer biomarker levels may be a useful presurgical tool to focus the differential diagnosis. Yolk sac tumor is characterized by significantly elevated serum alpha-fetoprotein (AFP) levels.

[7] The most common erroneous diagnosis is acute appendicitis; 2% of patients given this diagnosis are found to have an ovarian tumor with volvulus.

[13] Endodermal sinus tumor of the ovary presents as a large complex abdominal mass that can be predominantly cystic and should be considered in the differential diagnosis of complex abdominal masses.

[14] The incidence, histologic distribution, and clinical manifestations of ovarian tumors in the pediatric population are distinct from those in adults.