Littoral cell angioma

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Littoral cell angioma, abbreviated LCA, is an uncommon benign vascular lesion of the spleen.

Littoral cell angioma
Diagnosis in short
Template:Px
Littoral cell angioma. H&E stain.
LM anastoming, small vascular channels, cystic spaces - may have papillary projections
LM DDx hemangioendothelioma, angiosarcoma, hemangiopericytoma, splenic hamartoma
IHC lining cells: CD68 +ve, CD31 +ve, CD34 -ve, CD8 -ve
Site spleen
Clinical history weight loss
Signs pyrexia (fever), splenomegaly
Prevalence rare
Radiology solitary hypodense lesion
Prognosis benign

Angioma, in general, is dealt with in vascular malformations.

General

Features:[1]

  • Rare.
  • Benign.

Clinical:[1][2]

  • Pyrexia (fever), splenomegaly, weight loss, solitary hypodense lesion on imaging.

Note:

  • Littoral cells = line vascular channels of the red pulp.[1]
    • Littoral = related to shore.[3][4]

Microscopic

Features:[1]

  • Anastoming, small vascular channels.
  • Cystic spaces.
    • May have papillary projections.[2]

DDx:[1]

Images

  • Littoral cell angioma of the spleen - very low mag.jpg

    LCA - very low mag. (WC)

  • Littoral cell angioma of the spleen - low mag.jpg

    LCA - low mag. (WC)

  • Littoral cell angioma of the spleen - intermed mag.jpg

    LCA - intermed. mag. (WC)

  • Littoral cell angioma of the spleen - high mag.jpg

    LCA - high mag. (WC)

  • Littoral cell angioma of the spleen - very high mag.jpg

    LCA - very high mag. (WC)

www:

Littoral cell angioma of spleen Littoral cell angioma of spleen Littoral cell angioma of spleen Littoral cell angioma of spleen


Littorral cell angioma of spleen. A. Multiple irregular cysts and proliferated erythrocyte filled vessels. B. Plump lesional cells form the lining and are seen to lie within vascular lumens (arrows). C. Small papillae (arrows) appear to form. D. High power view shows them to be vascular lining cells, sometimes even columnar; they are enlarged endothelial cells positive for CD31, CD163, and CD68. Note the presence also of macrophages, some with hemosiderin pigment.

IHC

Normal littoral cells express CD8+, macrophage/histiocytic markers and vascular markers. In comparison, littoral cell angioma is:[1]

  • CD68 +ve (and other histiocytic markers)
  • CD31 +ve (and other vascular markers, e.g. Factor VIII, but not CD34)
  • CD34 -ve (normally in red pulp).[1]
  • CD8 -ve (normally in red pulp).[1]
  • CD21 +ve (at least focally)

See also

References

  1. ↑ 1.0 1.1 1.2 1.3 1.4 1.5 1.6 1.7 Tan, YM.; Chuah, KL.; Wong, WK. (Jul 2004). "Littoral cell angioma of the spleen.". Ann Acad Med Singapore 33 (4): 524-6. PMID 15329769. http://www.annals.edu.sg/pdf200408/V33N4p524.pdf.
  2. ↑ 2.0 2.1 Dascalescu, CM.; Wendum, D.; Gorin, NC. (Sep 2001). "Littoral-cell angioma as a cause of splenomegaly.". N Engl J Med 345 (10): 772-3. doi:10.1056/NEJM200109063451016. PMID 11547761. http://www.nejm.org/doi/full/10.1056/NEJM200109063451016.
  3. ↑ URL: http://www.merriam-webster.com/dictionary/littoral. Accessed on: 24 July 2011.
  4. ↑ URL: http://dictionary.reference.com/browse/littoral. Accessed on: 24 July 2011.
  5. ↑ URL: http://www.webpathology.com/image.asp?n=2&Case=382. Accessed on: 11 April 2014.
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