The husband of one breast cancer patient from Indonesia told us that he regretted for not subjecting his mother to chemotherapy after she had undergone surgery for her sarcoma. After the surgery, the cancer recurred and she had two more surgeries before she died.

I told this man, “You should not regret for not giving her the chemo. It is most likely that chemo would not be effective anyway.”

 

 

Review of Medical Literature on Sarcoma

Sarcoma is malignant tumor that can be divided into 2 groups:

1. Bone sarcomas, arising from bone or cartilage, and

2. Soft tissue sarcomas, arising from tissues such as fat, muscle, nerves and nerve sheath, blood vessels, and other connective tissues.

Soft tissue sarcomas are the most frequently occurring sarcomas. There are more than 50 different subtypes of soft tissue sarcoma. Some examples of soft tissue sarcoma:

• Angiosarcoma arises from blood vessels
• Kaposi’s sarcoma arises from blood vessels
• Fibrosarcoma arises from fibrous tissue
• Leiomyosarcoma arises from smooth muscle
• Liposarcoma arises from fat
• Malignant peripheral arises from nerve sheath tumor arises from Nerve tissue
• Rhabdomyosarcoma arises from skeletal muscle.

Sarcomas are more common among children, accounting for 15% of pediatric cancers, but become less frequent with age, accounting for about 1% of all adult cancers.

They can occur anywhere in the body.  Around 60% of sarcomas develop in the arms or legs. The rest begin in the gastrointestinal tract (25%), the back of the abdominal cavity and its internal organs, called the retroperitoneum (15-20%), or the head and neck area (9%).

Treatment

1. Surgery

Surgery has been the preferred primary treatment for soft tissue sarcoma. The aim of surgery is to completely remove or excise the tumor. A border or margin of 2-3 cm of normal tissue around the tumor is also desirable to minimize the chance that tumor cells have been left behind.

Nonetheless, treatment with marginal surgery has been associated with local failure rates of 30 to 50 percent. Generally, small, low grade tumors can be treated with surgery alone.

2. Radiotherapy

The standard practice for the treatment of soft tissue sarcomas is radiotherapy in combination with surgical resection.

3. Chemotherapy

One of the major deterrents to adjuvant chemotherapy has been the difficulty in justifying exposure to the significant toxicities of these drugs for potentially non-responding patients.

Even in the best of circumstances, only 30 to 50 percent of patients with soft-tissue carcinomas will respond to standard chemotherapeutic regimen.

Systemic cytotoxic chemotherapy is generally not considered curative for patients with metastatic soft tissue sarcomas.

Treatment of patients with soft tissue sarcomas remains a challenge.

Recurrent Disease

There is always a possibility that a soft tissue sarcoma will recur.

Surgery may be possible if it is a limited recurrence.

It may be difficult to give additional radiation if the tumor recurs in an area that has already received maximum radiation in the past.

Chemotherapy is often offered.

Metastasis

The incidence of metastasis in high-grade soft-tissue sarcomas is 20 to  50 percent when the primary tumour diameter is greater than 5 cm.

Fifty percent of soft tissue sarcoma patients will die from distant metastasis.

One common site of metastasis (50 percent) is the lung, followed in frequency by liver, bone and to a lesser degree, skin.

Patients with retroperitoneal sarcomas had a greater tendency for local recurrence and disseminated disease throughout the abdomen.

Patients with head and neck and truncal sarcomas had a higher local recurrence rate than those with extremity sarcomas.

Prognosis

The overall relative 5-year survival rate of people with soft tissue sarcomas is around 50% according to statistics from the National Cancer Institute (NCI).

The 5-year survival rates for soft tissue sarcomas have not changed much for many years. The 5-year survival rates were:

• 83% for localized sarcomas (56% of soft tissue sarcomas were localized when they were diagnosed)
• 54% for regional stage sarcomas; (19% were in this stage)
• 16% for sarcomas with distant spread (16% were in this stage)

References

http://sarcomaoncology.com/s_as_sarcoma.html

http://www.cancer.org/cancer/sarcoma-adultsofttissuecancer/detailedguide/sarcoma-adult-soft-tissue-cancer-survival-rates

I.J. Spiro et al. Soft tissue sarcoma (in Clinical Oncology, Ed. Philip Rubin. Health Science Asia, Elsevier Science).

A. Yasko et al. Sarcomas of soft tissue and bone (in Clinical Oncology. Ed. Raymond Lenhard, et al. Amer. Cancer Society).

Vernon Sondak & A. Chang. Clinical evaluation and treatment of soft tissue tumors (in Soft Tissue Tumours, Ed. Sharon Weiss and J. Goldblum, Heath Science Asia, Elsevier Science).