A case of recurrent angiosarcoma with isolated bone marrow metastasis presented as acute disseminated intravascular coagulation

Angiosarcoma is a rare type of soft-tissue sarcoma arising in the subcutaneous tissue of skin, soft tissue, and viscera.1, 2 Clinical manifestations vary considerably depending on the primary site and can include anemia, hepatic dysfunction, and disseminated intravascular coagulation (DIC). Final diagnosis is confirmed through histopathology and immunohistochemical staining results. Hematoxylin and eosin staining of the angiosarcoma demonstrate pleomorphic spindle endothelial cells with large hyperchromatic nuclei, and immunohistochemistry (IHC) shows most expression on CD31, CD34, FLI-1, and ERG. Angiosarcoma is aggressive, and unfavorable treatment response with local relapse or wide spreading is common, which explains its poor prognosis.3 We present a case of recurrent angiosarcoma with isolated bone marrow (BM) metastasis presented as acute DIC. A 78-year-old man with the history of scalp angiosarcoma, histologic grade (FNCLCC) 3, pT1a (Figure 1A), having undergone wide excision in May 2017 and radiation therapy with a total dose of 6000 cGy/30 fx, presented to the emergency department with 3-day history of multiple ecchymoses over all extremities and small amounts of blood-tinged sputum. He denied recent fever episodes and use of Chinese herbal medicine. Physical examination revealed multiple ecchymoses including on the right lateral thigh (10 × 10 cm2), right medial thigh (18 × 15.5 cm2), and left medial knee (4 × 6.5 cm2). Laboratory testing revealed thrombocytopenia (platelets: 19000/μL) with coagulopathy (PT [INR] 1.47, PT [P] 14.9 seconds, PT [C] 10.6 seconds, PTT [P] 39.3 seconds, PTT [C] 24.9 seconds), low fibrinogen (59.5 mg/dL), high D-dimer (>83.6 mg/dL), and high FDP (310 μg/dL). DIC was noted. No evidence of residual or recurrent tumor was detected through brain computed tomography. Positron emission tomography-computed tomography scanning revealed diffusely heterogeneous mildly reactive axial BM metabolism. He received BM biopsy after management with blood products; the pathologic report revealed large pleomorphic cells with ill-defined vascular channel formation and extravasated red blood cells. IHC revealed CD31 (+), factor VIII (−), CD117 (−), and LCA (−) in tumor cells, consistent with metastatic angiosarcoma (Figure 1B). He had persistent thrombocytopenia and coagulopathy and subsequently died from progression of the disease before cytotoxic chemotherapy. Several underlying conditions are responsible for initiating and propagating DIC, and the principle of DIC management is treatment of the underlying condition.4 The mechanism through which angiosarcomas trigger DIC is poorly understood. Malignancy may break the basement membrane of the endothelium, which is followed by blood cell trapping and lysis that induced clotting factors and tissue factor release.1 Yang et al. reported 42 patients with angiosarcoma, and 7 patients (17%) with unexplained DIC; all demonstrated metastatic disease. DIC resolves dependent on disease response and can be stabilized through systemic therapy, but coagulopathy worsens coincident with disease progression.5 Our case was challenging to diagnose because of the nonspecific appearance on imaging and no evidence of local recurrence. The patient presented with acute DIC and experienced rapid clinical deterioration and eventual death. Further studies of similar cases are warranted to identify an optimal management strategy. The authors would like to thank all members of the study team, the patient, and their family. All authors declare no conflict of interest.