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"Patients can present with a number of non-specific symptoms and findings, such as upper abdominal pain and fullness, fatigue, weight loss, splenomegaly and anemia.1,4,5,16–23 Splenic rupture can also be a presenting finding, sometimes resulting in acute hemoperitoneum in up to 30%.19 It has been proposed that splenic rupture does not relate to outcome,5 but splenectomy in both emergent and elective cases is rarely curative.2 "
[Show abstract][Hide abstract] ABSTRACT: Primary splenic angiosarcoma is a very rare neoplasm with a high propensity for metastatic disease and poor prognosis. There is a paucity of literature concerning this specific sarcoma subtype and the role of systemic therapy is not well defined. A retrospective review of the prospectively maintained University of Washington/Seattle Cancer Care Alliance Sarcoma Unit database was performed to identify patients with splenic angiosarcoma treated between 2007 and 2012. In total there were 19 patients with angiosarcoma treated at the Seattle Cancer Care Alliance from 2007 to 2012. The number of patients with splenic angiosarcoma was 2 (11%). The first patient was a woman aged 57 years who was referred with metastatic splenic angiosarcoma to the liver, post-splenectomy. She was treated with 4 cycles of weekly paclitaxel prior to metastatic resection and 4 cycles of the same drug in an adjuvant scenario, achieving a pathological complete response to treatment. She is alive and on third-line systemic therapy. The second patient was a male patient aged 30 years who presented with metastatic high-grade splenic angiosarcoma and was treated with 3 lines of systemic therapy, including doxorubicin, paclitaxel and gemcitabine+docetaxel, but developed a gastrointestinal metastasis with subsequent gastrointestinal bleeding. Splenic angiosarcoma is a very rare neoplasm. Surgery remains the mainstay of management for localized disease. Paclitaxel administered weekly proved to be well-tolerated and resulted in a good radiological response in one of our patients, enabling resection of metastatic disease. Durable clinical benefit can be achieved in metastatic splenic angiosarcoma with multi modality management.
[Show abstract][Hide abstract] ABSTRACT: Primary angiosarcoma of the spleen is rare, usually widespread or has a splenic rupture by the time of presentation, and almost always has poor prognosis; early radiological diagnosis is necessary for improving the survival rate. We report two special cases of this disease. Both patients did not have pathologic metastasis or splenic rupture, but their computed tomography (CT) manifestations were distinct from each other and had uncommon characteristics. We compared the CT features with pathological findings.
[Show abstract][Hide abstract] ABSTRACT: Primary angiosarcoma of the spleen is an extremely rare malignancy. Splenic angiosarcoma is difficult to diagnose, and treatment is often challenging. The prognosis is dismal, with a mean survival of less than 1 year. Currently, no standard treatment methods have been established. Anecdotal reports suggest that early diagnosis and prompt splenectomy followed by cytotoxic chemotherapy can be effective treatment modalities. We report a case of rapidly progressing primary splenic angiosarcoma with multiple liver and bone metastases and a fatal hemorrhagic outcome.