Angiosarcoma is an infrequent vascular malignancy with grave prognosis due to distant metastasis at initial presentation. The predominant metastatic sites of angiosarcoma are lung; and it is particularly important and should be approached with multimodality treatment because pulmonary metastasis of angiosarcoma frequently induces severe complications. Here, we reported a case of angiosarcoma in an old Indian male presented with cough and hemoptysis. 18-FDG PET-CT scan revealed multiple organ involvement by tumor, and excisional biopsy from lung and pleural nodule revealed malignant tumor composed of spindle shaped epithelioid cell. Strong positivity for D2-40, CD 31 & CD 34 favoured the diagnosis of angiosarcoma. Patient was planned for intravenous chemotherapy with paclitaxel along with zoledronic acid. After 3-cycles of chemotherapy, re-evaluation with PET-CT revealed good response. In view of stable disease, it was planned to continue the same regimen for three more cycles. However, after receiving two more cycles of chemotherapy, patient’s condition deteriorated rapidly and died within a month. The present case showed, paclitaxel has a good impact on metastatic angiosarcoma to diminish lung nodules. However, more effective treatment regimen should be explored to effectively treat metastatic angiosarcoma and increase survival.
R. J. Young, N. J. Brown, M. W. Reed, D. Hughes, P. J. Woll. Angiosarcoma. Lancet Oncol. 2010;11(10):983-91.
N. Penel, S. Marréaud, Y. M. Robin, P. Hohenberger. Angiosarcoma: state of the art and perspectives. Crit Rev Oncol Hematol. 2011;80(2):257-63.
N. Naka, M. Ohsawa, Y. Tomita, H. Kanno, A. Uchida, K. Aozasa. Angiosarcoma in Japan. A review of 99 cases. Cancer. 1995;75:989–996.
F. W. Stewart, N. Treves. Lymphangiosarcoma in postmastectomy lymphedema; a report of six cases in elephantiasis chirurgica. Cancer 1948;1(1):64–81.
I. Shimabukuro, K. Yatera, S. Noguchi, Y. Kawanami, T. Iwanami, C. Nishida et al. Primary pulmonary angiosarcoma presenting with hemoptysis and ground-glass opacity: A case report and literature review. Tohoku J Exp Med. 2015;237(4):273-8.
P. G. Casali et al. Bone sarcomas: ESMO-PaedCan-EURACAN Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann. Oncol. 29, iv79–iv95 (2018).
G. Treglia, G. Cardillo, P. Graziano. A rare case of primary pulmonary epithelioid angiosarcoma detected by (18)F-FDG PET/CT. Clin Nucl Med. 2014;39(5):450-2.
R. Wilson, S. Glaros, R. K. Brown, C. Michael, D. Reisman. Complete radiographic response of primary pulmonary angiosarcomas following gemcitabine and taxotere. Lung Cancer. 2008;61(1):131-6.
W.D. Travis, E. Brambilla, H. K. Muller-Hermelink, C. C. Harris, editors. World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of the Lung, Pleura, Thymus and Heart. IARC Press: Lyon; 2004
K. Kojima, I. Okamoto, S. Ushijima, T. Yoshinaga, M. Kitaoka, M. Suga et al. Successful treatment of primary pulmonary angiosarcoma. Chest. 2003;124(6):2397-2400.
E. Palmerini, A. Leithner, R. Windhager, G. Gosheger, K. Boye, M. Laitinen et al. Angiosarcoma of bone: a retrospective study of the European Musculoskeletal Oncology Society (EMSOS). Sci Rep 10, 10853 (2020). https://doi.org/10.1038/s41598-020-66579-5