Spindle Cell Sarcoma

What is Spindle Cell Sarcoma?

It is a rare form of cancer. It begins on the skin, soft tissues, and connective tissues.

The shape of the cancer cells is spindle and so it is named spindle cell sarcoma. It starts with just a small lump and inflammation and then the symptoms slowly progress as the cancer grows from one stage to another.

• Stage 1 – The lump is self-contained. You can barely notice it unless check under the microscope. It is important to discover spindle call sarcoma in the first stage as it can easily be treated through excision of the cancer formation.
• Stage 2 and 3 (advanced stage) – In these stages, most of the cancer cells already metastasized to other parts of the body making it hard to treat. The cancer cells could have spread to other important organs such as the lungs, kidneys, and liver.
• Spindle cell sarcoma stage 4 – This is a severe stage wherein the tumor has spread to other parts of the body including the lymph nodes. ^{(1, 2, 3, 4, 5)}

Image 1: A primary tumor categorized as intermediate grade spindle cell sarcoma. Figure B showing the MRI scan of the affected part of the body
Picture Source: journal.sajc.org

Picture 2: Figure shows a CT scan of spindle cell sarcoma and figure 2 shows the tumor after the resection surgery.
Photo Source: cancerwall.com

Spindle cell sarcoma causes

There are various factors that lead to the development of spindle cell sarcoma such as previous injuries, infections, and genetic predisposition. If a body suffers infection or inflammation, the connective tissues will initiate healing.

They will undergo cell division, which increases the risk of cancer. Another possible cause of spindle cell sarcoma is direct contact with carcinogenic chemicals. Anyone can have a spindle cell carcinoma, but it is more prevalent in people 40 years old and above. ^{(4, 5, 6)}

Spindle cell sarcoma symptoms

The type and severity of symptoms of spindle cell sarcoma depends on the size, location, and stage of malignancy. Some patients have ulcerated mass on the exposed areas of the skin. The patient feels exhausted and tired.

The patient also has fever and unexplained weight loss. Other clinical manifestations include swelling and tenderness in the affected area, reduced mobility on the affected area, and bone pain. ^{(6, 7)}

Types

Undifferentiated sarcoma of the bone

This type of sarcoma primarily involves the bones.

Malignant fibrous histiocytoma

It is a rare type, which affects the uppers and lower limbs. It commonly affects middle-aged people.

Fibrosarcoma

It primarily affects the thigh bone. ^{(7, 8, 9)}

Diagnosis

To accurately diagnose the condition of the patient, the doctor will perform a physical examination and will order various tests such as X-ray, bone scan, MRU, and biopsy. A biopsy is needed to accurately assess the cause of the symptoms.

The test is performed in a clinical lab and the sample is checked under the microscope. X-rays, CT scan and MRI do not accurately diagnose a spindle cell sarcoma. What they do is they give a clear view on the location and size of the tumor.

They also determine if the tumor has metastasized to other parts of the body. It is only the biopsy that will confirm the diagnosis of spindle cell sarcoma. However, it would take about two weeks to know the result of the biopsy.

Differential diagnosis are also performed if the diagnostic tests revealed that the patient is negative for spindle cell sarcoma. A number of health conditions with identical symptoms with spindle cell sarcoma are osteolytic osteosarcoma, metastatic carcinoma, and aneurysmal bone cyst.

However, if the patient tests positive for spindle cell sarcoma, the patient will be treated in a bone cancer center. ^{(2, 5, 7, 8)}

Treatment options

There are a variety of treatment modalities for spindle cell sarcoma such as radiology, surgery, and chemotherapy. There are instances when the affected part of the body needs to be amputated. The commonly performed surgery is called limb sparing surgery.

The tumor along with a margin of healthy tissue is removed to make sure that all tumor cells are thoroughly removed. It also lessens the possibility of recurrence. The tumor cells are carefully removed with consideration to the cosmetic appearance of the affected limb.

Examples of limb-sparing surgical procedures are:

• Resection ⁽⁶⁾
• Allograft
• Autograft
• Irradiation/reimplantation
• Metallic replacement ⁽¹⁾

The patient should undergo chemotherapy before or after the surgery, depending on the extent of the condition of the patient. Neoadjuvant therapy is given to the patient before the surgery. Its purpose is to destroy the tumor and control its spread.

Neoadjuvant therapy shrinks the size of the tumor. An adjuvant chemotherapy is performed after the surgical procedure. This is to make sure that the remaining tumor cells are destroyed. The commonly used drugs for chemotherapy are Cisplatin, Methotrexate, Doxorubicin, and Ifosamide. ^{(3, 7, 8, 9)}

Spindle cell sarcoma Prognosis

The prognosis is good if the condition is detected and treatment is initiated at an early stage. The tumor can be easily removed through surgical intervention. However, if the disease has advances in the end stage, the prognosis is poor. ^{(2, 3, 5)}

Survival rate

The maximum survival rate is five years for people with advanced stage spindle cell sarcoma. There are a lot of factors that could greatly affect the person’s chance of survival such as the type of sarcoma, location of the tumor, age of the patient, and the type of treatment received by the patient.

Generally, 90% of patient survived if diagnosed and treated at an early stage. Stage II sarcoma has 81% of survival while stage III has 56% survival rate. The rate of survival is extremely low if the sarcoma has metastasized to other parts of the body such as in the case of stage IV spindle cell sarcoma. ^{(1, 6, 7)}

References:

1. https://en.wikipedia.org/wiki/Spindle_cell_sarcoma
2. https://www.bcrt.org.uk/information/information-by-type/spindle-cell-sarcoma/
3. https://cancerwall.com/spindle-cell-sarcoma/
4. http://www.innovateus.net/innopedia/what-are-symptoms-spindle-cell-sarcoma
5. http://www.chaplainnorris.com/spindle-cell-sarcoma-a-rare-form-of-cancer/
6. https://www.cancercenter.com/soft-tissue-sarcoma/types/
7. http://cancer.ygoy.com/2011/03/26/what-is-spindle-cell-sarcoma-cancer/
8. https://www.sciencedirect.com/topics/veterinary-science-and-veterinary-medicine/spindle-cell-sarcoma
9. http://onlinelibrary.wiley.com/doi/10.1111/j.1349-7006.2003.tb01456.x/pdf