Sarcoma

Cancer is the abnormal growth of cells that can occur to anybody and any part of the body. The cancerous growth often is not diagnosed at the early stages, as many ignore the symptoms. The fact is also that the symptoms develop rapidly that when one tries to get treated, the stage of cancer is advanced. Cancer in the lungs, breast, prostate, intestines and the brain are often known to many. This is because many people suffer from these. But, a few types of cancers are less known, and one of the rare types of cancer is Sarcoma. This type of cancer is much different from the rest.

They occur in the kind of tissue that is much different from the rest. They grow in the connective tissues. These tissues support the other tissues of the body. For instance, the bones, muscles, cartilage and tendons are where the sarcoma is often found. But again they are not limited to these parts and can occur in any part of the body.

The sarcoma is classified into 2 major types. Soft tissue sarcoma and bone sarcoma. The latter is also called osteosarcoma. A surgical intervention is often required to remove the tumour. Sarcoma can be at higher risk in people who have blood relations suffering from this disease. When there is a bone disease called Paget’s disease, then sarcoma can occur. When people who work in places where radiation is high, for instance, the atomic power centres, the chances of being affected by sarcoma is higher. The genetic disorders like Gardner syndrome and neurofibromatosis can lead to the formation of sarcoma.

In fact, there are more than 50 types of sarcomas existing, but they fit into any of these major classifications. They have similar microscopic features and share the same set of symptoms.

A brief description of the popular types of sarcoma is listed here.

-  Undifferentiated pleomorphic sarcoma – this tumour is often found in the legs or arms. Also, it can affect the back of the abdomen

-  Gastrointestinal stromal tumour (GIST): The neuromuscular cells in the gut are affected by this type of sarcoma.

-  Liposarcoma: Fat tissues are affected by a tumour in this type

-  Leiomyosarcoma: the organs are covered by organ walls that are made of smooth muscles. This type of sarcoma affects these smooth muscles

-  Synovial sarcomas: the sarcomas that are found in the joints of arms and legs are classified thus

-  Malignant peripheral nerve sheath tumour: The term neurofibrosarcoma is used to denote this tumour, and the protective lining of the cells are affected by this tumour.

-  Rhabdomyosarcoma: This is a tumour that is common in kids compared with the adults. This tumour occurs in the muscles

-  Angiosarcomas: The tumour develops in the lymph vessels or the blood vessels

-  Fibrosarcomas: the tumour forms in the limbs that are formed from the fibroblasts. The fibroblasts are the most common type of connective tissues

-  Kaposi’s sarcoma: This type of sarcoma is caused by a virus. The organ affected is the skin. This disease is also linked to AIDS. This not only leads to lesions on the skin but can also affect the soft muscle tissues. Human Herpesvirus 8 causes this sarcoma.

-  Other types: the range of sarcomas run long. The dermatofibrosarcoma protuberance is a condition where skin lesions are caused. Myxoma affects elders and older adults, and often arms and legs are where the tumours are developed. A rare combination of various elements of sarcomas is called mesenchymomas.

Symptoms

The symptoms of sarcoma are often missed when they grow. This is because it is not easy to spot the growth of soft tissues, as they can grow anywhere and everywhere in the body. The often found symptom is the lump. This is painless until it gets bigger. When it goes bigger, it presses the nerves and makes this troublesome. Even breathing begins to be a tedious task with the bigger lumps. Unfortunately, it is not easy to find the symptoms before this stage, and no tests can identify the presence of these lumps, or sarcoma.

Pain is often the symptom but connected with various ailments. The nerves, tissues and muscles, when affected by the tumour, cause severe pain. Inflammation and swelling are on the cards, and this can grow as cancer grows.

There can be impairments based on the location of the sarcoma.

When it comes to the osteosarcoma the symptoms can be noticed way too early, compared with the soft tissue sarcoma. The bone where the growth happens, the pain is unbearable. This is even worse at the nights. When one starts experiencing pain, after a couple of weeks, can also see swelling in the area. When sarcoma affects legs, a limp is also seen.

When this type of sarcoma is present in the digestive tract, and the abdominal cavity, the internal bleeding results. Vomiting of blood or dark bowel motion result. The initial symptom is the pain in the abdominal region.

When a sarcoma is present in the lungs, suffocation and coughing happen often. Breathlessness also is caused. Based on the location, blockage of the organs can happen.

Compared with the elders, children and young adults are often the victims of sarcoma. Regrettably, the pain is associated with growing pains, as young kids and adults are often engaged in various activities, and the pain is mistaken. Especially, when the pain is worsening at nights, in children, consulting a doctor is necessary. Also, the pain sticks to one arm or one leg, and not on both. This is the major difference between sarcoma and growing pain.

Causes

The exact cause behind soft tissue sarcomas is not known. There are however many risk factors identified, and there are some genetic changes seen as a result. Cancer is caused by DNA mutation. The DNA mutation can be acquired over life rather than been caused by birth.

-  Radiotherapy:

Exposure to radiation can lead to sarcomas of less than 5%. When patients are given radiation to treat other types of cancer, the chances of developing sarcomas is higher. Once when a patient is given radiation, the diagnosis to find about sarcoma takes 10 years. However, thanks to the modern technology that ensures that radiation is done precisely to the cancerous spots, and the dosage is determined meticulously by the doctors. This implies that the number of people affected by sarcomas can be brought down considerably, provided the reason behind the disease is the radiation therapy.

-  Hereditary:

A few families have genetic mutations running in them. This can increase the risk of sarcomas and other types of cancers among the family.

Neurofibromatosis:

This type of benign tumours form under the skin in the nerves, and about 5% of people affected by this condition are affected by sarcoma. This condition is also called von Recklinghausen disease.

-  Gardner Syndrome:

The APC gene when affected causes this syndrome. This is a type of FAP, where multiple polyps are caused in the colon. Desmoid tumours are one of the problems caused by this syndrome. People affected by this are subject to soft tissue sarcoma and colon cancer.

-  Li-Fraumeni syndrome:

Defects in the TP53 gene cause this syndrome. The people who are affected by this syndrome are open to threats of various types of cancers, including sarcomas. However, the soft tissue sarcoma is caused only in 10 to 20% of the people affected by Li-Fraumeni syndrome. But, the risk percentage surges, when these people are also treated for other cancers with radiation therapy.

-  Retinoblastoma:

RB1 gene defects cause the eye cancer in children. They also have a higher risk of suffering from the soft tissue sarcoma. The risk percentage can go high if radiation therapy had been used to treat this type of cancer.

-  Werner syndrome:

The RECQL2 gene defects lead to this syndrome. Clogged arteries, cataracts, and skin changes that are related to elders are experienced by the children in this case. The risk of sarcomas is higher in these children.

-  Gorlin syndrome:

PTCH1 gene mutation is responsible for this syndrome. The affected people experience seizures and learning problems. Fibrosarcoma and rhabdomyosarcoma as the syndrome can lead to basal cell skin cancers.

-  Tuberous sclerosis:

TSC1 and/or TSC2 gene defects lead to this syndrome, and the affected area of higher risk of angiomyolipoma, cancer in kidneys. Risk of rhabdomyosarcoma is also higher.

-  Damaged lymph system:

A clear fluid called lymph is carried all through the body, by the lymph vessels. The lymph nodes are connected by these vessels. When these lymph nodes are damaged by radiation or removed, lymphedema is caused. This can also pave way for sarcoma.

-  Exposure to chemicals:

Exposure to various chemicals can lead to the formation of sarcomas in the liver. Arsenic can again lead to this disease.

-  How is it diagnosed?

A full set of exams and tests are required to diagnose this disease.

- A biopsy is required to diagnose the disease. For this, a small piece of flesh is taken from the affected area and sent for testing.

- CT scan, ultrasound scan and X rays are the imaging tests required to diagnose the severity and location of this disease. A CT guided needle biopsy is also used to detect this cancer.

- A bone scan might be required if the type of sarcoma is osteosarcoma.

- A PET scan is required to find how far cancer has spread. Instead of using multiple X rays, the PET scan gives the same results with single scanning. The radioactive deposits too can be found through this scan

- The extent of the tumour can be found by the MRI. The size of a tumour can be seen with this imaging test. It can also be helpful in finding from where this tumour begins.

Treatment

Various treatment types can be opted based on the type, severity and location of the sarcoma. Removal of a tumour through surgery is often on the cards. In case of osteosarcoma, the surgery removes only the cancer cells, and the organs like leg or arms are not removed.

Radiation therapy is used to shrink the cancer cells. Then it is followed by the surgery. The surgery kills the cells that are shrunk. When surgery cannot be done, this becomes the main treatment.

Chemotherapy

A single drug or combination of many drugs can be used to treat sarcoma. This is often the treatment that is started and works if cancer has not spread.

Based on the stages, the treatment options vary.

-  Stage 1: This is the initial stage of the disease. The surgery helps in curing the disease. This is in fact, the best way too. The tumour is very small, and less than 5 cm. But this works only if the cancer is not spread around the affected area. But, even in such cases, the radiation therapy is given to shrink the cancer cells. Then the surgery can be performed.

-  Stage 2: In this stage, cancer grows in size, and it becomes a little difficult to handle this cancer. As the cancer is spread over, removing it too can be a problem and affect the areas around the cancer cells, and if the cells are too large, then there is no way surgery can be used. So, obviously, radiation therapy is used to shrink the cells and then follows the surgery.

-  Stage 3: This is the final stage of the sarcoma. The cancer is now spread to the various parts. This is called metastasis. The cancer is spread wide across and very large in this stage. However, the therapies and surgery show some improvement in a few patients.

It has to be understood that the soft tissue sarcoma can come back even after successful treatment. The potency of the cancer cells can only determine this.

Prevention:

Prevention is not wholly possible. But one can always keep the risk away from staying off from the risk factors that can cause sarcoma. If the family members suffer from this cancer, regular tests to find if the cancer cells are starting to grow can help in faster treatment and efficient results.

Last updated on : 14-09-2018

Disclaimer : Docprime doesn’t endorse or take any guarantee of the accuracy or completeness of information provided under this article and these are the views strictly of the writer. Docprime shall not be held responsible for any aspect of healthcare services administered with the information provided on this article.

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