Elsevier

Gynecologic Oncology

Volume 97, Issue 3, June 2005, Pages 935-937
Gynecologic Oncology

Case Report
Pregnancy following recurrent angiosarcoma of the ovary—A case report and review of literature

https://doi.org/10.1016/j.ygyno.2005.02.027Get rights and content

Abstract

Background.

Ovarian angiosarcomas are rare tumors which may to be distinguished from other unusual primary ovarian tumors such as clear cell carcinoma, yolk sac tumor and leiomyosarcoma on the basis of histological appearance and immunohistochemistry. Angiosarcomas of the ovary occur in all age groups but are more frequent in women of child bearing age (less than 40 years). Surgery and radiotherapy have been the traditional treatment modalities.

Case.

The case we present is the only reported long-term survivor of recurrent ovarian angiosarcoma. Her initial treatment was surgical, both at presentation and relapse but since she wished conservation of fertility, radical surgery and radiotherapy were avoided and she underwent further adjuvant chemotherapy with doxorubicin and ifosfamide. She remains in remission 6 years after treatment of recurrence of the primary tumor and has had a successful pregnancy following treatment.

Conclusion.

Adjuvant chemotherapy of ovarian angiosarcoma with a combination of doxorubicin and ifosfamide appears effective and should be considered in women at risk of relapse who wish to conserve fertility.

Introduction

Soft tissue sarcomas account for less than 1% of all malignancies. Angiosarcomas comprise only 2% of all soft tissue sarcomas. Approximately 20 cases of angiosarcoma of the ovary are documented in MEDLINE. There is no known case of complete remission lasting more than 48 months following recurrence of ovarian angiosarcoma, and, to our knowledge, the case we are reporting is the only instance of a successful pregnancy following this rare and fatal cancer.

Section snippets

Case report

A 28 year old nulliparous woman on the oral contraceptive pill presented with acute right sided lower abdominal pain. An ovarian cyst with solid components was visualized on a diagnostic scan and a diagnosis of torsion of a dermoid cyst was made. An emergency laparotomy was performed, and a 20 × 25 cm right ovarian mass was found and right salpingo-oopherectomy carried out. The left ovary and tube and the uterus were entirely normal.

The histology of the ovary showed a variable appearance with

Discussion

Soft tissue sarcomas account for less than 1% of all cancers [1] and angiosarcomas comprise just 2% of all soft tissue sarcomas. They may occur in any region of the body but 60% arise in the skin and superficial soft tissues, predominantly in the head and neck region [1]. Angiosarcomas of the female reproductive system are very rare. They have most frequently been reported in the uterus and less commonly in the vagina, vulva, cervix and ovary. Overall, prognosis is poor and the reported 5-year

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