Littoral cell angioma
| 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)
- Pyrexia (fever), splenomegaly, weight loss, solitary hypodense lesion on imaging.
- Regarded as benign
- Case series show strong association with synchronous malignancy.[5]
Note:
Microscopic
Features:[3]
- Anastoming, small vascular channels.
- Cystic spaces.
- May have papillary projections.[4]
DDx:[3]
- Vascular lesions:
Images
LCA - very low mag. (WC)
LCA - low mag. (WC)
LCA - intermed. mag. (WC)
LCA - high mag. (WC)
LCA - very high mag. (WC)
www:
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
- ↑ 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.
- ↑ 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.
- ↑ 3.0 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.
- ↑ 4.0 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.
- ↑ 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.
- ↑ URL: http://www.merriam-webster.com/dictionary/littoral. Accessed on: 24 July 2011.
- ↑ URL: http://dictionary.reference.com/browse/littoral. Accessed on: 24 July 2011.
- ↑ URL: http://www.webpathology.com/image.asp?n=2&Case=382. Accessed on: 11 April 2014.