J Korean Surg Soc.  2009 Oct;77(4):291-295. 10.4174/jkss.2009.77.4.291.

Spontaneous Rupture of Primary Angiosarcoma of the Spleen

• Kim HH
• Hur YH
• Park CY
• Kim JC
• Kim SK

• KMID: 1464890
• DOI: http://doi.org/10.4174/jkss.2009.77.4.291

Abstract

Primary angiosarcoma of the spleen is an extremely rare malignancy, the pathogenesis of which is not completely understood, with high metastatic potential and an exceedingly poor prognosis, regardless of treatment regimen. The major complication is splenic rupture, which often leads to fatal hemoperitoneum. Overall, since 1879 when Langerhans described the first case of angiosarcoma of the spleen, there have been approximately 200 cases reported in the literature. Moreover, to the best of our knowledge, spontaneous rupture of primary splenic angiosarcoma and spontaneous rupture of remnant or recurred angiosarcoma is extremely rare, and no cases were reported in English literature. We report a case of spontaneous splenic rupture due to angiosarcoma in a 68-year-old man, and also review the existing literature.

Keyword

Primary angiosarcoma; Hemoperitoneum; Spontaneous splenic rupture

MeSH Terms

Aged
Hemangiosarcoma
Hemoperitoneum
Humans
Prognosis
Rupture, Spontaneous
Spleen
Splenic Rupture

Figure

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  Fig. 1 The abdominal CT scan. Abdominal CT scan revealed a large amount of hemoperitoneum due to splenic rupture, and hematoma, which replaces splenic parenchyma.

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  Fig. 2 Gross and microscopic finding. (A, B) Hemorrhagic and nodular lesions are seen in ruptured spleen. (C) Microscopic findings show presence of sinusoidal vascular spaces lined by large pleomorphic tumor cells with irregular hyperchromatic nuclei and abundant eosinophilic cytoplasm. (H&E stain, ×200). (D) Immunoreactivity of splenic angiosarcoma for CD31 is seen (×200).

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  Fig. 3 The follow-up abdominal CT scans (36th postoperative day). There is seen an angiomatous mass lesion (angiosarcoma, maximum 3×4 cm sized, white arrow) in splenectomy bed (A), with multiple hypervascular metastases in the liver (A, B).

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  Fig. 4 The follow-up abdominal CT scans (78th postoperative day). About two months later, the follow-up abdominal CT scan revealed a large amount of hematoma, possibly due to rupture of recurred mass lesion (angiosarcoma, maximum 10×9 cm sized, white arrow) in splenectomy bed (A), and more aggravation of multiple hypervascular metastases in the liver (A, B).

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