Littoral cell angioma of spleen: A seldom-vascular tumour and a rare cause of long-standing anaemia with thrombocytopenia

Abstract Splenic rupture in pregnancy is a rare event. It is generally due to trauma or an underlying splenic pathology. Timely diagnosis and intervention of splenic rupture is essential given the high rates of associated maternal and fetal morbidity and mortality. This case illustrates a 38 year old lady in the third trimester of pregnancy who presented with maternal collapse and a non-reassuring foetal heart rate tracing following a fall at home one week previously. A massive hemoperitoneum was identified at caesarean section and a splenectomy was performed. Histological examination revealed a littoral cell angioma of the spleen. This vascular tumour arising in the splenic red pulp sinuses is a rare entity and specific immunophenotypic features help distinguish it from other vascular tumours of the spleen. Both trauma and an underlying splenic pathology led to splenic rupture in this unique case.

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LITTORAL CELL ANGIOMA OF SPLEEN - A RARE CASE REPORT

10.36106/ijsr/7332224 ◽

2021 ◽

pp. 1-2

Author(s):

Nikhilkumar M. Sonagara ◽

Nidhi Dhingani ◽

Kamlesh Rabari

Keyword(s):

Thrombus Formation ◽

English Literature ◽

Benign Lesion ◽

Splenic Vein ◽

Malignant Potential ◽

Focal Lesion ◽

Vascular Tumour ◽

Littoral Cell ◽

Littoral Cell Angioma ◽

Operative Recovery

INTRODUCTION Littoral- cell angioma (LCA) is a rare benign vascular tumour of the spleen. There have been less then 250 cases reported in the literature. Recent reports have described it to be a Benign lesion with congenital and immunologic associations. We report a case of LCA of the spleen. PRESENTATION OF CASE A 09 -year-old male patient was admitted to hospital with a one month duration of intermittent upper abdominal pain and nausea. Imaging studies, including Ultrasonography and Colourdoppler Imaging of portal venous systems. Radiology show moderate gross enlargement-16cm & normal in echotexture with no focal lesion. Splenic vein-10mm in caliber with partial thrombus formation. An open splenectomy was performed and his post-operative recovery was uneventful. DISCUSSION Littoral cell angioma of the spleen is a benign vascular tumour that has been infrequently reported in the English literature. While it does have malignant potential, the vast majority are benign. Diagnosis depends on the expression of endothelial markers like CD31 and histiocytic markers like CD68.Malignant potential enhanced by the presence of splenomegaly as well. CONCLUSION This rare condition is made by the presence of rare vascular tumour in the spleens as well.

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

European Journal of Case Reports in Internal Medicine ◽

10.12890/2020_001863 ◽

2020 ◽

Author(s):

Maaike Ramael ◽

Patrick Schoeters ◽

Karl De Pooter ◽

Frederik Van Sonhoven ◽

Hilde Van Steelandt ◽

...

Keyword(s):

Vascular Tumour ◽

Review Of The Literature ◽

Littoral Cell ◽

Littoral Cell Angioma ◽

First Case ◽

Littoral Cells ◽

Splenic Red Pulp ◽

Underlying Pathology ◽

Red Pulp

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.

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Littoral cell angioma: A case and review of the literature

Radiology Case Reports ◽

10.2484/rcr.v6i3.324 ◽

2011 ◽

Vol 6 (3) ◽

Cited By ~ 3

Author(s):

Tarek Hanna ◽

Deborah A. Baumgarten ◽

Theodore M Friedman

Keyword(s):

Review Of The Literature ◽

Littoral Cell ◽

Littoral Cell Angioma

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LITTORAL CELL ANGIOMA

Diagnostic Pathology: Vascular ◽

10.1016/b978-0-323-37674-7.50086-6 ◽

2016 ◽

pp. 11-2-11-3

Keyword(s):

Littoral Cell ◽

Littoral Cell Angioma

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A Case of Littoral Cell Angioma with Multiple Liver Metastasis

The Japanese Journal of Gastroenterological Surgery ◽

10.5833/jjgs.2017.0012 ◽

2018 ◽

Vol 51 (3) ◽

pp. 222-227

Author(s):

Yoshihiro Takahara ◽

Yasuhiro Otsuka ◽

Takeshi Ogasawara ◽

Satoru Nomura ◽

Hidehiko Uno ◽

...

Keyword(s):

Liver Metastasis ◽

Littoral Cell ◽

Littoral Cell Angioma ◽

Multiple Liver Metastasis

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Littoral cell angioma of the spleen: A case report

SAGE Open Medical Case Reports ◽

10.1177/2050313x20959874 ◽

2020 ◽

Vol 8 ◽

pp. 2050313X2095987

Author(s):

Vaclav Opatrny ◽

Vladislav Treska ◽

Tomas Waloschek ◽

Jiri Molacek

Keyword(s):

Case Report ◽

Metastatic Lesion ◽

Vascular Tumors ◽

Rare Tumor ◽

Littoral Cell ◽

Littoral Cell Angioma ◽

Uncommon Disease ◽

Visceral Malignancy ◽

Red Pulp

Spleen tumors are an uncommon disease. Littoral cell angioma belongs to the group of vascular tumors. It is believed that this tumor originates from the tissue of the red pulp sinuses, specifically from the cells that are lining the sinuses. If this rare tumor is diagnosed, it is necessary to search for synchronous or metachronous visceral neoplasia. Littoral cell angioma can also mimic metastatic lesion of the spleen. This case report wants to draw attention on this rare tumor of the spleen which is very often associated with other visceral malignancy.

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