Introduction
The retroperitoneum is a part of the abdominal cavity that is situated between the space present between the peritoneum and the posterior abdominal wall. Each space contains parts of various organs such as the kidneys, pancreas, rectum, esophagus, aorta, adrenal glands, and some parts of the stomach. The anterior pararenal contains the head, neck, and pancreas. The posterior pararenal space is composed of blood vessels and fat. Sarcoma is a type of benign tumor or cancer that begins in soft tissues or bone and blood vessels.
What Is Retroperitoneal Sarcoma?
Retroperitoneal sarcomas are a rare type of tumor that slowly increases in size over time. Only one to two percent of the cases are malignant, and of all the sarcomas, they occur outside the retroperitoneum. Only 10 to 20 percent of sarcomas are retroperitoneal sarcomas. The size of these tumors is less than two inches, and that is why many a time are left unnoticed.
What Are the Types of Retroperitoneal Sarcoma?
The most common types of retroperitoneal sarcoma include:
-
Liposarcoma: Around 50 to 60 percent of cases are of liposarcoma. This type of cancer generally originates from fat cells and is a type of soft tissue sarcoma. It can occur in any part of the body where the fat cells are present (mostly in arms and legs). Most liposarcomas increase slowly and hardly cause any pain.
-
Leiomyosarcoma: Around 20 to 30 percent of cases are related to leiomyosarcoma. It is an aggressive type of cancer where the tumor grows rapidly. It originates from the smooth muscle, such as of intestines, stomach, and blood vessels. It is more common in adults than children.
What Causes Retroperitoneal Sarcoma?
There is no clear cause of retroperitoneal sarcoma, as it can affect anyone. Liposarcoma is said to affect fat cells, but there is no clear association with obesity. A mutation in the fat cells and smooth muscle is considered one of the causes that can lead to either liposarcoma or leiomyosarcoma. A gene mutation occurs when there is a change in a gene’s DNA (deoxyribonucleic acid) sequencing.
What Are the Symptoms of Retroperitoneal Sarcoma?
Some of the common symptoms include:
-
Swelling in the legs.
-
Abdominal mass.
-
Blood in stools.
-
Loss of appetite or feeling after eating a small amount of food.
-
Shortness of breath.
-
Exertion.
How Is Retroperitoneal Sarcoma Diagnosed?
The doctor will first clinically examine the patient and ask about the recent symptoms. The doctor will also look for a lump or swelling around the legs, abdomen, or arms. To further confirm the diagnosis, certain tests are done.
-
Computed Tomography Scan - In many cases, a computed tomography scan is considered a most useful diagnostic tool that also helps in diagnosing the deformities related to it. It is an imaging technique that uses computer technology and X-ray to get clear 3D images of the inside of the body. A computed tomography scan has a round or donut-shaped tube that rotates the X-rays around 360 degrees. First, a healthcare professional will ask the patient to remove any jewelry and other metal objects that can affect the scan. Next, the patient is asked to lie on the machine's table, which moves slowly in the scanner. Finally, the images of the affected area are taken and transferred to the computer. It takes around 10 to 30 minutes to complete the scan.
-
Needle Biopsy - A needle biopsy is done to detect the mass and cells in the body. After getting a computed tomography scan done, the doctor gets an idea of the targeted area from where the sample needs to be taken. To numb the area, anesthesia may be given. A fine needle is inserted into the skin, and a sample of cells is collected. The process is repeated a few times to collect enough cells. Once the sample is collected, it is sent to a laboratory for further testing.
How Is Retroperitoneal Sarcoma Managed?
Surgery is considered to be one of the most effective treatments for retroperitoneal sarcoma. After detecting the location of the sarcoma, a doctor will surgically remove it. In many cases, the tumor grows in size and spreads to the surrounding blood vessels and organs. In half of the retroperitoneal sarcoma surgeries, a kidney and a portion of large intestines are removed. Also, in some cases, the blood vessels are removed and reconstructed. These surgeries are complex and need proper monitoring. Chemotherapy and radiotherapy without surgery are rarely beneficial either used alone or in combination. These tumors are locally invasive, therefore extensive local resection of the tumor and any adjacent organs should be performed at the time of presentation.
What Are the Risk Factors for Retroperitoneal Sarcoma?
Some of the inherited conditions can increase an individual’s risk of getting retroperitoneal sarcoma.
-
Nevoid Basal Cell Carcinoma: It is a group of defects that are passed down from families. It involves the eyes, bones, and reproductive system.
-
Tuberous Sclerosis: Also known as tuberous sclerosis complex, it is a genetic disorder that leads to many noncancerous tumor growths in different parts of the body.
-
Werner Syndrome: Werner syndrome is a progressive disorder that is characterized by unusual accelerated aging (progeria) due to genetic mutation.
-
Neurofibromatosis Type 1: Neurofibromatosis type 1 is a condition that leads to changes in the color of the skin and tumors growth along the nerves.
Conclusion
Retroperitoneal sarcoma is a rare cancerous growth that occurs mainly in or around kidneys and can also spread to other organs. The size of this sarcoma is not that big, which is the prime reason it is left undiagnosed for a long time. Any symptoms that are related to this condition should not be neglected for a long time and consulted with the doctor immediately. Computed tomography scans and biopsy help in confirming the diagnosis. Surgical intervention helps in removing the tumor and stopping it from spreading to other organs. Continuous monitoring and regular visits to the doctor, even after a successful operation, help in avoiding the recurrence of this sarcoma.
