Low-grade, metastasizing splenic littoral cell angiosarcoma presenting with hepatic cirrhosis and splenic artery aneurysm

Brandon Larsen, Maria C. Bishop, Glenn C. Hunter, Stephen W. Renner

Research output: Contribution to journalArticle

5 Scopus citations


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.

Original languageEnglish (US)
Pages (from-to)618-626
Number of pages9
JournalInternational Journal of Surgical Pathology
Issue number6
StatePublished - Dec 1 2013
Externally publishedYes



  • cirrhosis
  • littoral cell angioma
  • littoral cell angiosarcoma
  • splenic artery aneurysm
  • splenic vascular neoplasm

ASJC Scopus subject areas

  • Anatomy
  • Surgery
  • Pathology and Forensic Medicine

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