Spontaneous rupture of primary splenic angiosarcoma: A case report

Xu, Baoyan; Xie, Xiaona; Zhou, Xiong; Zhai, Menghe; Yang, Wen‐Bin

doi:10.3892/ol.2015.3714

Cited by 8 publications

(7 citation statements)

References 15 publications

(19 reference statements)

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“…Rupture of the splenic tumor leads to early metastasis to other intraabdominal organs; hence, it is known as the worst prognostic factor [14]. Worth mentioning, in contrast to the slight male predominance reported earlier, most patients who presented with spontaneous splenic rupture due to primary angiosarcoma were females, with F/M ratio of 1.6:1 [15][16][17][18][19][20][21][22][23][24][25][26][27][28][29][30][31][32][33]. This surgical emergency may affect a wide age range of patients, with the youngest patient being 17 years old female [17], while the oldest was a female in her 80s, reported by Sivelli et al [25].…”

Section: Clinical Presentationmentioning

confidence: 99%

Primary Angiosarcoma of the Spleen: An Aggressive Neoplasm

2022

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Primary tumors of the spleen are rare, with an incidence rate of about 0.1%. These tumors could be benign, usually asymptomatic, or malignant which are usually symptomatic with abdominal pain being the most common symptom. Lymphoid neoplasms are the most common primary splenic tumors. Primary angiosarcoma is one of the extremely rare malignant vascular neoplasms of the spleen, which carries a dismal prognosis. It constitutes almost 7.4% of all primary malignant splenic neoplasms and is well known as an aggressive tumor with high local recurrence and distant metastasis rates. Overall survival is up to 12 months following diagnosis, regardless of management strategy. Due to the broad differential diagnosis of splenic tumors, this tumor is often forgotten, and is very challenging to diagnose early. Less than 300 cases of primary splenic angiosarcoma have been reported in the English literature. The main issue of this article is to review the current English literature to figure out the characteristic demographic features, clinical presentation, imaging findings and management of such tumors, in order to increase awareness of the treating physicians to improve diagnosis, management, as well as overall survival.

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Section: Clinical Presentationmentioning

confidence: 99%

Primary Angiosarcoma of the Spleen: An Aggressive Neoplasm

2022

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“…Common symptoms include abdominal pain, distension and splenomegaly ( 8 ). Additionally, patients may experience anemia, leucopenia, elevated lactate dehydrogenase levels and thrombocytopenia; however, these symptoms are occasionally reported ( 9 ). However, these vague presentations can lead to a delayed diagnosis, and such cases may initially resemble blood disorders, characterized by hematological issues and splenomegaly ( 10 ).…”

Section: Discussionmentioning

confidence: 99%

Metastatic splenic angiosarcoma presenting with anemia and bone marrow fibrosis mimicking primary myelofibrosis: A case report and literature review

Wu,

Li,

Luo

et al. 2024

Mol Clin Oncol

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Angiosarcomas, originating from endothelial cells, are infrequent soft tissue sarcomas characterized by a high propensity for metastasis and an unfavorable prognosis. Splenic angiosarcoma, an exceedingly rare and aggressive neoplasm, exhibits variable clinical manifestations. The present case report describes a patient initially exhibiting anemia and bone marrow fibrosis, mimicking primary myelofibrosis, ultimately diagnosed with splenic angiosarcoma. The findings of the present case report underscore the importance of considering splenectomy for histopathological confirmation. Employing a panel of vascular differentiation markers is invaluable for establishing the diagnosis of angiosarcoma.

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“…Life threatening rupture of spleen is the first manifestation of this malignancy in numerous cases. Early appearance of metastases is typical; liver, lung, lymph nodes, and gastrointestinal system are preferred sites of metastases [9]. Median survival is only 5 months [10].…”

Section: Discussionmentioning

confidence: 99%

Primary Angiosarcoma of the Spleen: Rare Diagnosis with Atypical Clinical Course

Kohutek

Badik

Bystricky

2016

Case Reports in Oncological Medicine

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Primary angiosarcoma of the spleen is a rare diagnosis with poor prognosis. Morphologically, it demonstrates conventional blood vessel differentiation. We present a case of 65-year-old female who underwent radical splenectomy for primary angiosarcoma of the spleen. After three-year disease-free interval, she was diagnosed with bone-only metastatic disease. Palliative radiotherapy and bisphosphonates kept her disease reasonably stable for another four years. After development of lung metastases, six cycles of single agent doxorubicin kept her progression-free for six years. Upon further progression in lungs, thirteen years after original diagnosis, lung biopsy confirmed metastatic splenic angiosarcoma in the lungs. She started weekly paclitaxel chemotherapy. Although splenic angiosarcoma generally carries grave prognosis, some patients may enjoy prolonged periods of disease stabilization. Durable benefit can be achieved in some patients with multimodality management. We review the literature focusing on systemic treatment for this rare tumor.

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Spontaneous rupture of primary splenic angiosarcoma: A case report

Cited by 8 publications

References 15 publications

Primary Angiosarcoma of the Spleen: An Aggressive Neoplasm

Primary Angiosarcoma of the Spleen: An Aggressive Neoplasm

Metastatic splenic angiosarcoma presenting with anemia and bone marrow fibrosis mimicking primary myelofibrosis: A case report and literature review

Primary Angiosarcoma of the Spleen: Rare Diagnosis with Atypical Clinical Course

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