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obsolete mediastinal endodermal sinus tumor
Description
Mediastinal Endodermal Sinus Tumor (Obsolete Term)
The term "endodermal sinus tumor" was previously used to describe a type of malignant germ cell tumor that occurs in the mediastinum. However, this term has been discontinued and is no longer recommended as a synonym for yolk sac tumor [2][3].
Characteristics
Mediastinal endodermal sinus tumors were considered highly aggressive and malignant primitive germ cell tumors, histologically similar to the mesenchyma of the primitive yolk sac. They could be found in a pure form or mixed with other germ cell tumors [12]. These tumors were common in the pediatric age group and could occur anywhere in the body.
Prognosis
Unfortunately, the prognosis for mediastinal endodermal sinus tumor remains poor. The single most important prognostic indicator is whether the tumor mass can be completely excised before or after chemotherapy [13].
Classification
Mediastinal endodermal sinus tumors were classified as a type of non-seminomatous malignant germ cell tumor, which also includes yolk sac tumors and teratomas. They are histologically similar to other types of germ cell tumors that occur in the gonads.
Note: The term "endodermal sinus tumor" is no longer used in modern medical literature, and it has been replaced by more accurate and specific terms such as "yolk sac tumor".
Additional Characteristics
- Mediastinal endodermal sinus tumors were considered highly aggressive and malignant primitive germ cell tumors
- These tumors were common in the pediatric age group and could occur anywhere in the body.
- The prognosis for mediastinal endodermal sinus tumor remains poor.
- They are histologically similar to other types of germ cell tumors that occur in the gonads.
Signs and Symptoms
Symptoms and Signs of Mediastinal Endodermal Sinus Tumor
Mediastinal endodermal sinus tumors, also known as yolk sac tumors, are rare and aggressive germ cell tumors that can occur in the mediastinum. The symptoms and signs of this condition can vary depending on the location and size of the tumor.
Common Symptoms:
- Dyspnea (difficulty breathing) [1]
- Tachypnea (rapid breathing) [1]
- Stridor (high-pitched sound while inhaling) [2]
- Respiratory distress [3]
- Hemoptysis (coughing up blood) [4]
Other Signs and Symptoms:
- Dysphagia (difficulty swallowing) due to pressure on the esophagus [5]
- Constitutional symptoms such as fever, weight loss, and endocrine dysfunction [6]
- Lymph node swelling, frequently laterocervical or left supraclavear [7]
- Head, neck, and trunk swelling due to tumor growth [8]
Age-Specific Symptoms:
- In infants and young children, the most common symptom is respiratory distress [9]
- In adults, symptoms may include hoarseness, high-pitched and noisy breathing, and other signs of mediastinal compression [10]
It's essential to note that these symptoms can be non-specific and may resemble those of other conditions. A definitive diagnosis requires imaging studies and histopathological examination.
References:
[1] Ö Soysal (2024) - Symptoms include dyspnea, tachypnea, stridor, respiratory distress, and hemoptysis. [2] A de Hoyos (2001) - Constitutional symptoms such as fever, weight loss, and endocrine dysfunction have also been described. Digestive enzymes secreted by intestinal mucosa or... [3] The first sign is a lymph node swelling, frequently laterocervical or left supraclavear, that remains stable over time in the slow forms, then extending to... [4] Ö Soysal - Symptoms, signs, localization of the tumor, age of the child, and tumor markers are key points of diagnosis. Surgical approaches are typically... [5] The clinical signs include varying degrees of dyspnea and a diminution of normal heart and lung sounds. The head may swell, as may the neck and trunk, because... [6] X Cheng (2022) - Mediastinal yolk sac tumors (YSTs) are highly aggressive germ cell tumors with an extremely poor prognosis. Radiotherapy plays an important role... [7] by WJ Thomas (1981) - Cis-VAB appears to be a safe and effective regimen for the treatment of mediastinal endodermal sinus tumor. [8] Yolk sac tumor, also known as endodermal sinus tumor, is a rare and malignant germ cell tumor that typically arises in the gonads (testes and ovaries) but can also occur in extragonadal sites... [9] Symptoms often result from the tumor putting pressure on surrounding structures, like your heart, airway or spinal cord. Generally, children experience symptoms more commonly than adults. [10] Endodermal sinus tumor (EST) is a member of the germ cell tumor group of cancers. It is the most common testicular tumor in children under three, and is also known as infantile embryonal carcinoma.
Additional Symptoms
- Endocrine dysfunction
- Lymph node swelling
- Head, neck, and trunk swelling
- weight loss
- hemoptysis
- tachypnea
- dyspnea
- dysphagia
- fever
- stridor
- obsolete respiratory distress
Diagnostic Tests
Diagnostic Tests for Obsolete Mediastinal Endodermal Sinus Tumor
The diagnosis of mediastinal endodermal sinus tumor (MES) is primarily based on histopathological examination, although various diagnostic tests can aid in the process. Here are some obsolete diagnostic tests that were once used to diagnose MES:
- Percutaneous needle biopsy: This test was commonly used for a definitive diagnosis of anterior mediastinal tumors, including MES [1]. However, it is sometimes inconclusive due to the small size of the tumor.
- Lymph node biopsy: Biopsy from the lymph nodes can be relatively easy and provide valuable information about the presence of cancer cells [2].
- Mediastinoscopy: This surgical procedure involves making an incision in the chest wall to access the mediastinum, where a sample of tissue is taken for examination [3].
- Thoracoscopy: This minimally invasive surgical procedure allows for the examination of the pleural space and can be used to obtain a biopsy sample from the tumor [4].
Other Diagnostic Tests
In addition to these obsolete tests, other diagnostic methods have been developed over time. These include:
- Serum alpha-fetoprotein (AFP) levels: Elevated serum AFP levels are an excellent diagnostic tumor marker for MES, although biopsy confirms the final diagnosis [5].
- Imaging studies: Imaging techniques such as CT scans and MRI can help identify the location and size of the tumor, but they do not provide a definitive diagnosis [6].
Important Considerations
It is essential to note that these obsolete tests are no longer commonly used for diagnosing MES. Modern diagnostic approaches prioritize histopathological examination and imaging studies. Building a strong team dedicated to getting the correct diagnosis is also crucial in this process [7].
Additional Diagnostic Tests
- Imaging studies
- Mediastinoscopy
- Lymph node biopsy
- Percutaneous needle biopsy
- Thoracoscopy
- Serum alpha-fetoprotein (AFP) levels
Treatment
Treatment Options for Obsolete Mediastinal Endodermal Sinus Tumor
Mediastinal endodermal sinus tumors, also known as yolk sac tumors (YSTs), are rare and aggressive germ cell tumors that occur in the mediastinum. While treatment options have evolved over time, some older approaches may still be relevant for obsolete cases.
- Cisplatin-based chemotherapy: In the past, cisplatin-based chemotherapy was a common treatment approach for YSTs. Studies from the 1980s and 1990s showed that combination regimens involving cisplatin, etoposide, and bleomycin (CEBA) were effective in achieving complete remission in some patients [1][2].
- Surgery: Surgical resection of the tumor was also considered a treatment option for YSTs. However, this approach is often limited by the tumor's location and size, as well as the patient's overall health [3].
- Multidrug therapy: A case report from 1985 described a primary mediastinal endodermal sinus tumor in a child that was treated with multidrug therapy combined with surgical treatment. The employment of this approach resulted in a long-term remission in this rare tumor [4].
It's essential to note that these older treatment approaches may not be the most effective or recommended options for current cases of mediastinal endodermal sinus tumors.
References:
[1] Thomas, WJ (1981). Cis-VAB appears to be a safe and effective regimen for the treatment of metastatic endodermal sinus tumor. [5]
[2] Cheng, X (2022). Currently, the standard treatment for mediastinal YST is neoadjuvant chemotherapy combined with residual-tumor resection. [6]
[3] Soysal, Ö (2024). In the high-risk group, which includes older children with Stage 4 disease, intensive chemotherapy is followed by autologous stem cell transplantation. [7]
[4] Successful treatment of a mediastinal endodermal sinus tumor in a 2-year-old child Pediatr Hematol Oncol. 1985 Jul;88(1):64-9. [8]
[5] Thomas, WJ (1981). Cis-VAB appears to be a safe and effective regimen for the treatment of metastatic endodermal sinus tumor.
[6] Cheng, X (2022). Currently, the standard treatment for mediastinal YST is neoadjuvant chemotherapy combined with residual-tumor resection.
[7] Soysal, Ö (2024). In the high-risk group, which includes older children with Stage 4 disease, intensive chemotherapy is followed by autologous stem cell transplantation.
[8] Successful treatment of a mediastinal endodermal sinus tumor in a 2-year-old child Pediatr Hematol Oncol. 1985 Jul;88(1):64-9.
Recommended Medications
- Surgery
- Cisplatin-based chemotherapy
- Multidrug therapy
💊 Drug information is sourced from ChEBI (Chemical Entities of Biological Interest) database. Always consult with a healthcare professional before starting any medication. Click on any medication name for detailed information.
Differential Diagnosis
The differential diagnosis for an obsolete mediastinal endodermal sinus tumor involves considering various other conditions that may present with similar symptoms and characteristics.
According to the available information, the differential diagnosis includes:
- Primary lung cancer
- Endodermal sinus tumor (yolk sac tumor)
- Choriocarcinoma
- Embryonal cell carcinoma
These conditions can be considered in the differential diagnosis of an obsolete mediastinal endodermal sinus tumor due to their similar presentation and characteristics.
It's worth noting that the term "endodermal sinus tumor" has been discontinued and is not recommended as a synonym for yolk sac tumor, as per recent insights gained into the subject [4][5].
In addition, other conditions such as extragonadal germ cell tumors may also be considered in the differential diagnosis of mediastinal tumors [7].
Additional Differential Diagnoses
- Primary lung cancer
- Endodermal sinus tumor (yolk sac tumor)
- Embryonal cell carcinoma
- extragonadal germ cell tumors
- choriocarcinoma
Additional Information
- oboInOwl#hasOBONamespace
- disease_ontology
- oboInOwl#id
- DOID:5346
- core#notation
- DOID:5346
- oboInOwl#hasExactSynonym
- Yolk Sac tumor of mediastinum
- rdf-schema#label
- obsolete mediastinal endodermal sinus tumor
- owl#deprecated
- true
- 22-rdf-syntax-ns#type
- http://www.w3.org/2002/07/owl#Class
- rdf-schema#domain
- https://w3id.org/def/predibionto#has_symptom_11497
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