Table 2: Hematologic and coagulation parameters associated with clinical features of the 7 angiosarcoma patients with DIC.

No.Age (yrs), sex (M/F), anatomic sitesSystemic therapy in metastatic diseaseISTH scoreDIC in 1st 30 daysDIC resolved with disease response/
DIC recurred at progressionBone marrow involvementTumor burden@Clinical thrombosis/
Survival in metastatic disease Remarks

137 M liver with metastasis to spleenPegylated liposomal doxorubicin7YesYesYesNoNonbulkyNo9 monthsMetastatic at first presentation. No evidence of clinically significant portal hypertension

242 F breast (de novo) with metastasis to chest wall, ribs, lungPegylated liposomal doxorubicin plus carboplatin, sirolimus, pazopanib, abraxane, bevacizumab plus vinorelbine5NoNAYesNot doneNonbulkyYes11 monthsLocalized to right breast at first presentation, had mastectomy, then 6 cycles adjuvant gemcitabine plus docetaxel followed by radiation.
Developed metastatic relapse within 11 months

339 M mediastinal with metastasis to bone, lung, brainDoxorubicin plus paclitaxel, ifosfamide plus cisplatin, gemcitabine plus vinorelbine5YesYesYesNot doneBulkyYes5 monthsMetastatic at first presentation

454 F cardiac with metastasis to lung, pleura, bones, bone marrowDoxorubicin plus ifosfamide7YesYesYesYesBulkyYes6 monthsMetastatic at first presentation

570 F breast (radiation related) with metastasis to lymph nodes, lungDoxorubicin plus ifosfamide6YesYesYesNoNonbulkyNo11 monthsMetastatic at first presentation; developed angiosarcoma 9 years following lumpectomy and radiation for early stage breast adenocarcinoma

666 F skull and meninges with metastasis to spinal cord, lungs, boneNil6YesNANANot doneNonbulkyNo1 monthMetastatic at first presentation; poor performance status precluded any therapy

778 M foot (bony) with metastasis to lungsNil 4*YesNANANot doneNonbulkyNo2 monthsMetastatic at first presentation; poor performance status precluded any therapy

 *Data available only for prothrombin time and platelets, giving a maximum possible ISTH score of 4.
@Bulky tumor is defined as the largest single or contiguous tumor mass 5 cm or more in diameter.
Development of DIC within 30 days of presentation with metastatic disease.