Spleen Rupture Due to Primary Angiosarcoma: A Case Report

Koutelidakis, Ioannis; Tsiaousis, Panagiotis; Papaziogas, B.; Patsas, Aristeides G.; Atmatzidis, Stefanos; Atmatzidis, Konstantinos

doi:10.1007/s12029-008-9034-y

Cited by 8 publications

(3 citation statements)

References 15 publications

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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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“…Patients may present with constitutional symptoms, abdominal pain from splenomegaly, anaemia and thrombocytopaenia. 71,72 Splenic angiosarcoma has a 6-month survival rate of 20%. 73 In cases of splenic rupture, the mean survival is only 4-5 months.…”

Section: Cardiac and Pulmonary Artery Angiosarcomamentioning

confidence: 99%

Angiosarcoma: clinical and imaging features from head to toe

Gaballah

Jensen

Palmquist

et al. 2017

The British Journal of Radiology

174

168

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Angiosarcoma is a rare, aggressive subtype of soft-tissue sarcoma with a propensity for local recurrence and metastasis associated with a generally poor prognosis, unless diagnosed early. Given the vascular endothelial cell origin of angiosarcoma, tumours may develop in essentially any organ; however, there is a predilection for the skin where half of all tumours arise, increasing in prevalence with age. The most common risk factors are chronic lymphoedema and history of radiation. We review the most important radiological findings along the spectrum of angiosarcoma from head to toe throughout the body, including uncommon and rare locations. Key imaging features of angiosarcoma across multiple organ systems will be described, as well as the impact on management and prognosis.

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“…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). In a subset of cases, splenic rupture occurs as an initial presentation, usually signifying a poor prognosis (9,(11)(12)(13). Throughout the course of the disease, metastases are common, often occurring early and extensively (14).…”

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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Spleen Rupture Due to Primary 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

Angiosarcoma: clinical and imaging features from head to toe

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

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