Primary ovarian angiosarcoma - review of the literature and report of a case with coexisting chylothorax

Background: Primary ovarian angiosarcoma is a very rare gynaecologic malignancy with poor prognosis and uncertain, up-to-date, treatment options. Its exact diagnosis is challenging for surgeons and difficult for pathologists. There are only a few cases reported in the international literature.

Case: We report a case of primary pure ovarian angiosarcoma with coexisting chylothorax which is, to the best of our knowledge, the first reported case. An extensive review of the literature analyzing all clinical and pathological parameters related to this condition is presented.

Result: In spite of all therapeutic efforts, surgical and medical, prognosis of ovarian angiosarcoma remains very poor in most cases.

Conclusion: Primary ovarian angiosarcoma is a rare and aggressive malignancy. The report of such cases is interesting in order to exchange knowledge and experience, and possibly to further improve our diagnostic and therapeutic capabilities.

Ovary; Primary angiosarcoma; Chylothorax, MAID chemotherapy

[1] Evtushenko N.T.: "Angiosarcoma of the ovary in a 7-year old girl". Akush Ginekol. (Mosk), I 958, 34, 105.

[2] Ongasuwan C.H., Taylor J., Tang Chik-Kwun, Prempree T "Angiosarcomas of the uterus and ovary: clinicopathologic report". Cancer, 1982, 49, 1469.

[3] Patel T., Ohri S.K., Sundaresan M., Jackson J., Desa L., Davey A.T. et al.: "Metastatic angiosarcoma of the ovary". Eu,: J. Surg Pathol., 1991, 17, 295.

[4] Bouchi J., El Asmar B., Couetil J.P, Acker M., Taleb N., Gedeon E. et al.: "Angiosarcome de l'ovaire avec metastases pulmonaires”. Ann. Chi1: Chir. thorac. cardio-vasc., 1993, 47, 740.

[5] Cunningham M.J, Brooks J., Noumo忏J.:'Treatment of primary ovarian angio- sarcoma with ifosfamide and doxorubicin" Gynecol. Oncol., 1994, 53, 265.

[6] Nara M., Sasaki T., Shimura S., Yamamoto M., Oshiro T., Kaiwa Y. et al.: "Diffuse alveolar haemon-hage caused by lung metastasis of ovarian angiosarcoma". Int. Med., 1996, 35, 653.

[7] Nielsen G., Young R., Prat J., Scully R.: "Primary angiosarcoma of the ovary. A report of seven cases and review of the literature" Int. J. Gyn. Pathol., 1997, 16, 378.

[8] Lifschitz-Mercer B., Leider-Trejo L., Messer G.. Peyser M.R., Czernobilsky B.: "Primary angiosarcoma of the ovary: a clinicopathologic, irnmunohistochemical and electronrnicroscopic study". Pathol. Res. Pract., 1998, 194, 183.

[9] Nucci M., Krausz T., Lifschitz-Mercer B., Chan J., Fletcher C "Angiosaroma of the ovary: immunohistochernical analysis of four cases with a broad morphologic spectrum". Am. J. Surg Pathol., 1998, 22, 620.

[10] Furihata M., Takeuc加T., Iwata J., Sonobe H., Ohtsuki Y., Wakatsuki A. et al.: "Primary ovarian angiosarcorna: a case report and literature review". Pathol. Int., 1998, 48, 967.

[11] Platt J., Rogers S., Flynn E., Taylor R.: "Primary angiosarcoma of the ovary: a case report and review of the literature". Gynecol Oncol., 1999, 73, 443.

[12] Jylling A., Jorgensen L., Holund B.: "Mucinous cystadenocarc1-norna in combination with hemangiosarcoma in the ovary" Pathol. Oneal. Res., 1999, 5, 318.

[13] Twu N.F., Juang C.M., Yeng M.S., Lu C.J., Lai C.Z., Chao K.C 'Treatment of primary pure angiosarcorna of the ovary with mu!tiple lung metastases: a case report". Eur. J. Gynaecol. Oneal., 1999, 20, 383.

[14] Pillay K., Essa A., Chetty R.: "Borderline serous cystadenocarc1一norna with coexistent angiosarcoma: an unusual form of ovarian carcinosarcoma". Int. J. Surg. Pathol., 2001, 9, 317.

[15] Davidson B., Abel er Y.: "Primary ovarian angiosarcoma presenting as malignant cells in ascites: case report and review of the literature". Diagn. Cytopathol., 2005, 32, 307

[16] Jha S., Chan K.K., Poole C.J., Rollason T.P.: "Pregnancy following recurrent angiosarcoma of the ovary-A case report and review of the literature". Gynecol. Oneal., 2005, 97, 935.

[17] Quesenberry C., Li C., Chen A., Zweizig S., Ball H.G.: "Primary angiosarcoma of the ovary: a case report of Stage l disease" Gynecol. Oneal., 2005, 99, 218.