Primary splenic angiosarcoma: a case series of a rare oncological entity and diagnostic challenge

Authors

  • Iris Dirven Department of Gastro-enterology, Universitair Ziekenhuis Brussel (UZ Brussel), Brussels, Belgium
  • Philippe Leclercq Department of Gastro-enterology, CHC MontLégia, Liège, Belgium
  • Lionel D'Hondt Department of Oncology, CHU UCL Namur site Godinne, Yvoir, Belgium
  • Valentine Delmotte Department of Gastro-enterology, CHC MontLégia, Liège, Belgium
  • Pierre Lefesvre Department of Pathology, Universitair Ziekenhuis Brussel (UZ Brussel), Brussels, Belgium
  • Hendrik Reynaert Department of Gastro-enterology, Universitair Ziekenhuis Brussel (UZ Brussel), Brussels, Belgium
  • Frederik Vandenbroucke Department of Radiology, Universitair Ziekenhuis Brussel (UZ Brussel), Brussels, Belgium
  • Magali Surmont Department of Gastro-enterology, Universitair Ziekenhuis Brussel (UZ Brussel), Brussels, Belgium

DOI:

https://doi.org/10.2340/1651-226X.2023.35412

Keywords:

Angiosarcoma, Liver Failure, Primary Splenic Angiosarcoma, Splenic imaging, Splenic Neoplasms

Abstract

Background and purpose: Primary angiosarcoma of the spleen (PAS), an exceptionally rare and aggressive neoplasm with high metastatic risk (70%–85%), is frequently diagnosed in an advanced or metastatic stage. It presents diagnostic challenges due to its nonspecific symptomatology and resemblance to benign vascular lesions in various imaging modalities.

Patients and methods: This case series aims to clarify the diagnostic difficulties by comparing imaging characteristics (CT-scan, MRI, and [18F]FDG-PET/CT) as well as pathological findings of three PAS cases diagnosed in different stages of the diseases (localized, metastatic, and metastatic with organ failure). Furthermore, a brief review on diagnostic and therapeutic features is included.

Results and interpretation: We suggest [18F]FDG-PET/CT as a differentiating tool between benign and malignant splenic lesions and propose a flowchart of a diagnostic algorithm for PAS. For treatment, we advocate for early splenectomy and when systemic therapy is warranted, paclitaxel emerges as a viable first-line option. While it is crucial to acknowledge that further trial data is required to evaluate the efficacy of emerging treatment regimens, designing and conducting trials for PAS is challenging given its scarcity and aggressive behavior. Therefore case reporting remains important.

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Published

2024-04-15

How to Cite

Dirven, I., Leclercq, P., D’Hondt, L., Delmotte, V. ., Lefesvre, P., Reynaert, H., … Surmont, M. (2024). Primary splenic angiosarcoma: a case series of a rare oncological entity and diagnostic challenge. Acta Oncologica, 63(1), 192–197. https://doi.org/10.2340/1651-226X.2023.35412