Multi-Focal Splenic Tumour in a Belgian Patient and a Brief Review of the Literature on Littoral Cell Angioma

Maaike Ramael; Patrick Schoeters; Karl De Pooter; Frederik Van Sonhoven; Hilde Van Steelandt; Jacqueline Swaegers; Wim Develter; Marc Ramael

doi:10.12890/2020_001863

Multi-Focal Splenic Tumour in a Belgian Patient and a Brief Review of the Literature on Littoral Cell Angioma

Eur J Case Rep Intern Med. 2020 Aug 29;7(11):001863. doi: 10.12890/2020_001863. eCollection 2020.

Authors

Maaike Ramael¹, Patrick Schoeters², Karl De Pooter³, Frederik Van Sonhoven⁴, Hilde Van Steelandt⁴, Jacqueline Swaegers⁵, Wim Develter⁶, Marc Ramael^{6

7}

Affiliations

¹ Faculty of Medicine and Health Sciences, University of Antwerp, Antwerp, Belgium.
² Department of Gastro-enterology, General Hospital AZ Herentals, Herentals, Belgium.
³ Department of Abdominal Surgery, General Hospital AZ Herentals, Herentals, Belgium.
⁴ Department of Medical and Molecular Imaging, General Hospital AZ Herentals, Herentals, Belgium.
⁵ Department of Radiology, Hôpital du Valais, Sion, Switzerland.
⁶ Department of Pathology and Molecular Pathology, Heilig Hartziekenhuis Lier, Lier, Belgium.
⁷ Laboratory of Histology and Cell Biology, University of Antwerp, Antwerp, Belgium.

Abstract

We describe the case of a 66-year-old woman with littoral cell angioma (LCA) confirmed by histopathology and immunohistochemistry, to our knowledge the first case in Belgium. LCA is an extremely rare primary vascular tumour of the splenic red pulp, probably originating from littoral cells. If a splenic mass and nodules are incidentally identified on imaging and the patient has no associated signs or symptoms, LCA should be suspected. Histopathology and adjacent techniques are mandatory for definitive diagnosis. Splenectomy followed by adequate follow-up is necessary to exclude underlying pathology.

Learning points: Littoral cell tumour, although a very rare neoplasm, must be included in the differential diagnosis of splenic lesions observed by imaging.As imaging cannot differentiate between benign and malignant lesions, a definitive diagnosis is made only by histopathology and immunohistochemistry.Individuals diagnosed with littoral cell angioma must be carefully evaluated to exclude associated primary, secondary and synchronous malignancies as well as accompanying inflammatory/autoimmune disease.

Keywords: CT scan; Littoral cell tumour; immunohistochemistry; spleen.