Splenic angiosarcomas are exceedingly rare tumors with a very poor prognosis.1
Only a few cases have been reported in the literature, since its original description by Langhans in 1879.2
Angiosarcomas have a highly variable histology and can be mistaken for vascular benign tumors or other non vascular malignant neoplasms. On microscopy most of them have a well defined nodular homogenous appearance and all of them show a vasoformative component. Most of the tumors express at least one immunohistochemical marker of histiocytic differentiation (CD68 and/or lysozyme) and two or more markers of vascular differentiation (CD 34, CD31, FVIII R Ag, VEGFR3).3
On radiological imaging, these tumors show an enlarged spleen (>12 cm), usually with either a solitary complex mass or multiple diffusely infiltrative heterogeneous masses.4
Metastases are a frequent finding at the time of diagnosis with up to 70% of metastatic deposits involving the liver. Common sites of metastatic disease include lung followed by bone and liver.5
Low platelet counts are seen in 33% of splenic angiosarcoma cases, which should be considered in the differential diagnosis of unexplained thrombocytopenia.6
In a retrospective study of 82 angiosarcoma patients, those with a primary resectable tumor had a 5 year disease specific survival (DSS) rate of 60%. Sixty seven percent of these patients received adjuvant radiation and 27% received chemotherapy. Tumors with intermediate or high grade pathology (76%) and those arising from previously irradiated or lymphedematous areas had worse disease specific survival.7
In another study looking at patients with advanced angiosarcomas, median disease-specific survival (DSS) for patients with locally recurrent disease and metastatic disease was 50 months (95% confidence interval (CI): 25.7–73.5 months) and 10 months (95% CI: 7.9–12 months) respectively.8
Most of these studies included patients with angiosarcomas arising from various sites including soft tissues, breast, skin, viscera and bone. Due to a paucity of data, it is however unclear if splenic angiosarcomas have a different natural course of progression than sarcomas arising from other organs.
Several chemotherapy treatment options have been tried in patients with locally recurrent/metastatic angiosarcoma, including Paclitaxel ± Gemcitabine, Doxorubicin ± Ifosfamide, Docetaxel ± Gemcitabine,9
Interferon alfa, Dacarbazine, Bevacizumab, Etoposide, Cisplatin and various combinations of these agents.10
Paclitaxel is an alkaloid ester extracted from the bark of the pacific yew tree (Taxus brevifolia
) and the European Yew tree (Taxus baccata
It stabilizes the microtubules by inhibiting the process of tubular depolymerization.12
This process occurs during the metaphase/anaphase transition of the mitotic cell cycle, resulting in an arrest of cell division.13
It also demonstrates antiangiogenic activity by inhibiting endothelial cell proliferation, motility and invasiveness, both in vivo
and in vitro
In a phase II study of paclitaxel therapy in advanced soft tissue sarcoma,15
a complete response was seen in a patient with angiosarcoma of the scalp. A retrospective study of patients with angiosarcoma of the scalp has shown that Paclitaxel is effective in the treatment of angiosarcoma of the scalp16
with a major response (complete response + partial response) seen in 8 out of 9 (89%) patients. The median duration of response was 5 months. A further phase II clinical trial of paclitaxel in advanced angiosarcoma suggested that weekly paclitaxel is well tolerated and is associated with a progression free survival rates of 74% and 45% at 2 months and 4 months respectively.17
At a median follow up of 8 months the study reported a progression free survival of 4 months and a median overall survival of 8 months.
Here we present a patient with locally advanced splenic angiosarcoma treated with single agent with neo-adjuvant Paclitaxel. Based on an extensive search of the available literature, we believe this is the first report of Paclitaxel facilitating surgical resection in locally advanced splenic angiosarcoma.