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sarcoma

Sarcoma

A rare class of cancers known as sarcomas develops in the body's connective tissues, which include the bones, muscles, fat, blood vessels, nerves, and fibrous tissues. Sarcomas form in the tissues that support and connect various body parts, as opposed to carcinomas, which originate from epithelial cells (such as those in the breast, lung, or colon).

Sarcomas can develop practically anywhere in the body due to their origin. They are frequently divided into two primary categories: soft tissue sarcomas and bone sarcomas, each of which has several subtypes. Although some types are more prevalent in one age group than the other, sarcomas can affect both adults and children.

What Are the Types of Sarcoma?

Sarcomas are broadly divided into:

  • Soft tissue sarcomas: These include cancers of the nerves (malignant peripheral nerve sheath tumors), blood vessels (angiosarcoma), muscle (leiomyosarcoma, rhabdomyosarcoma), fat (liposarcoma), and fibrous tissue (fibrosarcoma).
  • Bone sarcomas: Such as osteosarcoma, Ewing’s sarcoma, and chondrosarcoma.

There are more than 70 distinct subtypes of sarcoma, and they can differ significantly in how they behave. While some are indolent and grow slowly over many years, others are aggressive and grow quickly.

What Causes Sarcoma?

The cause of sarcoma is typically unknown. Nonetheless, a few risk factors have been found:

  • Genetic conditions such as familial retinoblastoma, neurofibromatosis type 1, and Li-Fraumeni syndrome raise the risk.
  • Previous radiation therapy, may eventually result in radiation-induced sarcoma, particularly in childhood or early adulthood.
  • Exposure to certain chemicals like dioxins and vinyl chloride have been connected to uncommon types of sarcoma.
  • Chronic swelling or lymphedema, especially after cancer treatment, can sometimes lead to angiosarcoma.
  • A history of bone disorders, such as Paget’s disease, may increase the risk of bone sarcoma in older adults.

That said, most people who develop sarcoma have no identifiable risk factor.

Signs and Symptoms

In their early stages, sarcomas frequently show few symptoms, which makes diagnosis difficult. The most typical presentations consist of:

  • A painless lump or swelling, often in the arm, leg, or abdomen
  • Persistent or worsening pain, especially in bone sarcomas
  • Limited mobility or nerve compression if the tumour presses against nearby structures
  • Fractures in bone sarcomas due to weakening of the bone
  • Weight loss, fatigue, or general malaise in advanced disease

These symptoms are occasionally disregarded or initially misdiagnosed because they can mimic benign conditions like lipomas or muscle strains. Any deep, firm, and growing lump needs to be examined further.

Diagnosis and Evaluation

Sarcoma diagnosis necessitates a combination of imaging, biopsy, and clinical evaluation:

  • MRIis frequently the recommended imaging method for soft tissue sarcomas, particularly those that affect the limbs.
  • CT scans may be applied to bone sarcomas or to detect metastases, especially to the lungs.
  • PET-CT can direct treatment choices and aid in assessing the full extent of the illness.
  • Core needle biopsy or surgical biopsy is necessary to determine the sarcoma subtype and confirm the diagnosis.

Following diagnosis, the tumor is staged and graded (low, intermediate, or high grade) to determine its aggressiveness, size, depth, and spread.

Treatment Options

A multidisciplinary team comprising surgical oncologists, medical oncologists, radiation oncologists, radiologists, and pathologists is frequently needed for the highly individualized treatment of sarcoma.

1. Surgery

The mainstay of treatment for the majority of localized sarcomas is surgery. Removing the tumor while leaving a visible margin of healthy tissue is the aim. When it is feasible, limb-sparing surgery is preferred to amputation for extremity sarcomas.

2. Radiation Therapy

To lower the chance of recurrence, radiation therapy can be administered either before or after surgery. It's particularly useful when getting precise surgical margins is challenging.

3. Chemotherapy

Chemotherapy can be administered either before (neoadjuvant) or after surgery and is frequently used for aggressive or high-grade sarcomas. Rhabdomyosarcoma and Ewing's sarcoma are two sarcoma subtypes that are especially sensitive to chemotherapy.

4. Targeted Therapy and Immunotherapy

According to tumor genetics and receptor status, more recent therapies like immunotherapy (e.g., immune checkpoint inhibitors in specific subtypes) or targeted therapy (e.g., pazopanib, trabectedin) may be taken into consideration in cases of advanced or metastatic disease.

Rehabilitation and Follow-Up

Following major surgery or radiation therapy, recovery from sarcoma treatment may entail:

  • Physical therapy to restore mobility and strength
  • Pain management
  • Psychological support to help cope with disfigurement or functional limitations

Because sarcomas can recur or spread even years after initial treatment, long-term follow-up is essential. Ongoing surveillance includes routine physical examinations and imaging.

Outlook and Survival

Prognosis in sarcoma depends on multiple factors:

  • Tumour size and depth
  • Grade and subtype
  • Whether it has spread (metastatic disease)
  • Patient’s age and overall health

When treated promptly, localized sarcomas have good survival rates, frequently exceeding 70–80%. Although advanced disease is more difficult to treat, many patients are finding that new treatments are giving them hope and extending their survival.

When to See a Specialist

It is crucial to be assessed and treated at a facility with experience in treating sarcomas due to their complexity and rarity. A sarcoma specialist can be consulted early to prevent misdiagnosis and guarantee the best possible treatment planning right away.

Medical oncologist Dr. Amit Badola actively treats sarcomas systemically. For soft tissue and bone sarcomas, he specializes in chemotherapy, targeted therapy, and immunotherapy, all of which are provided as a part of an all-encompassing, team-based approach that emphasizes evidence-based and customized care.