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March 29, 2026Case Reports in Oncology0 citationsOpen Access

Primary Angiosarcoma of the Ovary: A Case Report and Literature Review

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RERand Abou EidIAIbrahim AljnideAAAhmad Al-Bitar

Key Result

Surgical resection followed by four cycles of adjuvant carboplatin and paclitaxel resulted in no evidence of recurrence at six months in a 45-year-old woman with primary ovarian angiosarcoma.

Key Points

  • To present a rare case of primary angiosarcoma of the ovary and discuss its diagnosis and management.
  • Case report of a 45-year-old woman with abdominal pain and an ovarian mass.
  • Total abdominal hysterectomy with bilateral salpingo-oophorectomy performed.
  • Histopathological examination and immunohistochemistry used for diagnosis.
  • Tumor board review led to adjuvant chemotherapy with carboplatin and paclitaxel.
  • Histopathology revealed a high-grade pleomorphic tumor confirmed as primary ovarian angiosarcoma.
  • Patient remained asymptomatic with no recurrence after six months follow-up.
  • Strong positivity for CD31 and ERG helped confirm diagnosis, guiding treatment.

Study Design

Type

Case Report (n=1)

Structured PICO

P
Population
45-year-old woman with primary pure ovarian angiosarcoma (FIGO Stage II)
I
Intervention
Total abdominal hysterectomy with bilateral salpingo-oophorectomy followed by 4 cycles of adjuvant carboplatin and paclitaxel

This case report demonstrates the short-term efficacy of surgery combined with adjuvant carboplatin and paclitaxel for a rare primary ovarian angiosarcoma, while highlighting the necessity of immunohistochemistry for accurate diagnosis.

Abstract

Introduction: Primary angiosarcoma of the ovary is an exceptionally rare and aggressive malignancy, representing a diagnostic and therapeutic challenge. Due to its rarity, standardized management protocols are lacking, and prognosis remains poor. Case Presentation: A 45-year-old woman with diabetes and hypertension presented with abdominal pain and a palpable pelvic mass. Imaging revealed a complex right adnexal mass. She underwent total abdominal hysterectomy with bilateral salpingo-oophorectomy. Histopathological examination revealed a high-grade pleomorphic tumor. Immunohistochemistry was pivotal for diagnosis, showing strong positivity for CD31 and ERG, and negativity for epithelial markers, confirming a primary pure ovarian angiosarcoma (FIGO Stage II). Postoperative PET-CT showed no distant metastasis. Following a multidisciplinary tumor board review, she received four cycles of adjuvant carboplatin and paclitaxel. At six-month follow-up, she remained asymptomatic with no evidence of recurrence. Conclusion: This case underscores the importance of a comprehensive immunohistochemical panel in diagnosing rare ovarian malignancies, particularly to distinguish angiosarcoma from other poorly differentiated tumors. Multidisciplinary management is essential. While adjuvant chemotherapy may offer benefit, the prognosis for advanced-stage ovarian angiosarcoma remains guarded. This report adds to the limited literature on this entity and highlights the need for further research to establish optimal treatment strategies.

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Cite This Study

Eid et al. (2026) conducted a case report in Primary angiosarcoma of the ovary (n=1). Total abdominal hysterectomy with bilateral salpingo-oophorectomy and adjuvant carboplatin and paclitaxel was evaluated on Symptom status and recurrence. Surgical resection followed by four cycles of adjuvant carboplatin and paclitaxel resulted in no evidence of recurrence at six months in a 45-year-old woman with primary ovarian angiosarcoma.

synapsesocial.com/papers/69c8c1d7de0f0f753b39c0cahttps://doi.org/10.1159/000551775
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Also Consider

Synapse has enriched 5 closely related papers on similar clinical questions. Consider them for comparative context:

  1. 1Primary pure angiosarcoma of the ovary: A case report2025
  2. 2Primary ovarian angiosarcoma: a rare and recognizable ovarian tumor2021 · 15 citations
  3. 3Primary ovarian angiosarcoma: A case report and literature review1998 · 28 citations
  4. 4Ovarian angiosarcoma: a case report and review of the literature2014 · 24 citations
  5. 5Primary Ovarian Angiosarcoma: A Case Report and Review of the Literature2026