Spleen Cancer: Symptoms, Types, Causes and Treatment

Spleen cancer is a rare but serious condition that often poses diagnostic and therapeutic challenges. Unlike cancers of more common organs, tumors of the spleen are infrequent, but when they do occur, they can be aggressive and life-threatening. Understanding the symptoms, types, causes, and available treatments is crucial for early detection, effective management, and improved patient outcomes. In this article, we take a comprehensive look at spleen cancer, synthesizing current research to provide a clear, patient-focused guide.

Symptoms of Spleen Cancer

Recognizing the symptoms of spleen cancer can be challenging, as they often overlap with other conditions affecting the spleen or abdominal organs. However, certain signs are more commonly seen in patients with splenic malignancies. Early identification of these symptoms is vital for prompt diagnosis and treatment, which may improve prognosis.

Symptom	Description	Frequency/Prevalence	Source(s)
Splenomegaly	Enlarged spleen	Up to 92% of cases	1
Abdominal Pain	Pain/discomfort in abdomen	83% of cases	1
Weight Loss	Unintentional weight loss	40% of cases	1
Cytopenia	Low blood cell counts	91% of cases	1
Fever	Unexplained fever	10% of cases	1
Fatigue	Persistent tiredness	5% of cases	1
Splenic Rupture	Acute abdominal emergency	13% of cases	1

Table 1: Key Symptoms of Spleen Cancer

Common Symptoms and Their Meanings

Splenomegaly (Enlarged Spleen):
The most frequent sign of splenic cancer is an enlarged spleen, often detected during a physical examination or imaging study. Patients may not notice this themselves, but doctors may feel the spleen below the left rib cage during an exam. Splenomegaly may cause a sense of fullness in the abdomen and can be associated with other symptoms 1.

Abdominal Pain:
Most patients experience pain or discomfort in the upper left side of the abdomen. This can be due to the spleen pressing against nearby organs or stretching its capsule 1.

Systemic and Blood-Related Symptoms

Weight Loss and Fatigue:
Unintentional weight loss and persistent fatigue are classic symptoms of many cancers, including splenic malignancies. These may result from the body's response to the tumor or from blood cell deficiencies 1.

Cytopenia:
A significant number of patients develop cytopenias—deficiency of one or more types of blood cells (red cells, white cells, or platelets)—due to the spleen’s involvement in blood filtration and the tumor's effects on bone marrow function 1.

Fever:
Some patients report unexplained fevers. This symptom is less common but can indicate an immune system response to the cancer 1.

Acute and Emergency Symptoms

Splenic Rupture:
Though less frequent, a ruptured spleen is a medical emergency that can occur in advanced cases. Signs include sudden, severe abdominal pain and signs of internal bleeding. This requires immediate intervention 1.

Types of Spleen Cancer

Spleen cancer is not a single disease but encompasses various tumor types, broadly classified into lymphoid and non-lymphoid origins. Each type has unique features, behavior, and implications for treatment.

Cancer Type	Features/Origin	Relative Frequency	Source(s)
Angiosarcoma	Malignant vascular tumor	Most common primary non-lymphoid	1 3
Lymphoma	Cancer of lymphoid tissue	Most common primary overall	3 5
Hemangioma	Benign vascular tumor	Rare	2 3
Littoral Cell Angioma	Benign vascular tumor, unique to spleen	Very rare	2 3
Cystadenocarcinoma	Malignant epithelial tumor	Extremely rare	4 5
Metastatic Cancer	Secondary involvement from other organs	Less common	3 5

Table 2: Major Types of Spleen Cancer

Vascular Malignancies

Angiosarcoma:
This is the most aggressive and common primary non-lymphoid malignancy of the spleen. Angiosarcomas arise from blood vessel cells and are known for their rapidly progressive nature, high metastatic potential, and poor prognosis. Microscopically, they display diverse patterns, sometimes mimicking benign vascular lesions, which can complicate diagnosis 1 2 3.

Lymphoid Tumors

Lymphomas:
Lymphomas (both Hodgkin’s and non-Hodgkin’s types) are the most common splenic malignancies overall. They originate from the spleen’s lymphoid tissue and can present as primary splenic lymphoma or as part of systemic disease 3 5.

Benign Tumors

Hemangioma:
Although rare, hemangiomas represent the most common benign splenic tumor. They are composed of abnormal blood vessels and are typically found incidentally. Most do not require treatment unless symptomatic 2 3.

Littoral Cell Angioma:
Uniquely found in the spleen, these tumors arise from littoral cells lining the splenic red pulp sinusoids. They are typically benign and may be associated with immune system abnormalities 2 3.

Epithelial Malignancies

Serous Cystadenocarcinoma:
This is an exceptionally rare malignant epithelial tumor of the spleen, with only isolated case reports in medical literature. It may originate from displaced tissue or, rarely, arise primarily 4 5.

Secondary (Metastatic) Tumors

Metastatic Involvement:
The spleen is an uncommon site for metastasis, but when it occurs, the primary sources are usually melanoma, breast, or lung cancers 3 5.

Causes of Spleen Cancer

Understanding what causes spleen cancer is challenging due to its rarity and the diversity of tumor types. Nonetheless, certain risk factors and underlying mechanisms are recognized.

Cause/Risk Factor	Description	Associated Cancer Type	Source(s)
Unknown	Most cases have no identifiable cause	Angiosarcoma, others	1 3
Chronic Immune Stimulation	Ongoing immune disorders or inflammation	Littoral Cell Angioma	2 3
Prior Malignancy/Chemotherapy	History of other cancers and treatment	Angiosarcoma	1
Displaced Tissue/Implantation	Ectopic tissue from prior surgery	Cystadenocarcinoma	4 5
Metastasis from Other Cancers	Spread from primary tumors elsewhere	Metastatic tumors	3 5
Genetic/Unknown Factors	Possible but not well defined	All types	3

Table 3: Causes and Risk Factors of Spleen Cancer

Primary Causes and Risk Factors

Idiopathic (Unknown) Origins:
For most primary splenic malignancies, especially angiosarcomas and lymphomas, no clear causative factor is identified. Research continues to explore genetic, environmental, and immunological contributors 1 3.

Immune and Inflammatory Links

Chronic Immune Stimulation:
Tumors like littoral cell angioma are sometimes associated with chronic immune system activation or autoimmune conditions. This may create an environment in the spleen conducive to tumor development 2 3.

Association with Prior Disease or Therapy

Secondary to Other Malignancies or Treatments:
A history of previous cancers, particularly those treated with chemotherapy, may increase the risk of developing secondary splenic cancers such as angiosarcoma 1. However, such cases are rare.

Rare Pathways

Displaced or Implanted Tissue:
In extremely rare cases, splenic cancers such as serous cystadenocarcinoma may arise from non-native tissue accidentally implanted during prior abdominal surgery. This tissue can later undergo malignant transformation 4 5.

Metastatic Spread

Secondary Involvement from Other Cancers:
More commonly, the spleen may become involved by metastatic spread from cancers originating in other organs such as the skin (melanoma), breast, or lung 3 5.

Treatment of Spleen Cancer

Treatment strategies for spleen cancer depend on the tumor type, stage at diagnosis, and overall health of the patient. Management can involve surgery, systemic therapies, or emerging experimental approaches.

Treatment	Description/Approach	Indication/Use	Source(s)
Splenectomy	Surgical removal of the spleen	Most primary tumors	1 5
Chemotherapy	Systemic drug treatment	Lymphomas, some sarcomas	1 5
Radiation	Localized therapy	Adjunct for lymphoma	3 5
Immunotherapy	Stimulates body’s immune system	Experimental/lymphoma	6
Targeted Therapies	Drugs targeting specific pathways	Under investigation	6
Supportive Care	Management of symptoms/complications	All patients	1 5

Table 4: Main Treatment Approaches for Spleen Cancer

Surgical Management

Splenectomy:
Surgical removal of the spleen remains the cornerstone of treatment for most primary splenic tumors, whether benign or malignant. For angiosarcoma and other aggressive tumors, early splenectomy may be critical, especially before the development of metastatic disease. It is also indicated in cases of splenic rupture 1 5.

Systemic Therapies

Chemotherapy:
Particularly for lymphoid malignancies (lymphomas), chemotherapy is the mainstay of systemic treatment, often combined with or followed by splenectomy in some cases. Chemotherapy may also be used for angiosarcoma, though outcomes are generally poor due to the aggressive nature of the disease 1 5.

Radiation Therapy:
While not routinely used for most splenic tumors, radiation can play a role in controlling localized disease or as adjunct therapy for lymphomas 3 5.

Emerging and Experimental Treatments

Immunotherapy and Targeted Approaches:
Innovative therapies, such as spleen-targeted mRNA vaccines and immunostimulatory agents, are being explored in preclinical models. These approaches aim to boost the immune response against tumor cells and hold promise for future treatment of splenic and other cancers 6.

Supportive and Palliative Care

Symptom Management:
Regardless of the primary therapy, managing symptoms—such as pain, cytopenias, or complications of splenectomy (like infection risk)—is crucial to maintaining quality of life 1 5.

Conclusion

Spleen cancer, though rare, is a complex and serious disease that demands a nuanced approach to diagnosis and management. Understanding its symptoms, types, causes, and treatment options can empower patients, caregivers, and healthcare providers alike.

Key Takeaways:

• Spleen cancer most commonly presents with splenomegaly, abdominal pain, and blood cell abnormalities 1.
• The most frequent types are angiosarcoma and lymphoma, but a range of rare benign and malignant tumors can occur 1 2 3.
• Causes are often unknown, with some cases linked to immune disorders, prior malignancy, or metastatic spread from other cancers 1 3 4 5.
• Treatment typically centers on splenectomy, with chemotherapy, radiation, and emerging immunotherapies as important adjuncts or alternatives 1 5 6.
• Early recognition and multidisciplinary care are vital, given the aggressive nature of some splenic cancers and the potential for life-threatening complications 1.

Stay informed and consult with healthcare specialists for any unexplained spleen or abdominal symptoms—early detection can make a real difference.