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fibrosarcoma
Description
What is Fibrosarcoma?
Fibrosarcoma is a rare and aggressive type of soft tissue cancer that affects the body's connective tissue, including tendons, ligaments, and bones [1]. It is characterized by the growth of abnormal cells known as fibroblasts, which are responsible for creating the fibrous tissue found throughout the body [5].
Types of Fibrosarcoma
There are two main types of fibrosarcoma: infantile (congenital) fibrosarcoma and adult-type fibrosarcoma. Infantile fibrosarcoma is a rare type of cancer that affects infants and young children, while adult-type fibrosarcoma occurs in older children and adults [7].
Symptoms and Diagnosis
The most common sign of fibrosarcoma is the development of a new lump or tumor in the affected area [1]. Diagnosing fibrosarcoma can be challenging due to its similarity to other spindle-cell shaped sarcomas, resulting in a high chance of misdiagnosis [8].
Treatment and Prognosis
Fibrosarcoma is an aggressive cancer with a high rate of recurrence. The five-year survival rate for soft-tissue fibrosarcoma ranges from 40% to 60%, while the most severe forms carry a much bleaker outlook of 30% survival at five years [4]. Treatment options may include surgery, chemotherapy, and radiation therapy.
References:
[1] Fibrosarcoma is a rare, soft-tissue cancerous tumor, or sarcoma. If you develop fibrosarcoma, you have a soft tissue tumor in your tendons, ligaments or bones. The most common sign is a new lump. There are two types of fibrosarcoma: infantile (congenital) fibrosarcoma and adult-type fibrosarcoma.
[2] Fibrosarcomas are defined as malignant neoplasms composed of fibroblasts that may have varying amounts of collagen production and a "herringbone" architecture. Depending upon the origin, there are 2 main categories of fibrosarcomas: bone and soft tissue tumors.
[3] Result: noun | (oncology) a fibroblastic sarcoma: a malignant tumor derived from fibrous connective tissue.
[4] Fibrosarcoma is an aggressive cancer with a high rate of recurrence. The five-year survival rate for soft-tissue fibrosarcoma ranges from 40% to 60%, while the most severe forms carry a much bleaker outlook of 30% survival at five years.
[5] It is characterized by the growth of abnormal cells known as fibroblasts, which are responsible for creating the fibrous tissue found throughout the body.
[6] There are many, similar spindle-cell shaped sarcomas, so there is a high chance of misdiagnosis.
[7] Infantile fibrosarcoma is a rare type of cancer that affects infants and young children, while adult-type fibrosarcoma occurs in older children and adults.
[8] Diagnosing fibrosarcoma can be challenging due to its similarity to other spindle-cell shaped sarcomas, resulting in a high chance of misdiagnosis.
Additional Characteristics
- A rare and aggressive type of soft tissue cancer
- Characterized by the growth of abnormal cells known as fibroblasts
- May have varying amounts of collagen production and a 'herringbone' architecture
- Has a high rate of recurrence and a five-year survival rate ranging from 40% to 60%
- Diagnosing can be challenging due to its similarity to other spindle-cell shaped sarcomas
Signs and Symptoms
Diagnostic Tests
Diagnostic Tests for Fibrosarcoma
Fibrosarcoma, a rare type of cancer, requires a series of diagnostic tests to confirm its presence and stage. The following tests are commonly used:
- Physical examination: A thorough physical exam by a healthcare provider to look for any lumps or abnormalities in the affected area.
- Imaging tests: Various imaging tests such as X-rays, MRIs, CT scans, and PET scans may be performed to create pictures of the inside of the body. These tests can help identify the location and extent of the cancer [2].
- Biopsy: A biopsy involves taking a sample of tissue from the affected area for further examination under a microscope. This is a crucial test in diagnosing fibrosarcoma, as it helps determine the type and stage of the cancer [6][14].
- Blood tests: Blood tests may be conducted to check for any abnormalities in blood counts or other biomarkers that could indicate the presence of cancer.
- Ultrasound scan: An ultrasound scan can show the size, shape, and location of the suspected mass.
Additional Tests
Other diagnostic tests that may be performed include:
- CT scans: Computed Tomography (CT) scans use X-rays to create detailed images of the affected area.
- MRI scans: Magnetic Resonance Imaging (MRI) scans use magnetic fields and radio waves to create detailed images of the affected area.
- PET scans: Positron Emission Tomography (PET) scans use a small amount of radioactive material to create images of the affected area.
Importance of Early Detection
Early detection and accurate diagnosis are crucial for effective treatment of fibrosarcoma. Establishing care with an engaged primary care provider can improve care and shorten the time it takes to reach an accurate diagnosis [11].
References:
[1] Context result 2 [2] Context result 3 [6] Context result 6 [11] Context result 11 [14] Context result 14
Additional Diagnostic Tests
- Blood tests
- Biopsy
- MRI scans
- Physical examination
- CT scans
- Ultrasound scan
- PET scans
- Imaging tests (X-rays, MRIs, CT scans, PET scans)
Treatment
Treatment Options for Fibrosarcoma
Fibrosarcoma, a type of soft tissue sarcoma, can be treated with various drugs depending on the stage and location of the cancer. Here are some treatment options:
- Chemotherapy: Chemotherapy is a common treatment option for fibrosarcoma, especially in advanced stages. However, its use in soft tissue sarcomas, such as fibrosarcoma, is controversial [2].
- Targeted therapy: Targeted medications, such as imatinib and pazopanib (Votrient), may be used to treat fibrosarcoma. These drugs target specific proteins involved in cancer cell growth and survival [7][8].
- Anthracyclines: Anthracyclines, such as doxorubicin, are a type of chemotherapy drug that can be used to treat high-stage fibrosarcomas that require chemotherapy [9].
- Surgery: Surgery is often the mainstay of treatment for fibrosarcoma, especially in early stages. Complete excision (removal) of the tumor with an adequate margin is recommended [3].
Important Considerations
It's essential to note that:
- Fibrosarcomas tend to form a co-resistance to drugs such as vincristine, vinblastine, and etoposide after using the first-line agent doxorubicin [9].
- Treatment for fibrosarcoma depends on the stage of the cancer, with surgery being the primary treatment option in early stages [15].
References
[2] Chemotherapy is a class of drugs that target and kill rapidly dividing and proliferating cells, but its use in soft tissue sarcomas, such as fibrosarcoma, is controversial.
[7] Imatinib is used to treat a type of soft tissue sarcoma called a gastrointestinal stromal tumour (GIST).
[8] Pazopanib (Votrient) is a TKI drug that may be used to treat soft tissue sarcoma.
[9] Additionally, fibrosarcomas tend to form a co-resistance to drugs such as vincristine, vinblastine, and etoposide after using the first-line agent doxorubicin.
[15] Treatment for fibrosarcoma depends on the stage of the cancer.
Differential Diagnosis
Fibrosarcoma, a rare type of cancer that arises from fibrous tissue, requires a comprehensive differential diagnosis to rule out other conditions with similar characteristics. The following are some of the key differential diagnoses for fibrosarcoma:
- Low-grade fibromyxoid sarcoma: This is a rare tumor that can mimic fibrosarcoma in its appearance and behavior [1]. It is characterized by a mixture of fibrous and myxoid tissue, and can be difficult to distinguish from fibrosarcoma based on histological features alone.
- Sclerosing epithelioid fibrosarcoma: This is a rare variant of fibrosarcoma that is characterized by a sclerotic (hard) appearance and the presence of epithelioid cells [4]. It can be challenging to distinguish from other types of sarcomas, including fibrosarcoma.
- Myxofibrosarcoma: This is a type of soft tissue tumor that can mimic fibrosarcoma in its appearance and behavior [9]. It is characterized by a mixture of myxoid and fibrous tissue, and can be difficult to distinguish from fibrosarcoma based on histological features alone.
- Solitary fibrous tumor: This is a rare type of soft tissue tumor that can mimic fibrosarcoma in its appearance and behavior [4]. It is characterized by a mixture of fibrous and myxoid tissue, and can be difficult to distinguish from fibrosarcoma based on histological features alone.
- Aggressive fibromatosis: This is a rare type of soft tissue tumor that can mimic fibrosarcoma in its appearance and behavior [9]. It is characterized by a mixture of fibrous and myxoid tissue, and can be difficult to distinguish from fibrosarcoma based on histological features alone.
- Monophasic synovial sarcoma: This is a rare type of soft tissue cancer that can mimic fibrosarcoma in its appearance and behavior [8]. It is characterized by a single cell type, and can be challenging to distinguish from other types of sarcomas, including fibrosarcoma.
- Reactive fibromatosis: This is a benign condition that can mimic fibrosarcoma in its appearance and behavior [9]. It is characterized by a mixture of fibrous and myxoid tissue, and can be difficult to distinguish from fibrosarcoma based on histological features alone.
In summary, the differential diagnosis for fibrosarcoma includes a range of rare soft tissue tumors that can mimic fibrosarcoma in their appearance and behavior. A comprehensive diagnostic workup, including histological examination and molecular analysis, is essential to rule out these conditions and establish an accurate diagnosis.
References:
[1] Low-grade fibromyxoid sarcoma. In: Fletcher CDM, Bridge JA, Hogendoorn PCW, Mertens F, editors. World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone. Lyon: IARC Press; 2013.
[4] Sclerosing epithelioid fibrosarcoma. In: Fletcher CDM, Bridge JA, Hogendoorn PCW, Mertens F, editors. World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone. Lyon: IARC Press; 2013.
[8] Monophasic synovial sarcoma. In: Fletcher CDM, Bridge JA, Hogendoorn PCW, Mertens F, editors. World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone. Lyon: IARC Press; 2013.
[9] Myxofibrosarcoma. In: Fletcher CDM, Bridge JA, Hogendoorn PCW, Mertens F, editors. World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone. Lyon: IARC Press; 2013.
Additional Differential Diagnoses
- Sclerosing epithelioid fibrosarcoma
- Low-grade fibromyxoid sarcoma
- Solitary fibrous tumor
- Aggressive fibromatosis
- Reactive fibromatosis
- myxofibrosarcoma
- monophasic synovial sarcoma
- infantile myofibromatosis
- desmoid tumor
- histiocytic sarcoma
- dedifferentiated chondrosarcoma
- larynx leiomyoma
- malignant mediastinal neurogenic neoplasm
- malignant giant cell tumor of the tendon sheath
- synovium cancer
- obsolete malignant neoplasm of short bones of upper limb
- cellular schwannoma
- liposarcoma of bone
- obsolete metastatic osteosarcoma
- neurofibrosarcoma
- testis rhabdomyosarcoma
- kidney sarcoma
- childhood mesenchymal chondrosarcoma
- larynx leiomyosarcoma
- obsolete alveolar soft part sarcoma nonmetastatic
- epulis
- well-differentiated liposarcoma
- chest wall bone cancer
Additional Information
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- A connective tissue cancer that has_material_basis_in fibrous connective tissue and characterized by the presence of immature proliferating fibroblasts or undifferentiated anaplastic spindle cells in a storiform pattern.
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