Custom Search

\

        

   

 

Top 10 Facts about Littoral Cell Angioma of the Spleen

 

Dr Sampurna Roy MD   

 

 

Littoral cell angioma is a rare vascular tumor of the splenic red pulp, and is typically an incidental finding on abdominal imaging. The splenic lesion can only truly be differentiated from other splenic masses by histologic examination.

 

Top ten interesting facts about the tumour:

 

(1) Littoral-cell angioma a rare primary tumour of the spleen that was first described by Falk et al in 1991.

 

(2) It is a new subtype of benign vascular tumour that involves the cells that line the splenic sinus channels. The tumour expresses both vascular and histiocytic antigens.

 

(3) This splenic tumour can occur at any age.  It usually occurs in middle-aged men and women and has equal sex distribution.  (1-77 years; median age, 50 years).

                    

 

       

      

Littoral cell angioma of the spleen composed of small anastomosing and irregular channels reminiscent of splenic sinuses and covered of neoplastic plump "littoral" cells with low mitotic activity.

 

Source:  Johansson J, Björnsson B, Ignatova S, Sandström P, Ekstedt M. Littoral Cell Angioma in a Patient with Crohn’s Disease. Case Reports in Gastrointestinal Medicine. 2015;2015:474969.

 

(4) Clinically, patients with Littoral cell angioma present with splenomegaly, thrombocytopenia, anemia, or with constitutional symptoms, such as fever of unknown origin.

 

(5 ) Littoral cell angioma may appear as single or multiple nodular lesions in the spleen. Splenic lesions may range from 0.2 to 6.0 cm in maximal dimension.

 

(6) This unique tumour has characteristic morphologic and immunohistochemical characteristics that differentiate it from other vascular splenic tumours.

Morphological diagnosis is based on the presence of anastomosing narrow sinus-like vascular channels lined with tall endothelial cells, focal papillary fronds, and normal splenic sinuses at the periphery of the lesion. Combination of morphological and immunohistochemical analyses show a hybrid endothelial-histiocitic phenotype which helps in establishing the diagnosis.

 

  

 

 

  

The neoplastic cells express histiocytic antibody CD 68 as well as VIII whereas usual endothelial splenic cells only express VIII.

 

 

   

   

The neoplastic cells express FVIII.

 

(7) Immunohistochemistry: The neoplastic cells express histiocytic antibody CD68 as well as VIII whereas usual endothelial splenic cells only express VIII.

 

(8) A malignant subtype, littoral cell angiosarcoma has also been described and possesses cytologically atypical cells and malignant morphologic features, such as invasion of surrounding organs.

 

(9) The treatment for Littoral cell angioma is splenectomy, which is both diagnostic and therapeutic.

 

(10) There is a strong association between this neoplasm and a group of immunologic or oncologic entities, including Crohn disease, tumours of visceral organs, including colorectal, renal, hepatocellular, lung and pancreatic adenocarcinomas, malignant lymphoma], myelodysplastic syndrome, Wiskott Aldrich syndrom, ankylosing spondylitis, aplastic anaemia and Gaucher’s disease.

 

Further reading:

 

Littoral Cell Angioma in a Patient with Crohn’s Disease. Case Reports in Gastrointestinal Medicine. 2015;2015:474969.

 

Littoral Cell Angioma of the Spleen Treated by Laparoscopic Splenectomy

 

Littoral-cell angioma of the spleen: A case report

 

Littoral cell angioma, a rare cause of long standing anaemia: a case report

 

Incidental littoral cell angioma of the spleen

 

Littoral cell angioma of the spleen: report of three cases and literature review

 

Littoral cell angioma: A case report

 

 

 

 

 

 

Histopathology-India.net

 

Pathopedia-India.com

 

Surgical Pathology.com

 

Pathology-India.com

 

Dermpath-India

 

Infectious Disease Online

 

Pathology Quiz Online 

 

Paediatric Pathology Online

 

Pancreatic Pathology Online

 

Paraganglioma-Online

 

Endocrine Pathology Online

 

Eye Pathology Online

 

Ear Pathology Online

 

Cardiac Path Online

 

Pulmonary Pathology Online

 

Lung Tumour Online

 

Mesothelioma-Online

 

Nutritional Pathology Online

 

Environmental Pathology Online

 

Soft Tissue Tumour Online

 

GI Path Online-India

 

Gallbladder Pathology Online

 

E-book - History of Medicine  

 

Microscope - Seeing the Unseen

 

 

 

Disclaimer

Privacy Policy  

Advertising Policy

Copyright © 2016  histopathology-india.net