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spindle cell sarcoma

Description

What is Spindle Cell Sarcoma?

Spindle cell sarcoma is a rare and aggressive type of cancer that originates in the bone or soft tissue. It is characterized by its unique appearance under a microscope, where the cancerous cells appear long and narrow (spindle-shaped).

Types of Spindle Cell Sarcoma

There are several types of spindle cell sarcomas, including those found in muscle tissue, bone, and soft tissue. These tumors can occur anywhere in the body but are most commonly found in the arms, legs, and pelvis.

Demographics

Spindle cell sarcoma is more frequently diagnosed in adults over 40 years old. It affects slightly more males than females, and its incidence is extremely rare, making up only 2-5% of all primary bone cancer cases.

Symptoms

The symptoms of spindle cell sarcoma vary depending on the size and location of the tumor. Common signs include:

  • Bone pain that may be constant or intermittent
  • A broken bone (pathological fracture) due to weakened bone structure

Causes and Risk Factors

There is no known definite cause for spindle cell sarcoma, but it can sometimes occur as a result of previous radiotherapy.

References:

  • [1] Spindle cell sarcoma is a soft tissue tumor that originates in a bone. When viewed under a microscope, the cancerous cells appear long and narrow (spindle-shaped). While this rare malignancy can affect anyone, it is most frequently diagnosed in adults 40 and older.
  • [4] Spindle cell sarcomas of the bone are often found in the arms, legs and pelvis. They most commonly arise in patients over the age of 40 and are extremely rare, making up just 2-5% of all primary bone cancer cases.
  • [5] Spindle cell sarcoma can occur at any age but more commonly affects middle-aged and older people. It affects slightly more males than females.
  • [10] Spindle cell sarcoma symptoms vary depending on tumor size, the number of tumors and which bones (or other body parts) contain cancer cells.
  • [13] What is spindle cell sarcoma? Spindle cell sarcoma is a rare malignant (cancerous) tumour which can develop in the bone or soft tissue.

Additional Characteristics

  • Spindle cell sarcoma is a rare and aggressive type of cancer that originates in the bone or soft tissue.
  • It affects slightly more males than females, and its incidence is extremely rare, making up only 2-5% of all primary bone cancer cases.
  • Symptoms include bone pain and pathological fracture due to weakened bone structure.
  • There is no known definite cause for spindle cell sarcoma, but it can sometimes occur as a result of previous radiotherapy.
  • It most commonly affects adults over 40 years old.

Signs and Symptoms

Diagnostic Tests

Diagnostic Tests for Spindle Cell Sarcoma

Spindle cell sarcoma diagnosis involves a combination of imaging studies, biopsies, and histopathological analysis to confirm the presence of this rare type of cancer.

  • Imaging tests: These include X-rays, Computerized Tomography (CT) scans, Magnetic Resonance Imaging (MRI), and Fluorescence in situ hybridization (FISH) testing. [1][2][8]
  • Biopsy: A biopsy is a crucial diagnostic tool for spindle cell sarcoma, where a sample of the tumor tissue is taken and examined under a microscope to confirm the presence of cancer cells. [3][6]
  • Physical examination: A physical exam by a healthcare provider can help identify symptoms and provide clues that may aid in diagnosis. [11]
  • Immunohistochemical markers: These are used as reliable surrogates for molecular testing in spindle cell sarcoma, particularly in cases where the tumor's genetic characteristics cannot be classified. [12]

Diagnostic Approach

The diagnostic approach for spindle cell sarcoma typically involves a combination of imaging studies and core needle biopsy performed prior to undertaking surgery. [9] This comprehensive approach helps ensure accurate diagnosis and effective treatment planning.

References:

[1] Apr 6, 2018 - A CT scan uses x-rays to make detailed cross-sectional images of your body. [2] Tests to diagnose soft tissue sarcoma, such as x-ray, CT, MRI and biopsy, are done when symptoms are present. [3] The diagnostic approach should include imaging studies and core needle biopsy performed prior to undertaking surgery. [8] Apr 5, 2024 - Fluorescence in situ hybridization (FISH) testing is used to identify common translocations that can aid in the diagnosis of soft tissue ... [9] by XG del Muro · 2016 · Cited by 61 — The diagnostic approach should include imaging studies and core needle biopsy performed prior to undertaking surgery. [11] A physical exam. Your doctor will likely do a physical exam to better understand your symptoms and look for other clues that will help with your diagnosis. [12] Synovial sarcoma is a spindle cell sarcoma that arises most commonly in the deep soft tissue of adolescents and young adults, often as a circumscribed mass adjacent to a joint (although it is unrelated to synovium). ... there now exists a multitude of immunohistochemical markers that can act as reliable surrogates for molecular testing in the ...

Treatment

Treatment Options for Spindle Cell Sarcoma

Spindle cell sarcoma, a rare and aggressive form of cancer, requires prompt and effective treatment to manage its progression. While surgery is the primary treatment method, drug therapy plays a crucial role in addressing this condition.

  • Chemotherapy: Chemotherapy uses anti-cancer drugs to destroy cancer cells. It may be used as an adjuvant therapy after surgery or as a standalone treatment for advanced cases (12).
  • Targeted Therapy: Targeted medications, such as pazopanib and regorafenib, have shown promise in treating spindle cell sarcoma by blocking specific enzymes that promote cancer growth (3, 11). These drugs may be used to treat advanced cases or as a maintenance therapy.
  • Immunotherapy: Immunotherapies stimulate the immune system to fight cancer cells. While not specifically mentioned for spindle cell sarcoma, immunotherapies have shown efficacy in treating other types of soft tissue sarcomas (14).

Key Points

  • Surgery is the primary treatment method for spindle cell sarcoma.
  • Chemotherapy and targeted therapy are used as adjuvant or standalone treatments for advanced cases.
  • Immunotherapy may be explored for its potential benefits in treating this condition.

References

[3] This page lists cancer drugs approved by the Food and Drug Administration (FDA) for soft tissue sarcoma. The list includes generic names and brand names. The drug names link to NCI's Cancer Drug Information summaries. There may be drugs used in soft tissue sarcoma that are not listed here.

[11] The multi-targeted drug pazopanib, a tyrosine kinase inhibitor targeting VEGFR, a platelet-derived growth factor receptor, and a c-kit were not included, as the treatment is given to many sarcoma patients with genetic testing with good clinical responses [22,23,24,25,26,27,28]. The same is true for regorafenib [29,30].

[12] The first treatment method for spindle cell sarcoma is surgery. The surgeon will remove the tumour and will aim to take out an area of normal tissue too – this is known as “taking a margin”. This is to make sure that all of the cancer has been removed ... This treatment uses anti-cancer drugs to destroy cancer cells. It is sometimes used ...

[14] Chemotherapy is a treatment for soft tissue sarcoma that uses drugs to attack cancer cells. It generally works by attacking rapidly dividing cells in the body. Other therapies target the genetic mutations found in tumors or stimulate the immune system to fight the cancer.

Differential Diagnosis

Additional Information

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