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Soft Tissue Sarcoma - Stages and Types of Soft Tissue Sarcoma

Home> Soft Tissue Sarcoma> Overview and Types

Soft tissue sarcoma occurs when the cells present in the soft tissues start dividing uncontrollably and form a tumor. There are several types of soft tissue sarcoma. The classification is required to determine the prognosis and appropriate treatment strategy. The staging of this condition is done according to the grade (aggressiveness) and size of the tumor.

How common is Soft Tissue Sarcoma?

Soft tissue sarcoma is a rare condition, and its incidence stands at 1.8–5 per 100,000 people per year. This sarcoma type constitutes 1–2% of all solid malignant neoplasms.

What Is Soft Tissue Sarcoma?

Several soft tissues are present in the body. These include tendons, muscles, blood vessels, nerves, and joint tissues. Soft tissue sarcoma occurs when the cells of these soft tissues grow uncontrollably, resulting in the formation of tumors, and it may develop in any soft tissue in the body. The most commonly affected soft tissues are the legs, arms, and abdomen. Soft tissue sarcomas are relatively rare compared to other types of cancer. This condition can affect children and adults, but adults are more commonly affected. The exact cause of this condition remains unknown, and the condition may occur spontaneously. Environmental factors and the presence of certain genetic factors enhance the risk of this condition.

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Types of Soft Tissue Sarcoma

There are several types of soft tissue sarcomas, and they are classified based on the tissues in which they occur. Some of the common types of soft tissue sarcoma in adults and children include:

Types of Soft Tissue Sarcoma in Adults

Certain types of soft tissue sarcomas are more common in adults. The exact reason for this is unknown. The following are the types of soft tissue sarcomas commonly occurring in adults:

Liposarcoma

Liposarcoma is a soft tissue sarcoma tumor that develops from the connective tissues, particularly the fat cells. It is one of the most common soft tissue sarcomas in adults and accounts for about 20% of all adult soft tissue sarcomas. Liposarcoma can occur in any body part but is most frequent in the extremities (thighs or upper arms), the back of the abdominal cavity, and the inguinal area.

Some liposarcoma types include well-differentiated liposarcoma, myxoid liposarcoma, and dedifferentiated liposarcoma.

Patients with liposarcoma experience painless swelling or lumps, and the condition is usually diagnosed with imaging studies (CT and MRI) and a biopsy. The prognosis of liposarcoma depends on various factors, including the stage of diagnosis, response to treatment, and the affected body organ.

Leiomyosarcoma

It is also a common subtype of soft tissue sarcoma in adults. It occurs in the smooth muscle cells. These cells are present in various body parts, including the gastrointestinal tract, uterus (uterine leiomyosarcoma), and blood vessels. Uterine leiomyosarcoma is a rare but aggressive form of leiomyosarcoma. Patients may have a painful palpable mass and other symptoms specific to the tumor location. Regarding prognosis, uterine leiomyosarcoma has a relatively poor prognosis compared to other leiomyosarcoma subtypes.

Undifferentiated pleomorphic sarcoma

Undifferentiated pleomorphic sarcoma was previously known as malignant fibrous histiocytoma (MFH). This condition is characterized by a lack of differentiation. In this sarcoma, the tumor cells do not resemble any specific type of normal tissue cells. Undifferentiated pleomorphic sarcoma is a high-grade and aggressive form of sarcoma and can occur in various soft tissues, including the legs, arms, trunk, and retroperitoneum. It grows rapidly and has the potential to metastasize to other body parts quickly. Given its aggressive nature and rapid metastasis, early diagnosis and prompt management improve outcomes in patients with undifferentiated pleomorphic sarcoma.

Types of Soft Tissue Sarcoma in Children

Certain types of soft tissue sarcoma are more common in children, and they include:

Rhabdomyosarcoma

Rhabdomyoblasts are the precursors to skeletal muscle cells. Rhabdomyosarcoma arises from rhabdomyoblasts. It is one of the most common types of soft tissue sarcoma in children. The two main types of rhabdomyosarcoma are embryonal rhabdomyosarcoma and alveolar rhabdomyosarcoma. The most common sites for this condition include the head and neck region and the genitourinary tract. The symptoms include pain, the presence of a visible mass, and swelling. The other signs are specific to the location of the tumor. Compared to adults, the overall survival rate for rhabdomyosarcoma is better in children.

Ewing sarcoma in soft tissue

It is also a type of soft tissue sarcoma in children. Ewing sarcoma usually occurs in the bones. However, it may also occur in the soft tissues of some patients. Ewing sarcoma of the soft tissues commonly occurs in the abdominal wall, chest wall, and other soft tissue locations. The symptoms experienced by the patients include pain, swelling, lumps, functional impairment, and limited range of motion. The diagnosis of the disease is confirmed by the presence of an EWSR1 gene rearrangement detected through genetic testing. The prognosis is relatively better in patients with early disease diagnosis and prompt treatment.

How serious is Soft Tissue Sarcoma?

Several factors affect the seriousness and severity of this condition. These factors include types of soft tissue sarcoma, tumor location, size, grade of tumor, and extent of tumor spread. High-grade aggressive sarcomas have a relatively poor prognosis. Further, sarcomas of the extremities, such as legs or arms, have a relatively better prognosis than those in critical locations, such as the neck, head, or retroperitoneum. Some subtypes of soft tissue sarcoma have high resistance to treatment and have high recurrence potential. Patients with those subtypes have a poor prognosis.

What are the Different Soft Tissue Sarcoma Stages?

Stages of soft tissue sarcoma include:

Stage 1

It is an early-stage soft tissue sarcoma. At stage 1, the tumor is of low grade (grade 1) and may be of any size. Nearby lymph nodes or distant organs are not affected.

Stage 2

The tumor at stage 2 is of grade 2 or grade 3. The tumor size is <5 cm. It has not affected the surrounding lymph nodes or the distant organs.

Stage 3

Stage 3 soft tissue sarcoma is of grade 2 or 3. The size is greater than 5 cm but not more than 10 cm (stage 3A) or greater (stage 3B). It has not affected the surrounding lymph nodes or the distant organs.

Stage 4

The stage 4 soft tissue sarcoma refers to a tumor that may be of any size and either spread to the nearby lymph nodes (stage 4A) or distant organs such as the liver (stage 4B). The tumor may be of any grade.



Conclusion

Soft tissue sarcoma originates from the soft tissues, such as connective tissues, blood vessels, and nerves. The types of soft tissue sarcomas include liposarcoma, leiomyosarcoma, and undifferentiated pleomorphic sarcoma in adults and rhabdomyosarcoma and Ewing sarcoma in soft tissue in children. Based on the extent of the disease’s growth and spread, this condition is staged between stages 1 and 4, with stage 1 referring to the early-stage disease and stage 4 referring to the advanced stage of the disease.

Frequently Asked Questions

Is soft tissue sarcoma curable?

The success rate depends on several factors. These include the disease diagnosis stage, the patient's overall health, response to treatment, subtypes of soft tissue sarcoma, and potential for recurrence.

The growth rate of soft tissue sarcoma depends on its type. Some types have a faster growth rate, while others have a slower growth rate.

These are low-grade tumors that may be of any size. The initial symptoms include swelling and pain.

Sarcoma is a type of cancer that originates from the mesenchymal tissues. Examples of sarcoma tumors include bone sarcoma and soft tissue sarcoma, based on the origin of sarcoma. In other words, soft tissue sarcoma is a type of sarcoma that forms in the body’s soft tissues.

Undifferentiated pleomorphic sarcoma is often considered one of the more aggressive types of soft tissue sarcoma.