Low-Grade, Metastasizing Splenic Littoral Cell Angiosarcoma Presenting With Hepatic Cirrhosis and Splenic Artery Aneurysm

The behavior of littoral cell neoplasms ranges from benign (littoral cell angioma, LCA) to highly malignant (angiosarcoma). Two unusual cases of low-grade metastatic littoral cell angiosarcoma (LCAS) have been reported with late recurrence and bulky metastases. We present the third case of this rare...

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Bibliographic Details
Published in:International journal of surgical pathology 2013-12, Vol.21 (6), p.618-626
Main Authors: Larsen, Brandon T., Bishop, Maria C., Hunter, Glenn C., Renner, Stephen W.
Format: Article
Language:English
Subjects:
Adult
Aneurysm - etiology
Aneurysm - pathology
Biomarkers, Tumor - analysis
Hemangioma - complications
Hemangioma - metabolism
Hemangioma - pathology
Humans
Immunohistochemistry
Liver Cirrhosis - etiology
Male
Neoplasm Grading
Neoplasm Metastasis
Splenic Artery - pathology
Splenic Neoplasms - complications
Splenic Neoplasms - metabolism
Splenic Neoplasms - pathology
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Summary:The behavior of littoral cell neoplasms ranges from benign (littoral cell angioma, LCA) to highly malignant (angiosarcoma). Two unusual cases of low-grade metastatic littoral cell angiosarcoma (LCAS) have been reported with late recurrence and bulky metastases. We present the third case of this rare neoplasm in a 38-year-old man with cirrhosis and a large splenic artery aneurysm, without extrasplenic masses. The spleen showed nodules resembling LCA, immunoreactive for CD31, factor VIII, CD68, and CD163 but not CD8 or CD34. Also present were solid areas of immunophenotypically identical bland spindle cells, although lighter CD31 immunostaining distinguished them from LCA-like angiomatous channels. Similar cells diffusely infiltrated the cirrhotic liver. After splenectomy, pancytopenia resolved, and he is asymptomatic 19 months later. Low-grade LCAS is a previously unreported cause of cirrhosis and may metastasize without forming masses. In cases of LCA, CD31 immunohistochemistry may facilitate detection of LCAS and indicate metastatic potential.
ISSN:1066-8969
1940-2465
DOI:10.1177/1066896913476737