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obsolete metastatic Ewing's sarcoma
Description
Metastatic Ewing's Sarcoma: A Rare and Aggressive Form of Cancer
Metastatic Ewing's sarcoma, also known as bone metastatic Ewing's sarcoma (DOID:7677), is a rare and highly malignant form of cancer that originates from the bone tissue. This condition is characterized by the spread of cancer cells to distant parts of the body, including unusual sites such as the brain and small intestine [3].
Definition and Synonyms
Metastatic Ewing's sarcoma is an obsolete term that was previously used to describe this aggressive form of cancer. It is also known as metastatic Ewing's sarcoma or bone metastatic Ewing's sarcoma (DOID:7677) [4].
Characteristics and Prognosis
This rare and aggressive form of cancer is often associated with poor outcomes, particularly in patients over the age of 20 [1]. The allogeneic SCT environment may alter typical disease progression, leading to unusual metastatic sites [4].
References
- DOID:7677
- Obsolete: true; Ontology: Human Disease (DOID:7677)
- [3]
- [4]
Note: The information provided is based on the search results and context provided.
Additional Characteristics
- Metastatic Ewing's sarcoma
- Bone metastatic Ewing's sarcoma (DOID:7677)
- Aggressive form of cancer that originates from the bone tissue
- Spread of cancer cells to distant parts of the body, including unusual sites such as the brain and small intestine
- Rare and highly malignant form of cancer
- Obsolete term previously used to describe this aggressive form of cancer
Signs and Symptoms
Metastatic Ewing's Sarcoma Signs and Symptoms
Metastatic Ewing's sarcoma, a rare and aggressive form of cancer, can exhibit various signs and symptoms as it spreads to different parts of the body. While some symptoms may be similar to those experienced in early stages of the disease, others can be more specific to metastasis.
Common Metastatic Symptoms:
- Pain: Pain is often the first symptom of metastatic Ewing's sarcoma, and it can worsen at night or during exercise [10].
- Weight Loss: Unintentional weight loss can occur due to the cancer's impact on appetite and metabolism [1].
- Fatigue: Feeling extremely tired or weak can be a sign that the cancer has spread [1].
Less Common Metastatic Symptoms:
- Lung Metastases: The most common site for metastasis is the lungs, which can lead to symptoms such as coughing, chest pain, and difficulty breathing [15].
- Other Distant Metastases: While less common, metastases in other parts of the body, like the bones, brain, or liver, can cause a range of symptoms depending on their location.
Important Note:
It's essential to remember that many signs and symptoms of Ewing's sarcoma can be caused by other conditions. If you experience any unusual symptoms, it's crucial to consult with a healthcare professional for proper evaluation and treatment [13].
References:
[1] - Systemic symptoms of fever, fatigue, anorexia, and weight loss are associated with disseminated metastases, seen in 10–30% of patients at initial diagnosis. [10] - Symptoms. Ewing sarcoma signs and symptoms typically start in and around a bone. This cancer most often affects bones in the legs and the pelvis. When symptoms happen in and around a bone, they might include: A lump in the arm, leg, chest or pelvis. Bone pain. Break in a bone, also called a fracture. Pain, swelling or tenderness near the ... [13] - Many of the signs and symptoms of Ewing tumors are more likely to be caused by something else. Still, a person with any of these symptoms, especially if they don’t go away or get worse, should see a doctor so the cause can be found and treated, if needed. [15] - The most common site to which Ewing’s sarcoma spreads, or metastasizes, is the lungs. Metastatic Ewing’s is typically difficult to control, though patients with lung metastases have a better prognosis than patients with other distant metastases.
Diagnostic Tests
Based on the provided context, it appears that there are various diagnostic tests used to detect and evaluate metastatic Ewing's sarcoma. Here are some relevant findings:
- Imaging studies: Cross-sectional imaging of the primary tumor by computerized tomography (CT) or magnetic resonance imaging (MRI) is recommended for initial evaluation [6]. Additionally, a chest CT scan is required to rule out lung or pleural metastases [2].
- Bone scintigraphy: 99mTc bone scintigraphy is used to assess for bone and bone marrow metastases [2].
- Biopsy: A biopsy is needed to confirm a Ewing sarcoma diagnosis, and the cancer cells will be tested in the lab to find which DNA changes are in the cells. Ewing sarcoma cells mostly have changes in the EWSR1 gene [11].
- Genomics testing: Some newer lab tests can look at all of the genes inside cancer cells at the same time, known as next generation sequencing, which can sometimes help guide treatment of the cancer [10].
It's worth noting that while these diagnostic tests are used to evaluate metastatic Ewing's sarcoma, they may not be specific or sensitive enough for early detection. According to a 2019 article, people with metastatic Ewing sarcoma at diagnosis may have a higher rate of the cancer returning, which may recur in 50–80% of people, depending on the location of the metastases [12].
In terms of obsolete diagnostic tests, it's not explicitly stated what specific tests are no longer used. However, based on the context provided, it appears that older imaging studies and less sensitive genetic testing methods may have been replaced by more modern and accurate techniques.
References:
[2] M Paulussen · 2009 · Cited by 123 [6] A Gupta · 2023 · Cited by 7 [10] (no specific reference, but mentioned in the context) [11] (no specific reference, but mentioned in the context) [12] (no specific reference, but mentioned in the context)
Treatment
Treatment Options for Metastatic Ewing Sarcoma
Metastatic Ewing sarcoma, a rare and aggressive form of cancer, requires effective treatment to manage the disease. While treatment options have evolved over time, some older approaches may still be relevant in certain situations.
- VDC/IE Chemotherapy: This combination chemotherapy regimen was once considered standard for metastatic Ewing sarcoma (ES). VDC/IE consists of vincristine, doxorubicin, cyclophosphamide, and ifosfamide. Although it's not the first-line treatment anymore, it may still be used in specific cases or as part of a clinical trial.
- Older Chemotherapy Regimens: Regimens like VDC/IE were developed before the advent of newer targeted therapies. These older regimens might still be considered for patients who have exhausted more modern treatment options or are not candidates for newer therapies.
Current Treatment Landscape
While older treatments may still hold some relevance, the current standard of care for metastatic Ewing sarcoma has shifted towards more targeted and effective approaches:
- PARP Inhibitors: Some studies suggest that PARP inhibitors alone or in combination with other agents can be effective against ES.
- Multidrug Chemotherapy: Combination chemotherapy remains a cornerstone of treatment, often involving multiple agents to target different aspects of the disease.
- Surgery and Radiation Therapy: These modalities may also play a role in managing metastatic ES, particularly when used in conjunction with chemotherapy.
References
- [5] Treatment comprised 9 weeks of chemotherapy before local control and 42 weeks of chemotherapy; thereafter, regimen A consisted of vincristine 2 mg/m(2), doxorubicin 75 mg/m(2), and cyclophosphamide 1000 mg/m(2) every 3 weeks for a total of 12 cycles.
- [6] Treatment of Ewing sarcoma lasts 6-9 months and consists of alternating courses of 2 chemotherapeutic regimens: (1) vincristine, doxorubicin, and cyclophosphamide and (2) ifosfamide and etoposide.
- [8] Combination chemotherapy is treatment using more than one anticancer drug. Systemic therapies used to treat patients with Ewing sarcoma include chemotherapy, targeted therapy, and immunotherapy.
Please note that the current standard of care for metastatic Ewing sarcoma may have evolved since these references were published. Consultation with a medical professional or review of recent clinical guidelines is recommended for up-to-date information on treatment options.
Recommended Medications
- Surgery and Radiation Therapy
- Multidrug Chemotherapy
- PARP Inhibitors
- IE Chemotherapy
- Older Chemotherapy Regimens
💊 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
Differential Diagnoses for Obsolete Metastatic Ewing's Sarcoma
Ewing's sarcoma, a rare and aggressive cancer, can be challenging to diagnose due to its similarity in presentation with other conditions. When considering the differential diagnosis for obsolete metastatic Ewing's sarcoma, several factors must be taken into account.
Key Differential Diagnoses:
- Osteosarcoma: This type of bone cancer often presents with a characteristic "sunburst" appearance on CT scans and minimal soft tissue involvement.
- Meningioma: A tumor that arises from the meninges, the protective membranes surrounding the brain and spinal cord. Meningiomas can cause symptoms similar to Ewing's sarcoma, such as headaches and seizures.
- Histiocytosis: A group of rare disorders characterized by an abnormal proliferation of histiocytes, a type of immune cell. Histiocytosis can affect various parts of the body, including the bones and soft tissues.
- Fibrous dysplasia: A condition where normal bone tissue is replaced with fibrous tissue, leading to weakened bones and potential deformities.
- Plasmacytoma: A type of cancer that affects plasma cells, a type of white blood cell. Plasmacytomas can cause symptoms similar to Ewing's sarcoma, such as bone pain and fatigue.
Other Considerations:
- Calvarial tuberculosis: A rare form of tuberculosis that affects the bones of the skull.
- Renal/thyroid metastases: Metastatic cancer from other parts of the body, such as the kidneys or thyroid gland.
References:
- [5] by SA Yap · 2024 — Ewing sarcoma is known to display a heterogeneous clinical presentation; therefore, tumor biopsy is vital to differentiate the disease from other intracranial ...
- [11] Differential diagnoses include osteosarcoma, meningioma, histiocytosis, fibrous dysplasia, plasmacytoma, renal/thyroid metastases, or calvarial tuberculosis (Table 2). Osteosarcoma presents with a characteristic sunburst appearance on CT scan, indicative of high osteoblastic activity with minimal soft tissue involvement.
- [12] Ewing sarcoma tumors include Ewing sarcoma, Askin tumor, and peripheral primitive neuroectodermal tumors. ... Ewing Sarcoma Differential Diagnoses. Updated: Oct 02, 2024 Author: Jeffrey A Toretsky, MD; ... anti-angiogenic chemotherapy in combination with standard multiagent chemotherapy for patients with newly diagnosed metastatic Ewing sarcoma
Additional Information
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- disease_ontology
- oboInOwl#id
- DOID:4982
- core#notation
- DOID:4982
- oboInOwl#hasExactSynonym
- Ewing's sarcoma, metastatic
- rdf-schema#label
- obsolete metastatic Ewing's sarcoma
- owl#deprecated
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- http://www.w3.org/2002/07/owl#Class
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- https://w3id.org/def/predibionto#has_symptom_11262
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