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Difference between revisions of "Littoral cell angioma"
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haemangioendothelioma variant
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m (haemangioendothelioma variant) |
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| LMDDx = [[hemangioendothelioma]], [[angiosarcoma]], [[hemangiopericytoma]], [[splenic hamartoma]] | | LMDDx = [[hemangioendothelioma]], [[angiosarcoma]], [[hemangiopericytoma]], [[splenic hamartoma]] | ||
| Stains = | | Stains = | ||
| IHC = | | IHC = lining cells: CD68 +ve, CD31 +ve, CD34 -ve, CD8 -ve | ||
| EM = | | EM = | ||
| Molecular = | | Molecular = | ||
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| Symptoms = | | Symptoms = | ||
| Prevalence = rare | | Prevalence = rare | ||
| Bloodwork = | | Bloodwork = | ||
| Rads = solitary hypodense lesion | | Rads = solitary hypodense lesion | ||
| Endoscopy = | | Endoscopy = | ||
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| Tx = | | Tx = | ||
}} | }} | ||
'''Littoral cell angioma''', abbreviated '''LCA''', is an uncommon | '''Littoral cell angioma''', abbreviated '''LCA''', is an uncommon [[vascular lesions|vascular lesion]] of the [[spleen]]. Regarded as benign, rare cases have shown malignant behaviour and have been labelled as [[littoral cell hemangioendothelioma]].<ref>{{Cite journal | last1 = Ben-Izhak | first1 = O. | last2 = Bejar | first2 = J. | last3 = Ben-Eliezer | first3 = S. | last4 = Vlodavsky | first4 = E. | title = Splenic littoral cell haemangioendothelioma: a new low-grade variant of malignant littoral cell tumour. | journal = Histopathology | volume = 39 | issue = 5 | pages = 469-75 | month = Nov | year = 2001 | doi = | PMID = 11737304 }}</ref><ref name=pmid24420309>{{Cite journal | last1 = He | first1 = P. | last2 = Yan | first2 = XD. | last3 = Wang | first3 = JR. | last4 = Guo | first4 = RC. | last5 = Zhang | first5 = HB. | title = Splenic littoral cell hemangioendothelioma: report of a case with hepatic metastases and review of the literature. | journal = J Clin Ultrasound | volume = 42 | issue = 5 | pages = 308-12 | month = Jun | year = 2014 | doi = 10.1002/jcu.22120 | PMID = 24420309 }}</ref> | ||
''Angioma'', in general, is dealt with in ''[[vascular malformations]]''. | ''Angioma'', in general, is dealt with in ''[[vascular malformations]]''. | ||
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Features:<ref name=pmid15329769>{{Cite journal | last1 = Tan | first1 = YM. | last2 = Chuah | first2 = KL. | last3 = Wong | first3 = WK. | title = Littoral cell angioma of the spleen. | journal = Ann Acad Med Singapore | volume = 33 | issue = 4 | pages = 524-6 | month = Jul | year = 2004 | doi = | PMID = 15329769 | url=http://www.annals.edu.sg/pdf200408/V33N4p524.pdf }}</ref> | Features:<ref name=pmid15329769>{{Cite journal | last1 = Tan | first1 = YM. | last2 = Chuah | first2 = KL. | last3 = Wong | first3 = WK. | title = Littoral cell angioma of the spleen. | journal = Ann Acad Med Singapore | volume = 33 | issue = 4 | pages = 524-6 | month = Jul | year = 2004 | doi = | PMID = 15329769 | url=http://www.annals.edu.sg/pdf200408/V33N4p524.pdf }}</ref> | ||
*Rare. | *Rare. | ||
*Benign | *Benign (except for rare cases - see above) | ||
Clinical:<ref name=pmid15329769/><ref name=pmid11547761>{{Cite journal | last1 = Dascalescu | first1 = CM. | last2 = Wendum | first2 = D. | last3 = Gorin | first3 = NC. | title = Littoral-cell angioma as a cause of splenomegaly. | journal = N Engl J Med | volume = 345 | issue = 10 | pages = 772-3 | month = Sep | year = 2001 | doi = 10.1056/NEJM200109063451016 | PMID = 11547761 | url=http://www.nejm.org/doi/full/10.1056/NEJM200109063451016 }}</ref> | Clinical:<ref name=pmid15329769/><ref name=pmid11547761>{{Cite journal | last1 = Dascalescu | first1 = CM. | last2 = Wendum | first2 = D. | last3 = Gorin | first3 = NC. | title = Littoral-cell angioma as a cause of splenomegaly. | journal = N Engl J Med | volume = 345 | issue = 10 | pages = 772-3 | month = Sep | year = 2001 | doi = 10.1056/NEJM200109063451016 | PMID = 11547761 | url=http://www.nejm.org/doi/full/10.1056/NEJM200109063451016 }}</ref> | ||
*Pyrexia (fever), splenomegaly, weight loss, solitary hypodense lesion on imaging. | *Pyrexia (fever), splenomegaly, weight loss, solitary hypodense lesion on imaging. | ||
*Regarded as benign | |||
*Case series show strong association with synchronous malignancy.<ref name=pmid24987971>{{Cite journal | last1 = Sarandria | first1 = JJ. | last2 = Escano | first2 = M. | last3 = Kamangar | first3 = F. | last4 = Farooqui | first4 = S. | last5 = Montgomery | first5 = E. | last6 = Cunningham | first6 = SC. | title = Massive splenomegaly correlates with malignancy: 180 cases of splenic littoral cell tumors in the world literature. | journal = Minerva Chir | volume = 69 | issue = 4 | pages = 229-37 | month = Aug | year = 2014 | doi = | PMID = 24987971 }}</ref> | |||
Note: | Note: | ||
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**[[Hemangiopericytoma]]. | **[[Hemangiopericytoma]]. | ||
**[[Splenic hamartoma]]. | **[[Splenic hamartoma]]. | ||
**Splenic [[hemangioma]].<ref>URL: [http://www.webpathology.com/image.asp?n=2&Case=382 http://www.webpathology.com/image.asp?n=2&Case=382]. Accessed on: 11 April 2014.</ref> | |||
===Images=== | ===Images=== | ||
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*[http://www.webpathology.com/image.asp?n=5&Case=382 LCA - low mag. (webpathology.com)]. | *[http://www.webpathology.com/image.asp?n=5&Case=382 LCA - low mag. (webpathology.com)]. | ||
*[http://www.webpathology.com/image.asp?n=6&Case=382 LCA - high mag. (webpathology.com)]. | *[http://www.webpathology.com/image.asp?n=6&Case=382 LCA - high mag. (webpathology.com)]. | ||
[[File:3 18031130010902 sl 1.png|Littoral cell angioma of spleen]] | |||
[[File:3 18031130010902 sl 2.png|Littoral cell angioma of spleen]] | |||
[[File:3 18031130010902 sl 3.png|Littoral cell angioma of spleen]] | |||
[[File:3 18031130010902 sl 4.png|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== | ==IHC== | ||
Normal littoral cells express CD8+, macrophage/histiocytic markers and vascular markers. In comparison, littoral cell angioma is:<ref name=pmid15329769/> | |||
*CD68 +ve | *CD68 +ve (and other histiocytic markers) | ||
*CD31 +ve | *CD31 +ve (and other vascular markers, e.g. Factor VIII, but ''not'' CD34) | ||
*CD34 -ve (normally in red pulp).<ref name=pmid15329769/> | *CD34 -ve (normally in red pulp).<ref name=pmid15329769/> | ||
*CD8 -ve (normally in red pulp).<ref name=pmid15329769/> | *CD8 -ve (normally in red pulp).<ref name=pmid15329769/> | ||
* | *CD21 +ve (at least focally) | ||
==See also== | ==See also== | ||