fbpx
Blog, Soft Tissue Sarcoma

Soft Tissue Sarcoma-Treatment


The soft tissue sarcoma treatment usually depends on many factors, including but not limited to the type of sarcoma, stage of disease, the location of the disease, patient’s age and overall health, side effects associated with the treatment, patient’s preference, along with other factors. Following are the preferred treatment approaches for different stages of soft tissue sarcoma, but the final decision is taken after clinical assessment of the patient by an oncologist.

1.STSs of the Trunk, Extremities (limbs), Head and Neck, Abdominal/Thoracic Visceral Organs, and Retroperitoneum:

Stage Preferred Treatment
Stage I Stage I-STSs are low-grade tumors of any size. If possible, surgical resection with negative margins is considered as the standard treatment.

 

In a case when negative margins were not obtained, further surgical resection to remove all cancerous tissue is the preferred approach. If further surgery is not possible, radiotherapy may be employed to decrease the chances of disease recurrence.

 

If the tumor is present at such a location (e.g. retroperitoneum, trunk, and head and neck,) that it cannot be entirely removed without functional disability, radiotherapy may be employed as the first-line treatment. This may cause the tumor to shrink sufficiently that it can be removed surgically.

 

The patient should be followed-up closely after treatment for any sign of recurrence.

Stage II or III Most Stage II- and III-STSs are high-grade tumors that tend to spread quickly and some of them have already spread to nearby lymph nodes. If possible (without any significant functional disability), surgical resection with negative margins is considered as the standard treatment. Any lymph node with the sign of disease spread should also be removed. Radiotherapy with/without chemotherapy may be considered after complete removal of the tumor.

In case of the positive margin, further surgical resection to remove all cancerous tissue is the preferred approach. If further surgery is not possible, radiotherapy may be employed to decrease the chances of disease recurrence.

 

If tumor is too large or present at such a location (e.g. retroperitoneum, trunk, and head and neck,) that it cannot be entirely removed without functional disability, or if surgery is not possible due to overall health of the patient, radiotherapy with or without chemotherapy may be employed as the first-line treatment. This may cause the tumor to shrink sufficiently that it can be removed with surgery. Further treatment, such as more radiotherapy/chemotherapy and/or supportive care or any other treatment may be provided based on the response to initial treatment.

 

The patient should be followed-up closely after treatment for any sign of recurrence.

Stage IV Stage IV-STSs are cancers that have spread to a distant body parts. For patients with a single site of disease spread and a small primary tumor that can be entirely removed with surgery, a surgical resection with the negative surgical margins for the primary tumor and the distant site is considered as the preferred treatment. As appropriate, tumor ablation, embolization, or radiotherapy may be employed instead of surgical resection for the secondary tumors.

For patients with a widespread disease or when the primary and secondary tumors cannot be removed by surgery, chemotherapy is usually employed. Any other suitable treatment may be employed as palliative treatment.

 

2.Gastrointestinal Stromal Tumors (GISTs)

Tumor characteristic Preferred Treatment
Localized, resectable tumors For small, resectable, low-grade tumors, surgical resection to remove all cancerous tissue is the preferred approach. Targeted drug (e.g. Imatinib) should be employed after surgical resection to prevent disease recurrence in case of intermediate or high risk disease.

 

The patient should be followed-up closely after treatment for any sign of recurrence.

Localized, marginally resectable tumors For localized GISTs that cannot be completely removed with surgery (marginally resectable tumors), targeted therapy should be considered as the first-line treatment. This should be continued until the cessation of clinical benefit. In case of a good response to treatment (sufficient shrinkage to resectable disease), surgical resection to remove all cancerous should be attempted. After surgery, a continuation of the targeted drug is generally recommended to prevent disease recurrence.

 

In case of the cessation of clinical benefit and unresectable disease, the dose of the targeted drug should be increased or a different drug may be employed.

 

The patient should be followed-up closely during and after treatment for any sign of recurrence.

Disseminated, unresectable tumors For disseminated GISTs that cannot be removed with surgery (unresectable tumors), targeted therapy should be considered as the first-line treatment. This should be continued until the cessation of clinical benefit. In case of a good response to treatment (sufficient shrinkage to resectable disease), surgical resection to remove all cancerous should be attempted.

In the case of the cessation of the clinical benefit and unresectable disease, the dose of the targeted drug should be increased or a different drug may be employed.

 

3.Rhabdomyosarcoma (RMS):

The standard treatment approach for RMS is not yet established and it is still evolving with the availability of new treatment options. Pleomorphic RMSs are usually treated with a treatment approach similar to that for other STSs. For the treatment of embryonal and alveolar RMS, patients should refer to institutions with experience in treating RMS. All children and adults with RMS are treated with a multimodality treatment approach. Chemotherapy, surgery and/or radiotherapy is usually the part of the multimodality treatment approach. The surgery may be performed before chemotherapy, if possible, without any significant functional disability or other major cosmetic issues. Otherwise, chemotherapy and/or radiotherapy are employed as first-line treatment. In case of the sufficient shrinkage in the tumor to render it respectable, surgery may be employed to remove all cancerous tissue. All these treatments may be employed separately or in combination when the tumor continues to grow or comes back after the initial treatment.

 

4.Kaposi sarcoma (KS):

Type Preferred Treatment
Epidemic/AIDS-related KS For patients with AIDS-related KS, treatment of the HIV infection with highly active antiretroviral therapy (HAART) is considered the primary treatment. Treatment of AIDS leads to better immune function and relieves the symptoms of KS as well. Prophylactic treatment for opportunistic infection or immediate treatment of any infection should be provided as supportive care.

 

For patients with localized disease (good risk patients) and presence of symptoms, besides HAART, a local treatment is usually employed, which may include radiation therapy, cryosurgery, a topical retinoid, intralesional chemotherapy, or local excision.

 

For patients with widespread disease (patients with poor risk), lymphedema, or other serious complications, HAART and systemic chemotherapy are usually employed to control disease symptoms. Targeted drugs or immunotherapeutic agents may be employed alone or in combination with chemotherapy to slow down or delay the progression of the disease. HAART should be continued with the cessation of chemotherapy and/or targeted therapy upon resolution of skin lesions.

Classic/Mediterranean KS Classic KS grow and spread very slowly and do not cause any symptom unless it has spread to vital body parts. These patients are also at a high risk of developing secondary cancer and should be followed up closely to monitor the same.

 

For patients with localized disease, surgery, radiotherapy, or intralesional chemotherapy is considered as the preferred treatment.

 

For patients with disseminated disease, chemotherapy is usually employed to control disease symptoms. Radiotherapy may be added to chemotherapy for local/superficial disease control.

Endemic/African KS Endemic Ks is usually observed in poor countries where treatment options are limited. When possible treatment similar to that of classic KS should be employed for endemic KS.
Iatrogenic/transplant-related KS For patients with transplant-related KS, cessation or dose reduction of the immunosuppressive agents is recommended to ease the disease symptoms. In place of conventional immunosuppressive drugs, angiogenesis inhibitor (e.g. sirolimus) may be employed as anti-rejection treatment. This enables the immune system to recover and fight against the KS cells. Radiotherapy and other local treatments can be employed to relieve the local disease symptoms.

 

Following is the brief description of various treatment types employed for different STSs:

Surgery: Surgery is the treatment of choice for most early-stage and some higher stage STSs that has not spread to distant body parts and can be completely removed. The goal of surgery is to remove entire cancerous tissue along with some healthy tissue. This is possible for some early-stage disease where the tumor is confined to a specific area, and complete resection can be performed with ease. However, a complete resection is not always possible, such as in the case of advanced-stage disease, the disease involving a vital organ/structure, or when a surgical resection will lead to a significant functional disability. Surgery may also be performed to collect biopsy sample in some cases.

Radiation Therapy: Radiation therapy uses high-energy x-rays or other high-energy radiations which are directed to the affected area to kill cancerous cells. Radiotherapy can be employed either by using an external radiation source (external beam radiation therapy) or by directly placing the source of radiation near the cancer tissue (brachytherapy). It can be employed alone as the first-line treatment for certain early-stage STSs or can be combined with other treatment options, such as surgery and/or chemotherapy for some advanced-stage STSs. It may be considered in some patients who cannot tolerate surgery or in case of disease recurrence after primary treatment. It is sometimes used for palliation of symptoms of the disease such as pain, bleeding, and obstructive problems

Chemotherapy: Chemotherapy means treatment with anti-cancer drugs that kill or decrease the growth of rapidly growing cancer cells. Chemotherapy is generally employed for the treatment of advanced stage STS that has spread to distant body parts and cannot be removed completely with surgical resection. Depending on the physician’s preference and patient’s condition, it may also be combined with other treatment options, like radiotherapy, to accelerate the benefit achievement. Many pharmaceutical companies are conducting a number of clinical trials to find out new drugs and drug-combinations with increased efficacy and specificity to target cancer cells. Chemotherapy may be associated with side effects due to its effect on normal body cells apart from cancerous cells.

Targeted Therapy: Targeted drugs works differently than chemotherapy drugs that they target a specific gene or protein characteristic of the cancer cells that help them to divide and grow indefinitely. For example, Olaratumab targets PDGFR-alpha, Pazopanib targets tyrosine kinases, Imatinib targets both the KIT and PDGFRA proteins, etc. They are generally used alone or in combination with chemotherapy for the treatment of advanced-stage STSs or STSs that possess specific proteins.

Ablation and Embolization: These techniques are generally employed to treat GISTs that have spread to the lungs or liver. In ablation, small secondary tumors are destroyed without actual removal from the body. High-energy radio waves are utilized in radiofrequency ablation (RFA), microwaves are used in microwave ablation (MWA) technique, while very cold gases are used in cryoablation to destroy tumors. In embolization, small (relatively larger than ablation) secondary cancers are destroyed by blocking the blood supply to the cancer cells with the help of some inert tiny particles (particles are loaded with drugs in chemo-embolization and with a radioactive substance in radio-embolization) that are injected directly in the artery supplying blood to cancer cells. Embolization can sometimes be employed in conjunction with ablation. Imaging techniques are utilized along with these techniques to accurately locate the target tumors.

Local Therapy for Kaposi Sarcoma: Patients with Kaposi sarcoma (KS) generally required local therapy for controlling localized/superficial disease lesions. Topical agents, for example, alitretinoin, a retinoid drug may be used topically to relieve pain and swelling associated with these lesions. Local excision may be employed using simple surgical excision, curettage (tumor removal by scraping), electrodesiccation (tumor cell destruction using electric current), or cryosurgery (killing cancer cells by intense cooling). Intralesional chemotherapy (injecting the chemotherapeutic drug directly into the KS lesion) can also be used for local disease control. These treatments help in controlling local disease symptoms and do not cause systemic side-effects.

Palliative Treatment: It do not directly treat STSs but helps in improving the overall quality of life by providing relief from the symptoms and by reducing the suffering caused by the disease. It is generally given as supportive care for advanced staged STSs along with other treatments. It may include but not limited to: using drugs or other interventions to reduce swelling, pain, bleeding, and other symptoms such as nausea, vomiting, and diarrhea; surgical interventions or radiotherapy may also be employed.

It is very important to assess the benefits of each treatment option versus the possible risks and side effects before making a treatment decision. Sometimes, patient’s choice and health condition are also important to make a treatment choice. Following are ultimate goals for soft tissue sarcoma treatment:

  • Prolongation of life.
  • Reduction of symptoms.
  • Improvement of overall quality of life.

Join our community of fighters and survivors, motivate and uplift each other to fight cancer together.


Leave a Reply

 

No Logo Added!
s