Angiosarcoma of the spleen presenting as spontaneous splenic rupture: A rare case report and review of the literature

Badiani, Raj; Schäller, G.; Jain, Kapil; Swamy, Rajiv; Gupta, Sudhir Kumar

doi:10.1016/j.ijscr.2013.06.007

Cited by 21 publications

(20 citation statements)

References 5 publications

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“…Clinical symptoms were non-specific and varied from asymptomatic diseases, found only on incidental investigations, to splenic rupture or lethal hemorrhage [15,22,23]. From the present study, a series of manifestations could be concluded as first presentations in PSA.…”

Section: Discussionmentioning

confidence: 54%

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

Zhang

et al. 2018

Cell Physiol Biochem

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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.

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Section: Discussionmentioning

confidence: 54%

“…Since the first case reported by Theodor Langhans in 1879，only approximately 300 cases have been reported in the literature worldwide, largely as isolated case reports [11,15]. Large-scale systemic analyses are still lacking.…”

Section: Introductionmentioning

confidence: 99%

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

Zhang

et al. 2018

Cell Physiol Biochem

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“…There is slight male preponderance, but no genetic predisposition has been identified. Although the pathogenesis of this tumour is not clear, exposure to ionizing radiation, arsenic, vinyl chloride and chemotherapy for lymphoma have been implicated as causative factors [1][2][3][4].…”

Section: Discussionmentioning

confidence: 99%

“…Other complaints include fatigue, weight loss, and anorexia [3]. Splenomegaly is the most common physical examination finding and anaemia is the most common laboratory abnormality [1,4]. Definitive preoperative diagnosis of splenic angiosarcoma remains challenging.…”

Section: Discussionmentioning

confidence: 99%