Littoral Cell Splenic Angioma

What is Littoral Cell Splenic Angioma?

Littoral cell angioma (LCA) is a rare type of tumor found in the spleen. It was first recognized and named by Falk and his team in 1991. Most of these tumors are benign, or non-cancerous, and can occur as a single or multiple recurring growths the spleen. The location of these tumors is specific to the cells of the reticuloendothelial system (RES), found in the red sinus areas of the spleen, which is part of the body’s immune system.

A characteristic feature of LCA is that the tumor cells express both endothelial and histiocytic antigens. To put it simply, these cells show signs of two different types of cell changes. This is in contrast to normal spleen cells that only express endothelial markers, which are signs of one type of cell change. The identification of LCA involves examining its structure and performing biochemical tests called immunohistochemical studies.

In some cases, a procedure called fine needle aspiration cytology can be helpful. This is where a thin needle is used to extract cells from the tumor, which can help doctors identify it before it’s surgically removed.

What Causes Littoral Cell Splenic Angioma?

Littoral cell angioma, a type of blood vessel tumor in the spleen, has no clearly defined causes. However, many cases have shown its occurrence alongside other tumors and inflammation, suggesting an issue with the immune system. These tumors are different from other spleen blood vessel tumors, like angiosarcoma, because they arise specifically from cells that line the blood vessels of the red pulpy substance in the spleen, hence being named ‘littoral cells’.

These littoral cell angioma tumors have certain characteristics when examined under a microscope, showing markers found on both blood vessel cells (known as endothelial markers) and immune cell types known as macrophages. This presence of CD 68 and lysozyme (macrophage markers) and factor VIII and CD 31 (endothelial markers) in the cells of the tumor suggests that the tumor originates from the cells lining the blood sinuses, or vessels, of the spleen.

Risk Factors and Frequency for Littoral Cell Splenic Angioma

Littoral cell angioma (LCA) is a seldom-seen tumor found in the blood vessels of the spleen. People usually discover it by chance or when they feel pain in their belly. This condition can affect people of any age, but it is mainly reported in adults of middle age, and rarely in children. It is important to note that it affects both genders equally.

Signs and Symptoms of Littoral Cell Splenic Angioma

The LCA, or left colonic artery, may be discovered unexpectedly in a patient who doesn’t show any symptoms. It can also cause abdominal pain. A check-up may reveal an enlarged spleen in some instances. Less frequently seen symptoms can involve low red blood cell counts (anemia), reduced platelets (thrombocytopenia), liver inflammation (hepatitis), liver scarring (cirrhosis), and increased pressure in the vein carrying blood to the liver (portal hypertension). There are also some documented cases that associate LCA with extramedullary hematopoiesis, which is the production of blood cells outside the bone marrow.

• Abdominal pain
• Enlarged spleen
• Anemia (low red blood cell count)
• Thrombocytopenia (reduced platelets)
• Hepatitis (liver inflammation)
• Cirrhosis (liver scarring)
• Portal hypertension (increased blood pressure in the vein to the liver)
• Extramedullary hematopoiesis (blood cell production outside the bone marrow)

Testing for Littoral Cell Splenic Angioma

To diagnose Littoral Cell Angioma (LCA), a type of splenic tumor, doctors often need to examine the tissue under a microscope, a process known as histopathological examination. One potential method is Fine Needle Aspiration Cytology (FNAC), which involves using a thin needle to collect cells from the spleen. However, this procedure is not typically recommended because it lacks specificity and there’s a risk of causing bleeding and spreading tumor cells if the tumor turns out to be malignant (cancerous).

Imaging studies, like magnetic resonance imaging (MRI) and computed tomography (CT) scans, aren’t usually effective in diagnosing LCA. This is because it’s challenging to distinguish LCA from other types of spleen tumors, including angiosarcomas, lymphomas, and metastatic tumors, using these methods. Even though the MRI might show low-density areas due to the presence of a form of iron in the tumor cells, it is still hard to identify the disease accurately. Similarly, ultrasound evaluations aren’t helpful either. They often show inconsistent findings and don’t clearly show the presence of any lesions (abnormal areas).

Treatment Options for Littoral Cell Splenic Angioma

The current recommended treatment for Littoral Cell Angioma (LCA), a rare condition that can potentially lead to cancer, is the removal of the spleen (splenectomy). Often, LCA is associated with an enlarged spleen (splenomegaly), which can make the surgery challenging. That’s why it’s important that the surgery is done by someone with a lot of experience.

In a procedure called laparoscopic splenectomy (LS), the spleen is removed through a few small cuts in the abdomen. While this method is less invasive, it’s crucial to make sure the spleen doesn’t rupture or break open, as this could allow any potentially cancerous cells in the spleen to spread.

A study by Cai et al. showed that it is possible and safe to perform LS on patients with LCA. However, there may be times when a different approach is necessary, such as when the spleen is significantly larger than normal or if there are extensive adhesions – scar tissues binding structures together. In these cases, doctors may have to use a technique known as hand-assisted laparoscopic splenectomy (HALS), or even perform traditional open surgery.

The primary goal in all cases, especially when dealing with potential malignant tumor, is to ensure the spleen is removed safely, without causing further harm to the patient.

What else can Littoral Cell Splenic Angioma be?

When examining a patient, doctors might need to differentiate between certain types of blood vessel-related tumors in the spleen. These could include:

• Splenic hemangioma
• Lymphangioma
• Hamartoma
• Angiosarcoma

These possible conditions can be distinguished from each other based on their different features as seen under the microscope and the unique markers they express, as detailed previously. However, imaging tests like MRI, CT scans, and ultrasounds have not shown to be helpful in differentiating between these conditions.

What to expect with Littoral Cell Splenic Angioma

LCA, or Littoral cell angioma, is generally harmless and people usually recover well after a spleen-removal operation (splenectomy). But, there have been instances where LCA has turned into a harmful condition.

Studies, like one by Sarandria et al. of 180 LCA cases, showed that having a particularly enlarged spleen (medically known as massive splenomegaly. This term refers to a spleen that weighs 1500 gm or more and/or is bigger than 20 cm preoperatively) is linked with LCA transforming into a harmful condition.

Patients with an extremely enlarged spleen who are preparing for a spleen-removal operation should have regular check-ups after the operation. This is to make sure there are no recurrences and to look out for the spread of any harmful cells.

Possible Complications When Diagnosed with Littoral Cell Splenic Angioma

The primary problems that can come up with littoral cell angioma involve exaggerated spleen functions, or hypersplenism, causing a condition of low red blood cells or anemia, low platelets or thrombocytopenia, belly pain, and weight loss. Also, complications can occur soon after a spleen removal surgery, this being the usual treatment for LCA, such as bleeding, wound infection, hernia, collapsed lungs or atelectasis, lung and abdomen infection, inflammation of the pancreas, and blood clot in the lung. Patients without a spleen are at a higher risk for life of getting severe infections and infections with encapsulated organisms, even if they have gotten all the necessary shots. For instance, even with the pneumonia vaccine, the risk isn’t completely gone.

Possible Complications:

• Anemia
• Thrombocytopenia (low platelets)
• Abdominal pain
• Weight loss
• Bleeding post-surgery
• Site infection
• Hernia
• Atelectasis (Collapsed lungs)
• Lung and abdomen infection
• Pancreatitis (Inflammation of the pancreas)
• Pulmonary embolism (Blood clots in the lung)
• Increased lifelong risk of severe infections
• Infections with encapsulated organisms despite being vaccinated, like Pneumonia

Preventing Littoral Cell Splenic Angioma

LCA, or littoral cell angioma, is often discovered by chance in patients who are not showing any symptoms. However, it’s important that these patients continually check-in with their doctors to monitor any suspected issues with their spleen. After a spleen removal surgery, it’s also crucial to follow the doctor’s advice on getting immunizations. This is because patients without a spleen are vulnerable to severe infections from certain bacteria, and vaccinations can prevent these life-threatening conditions.