Clinical Characteristics and Prognostic Factors of Primary Splenic Angiosarcoma: A Retrospective Clinical Analysis from China

Abstract

Background/Aims: Primary splenic angiosarcoma is an aggressive malignancy originating from endothelial cells with a particularly poor outcome despite radical therapy. Owing to its extremely low incidence, available data for splenic angiosarcoma are limited. The present study aimed to address this limitation by presenting a thorough retrospective analysis of Chinese primary splenic angiosarcoma patients over a 53-year period (1963-2016). Methods: To determine the characteristics of Chinese primary splenic angiosarcoma and identify factors that impact the outcomes of this histology, we retrospectively retrieved reports of 110 Chinese primary splenic angiosarcoma cases published between 1963-2012. Results: In total, 61 males and 49 females diagnosed with primary splenic angiosarcoma were included in the present study. The median age at diagnosis was 50 years (range 2.5–76 years). Of these patients, 25.5% had received prior radiotherapy. The rate of splenic rupture was 59.11%. The 1-year overall survival rate was 19.1% with a median overall survival time of 8.1 months. Age, gender, and radiation history showed no correlation with survival rate. However, by univariate analysis, we found that significant adverse predictors of survival were splenic rupture before surgery and large tumor size (> 5 cm), while adjuvant chemotherapy was a favorable predictor. Furthermore, multivariate analysis revealed that splenic rupture and adjuvant chemotherapy were independent adverse and favorable predictors, respectively. Conclusion: Our large series describes and confirms the characteristics and poor prognosis of Chinese primary splenic angiosarcoma, thus indicating a critical role for early diagnosis and surgical intervention (prior to rupture) in management, and highlights the promising potential of adjuvant chemotherapy for improving the outcome in these cases.

Primary splenic angiosarcoma: a rare entity often associated with rupture and hemoperitoneum

“…The diagnosis is challenging and is often made only after splenectomy. More than 300 PSA cases have been described 4. Azevedo et al5 reported the case of a 57-year-old woman previously treated for breast ductal carcinoma, and as far as we know, to date, our case was the first case report of PSA with spontaneous rupture in a patient in Brazil.…”

“…PSA has been reported in association with environmental or occupational exposure to carcinogens as ionizing radiation (e.g. thorium dioxide), arsenic, vinyl chloride, and chemotherapy for lymphoma, these causal relationships have not been unequivocally substantiated, and most cases occur without identifiable risk factors 4 , 14 , 15. PSA is generally a neoplasm of middle-aged and older people with the mean age at presentation of 50–60 years, but cases in young people and children also have been reported 7 , 16 - 19.…”

“…In a review 4 of 110 Chinese PSA cases, nearly 70% had metastases by the time of diagnosis, and 60% presented as splenic rupture. Moreover, some series reported a rate of 100% of metastasis to liver, bone, and bone marrow, as well as a poor prognosis with a survival rate of 20% in 6 months 3 , 4 , 7 , 12 , 14 - 17. Splenic biopsy is generally contraindicated because of the high risk of inducing rupture and may not render the diagnosis.…”

Prognostic Nomograms for Predicting Overall Survival and Cancer-Specific Survival in Patients with Angiosarcoma, a SEER population-based study

“…Biing Luen Lee reported male sex was the possible predictor of poor prognosis in AS 10 . However, some research suggested gender appeared no obvious correlation with OS probably due to the limited sample size 11,12 . As displayed in the nomogram, age was also observed as an independent factor.…”

Angiosarcoma esplénico primario con rotura capsular y diseminado: reporte de un caso

“…Del 25% al 69% de los casos se presentan con rotura esplénica. El tratamiento de primera opción es la esplenectomía, pero su pronóstico es casi siempre fatal a los pocos meses del diagnóstico [1][2][3] .…”