Primary angiosarcoma of the spleen, a rare indication for splenectomy: a case report

Matthew Wheelwright; Ellen J Spartz; Keith Skubitz; Hira Yousaf; Paari Murugan; James V Harmon

doi:10.1016/j.ijscr.2021.105929

Primary angiosarcoma of the spleen, a rare indication for splenectomy: a case report

Int J Surg Case Rep. 2021 May:82:105929. doi: 10.1016/j.ijscr.2021.105929. Epub 2021 Apr 29.

Authors

Matthew Wheelwright¹, Ellen J Spartz², Keith Skubitz³, Hira Yousaf⁴, Paari Murugan⁴, James V Harmon⁵

Affiliations

¹ Department of Surgery, University of Minnesota Medical School, Minneapolis, MN 55414, United States of America. Electronic address: wheel381@umn.edu.
² University of Minnesota Medical School, Minneapolis, MN 55414, United States of America.
³ Department of Medicine, Division of Hematology, Oncology, and Transplantation, University of Minnesota Medical School, Minneapolis, MN 55414, United States of America.
⁴ Department of Laboratory Medicine and Pathology, University of Minnesota Medical School, Minneapolis, MN 55414, United States of America.
⁵ Department of Surgery, University of Minnesota Medical School, Minneapolis, MN 55414, United States of America.

Abstract

Introduction and importance: Primary angiosarcoma of the spleen is a rare condition with a nonspecific clinical presentation and is associated with a poor prognosis. We describe two patients with primary splenic angiosarcoma successfully treated with splenectomy and adjuvant chemotherapy.

Case presentations: Case 1: A 50-year-old female presented with fatigue and left-sided rib, shoulder, and abdominal pain. A CT scan demonstrated a large splenic mass, and biopsy was diagnostic of angiosarcoma. An open en bloc resection of the spleen was performed, and pathologic examination confirmed high-grade angiosarcoma; the surgical margins were negative. The patient received pegylated liposomal doxorubicin (PLD) and ifosfamide; she demonstrated no evidence of recurrence with four years of follow-up. Case 2: A 70-year-old male presented with acute back pain. A CT scan demonstrated a splenic mass; biopsy was diagnostic of angiosarcoma. The patient underwent open splenectomy, and pathology revealed high-grade angiosarcoma; the surgical margins were positive. The patient received PLD and ifosfamide but presented three years later with metastatic tumor to the spine. The patient had a favorable tumor response to pembrolizumab. The patient's tumor burden remains stable at 5 years following splenectomy.

Clinical discussion: Angiosarcoma of the spleen is a rare clinical entity and is often challenging to diagnose early. Moratality is high, especially in the case of metastasis or spontaneous rupture.

Conclusion: Due to the rare nature of this tumor, optimal treatment is not known. Here, we show excellent response in two patients to surgery combined with adjuvant therapy.

Keywords: Angiosarcoma; Spleen; Splenectomy.