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

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.

Primary Angiosarcoma of the Spleen: An Aggressive Neoplasm

“…Splenic angiosarcoma is highly refractory to chemo/radiotherapy treatment, and up to date, there is no scientific evidence to suggest these treatment modalities for such tumors [9,12]. Some recent reported cases are exceptional to the previously mentioned statement: Wheelwright et al [34] reported two cases of splenic angiosarcoma treated by surgical means and adjuvant chemotherapy. One patient, treated by adjuvant doxorubicin and ifosfamide-based chemotherapy, showed no evidence of recurrence at 4 years of clinical and radiological follow-up.…”

“…Physical examination of patients with primary angiosarcoma of the spleen can be without abnormal findings [ 34 ], while on the other hand abdominal distention, due to hemoperitoneum in the case of rupture, could be found. As most patients usually exhibit a replacement of the normal splenic parenchyma by tumor cells, diffuse enlargement of the entire spleen with splenomegaly (77-94%) is the most common physical finding [ 3 , 5 ].…”

Images in Immunotherapy and Precision Oncology: Angiosarcoma of the Spleen and Liver

“…[ 7 , 8 ] Immunotherapies targeting PD-L1 or the programmed cell death protein 1 PD-1 receptor have also demonstrated efficacy in treating primary splenic angiosarcomas, as recent case reports have shown significant clinical benefit with pembrolizumab monotherapy as well as with the anti-PD-1 toripalimab combined with the multikinase inhibitor anlotinib. [ 9 , 10 ] In addition to these novel therapies, other tyrosine kinase inhibitors that also inhibit the vascular endothelial growth factor (VEGF)/VEGF receptor (VEGFR) signaling pathway, such as sunitinib or sorafenib, have demonstrated clinical benefit in treating recurrent metastatic angiosarcomas. [ 11 , 12 ]…”

A Case of Primary Splenic Angiosarcoma and Multiple Liver Metastases Diagnosed after an Intraabdominal Hemorrhage

“…DepartmentsofGeneralSurgery 1) andPathology 2) ,TokyoKitaMedicalCenter A75-year-oldmanwithcomplaintsofdimmedvision,abdominalpain,anddyspneawastransferred toourhospital.Computedtomographyrevealedmultiplehepaticandsplenictumorsandhemorrhagicascites.Intraabdominalhemorrhagecausedbytheruptureofeitherofthetumorswassuspected.Because hisbloodpressurewaswellmaintainedthroughfluidreplacementandtransfusion,weperformedsplenectomy and lateral segmentectomy of the liver the next day. After pathological examination, the patient wasdiagnosedwithprimarysplenicangiosarcomaandmultiplelivermetastases.Intraabdominalhemor-rhagesonthe32ndand34thpostoperativedaysweremanagedusingtranscatheterarterialembolization.…”