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.