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Systemic therapy in primary angiosarcoma of the spleen
Bruna Pellini Ferreira1, Eve T Rodler, Elizabeth T Loggers
1School of Medicine, Federal University of Rio Grande do Sul, RS, Brazil;
Rare Tumors
|February 2, 2013
Summary
Primary splenic angiosarcoma is rare, with limited systemic therapy data. Weekly paclitaxel showed promise in one patient, enabling metastatic resection and achieving a complete response, suggesting multi-modality management benefits.
Area of Science:
- Oncology
- Sarcoma Research
- Medical Oncology
Background:
- Primary splenic angiosarcoma is an exceptionally rare malignancy.
- It has a high tendency for metastasis and a poor prognosis.
- Literature on this specific sarcoma subtype and systemic therapy is scarce.
Purpose of the Study:
- To review cases of splenic angiosarcoma treated at the Seattle Cancer Care Alliance.
- To evaluate the role and efficacy of systemic therapy in splenic angiosarcoma management.
Main Methods:
- Retrospective review of a prospectively maintained database.
- Identification of patients with splenic angiosarcoma treated between 2007 and 2012.
- Analysis of treatment regimens and patient outcomes.
Main Results:
- Two cases (11%) of splenic angiosarcoma were identified among 19 angiosarcoma patients.
- One patient with metastatic disease achieved a pathological complete response with weekly paclitaxel, enabling metastatic resection.
- The second patient received multiple lines of therapy but experienced gastrointestinal metastasis and bleeding.
Conclusions:
- Surgery is the primary treatment for localized splenic angiosarcoma.
- Weekly paclitaxel demonstrated tolerability and efficacy in enabling metastatic resection in one case.
- Multi-modality management may lead to durable clinical benefit in metastatic splenic angiosarcoma.
