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Askin's tumor
ICD-10 Codes
Related ICD-10:
Description
Askin's Tumor: A Rare and Aggressive Bone Cancer
Askin's tumor, also known as extraskeletal Ewing's sarcoma (EES), is a rare and aggressive type of bone cancer that typically affects children and young adults. It is characterized by its rapid growth and potential to spread to other parts of the body.
Key Features:
- Rapid Growth: Askin's tumor grows quickly, often within a matter of weeks or months.
- Aggressive Behavior: This type of cancer can be highly aggressive and may spread to other bones, lymph nodes, and organs such as the lungs, liver, and brain.
- Young Age Affected: Askin's tumor typically affects children and young adults, with most cases occurring in individuals under the age of 20.
Symptoms:
- Pain: Pain is a common symptom of Askin's tumor, often described as a dull ache or sharp pain that worsens over time.
- Swelling: The affected area may become swollen, red, and warm to the touch.
- Weakness: Patients may experience weakness or fatigue due to the cancer's impact on their overall health.
Diagnosis:
- Imaging Studies: Askin's tumor is typically diagnosed using imaging studies such as X-rays, CT scans, and MRI scans.
- Biopsy: A biopsy of the affected tissue is often performed to confirm the diagnosis and rule out other conditions.
Treatment:
- Surgery: Surgical removal of the tumor may be possible in some cases, but this is often not feasible due to the cancer's aggressive nature.
- Chemotherapy: Chemotherapy is a common treatment approach for Askin's tumor, involving the use of powerful medications to kill cancer cells.
- Radiation Therapy: Radiation therapy may also be used to treat the cancer and alleviate symptoms.
Prognosis:
- Poor Prognosis: Unfortunately, Askin's tumor has a poor prognosis due to its aggressive nature and potential for rapid spread.
- High Mortality Rate: The mortality rate for Askin's tumor is high, with many patients succumbing to the disease despite treatment efforts.
According to [1], Askin's tumor accounts for only 2-3% of all bone cancers in children. Despite its rarity, this aggressive cancer requires prompt and effective treatment to improve patient outcomes.
References:
[1] Askin's tumor: A rare and aggressive form of extraskeletal Ewing's sarcoma. (Source: Search Result 1)
Additional Characteristics
- Askin's tumor, also known as extraskeletal Ewing's sarcoma (EES), is a rare and aggressive type of bone cancer.
- Rapid Growth: Askin's tumor grows quickly, often within a matter of weeks or months.
- Aggressive Behavior: This type of cancer can be highly aggressive and may spread to other bones, lymph nodes, and organs such as the lungs, liver, and brain.
- Young Age Affected: Askin's tumor typically affects children and young adults, with most cases occurring in individuals under the age of 20.
- Pain is a common symptom of Askin's tumor, often described as a dull ache or sharp pain that worsens over time.
- The affected area may become swollen, red, and warm to the touch.
- Patients may experience weakness or fatigue due to the cancer's impact on their overall health.
- Askin's tumor has a poor prognosis due to its aggressive nature and potential for rapid spread.
- Unfortunately, Askin's tumor accounts for only 2-3% of all bone cancers in children.
Signs and Symptoms
Askin's tumor, also known as extraosseous Ewing sarcoma, can present with a range of symptoms and signs. Here are some of the common ones:
- Respiratory problems: Patients may experience pain, dyspnea (shortness of breath), cough, chest tightness, and shortness of breath [1][7].
- Chest wall mass: A lump or bump can be felt on the chest wall, which may feel soft or warm if the sarcoma has progressed out of the bone [4].
- Bone pain: Pain at the site of the tumor is a common symptom [4].
- Fever and anorexia: Some patients may experience fever, loss of appetite (anorexia), and weight loss [2][5].
- Swelling and redness: Swelling and redness around the painful areas can be observed during physical examination [3].
- Lump or bump: A lump or bump can be felt on the affected area, which may be soft or warm to touch [4].
It's worth noting that most patients with Askin tumor are asymptomatic at clinical presentation, although some may experience fever, anorexia, and weight loss [5]. Early detection and diagnosis are crucial for effective treatment.
References: [1] Singh A (2016) - Cited by 10 [2] Our patient presented some clinical signs and symptoms commonly seen in Askin's tumors... [3] Physical exam: Your doctor will start out with this, especially checking around the painful areas for signs such as lumps, redness, and swelling... [4] Bone pain at the site of the tumor · Lump or bump that may feel soft or warm, if the sarcoma has progressed out of the bone · Broken bones from even moderate... [5] Covello B (2021) - Most patients with Askin tumor are asymptomatic at clinical presentation, although fever, anorexia, and weight loss have been reported [1]. [7] Zhang KE (2016) - Clinically, Askin's tumor commonly presents with the symptoms of fever, cough, chest pain, chest tightness and shortness of breath.
Additional Symptoms
- Respiratory problems
- Swelling and redness
- Fever and anorexia
- Lump or bump
- mass in chest
- bone pain
Diagnostic Tests
Askin's tumor, also known as Ewing sarcoma, can be diagnosed through various diagnostic tests. Here are some of the common procedures used to diagnose this condition:
- Physical examination: A thorough physical examination is usually the first step in diagnosing Askin's tumor. The doctor will examine the patient's chest wall and mediastinum for any abnormalities.
- Imaging studies: Imaging studies such as computed tomography (CT) scans, magnetic resonance imaging (MRI), and positron emission tomography (PET) CT are used to visualize the tumor and its extent. These tests can help identify the location and size of the tumor [1].
- Histopathology and immunohistochemistry: Histopathological examination of tissue samples from the tumor is essential for diagnosing Askin's tumor. Immunohistochemical analysis can also be performed to confirm the diagnosis [2][4].
- Cytologic smears: Cytologic smears of the tumor can reveal small round cells, which are characteristic of Askin's tumor [3].
It's worth noting that a combination of these diagnostic tests is often used to confirm the diagnosis of Askin's tumor.
References:
[1] 5. by T Xia · 2014 · Cited by 17 — In summary, CT and FDG-PET/CT images of Askin tumors showed heterogeneous soft tissue masses in the chest wall and the mediastinum, accompanied by rib ...
[2] 4. by KE ZHANG · 2016 · Cited by 25 — Histopathology and immunohistochemical analysis are the basis for Askin's tumor diagnosis.
[3] 4. by KE ZHANG · 2016 · Cited by 25 — Cytologic smears of the tumor revealed small round cells, which are characteristic of Askin's tumor.
[5] 5. by T Xia · 2014 · Cited by 17 — In summary, CT and FDG-PET/CT images of Askin tumors showed heterogeneous soft tissue masses in the chest wall and the mediastinum, accompanied by rib ...
Additional Diagnostic Tests
- Physical examination
- Imaging studies (CT scans, MRI, PET CT)
- Histopathology and immunohistochemistry
- Cytologic smears
Treatment
Treatment Options for Askin's Tumor
Askin's tumor, also known as extrapulmonary Ewing's sarcoma, is a rare and aggressive type of cancer that requires prompt and effective treatment. While there is no standard therapy available due to its rarity, various treatment options have been explored and reported in medical literature.
Multimodal Therapy
A multimodal approach consisting of surgical resection, chemotherapy, and radiotherapy has shown some benefit in treating Askin's tumor [2]. This combination of treatments aims to remove the tumor surgically, followed by chemotherapy and radiation therapy to eliminate any remaining cancer cells.
Chemotherapy Protocols
Several chemotherapy protocols have been reported in medical literature. For example, a study published in 2016 described a patient who was started on a combination of six drugs chemotherapy protocol (VACAc/IE): Vincristine, doxorubicin, cyclophosphamide, ifosfamide, and etoposide [3]. Another study mentioned the use of vincristine, doxorubicin, and cyclophosphamide in an alternate scheme [6].
Investigational Therapies
Recent investigations have explored the use of monoclonal antibodies, such as bevacizumab (Avastin) and sunitinib, in treating Askin's tumor [4]. These investigational therapies aim to target specific molecular mechanisms involved in cancer growth.
Current Treatment Guidelines
The current treatment guidelines for Ewing sarcoma, which includes Askin's tumor, typically involve chemotherapy and/or radiation followed by surgery to remove any remaining cancer [7].
Survival Outcomes
Studies have reported improved survival outcomes with a multimodal treatment approach that includes surgery, chemotherapy, and radiotherapy [8]. However, more research is needed to establish the most effective treatment protocols for Askin's tumor.
In summary, while there is no standard therapy available for Askin's tumor, various treatment options have been explored and reported in medical literature. A multimodal approach consisting of surgical resection, chemotherapy, and radiotherapy has shown some benefit, and investigational therapies such as monoclonal antibodies are being investigated.
References:
[1] XUE DOU · 2013 · Cited by 17 [2] I Takanami · 2002 · Cited by 32 [3] A Singh · 2016 · Cited by 10 [4] TG Korol · 2022 · Cited by 1 [5] XUE DOU · 2013 · Cited by 17 [6] [7] Feb 22, 2024 [8] S Triarico · 2018 · Cited by 8
Recommended Medications
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Differential Diagnosis
What is Differential Diagnosis?
Differential diagnosis refers to the process of identifying and ruling out other possible causes of a patient's symptoms or condition, in order to arrive at an accurate diagnosis [10].
Askin's Tumor: A Rare Primitive Neuroectodermal Tumoral
Askin's tumor, also known as Askin tumor, is a rare primitive neuroectodermal tumoral developing from the soft tissues of the chest wall. Its diagnosis approach is complex and requires a multidisciplinary team [13].
Differential Diagnosis of Askin's Tumor
The differential diagnosis of Askin's tumor includes other sarcomas such as chondrosarcoma, pleuropulmonary blastoma, lymphoma, and infection [3]. In children and adolescents, the differential diagnosis should also include Ewing sarcoma, neuroblastoma, Langerhans's cell histiocytosis, Wilms tumor, and non-Hodgkin lymphoma [1].
Other Conditions to Consider
Older age at presentation further broadens the differential diagnosis to more likely include rhabdomyosarcoma, chondrosarcoma, lymphoma, neurofibroma, as well as a metastatic disease [11]. When the mass is mediastinal or paraspinal and in a child, neuroblastoma should also be considered.
Importance of Accurate Diagnosis
Accurate diagnosis of Askin's tumor is crucial for optimal treatment. Early clinical suspicion is essential, and Askin's tumor must be kept in mind in the differential diagnosis of small round cell malignant tumors of the thorax [8].
References:
[1] Context 1 [3] Context 3 [8] Context 8 [11] Context 11 [13] Context 13
Additional Differential Diagnoses
- Askin's tumor
- metastatic disease
- infection
- Langerhans's cell histiocytosis
- Wilms tumor
- lymphoma
- non-Hodgkin lymphoma
- Ewing sarcoma
- chondrosarcoma
- rhabdomyosarcoma
- neurofibroma
- pleuropulmonary blastoma
- neuroblastoma
Additional Information
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- IAO_0000115
- An Ewing sarcoma that arises from the soft tissues of the chest wall that tend to recur locally and not widely disseminated.
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- Askin's tumour
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- owl#annotatedSource
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- DOID:0050608
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- Askin's tumor
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