small intestine leiomyosarcoma

Small Intestine Leiomyosarcoma: A Rare and Aggressive Cancer

Leiomyosarcoma (LMS) of the small intestine is a rare and aggressive type of cancer that originates from the smooth muscle cells in the small bowel. It is considered an extremely rare tumor, accounting for less than 5% of all gastrointestinal malignancies [2]. According to various studies, LMS represents only about 1.2% of all small bowel tumors, with sarcomas ranking fifth among these cases [2].

Characteristics and Symptoms

LMS of the small intestine is characterized by a proliferation of neoplastic spindle cells [6]. The tumor often presents as chronic nonspecific symptoms, including abdominal pain, melena (blood in stool), or chronic diarrhea [4]. In some cases, severe and persistent vomiting may be observed if the tumor affects the proximal small intestine [8].

Diagnosis and Treatment

The diagnosis of LMS is challenging due to its rarity and slow growth rate. Immunohistochemistry is essential to confirm the diagnosis [1]. Surgery is considered the only effective treatment for LMS, but it has a low response rate to chemotherapy [1]. Early diagnosis is crucial for improving patient outcomes.

Incidence and Demographics

LMS of the small intestine affects both men and women, with a slight male predominance observed in some studies [12]. The condition can develop in people of any age, but it's more common in individuals over 50 years old. According to the American Cancer Society, approximately 15,000 people are diagnosed with soft tissue sarcoma every year, which includes LMS [11].

References

[1] Keywords: Leiomyosarcoma, Small bowel, Ileal neoplasms, Case report. [2] Tumours of the small bowel account for less than 5% of all gastrointestinal malignancies. [4] by E Wilt · 2024 — Leiomyosarcoma is a rare small bowel malignancy that often presents as chronic nonspecific symptoms including abdominal pain, melena, or chronic ... [6] An aggressive malignant smooth muscle neoplasm, arising from the small intestine. It is characterized by a proliferation of neoplastic spindle cells. [8] The most common type of sarcoma that affects your small intestine is called a gastrointestinal stromal tumor (GIST). Previously, these tumors were called leiomyosarcomas. [11] Leiomyosarcoma affects men and women, but it occurs more often in women. Though the condition can develop in people of any age, it’s more common in people over the age of 50. [12] Leiomyosarcoma of Small Intestine is a very rare tumor that is observed in adults (age range 50-70 years); only about 2-3% of all small bowel malignancies are leiomyosarcomas

Common Signs and Symptoms

Small intestine leiomyosarcoma, a rare type of cancer, can exhibit various symptoms depending on the tumor's size and location. Some common signs and symptoms include:

• Abdominal pain: Pain in the belly (abdomen) is often one of the first symptoms to appear [5][9].
• Weight loss: Unexplained weight loss can occur due to the tumor's growth and its impact on digestion [10][11].
• Nausea and vomiting: Gastrointestinal issues, such as nausea and vomiting, may arise as the tumor compresses or pushes away nearby organs or connective tissues [15].
• Weakness and fatigue: Feeling very weak or tired (fatigue) can be a symptom of small intestine leiomyosarcoma [5][10].
• Dark-colored stools: Blackened stools can occur if the tumor affects the digestive system's ability to properly process food [11].

Other Possible Symptoms

In some cases, people may experience additional symptoms as the tumor grows and becomes more prominent. These can include:

• Painful lumps or swelling: A growing lump or swelling that can be felt through the skin may develop in the affected area [11].
• Abdominal bloating: Bloating of the abdomen can occur due to the tumor's impact on digestion [10].

Early-Stage Symptoms

It is essential to note that small intestine leiomyosarcoma can be asymptomatic in its early stages, making it challenging to diagnose. As the tumor grows and becomes more prominent, symptoms may become more apparent.

References:

[5] - May 17, 2023 — Signs and symptoms of small intestine cancer include unexplained weight loss and abdominal pain. [9] - May 17, 2023 — Signs and symptoms of small intestine cancer include unexplained weight loss and abdominal pain. These and other signs and symptoms may be ... [10] - People may have varying symptoms depending on how big the tumor is and where it’s located. Some people don’t experience symptoms early on, but may notice certain signs as the tumor grows, such as: Pain. Tiredness. Abdominal bloating. Nausea and vomiting. Weight loss. Fever. A lump under your skin. [11] - Signs and symptoms of leiomyosarcoma depend on where the cancer starts. They might include pain, weight loss, and a growing lump or swelling that can be felt through the skin. [15] - As with many bowel malignancies the presenting symptoms are often vague and nonspecific including abdominal pain, fatigue, anemia, and changes ...

Diagnostic Tests for Small Intestine Leiomyosarcoma

Small intestine leiomyosarcoma, a rare and malignant tumor, requires accurate diagnosis to determine the presence, size, and location of the tumor. The following diagnostic tests are commonly used:

• Imaging tests: These include:
  • Computed Tomography (CT) scan [10][15]
  • Magnetic Resonance Imaging (MRI) [3][13]
  • Positron Emission Tomography (PET) scan [11][15] These imaging tests help doctors visualize the small intestine and surrounding areas to detect growths and determine tumor presence, size, and location.
• Endoscopy: This involves inserting an endoscope into the mouth to examine the esophagus, stomach, and first part of the small intestine [5].
• Capsule endoscopy: A small camera is swallowed to take pictures of the inside of the small intestine [14]. If something is found on a capsule endoscopy, other tests may be needed to determine what it is.
• Enteroscopy: This uses special devices to guide an endoscope into the small intestine, allowing doctors to see more of the small intestine than with upper endoscopy [14].
• Biopsy: A small needle is used to collect a sample of tissue for testing. If a mass is detected, a biopsy may be performed or surgery may be done directly [15].

Additional Tests

Other tests that may be used to diagnose leiomyosarcoma and determine tumor presence, size, and location include:

• Blood tests: To check for signs of cancer, such as anemia [2][6]
• Physical exam: A doctor will examine you to gather more clues about what's causing any signs and symptoms you may be experiencing [12]

It is essential to note that the prognosis of leiomyosarcoma of small intestine is generally poor in a majority of cases, with an overall 5-year survival rate of about 28-48% [10].

Differential Diagnosis of Small Intestine Leiomyosarcoma

Small intestine leiomyosarcoma (LMS) is a rare type of cancer that can be challenging to diagnose due to its similarity in presentation with other gastrointestinal tumors. The differential diagnosis for small intestine LMS includes:

• Gastrointestinal Stromal Tumors (GISTs): GISTs are the most common mesenchymal neoplasms of the GI tract, and they often present with similar symptoms as LMS [9][11]. However, GISTs can be differentiated from LMS through immunohistochemical methods, such as the lack of CKIT expression in LMS [4].
• Retroperitoneal Soft Tissue Tumors: Retroperitoneal soft tissue tumors are another differential diagnosis for small intestine LMS. These tumors often present with similar symptoms and can be challenging to differentiate from LMS based on imaging studies alone [13].

Key Diagnostic Features

To accurately diagnose small intestine LMS, it is essential to consider the following key diagnostic features:

• Immunohistochemistry: Immunohistochemical methods are crucial in differentiating LMS from GISTs and other soft tissue tumors. The lack of CKIT expression in LMS is a key feature that distinguishes it from GISTs [4].
• Histopathology: Characteristic histopathological features, such as the presence of smooth muscle cells and their mesenchymal cell precursors, are essential for diagnosing LMS [12].

Clinical Presentation

Small intestine LMS often presents with nonspecific symptoms, such as weight loss, nausea, vomiting, abdominal pain, and anemia. These symptoms can be similar to those presented by GISTs and other soft tissue tumors, making differential diagnosis challenging [14][15].