retroperitoneal sarcoma

What is Retroperitoneal Sarcoma?

Retroperitoneal sarcoma (RPS) is a rare type of cancer that occurs in the retroperitoneum, an area at the back of the abdominal cavity near the kidneys and pancreas. It accounts for approximately 15% of all soft tissue sarcomas and 0.1-0.2% of all malignancies [4][14].

Types of Retroperitoneal Sarcoma

The most common type of RPS is liposarcoma, which arises from fat cells. Other types include leiomyosarcoma (smooth muscle), solitary fibrous tumor, and undifferentiated pleomorphic sarcoma [6][11]. The exact histological subtype can be challenging to identify and is crucial for determining the optimal treatment plan.

Symptoms of Retroperitoneal Sarcoma

The symptoms of RPS can be vague and nonspecific, making it difficult to diagnose early. Common symptoms include:

• A noticeable lump in the abdomen
• Increase in abdominal girth
• Dull pain in the abdomen or back
• Intense abdominal pain with bleeding [5][12]

Characteristics of Retroperitoneal Sarcoma

RPSs are often large at presentation, with nearly 50% being larger than 20 cm in diameter [2]. They can grow to massive sizes, weighing up to 30 pounds. The average tumor size at presentation is around 15 cm, and the average age at diagnosis is 54 years old [13].

Treatment of Retroperitoneal Sarcoma

Due to its rarity, RPS requires a multidisciplinary team in a specialized center for optimal management. Treatment depends on the anatomical characteristics and histotype of the tumor. It's essential to identify the histologic subtype accurately, as this can significantly impact treatment decisions [10][11].

References:

[1] INTRODUCTION. Retroperitoneal sarcoma (RPS) is a rare tumor that accounts for 0.15% of all malignant tumors and approximately 15% of all sarcomas.

[2] Nearly 50% of RPSs are larger than 20 cm in diameter.

[3] The average age at presentation is 54 years old, with an equal male-to-female distribution.

[4] Retroperitoneal sarcoma accounts for approximately 0.1-0.2% of all malignancies.

[5] Common symptoms include a noticeable lump in the abdomen, increase in abdominal girth, dull pain in the abdomen or back, and intense abdominal pain with bleeding.

[6] The most common type of RPS is liposarcoma, which arises from fat cells.

[7] Other types include leiomyosarcoma (smooth muscle), solitary fibrous tumor, and undifferentiated pleomorphic sarcoma.

[8] The exact histological subtype can be challenging to identify and is crucial for determining the optimal treatment plan.

[9] RPSs are often large at presentation, with nearly 50% being larger than 20 cm in diameter.

[10] Treatment depends on the anatomical characteristics and histotype of the tumor.

[11] Due to its rarity, RPS requires a multidisciplinary team in a specialized center for optimal management.

Symptoms of Retroperitoneal Sarcoma

Retroperitoneal sarcomas can present with a range of symptoms, which can vary depending on the size and location of the tumor. Here are some common signs and symptoms:

• Abdominal discomfort or fullness: Many patients experience a sensation of heaviness or fullness in their abdomen, which can be a vague and nonspecific symptom.
• Weight loss: As the tumor grows, it can cause weight loss due to decreased appetite or difficulty eating.
• Early satiety: Some patients may feel full after eating a small amount of food, leading to early satiety.
• Hernia: In some cases, the tumor can cause a hernia, which is a protrusion of tissue through a weakened area in the abdominal wall.
• Anaemia: The tumor can also cause anaemia due to blood loss or decreased red blood cell production.
• Nonspecific symptoms: Other nonspecific symptoms include weakness, fever, and loss of appetite.

Rare Symptoms

In some cases, retroperitoneal sarcomas can cause more specific symptoms, such as:

• Shortness of breath: Large tumors can compress the lungs or diaphragm, leading to shortness of breath.
• Reflux: The tumor can also cause reflux by compressing the stomach or intestines.
• Constipation: Some patients may experience constipation due to compression of the bowel.
• Leg swelling: In rare cases, the tumor can cause leg swelling due to compression of the veins.

Important Note

It's essential to note that symptoms of retroperitoneal sarcoma can be vague and nonspecific, making it challenging to diagnose. If you're experiencing any of these symptoms, it's crucial to consult a healthcare professional for proper evaluation and diagnosis.

References:

• [1] Symptoms of retroperitoneal sarcomas can vary depending on the size and location of your tumour.
• [2] Other rare symptoms include early satiety (meaning feeling full after eating a small amount of food), weight loss, hernia or anaemia.
• [3] The first warning sign may be a sensation of heaviness or fullness in the abdomen.
• [4] Occasionally, retroperitoneal sarcoma can cause symptoms when the tumor is compressing or involving a nerve or blood vessel.

Diagnostic Testing for Retroperitoneal Sarcoma

Retroperitoneal sarcoma, a rare and aggressive type of cancer, requires accurate diagnostic testing to confirm the presence of the tumor and determine its size, location, and potential spread. The following tests are commonly used in the diagnosis of retroperitoneal sarcoma:

• Imaging Tests: Computed Tomography (CT) scans, Magnetic Resonance Imaging (MRI) scans, and Ultrasound are typically ordered to visualize the tumor and evaluate its size and location [1]. These imaging tests help identify the tumor's extent and potential involvement with surrounding structures.
• Needle Biopsy: A core needle biopsy is performed to obtain tumor tissue for histopathological examination [2]. This procedure is usually done by a radiologist and is an outpatient process. The biopsy sample helps confirm the diagnosis of retroperitoneal sarcoma and determine its specific type.
• Image-guided Percutaneous Coaxial Core Needle Biopsy: This is considered the most accurate diagnostic modality for establishing a definitive diagnosis [3]. It involves using imaging guidance to insert a needle into the tumor, allowing for the collection of tissue samples for further examination.

Additional Diagnostic Considerations

In addition to these tests, other factors are taken into account during the diagnostic process:

• Clinical manifestations: Symptoms secondary to retroperitoneal lesions appear late in the course of disease and are associated with the displacement of organs and obstructive phenomena [4].
• Histopathological types and behaviors: The management of retroperitoneal sarcomas can be challenging due to their variety of presentation, histopathological types, and behaviors [5].

References

[1] To visualize the tumor and evaluate its size and location, a physician will typically order images, such as a computed tomography (CT) scan, magnetic resonance imaging (MRI) scan or ultrasound.

[2] To accurately diagnose retroperitoneal sarcoma, a core needle biopsy is performed to obtain tumor tissue. This is an outpatient procedure done by a radiologist.

[3] Image-guided percutaneous coaxial core needle biopsy (14 or 16 gauge) is the most accurate diagnostic modality and the preferred method to establish a definitive diagnosis.

[4] Symptoms secondary to retroperitoneal lesions appear late in the course of disease and are associated with the displacement of organs and obstructive phenomena.

[5] The management of retroperitoneal sarcomas can be challenging due to their variety of presentation, histopathological types, and behaviors.

The management of retroperitoneal sarcomas can be challenging due to their varied presentation, histopathological types, and behaviors [1]. While surgical removal of the tumor is the main form of treatment for retroperitoneal sarcoma, chemotherapy or radiation therapy may also be administered in some cases [4].

Biopsy is recommended in all major retroperitoneal sarcoma treatment guidelines (NCCN, TARPSWG, ESMO) to accurately diagnose and stage the cancer [2]. The use of chemotherapy as a treatment for retroperitoneal sarcomas is dependent on the types of cells that make up the tumour [9].

Anthracycline-based chemotherapy (doxorubicin or epirubicin) is considered a first-line treatment, and the association with ifosfamide or dacarbazine can be considered in certain cases [8]. Neoadjuvant therapies (chemotherapy, external beam radiation, or combination radiation and chemotherapy) are also safe in well-selected patients and may be used to shrink tumors before surgery [7].

It's worth noting that treatment should be carried out in a sarcoma center of excellence, where multidisciplinary evaluation from surgical oncology, medical oncology, and radiation oncology can optimize clinical outcomes [11].