Introduction
Splenic tumors are uncommon and can be categorized as primary or secondary. Secondary tumors are less common and arise as a spread from a distant source, such as colon, ovaries, lung, breast, or melanoma. Primary splenic tumors have an incidence rate of approximately 0.1 percent overall, making them exceedingly rare. This article discusses primary splenic angiosarcoma in detail, as well as its causes, symptoms, and management.
What Is Primary Splenic Angiosarcoma?
Rare and aggressive malignant neoplasms originating from the splenic vascular endothelium are called primary splenic angiosarcomas. It is one of the rarest forms of neoplasm, with an estimated yearly frequency of 0.14 to 0.25 cases per million persons. It was first identified by Langhans in 1879. The only known treatment that has the potential to produce long-term, disease-free survival is splenectomy. Because primary splenic angiosarcomas have shown to be extremely resistant to radiation therapy and chemotherapy, they are linked to a very bad prognosis. Regardless of the course of therapy, the majority of patients pass away within a year after diagnosis. To date, the available research contains only a few evaluations regarding primary splenic angiosarcoma.
What Are the Causes of Primary Splenic Angiosarcoma?
The cause of splenic angiosarcomas is unidentified. A small number of people have been reported to develop splenic angiosarcomas years after undergoing radiation treatment for other cancers. But as far as researchers know, no conclusive link between radiation exposure and any of the following has been found. According to certain sources, hemangiomas (benign vascular tumors), lymphangiomas (benign malformations of the lymphatic system), and hemangioendothelioma (rare cancer of the blood vessels) are examples of benign splenic tumors that may malignly convert into primary splenic angiosarcoma. Vinyl chloride, arsenic, and thorium dioxide have all been linked to the development of hepatic angiosarcomas; however, no clear causal relationship between these compounds and splenic angiosarcomas has been shown.
What Are the Clinical Features of Primary Splenic Angiosarcoma?
There are no defined clinical diagnostic guidelines for primary splenic angiosarcoma. Upper abdominal discomfort is the most common presenting symptom in more than 80 percent of cases. While fever, exhaustion, and weight loss are common constitutional symptoms associated with malignancy, they are not always the first signs to appear (less than 10 percent of cases). Physical examination frequently reveals splenomegaly and a palpable lump in the left upper quadrant. There have been reports of 13 percent to 32 percent of cases of splenic rupture. Anemia is the most prevalent laboratory anomaly in 75 percent to 81 percent of cases, with thrombocytopenia occurring in 10 percent to 40 percent of patients; leukocytosis is also common.
How Is Primary Splenic Angiosarcoma Diagnosed?
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Primary angiosarcoma of the spleen is a difficult tumor to diagnose because it is extremely rare and presents with nonspecific symptoms. A combination of physical, radiographic, and histological features is required for the diagnosis of these cancers.
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In patients with primary splenic angiosarcoma, physical examination may reveal no abnormalities; nonetheless, in the event of a rupture, abdominal distention may result from hemoperitoneum. The most common physical observation is diffuse swelling of the entire spleen and splenomegaly (77 to 94 percent), as most patients often have tumor cells replacing the normal splenic parenchyma.
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A complicated mass or masses in an enlarged spleen was the most prevalent clinical finding in another investigation, showing up in nearly 60 % of patients. As previously stated, a serious surgical emergency may be the initial symptom of spontaneous rupture of the original tumor and hemodynamic instability.
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Despite recent considerable technological advancements in imaging modalities, radiographic identification of primary splenic angiosarcoma remains extremely difficult. The primary cause of the challenge is the radiological overlap between this tumor and other splenic cancers that are nonvascular (lymphomas) and vascular (hemangiomas and epithelioid vascular tumors).
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Currently, several radiographic diagnostics are available for splenic angiosarcoma diagnosis and staging assessment. These consist of an MRI (magnetic resonance imaging), CT (computed tomography) scan, and abdominal ultrasound. Splenomegaly is the most frequent radiological finding, observed in all of the previously described methods.
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The most frequent finding on ultrasound examination is a heterogeneous complicated mass that includes areas of necrosis and bleeding. Typically, the necrotic and hemorrhagic regions within the splenic mass are shown as cystic patches. The results of a CT scan are usually determined by whether or not the initial tumor bursts.
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The results of the CT scan are comparable to the ultrasound results previously indicated when there is an undisturbed tumor. Tests using immunohistochemistry and histopathology are typically used to get the exact diagnosis of primary angiosarcoma of the spleen.
How Is Primary Splenic Angiosarcoma Managed?
Treatment of primary angiosarcoma of the spleen is not well established due to the small number of documented cases and the lack of investigations. Based on the physician's experience, each case is handled independently. Therefore, treating this patient group with a multidisciplinary team of doctors made up of radiologists, experienced oncologists, and qualified surgeons is highly recommended.
The standard course of treatment consists of chemotherapy, radiation therapy, and splenectomy. Surgical resection, or splenectomy, is the basis for treatment even though it is rarely beneficial particularly when there is splenic local illness or spontaneous rupture. Splenic angiosarcoma is very resistant to chemotherapy and radiation therapy. Primary splenic angiosarcoma has an extremely bad prognosis. The majority of patients (69 to 100 percent) experience invasive spreading. The most commonly afflicted organs are the liver, lungs, lymphatic system, and skeletal system.
Conclusion
Primary splenic angiosarcoma is an exceptionally rare aggressive tumor with a poor prognosis. The medical team treating this unusual tumor faces significant diagnostic difficulty due to its confusing clinical presentations. The fundamental challenge is early detection and differential diagnosis between aggressive angiosarcoma tumors and other splenic primary tumors. Because of the scarcity and relative lack of scientific data related to this rare cancer, it is necessary to thoroughly analyze all factors in many aspects, such as demographic traits, clinical appearance, imaging tests, and histological findings to confirm the diagnosis.
