Uncertain Differentiation/Undifferentiated Pleomorphic Sarcoma

Uncertain differentiation refers to a group of rare soft tissue sarcomas that do not resemble other sarcoma types or normal cells under a microscope. These types of sarcoma are usually hard to diagnose. They are malignant sarcomas that tend to grow and spread fast to other parts of your body from the primary tumor site.

We treat various subtypes of uncertain differentiation, including undifferentiated pleomorphic sarcoma, a unique high-grade aggressive sarcoma because of its cellular makeup. 

At the Sarcoma Oncology Center, we design the most effective course of treatment for our patients. We ensure that they receive proper attention and care from prestigious oncologists who are specialists in sarcoma and its numerous types.

Understanding Uncertain Differentiation Sarcomas

Uncertain differentiation sarcoma is a sarcoma type that shares no common cellular resemblance to other soft tissue sarcomas. It represents approximately 20% of all soft tissue sarcomas. 

Differentiation means how different the sarcoma cells look under a microscope compared to normal cells. It is used in tumor grading systems, which are different for each type of cancer. Well-differentiated tumor cells look more like normal cells and tend to grow and spread more slowly than poorly differentiated or undifferentiated cancer cells. 

For the most part, surgery tends to be the best option for these types of sarcomas. It may also be combined with other treatment methods. 

Uncertain differentiation sarcoma has various subtypes that are characterized according to their appearance. 

Undifferentiated Pleomorphic Sarcoma or MFH Cancer

Undifferentiated pleomorphic sarcoma, formerly called malignant fibrous histiocytoma or MFH cancer, is a high-grade aggressive sarcoma that typically occurs in the arms or legs. In rare cases, it can occur in the area behind the abdominal organs, called the retroperitoneum. MFH cancer may also occur in a part of the body where a patient previously received radiation therapy. 

The name comes from the way the tumor cells appear under the microscope. Undifferentiated means the tumor cells lack any clear resemblance to normal cells and it is difficult to tell where in the body they originated from their appearance. Pleomorphic indicates that the tumor cells grow in multiple shapes and sizes without following the regular pattern of a normal cell. 

Undifferentiated pleomorphic sarcoma is a malignant sarcoma that grows quickly and spreads to other parts of the body, including the lungs. It usually occurs in older adults and may sometimes occur as a second sarcoma in patients who have retinoblastoma, a kind of eye cancer that starts in the retina. 

Epithelioid Sarcoma

Epithelioid sarcoma usually develops under the skin of the hands, forearms, feet, or lower legs. It often spreads to other body parts in the early stage of the tumor.

Epithelioid sarcoma causes firm growth under the skin, which is called a nodule. Sometimes, the growths may cause sores on the skin that do not heal. It often affects teenagers and young adults but can also affect older people.

Synovial Sarcoma

Synovial sarcoma is a malignant sarcoma that typically affects the leg, foot, hand, and knee joints. It begins as a growth of cells that multiplies quickly. The first symptom is usually a lump under the skin that may or may not hurt.

Alveolar Soft Part Sarcoma

In adults, this type of sarcoma is often found as a painless lump in the thigh or buttock. It typically affects men more than women. It can be difficult to diagnose in the early stage because the tumor forms a painless swelling. It can spread to other body parts, including the lungs, chest cavity, liver, and bone.

Clear Cell Sarcoma

Clear cell sarcoma is a rare tumor that shares features of both sarcoma and melanoma. It can travel to lymph nodes (typical of melanoma) and to the lung (typical of sarcomas). Although the origin of this cancer is unknown, it often develops in arm or leg tendons.

Extraskeletal Myxoid Chondrosarcoma

This type of uncertain differentiation sarcoma is a rare, slow-growing type of sarcoma that forms in soft tissues outside the bone. It may grow big and spread to nearby tissue or other body parts, especially the lungs. Extraskeletal myxoid chondrosarcoma is the second most common primary bone cancer of cartilage cells.

PNET/Extraskeletal Ewing Sarcoma

Ewing sarcoma develops in bones and the soft tissue around the bones. It is the third most common primary type of bone cancer. Although it can occur in any bone, this tumor typically begins in the leg bones and the pelvis. 

Desmoplastic Small Round Cell Tumor

This type of uncertain differentiation sarcoma grows in the abdomen and pelvic areas of the body and tends to spread further to the liver and lungs. This subtype may induce symptoms such as pain, nausea, vomiting, and swelling in the abdomen.

Causes & Symptoms of Uncertain Differentiation Sarcomas

Uncertain differentiation sarcomas likely arise from genetic predisposition and environmental factors. The exact cause of uncertain differentiation is not clear.

Like other subtypes of soft tissue sarcoma, uncertain differentiation may not have noticeable symptoms until it has reached an advanced stage or spread to another part of the body. Therefore, it is critical to pay attention to any abnormal signs and symptoms you may experience to ensure timely treatment for malignant tumors.

Symptoms of uncertain differentiation may include:

  • Lump formation
  • Painful swelling
  • Weight loss 
  • Fatigue
  • Nausea and vomiting
  • Breathing difficulty
  • Muscle weakness

Diagnosis of Uncertain Differentiation Sarcomas

Diagnosis of uncertain differentiation sarcoma depends on where the tumor has formed in your body. Your doctor may ask for the following tests: 

  • Physical Examination: Your doctor may examine your body to check for any abnormal symptoms, such as signs of lumps or swelling. They may also ask for your medical history.
  • Imaging Tests: Imaging tests like X-rays, CT scans, and MRIs may be conducted to take detailed images of the tumor in the affected area.
  • Biopsy: In a biopsy, a sample tissue from the tumor site is taken to determine the nature of the tumor, such as its size and whether it is benign or malignant. A biopsy may be performed via surgery or as a minimally invasive procedure.

Treatment of Uncertain Differentiation Sarcomas

The treatment for uncertain differentiation sarcoma comprises a personalized multimodal approach. It often combines standard treatments, such as surgery and radiation therapy, with new types of treatment, like clinical trials and targeted therapy.

At Sarcoma Oncology Center, our team collaborates with surgeons, radiologists, and radiation oncologists from prominent institutions to ensure our patients receive personalized attention. 

The following treatment options may be considered for treating uncertain differentiation sarcomas.

  • Surgery: Surgery is used to remove the tumor cells from the body. However, in cases where the tumor cells invade the surrounding tissue and vital organs, it may not be possible to remove all tumor cells by surgery.
  • Radiation Therapy: Radiation therapy is used to kill or shrink tumor cells using high-energy radiation beams. A radiation oncologist can perform either external or internal radiation, depending on the location of the tumor.
  • Chemotherapy: Chemotherapy uses strong medication to stop the growth of tumor cells. Your oncologist may also choose to perform chemotherapy before surgery.
  • Clinical Trial: Clinical trials use new treatment approaches for uncertain differentiation sarcomas. You may need clinical trials or more cutting-edge personalized treatments when standard treatments are not effective. 

Treatment of Uncertain Differentiation Sarcomas at Sarcoma Oncology Center

Uncertain differentiation sarcoma is unlike other types of soft tissue sarcoma. The treatment plan for this type of sarcoma is based on the tumor stage, prior treatments (if any), growth of the tumor, and biopsy analysis. 

At our office in Santa Monica, we design personalized and innovative plans for treating skeletal muscle sarcoma.

To schedule a consultation, please call us at 310-552-9999 or request an appointment online.

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