endodermal sinus tumor

Endodermal Sinus Tumor (EST) Description

The Endodermal Sinus Tumor (EST), also known as Yolk Sac Tumor, is a rare and aggressive malignant germ cell tumor that arises from the primitive yolk sac. It can occur in various parts of the body, including the ovaries, testes, and extragonadal sites such as the mediastinum, retroperitoneum, and sacrococcygeal region [5][9].

Key Characteristics:

• Rare Tumor: EST is a rare tumor in its pure form, representing only a small fraction of adult germinal tumors [13].
• Malignant Germ Cell Tumor: It is a malignant germ cell tumor that typically arises in the gonads (testes and ovaries) but can also occur in extragonadal sites [14][15].
• Highly Malignant: ESTs are rapidly growing and highly malignant tumors that respond well to chemotherapy [6].
• Common in Children: These tumors are most commonly found in children and young adults, with the majority of cases occurring before the age of two years [11].

Symptoms and Treatment:

• Quick Growth: Endodermal sinus tumors tend to grow quickly and spread to other parts of the body if not treated.
• Treatment Options: The treatment options for EST include surgery, chemotherapy, and radiation therapy.

Note: The information provided is based on the search results and may not be an exhaustive list of all possible characteristics or symptoms.

Common Signs and Symptoms of Endodermal Sinus Tumor

Endodermal sinus tumors, also known as yolk sac tumors, can exhibit a range of symptoms depending on their location in the body. Here are some common signs and symptoms associated with this type of cancer:

• Pelvic Mass: In females, endodermal sinus tumors often present as a large pelvic mass, which can cause abdominal pain.
• Abdominal Pain: Both males and females may experience abdominal pain due to the tumor's growth.
• Painless Testicular Mass: In males, endodermal sinus tumors can appear as a painless, unilateral testicular mass.
• Elevated Serum Alpha-Fetoprotein (AFP): Elevated levels of AFP in the blood are often indicative of an endodermal sinus tumor.

Additional Symptoms

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

• Headache: In rare instances, brain metastases can cause headaches.
• Vomiting: Vomiting may occur due to increased intracranial pressure.
• Rapidly Enlarging Mass: A rapidly enlarging mass in the abdomen or pelvis can be a sign of an endodermal sinus tumor.

Important Note

It's essential to note that not all individuals with endodermal sinus tumors will exhibit these symptoms. In some cases, the tumor may grow and spread without causing noticeable signs or symptoms.

References:

• [1] Talerman A. Endodermal sinus (yolk sac) tumor elements in testicular germ-cell tumors in adults: comparison of prospective and retrospective studies. Cancer. 1980 Sep 1. 46(5):1213-7.
• [2] Acute symptoms can alternatively be caused by torsion of the tumor and may lead to the diagnosis of acute appendicitis or a ruptured ectopic pregnancy.
• [4] More common characteristics of ESTs include a mixed solid-cystic appearance upon imaging, hemorrhage and marked enhancement.
• [8] The most common presenting symptoms for patients with endodermal sinus tumors are a rapidly enlarging mass and pain.

Diagnostic Tests for Endodermal Sinus Tumor

Endodermal sinus tumors (ESTs) are a type of germ cell tumor that can be challenging to diagnose. However, several diagnostic tests can help confirm the presence of this condition.

• Immunohistochemical testing: This test can be helpful in diagnosing endodermal sinus tumor, especially when considering the diagnosis of EST in combination with other types of germ cell tumors [3]. The use of immunohistochemical testing for alpha fetoprotein (AFP) can aid in identifying this condition.
• Eosinophilic hyaline globules: These are a diagnostic feature of endodermal sinus tumor and can be identified through histopathological examination [4].
• Alpha fetoprotein (AFP) levels: Elevated serum AFP is a common laboratory finding in patients with endodermal sinus tumors [5, 7]. This test can help confirm the diagnosis.
• Imaging studies: Complete blood count (CBC), differential, and platelet count; Glomerular filtration rate (GFR) or creatinine clearance rate; and other imaging studies may be used to assess the extent of disease and monitor response to treatment [6].
• Placental alkaline phosphatase (PLAP): Approximately 40-80% of cases of yolk sac tumors (YSTs), which are also known as endodermal sinus tumors, are positive for PLAP, a marker of several different germ cell tumors [8].

These diagnostic tests can help confirm the presence of an endodermal sinus tumor and aid in determining the best course of treatment.

References: [3] CE Margo · 1983 · Cited by 45 — When considering the diagnosis of endodermal sinus tumor, the use of immunohistochemical testing for alpha fetoprotein can be helpful. [4] Eosinophilic hyaline globules, immunoreactive for α-fetoprotein, are a diagnostic feature. Endodermal sinus tumors are usually highly invasive. Sometimes they ... [5] Elevated serum alpha fetoprotein is a common laboratory finding. [6] Aug 16, 2023 — Laboratory Studies · Complete blood count (CBC), differential, and platelet count · Glomerular filtration rate (GFR) or creatinine clearance rate ... [7] Elevated serum alpha fetoprotein is a common laboratory finding. Synonym(s): Endodermal sinus tumor. [8] Dec 29, 2021 — Approximately 40-80% of cases of yolk sac tumors (YSTs) are positive for PLAP, a marker of several different germ cell tumors.

Treatment Options for Endodermal Sinus Tumor

Endodermal sinus tumors, also known as yolk sac tumors, are typically treated with a combination of surgery and chemotherapy.

• Surgery: Surgery is often the first line of treatment for endodermal sinus tumors. The goal of surgery is to remove the tumor in its entirety, which can help improve survival rates.
• Chemotherapy: Chemotherapy is also an essential part of treating endodermal sinus tumors. The most commonly used chemotherapy regimen is a combination of bleomycin, etoposide, and cisplatin (BEP). This treatment has been shown to be effective in improving outcomes for patients with this type of tumor.

Specific Chemotherapy Regimens

• BEP (Bleomycin, Etoposide, and Cisplatin): This is the most commonly used chemotherapy regimen for endodermal sinus tumors. The BEP regimen has been shown to be effective in improving outcomes for patients with this type of tumor.
• CEBA (Cisplatin, Etoposide, Bleomycin, and Actinomycin-D): This is another chemotherapy regimen that may be used to treat endodermal sinus tumors. The CEBA regimen has been shown to be effective in some cases.

Other Treatment Options

• Thalidomide: Thalidomide is a medication that has been studied as a potential treatment for endodermal sinus tumors. However, more research is needed to determine its effectiveness.
• Epirubicin, Gemcitabine, and Paclitaxel: These medications have also been studied as potential treatments for endodermal sinus tumors.

References

• [6] Chemotherapy has dramatically improved the prognosis of endodermal sinus tumors. Current treatment consists of surgery followed by bleomycin, etoposide, and cisplatin (BEP) chemotherapy.
• [3] Modern therapy for ESTs consists of bleomycin, etoposide, and cisplatin (BEP). Negative prognostic factors include unsatisfactory decline in tumor size after initial treatment.
• [4] Women with endodermal sinus tumors are treated with surgery and cisplatin-based chemotherapy. Those women who do not respond to chemotherapy have a poor prognosis.

Note: The information provided is based on the search results and may not be comprehensive or up-to-date. It's always best to consult with a healthcare professional for personalized advice and treatment options.

Differential Diagnosis of Endodermal Sinus Tumor

Endodermal sinus tumors (ESTs), also known as yolk sac tumors, are highly malignant germ cell tumors that can be challenging to diagnose due to their similarity in appearance with other types of tumors. The differential diagnosis for ESTs includes several other conditions that need to be ruled out through histopathological examination and clinical presentation.

Key Points to Consider:

• Seminoma: A type of germ cell tumor that is often confused with ESTs due to its similar microscopic appearance. However, seminomas typically have a more uniform and compact growth pattern compared to the loose, reticular pattern seen in ESTs [10].
• Embryonal Carcinoma: Another type of germ cell tumor that can be difficult to distinguish from ESTs. Embryonal carcinomas often show a mixture of glandular and solid patterns, whereas ESTs typically exhibit a microcystic or reticular pattern [12].
• Teratoma: A type of germ cell tumor that contains several different types of tissues, including epithelial, mesenchymal, and neural elements. Teratomas can be confused with ESTs due to their similar microscopic appearance, but they typically have a more complex and heterogeneous growth pattern [10].
• Dysgerminoma: A type of germ cell tumor that is often found in the ovary and can be difficult to distinguish from ESTs. Dysgerminomas typically show a solid or sheet-like growth pattern with large cells and prominent nucleoli, whereas ESTs exhibit a more loose and reticular pattern [11].

Clinical Presentation:

• Elevated Alpha-Fetoprotein (AFP) Levels: Elevated AFP levels are a critical diagnostic tool for ESTs. However, it's essential to note that other conditions such as hepatocellular carcinoma, embryonal carcinoma, and teratoma can also cause elevated AFP levels [11].
• Pelvic Pain and Mass: Young women with pelvic pain and a mass may present with a differential diagnosis that includes ESTs, but also other conditions such as ovarian cysts, endometriosis, or adnexal torsion [14].

Conclusion:

The differential diagnosis for endodermal sinus tumors is broad and requires careful consideration of histopathological features, clinical presentation, and laboratory results. A thorough understanding of these factors can help clinicians accurately diagnose ESTs and provide appropriate treatment.

References:

[10] Yolk sac tumor (endodermal sinus tumor): A review of the literature. [11] Alpha-fetoprotein as a diagnostic marker for yolk sac tumors. [12] Differential diagnosis of primary mediastinal germ cell tumors. [14] Radiographical findings and clinical presentation of endodermal sinus tumors.