Littoral cell angioma

From Libre Pathology
Jump to: navigation, search
Littoral cell angioma
Diagnosis in short

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

Littoral cell angioma, abbreviated LCA, is an uncommon vascular lesion of the spleen. Regarded as benign, rare cases have shown malignant behaviour and have been labelled as littoral cell hemangioendothelioma.[1][2]

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

General

Features:[3]

  • Rare.
  • Benign (except for rare cases - see above)

Clinical:[3][4]

  • Pyrexia (fever), splenomegaly, weight loss, solitary hypodense lesion on imaging.
  • Regarded as benign
  • Case series show strong association with synchronous malignancy.[5]

Note:

  • Littoral cells = line vascular channels of the red pulp.[3]
    • Littoral = related to shore.[6][7]

Microscopic

Features:[3]

  • Anastoming, small vascular channels.
  • Cystic spaces.
    • May have papillary projections.[4]

DDx:[3]

Images

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:[3]

  • 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).[3]
  • CD8 -ve (normally in red pulp).[3]
  • CD21 +ve (at least focally)

See also

References

  1. Ben-Izhak, O.; Bejar, J.; Ben-Eliezer, S.; Vlodavsky, E. (Nov 2001). "Splenic littoral cell haemangioendothelioma: a new low-grade variant of malignant littoral cell tumour.". Histopathology 39 (5): 469-75. PMID 11737304.
  2. He, P.; Yan, XD.; Wang, JR.; Guo, RC.; Zhang, HB. (Jun 2014). "Splenic littoral cell hemangioendothelioma: report of a case with hepatic metastases and review of the literature.". J Clin Ultrasound 42 (5): 308-12. doi:10.1002/jcu.22120. PMID 24420309.
  3. 3.0 3.1 3.2 3.3 3.4 3.5 3.6 3.7 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.
  4. 4.0 4.1 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.
  5. Sarandria, JJ.; Escano, M.; Kamangar, F.; Farooqui, S.; Montgomery, E.; Cunningham, SC. (Aug 2014). "Massive splenomegaly correlates with malignancy: 180 cases of splenic littoral cell tumors in the world literature.". Minerva Chir 69 (4): 229-37. PMID 24987971.
  6. URL: http://www.merriam-webster.com/dictionary/littoral. Accessed on: 24 July 2011.
  7. URL: http://dictionary.reference.com/browse/littoral. Accessed on: 24 July 2011.
  8. URL: http://www.webpathology.com/image.asp?n=2&Case=382. Accessed on: 11 April 2014.