Difference between revisions of "Vascular tumours"

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[[Image:Capillary_hemangioma_-_very_high_mag.jpg|thumb|right|[[Micrograph]] showing a [[capillary hemangioma]], a common type of vascular tumour. [[H&E stain]].]]
This article covers [[soft tissue lesions|soft tissue]] '''vascular tumours'''.  Vascular malformations are covered in the ''[[vascular malformations]]'' article.
This article covers [[soft tissue lesions|soft tissue]] '''vascular tumours'''.  Vascular malformations are covered in the ''[[vascular malformations]]'' article.


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=Distinct entities=
=Distinct entities=
==Hemangioma==
==Hemangioma==
===General===
{{Main|Hemangioma}}
*May be found in the liver.
{{Main|Liver hemangioma}}
**Classically subcapsular.
***May rupture and be life-threatening.<ref>{{Cite journal  | last1 = Vokaer | first1 = B. | last2 = Kothonidis | first2 = K. | last3 = Delatte | first3 = P. | last4 = De Cooman | first4 = S. | last5 = Pector | first5 = JC. | last6 = Liberale | first6 = G. | title = Should ruptured liver haemangioma be treated by surgery or by conservative means? A case report. | journal = Acta Chir Belg | volume = 108 | issue = 6 | pages = 761-4 | month =  | year =  | doi =  | PMID = 19241936 }}</ref>
 
====Childhood====
Common childhood hemangiomas:<ref name=pmid10410855>{{cite journal |author=Prieto VG, Shea CR |title=Selected cutaneous vascular neoplasms. A review |journal=Dermatol Clin |volume=17 |issue=3 |pages=507–20, viii |year=1999 |month=July |pmid=10410855 |doi= |url=}}</ref>
*Tufted - small clusters of blood vessels.
*Microvenular hemangioma.
*Glomeruloid hemangioma - associated with [[POEMS syndrome]], [[Castleman disease]].<ref name=pmid16860182>{{cite journal |author=Uthup S, Balachandran K, Ammal VA, ''et al.'' |title=Renal involvement in multicentric Castleman disease with glomeruloid hemangioma of skin and plasmacytoma |journal=Am. J. Kidney Dis. |volume=48 |issue=2 |pages=e17–24 |year=2006 |month=August |pmid=16860182 |doi=10.1053/j.ajkd.2006.04.089 |url=}}</ref><ref name=Ref_WMSP618>{{Ref WMSP|618}}</ref>
*Epithelioid hemangioma.
*Targetoid hemosideric hemangioma.
====Soft tissue====
Several types are seen in soft tissue:<ref>{{Ref WMSP|602}}</ref>
*Capillary.
*Cavernous.
*Arteriovenous.
*Venous.
*Intramuscular.
*Synovial.


===Microscopic===
==Lymphangioma==
Features:
*[[AKA]] ''cystic hygroma''.<ref name=Ref_WMSP12>{{Ref WMSP|12}}</ref>
*Abundance of benign small blood vessels. (???)


Images:
*[[WC]]:
**[http://commons.wikimedia.org/wiki/File:Capillary_hemangioma_-_intermed_mag.jpg Capillary hemangioma - intermed. mag. (WC)].
**[http://commons.wikimedia.org/wiki/File:Capillary_hemangioma_-_very_high_mag.jpg Capillary hemangioma - very high mag. (WC)].
*www:
**[http://path.upmc.edu/cases/case277.html Diffuse hemangioma - several images (upmc.edu)].
==Kaposi sarcoma==
===General===
===General===
*Not really a sarcoma.
*Benign.
*Caused by [[Human herpesvirus-8]] (HHV-8).
*Classically in the left neck.<ref name=Ref_WMSP12>{{Ref WMSP|12}}</ref>
*Associated with immunodeficiency, e.g. [[HIV]]/AIDS.
*May be seen in [[Turner syndrome]].


===Stages===
Treatment:
It is seen in different stages:<ref>URL: [http://www.histopathology-india.net/KS.htm http://www.histopathology-india.net/KS.htm]. Accessed on: 31 January 2010.</ref><ref>URL: [http://emedicine.medscape.com/article/1083998-clinical#a0217 http://emedicine.medscape.com/article/1083998-clinical#a0217]. Accessed on: 17 November 2011.</ref>
*Surgical excision.
#Patch stage.
#Plaque stage.
#Nodular stage.
#Exophytic stage.
#Infiltrative stage.
#Lymphadenopathic stage.


===Microscopic===
===Microscopic===
Features:<ref name=Ref_Klatt23>{{Ref Klatt|23}}</ref>- '''key feature'''.
Features:<ref name=Ref_WMSP489>{{Ref WMSP|489}}</ref><ref name=Ref_WMSP12>{{Ref WMSP|12}}</ref>
*+/-Nuclear atypia.
*Thin-walled channels lined by endothelium.
*[[Hyaline globules]] (intracytoplasmic)<ref name=pmid7528163>{{cite journal |author=del Rosario AD, Bui HX, Singh J, Ginsburg R, Ross JS |title=Intracytoplasmic eosinophilic hyaline globules in cartilaginous neoplasms: a surgical, pathological, ultrastructural, and electron probe x-ray microanalytic study |journal=Hum. Pathol. |volume=25 |issue=12 |pages=1283–9 |year=1994 |month=December |pmid=7528163 |doi= |url=}}</ref> - pale pink globs (that are paler than RBCs) - '''important feature'''.
*+/-Eosinophilic intraluminal material.
*+/-Hemosiderin deposits.
*+/-Clusters of intraluminal lymphocytes.
*+/-Occasional [[RBC]]s.


DDx:
DDx:
*Angiosarcoma (have many mitoses, nuclear atypia).
*[[Hemangioma]].
*Masson's hemangioma (Intravascular papillary endothelial hyperplasia).
*Benign lymphangioendothelioma.<ref>{{Cite journal  | last1 = Guillou | first1 = L. | last2 = Fletcher | first2 = CD. | title = Benign lymphangioendothelioma (acquired progressive lymphangioma): a lesion not to be confused with well-differentiated angiosarcoma and patch stage Kaposi's sarcoma: clinicopathologic analysis of a series. | journal = Am J Surg Pathol | volume = 24 | issue = 8 | pages = 1047-57 | month = Aug | year = 2000 | doi =  | PMID = 10935645 }}</ref>
**Histologically very similar.<ref>URL: [http://path.upmc.edu/cases/case134/dx.html http://path.upmc.edu/cases/case134/dx.html]. Accessed on: 5 January 2012.</ref>
 
Notes:
*Hyaline globules have a DDx ([[hepatocellular carcinoma]], lung adenocarcinoma, [[chondrosarcoma]]s + others).<ref name=pmid7528163/>
*Promontory sign - small vessel protruding into an abnormal vascular space.<ref name=pmid19318797>{{cite journal |author=Lazova R, McNiff JM, Glusac EJ, Godic A |title=Promontory sign--present in patch and plaque stage of angiosarcoma! |journal=Am J Dermatopathol |volume=31 |issue=2 |pages=132–6 |year=2009 |month=April |pmid=19318797 |doi=10.1097/DAD.0b013e3181951045 |url=}}</ref>
**Not pathognomonic for KS.<ref name=pmid20577080>{{cite journal |author=Fernandez-Flores A, Rodriguez R |title=Promontory Sign in a Reactive Benign Vascular Proliferation |journal=Am J Dermatopathol |volume= |issue= |pages= |year=2010 |month=June |pmid=20577080 |doi=10.1097/DAD.0b013e3181cf0ae5 |url=}}</ref>
**Image: [http://www.diagnosticpathology.org/content/3/1/31/figure/F1 Promontory sign (diagnosticpathology.org)].


Images:
Images:
*[http://commons.wikimedia.org/wiki/File:Kaposi_sarcoma_high_mag.jpg Kaposi sacoma - high mag. (WC)].
*[http://www.webpathology.com/image.asp?n=5&Case=412 Lymphangioma - low mag. (webpathology.com)].
*[http://commons.wikimedia.org/wiki/File:Kaposi_sarcoma_low_intermed_mag.jpg Kaposi sarcoma - intermed. mag. (WC)].
*[http://www.webpathology.com/image.asp?case=412&n=6 Lymphangioma - high mag. (webpathology.com)].


===IHC===
===IHC===
*CD31 +ve.  
Features:<ref name=pmid19098468>{{Cite journal  | last1 = Kalof | first1 = AN. | last2 = Cooper | first2 = K. | title = D2-40 immunohistochemistry--so far! | journal = Adv Anat Pathol | volume = 16 | issue = 1 | pages = 62-4 | month = Jan | year = 2009 | doi = 10.1097/PAP.0b013e3181915e94 | PMID = 19098468 }}</ref><ref name=pmid11950918>{{Cite journal  | last1 = Kahn | first1 = HJ. | last2 = Bailey | first2 = D. | last3 = Marks | first3 = A. | title = Monoclonal antibody D2-40, a new marker of lymphatic endothelium, reacts with Kaposi's sarcoma and a subset of angiosarcomas. | journal = Mod Pathol | volume = 15 | issue = 4 | pages = 434-40 | month = Apr | year = 2002 | doi = 10.1038/modpathol.3880543 | PMID = 11950918 }}</ref>
*CD34 +ve.  
*D2-40 +ve.
*[[HHV-8]] +ve.
 
==Kaposi sarcoma==
{{Main|Kaposi sarcoma}}


==Masson hemangioma==
==Masson hemangioma==
===General===
*Benign non-neoplastic lesion - a vessel that has thrombosed and recanalized.
*[[AKA]] ''intravascular papillary endothelial hyperplasia''.<ref name=pmid15865098>{{cite journal |author=Korkolis DP, Papaevangelou M, Koulaxouzidis G, Zirganos N, Psichogiou H, Vassilopoulos PP |title=Intravascular papillary endothelial hyperplasia (Masson's hemangioma) presenting as a soft-tissue sarcoma |journal=Anticancer Res. |volume=25 |issue=2B |pages=1409–12 |year=2005 |pmid=15865098 |doi= |url=}}</ref>  
*[[AKA]] ''intravascular papillary endothelial hyperplasia''.<ref name=pmid15865098>{{cite journal |author=Korkolis DP, Papaevangelou M, Koulaxouzidis G, Zirganos N, Psichogiou H, Vassilopoulos PP |title=Intravascular papillary endothelial hyperplasia (Masson's hemangioma) presenting as a soft-tissue sarcoma |journal=Anticancer Res. |volume=25 |issue=2B |pages=1409–12 |year=2005 |pmid=15865098 |doi= |url=}}</ref>  
*Histomorphologically may be confused with low-grade angiosarcoma or other soft tissue sarcomas.<ref name=pmid15865098/>
*[[AKA]] ''Masson tumor''.<ref>URL: [http://path.upmc.edu/cases/case544/dx.html http://path.upmc.edu/cases/case544/dx.html]. Accessed on: 25 January 2012.</ref>
===Microscopic===
{{Main|intravascular papillary endothelial hyperplasia}}
Features:
*Well-circumscribed - '''key (low power) feature'''.  
*Abundant small vascular channels with benign endothelium.
 
Notes:
*Looks like [[Kaposi sarcoma]] at high power.


==Angiosarcoma==
==Angiosarcoma==
===General===
{{Main|Angiosarcoma}}
*Malignant tumour - with a horrible prognosis.<ref name=pmid20537949>{{cite journal |author=Young RJ, Brown NJ, Reed MW, Hughes D, Woll PJ |title=Angiosarcoma |journal=Lancet Oncol |volume= |issue= |pages= |year=2010 |month=May |pmid=20537949 |doi=10.1016/S1470-2045(10)70023-1 |url=}}</ref>
*Classically on the scalp or head & neck.
 
Epidemiology:
*May arise secondary to therapeutic radiation or chronic lymphoedema related to breast carcinoma.
*Liver angiosarcomas are assoc. with vinyl chloride exposure.<ref>{{Ref PCPBoD8|212}}</ref>
 
===Microscopic===
Features:
*Very many small capillaries of irregular shape lined with:
**Pleomorphic nuclei.
***May have hobnail morphology.
*Mitoses.
*Cytoplasmic vacuoles.
**Cells trying to form lumina - embryologic.
 
Notes:
*Epithelioid variant (with abundant cytoplasm & sheeting architecture) may resemble ''[[melanoma]]'' or ''[[hepatocellular carcinoma]]''.
 
Images:
*[http://commons.wikimedia.org/wiki/File:Epithelioid_angiosarcoma_-_very_low_mag.jpg Epithelioid angiosarcoma - very low mag. (WC)].
*[http://commons.wikimedia.org/wiki/File:Epithelioid_angiosarcoma_-_intermed_mag.jpg Epithelioid angiosarcoma - intermed mag. (WC)].
*[http://commons.wikimedia.org/wiki/File:Epithelioid_angiosarcoma_-_very_high_mag.jpg Epithelioid angiosarcoma - very high mag. (WC)].
 
===IHC===
*CD34 +ve.
*D2-40 +ve. (???)
*CD31 +ve.
*FLI-1 +ve.<ref name=pmid15001993>{{Cite journal  | last1 = Rossi | first1 = S. | last2 = Orvieto | first2 = E. | last3 = Furlanetto | first3 = A. | last4 = Laurino | first4 = L. | last5 = Ninfo | first5 = V. | last6 = Dei Tos | first6 = AP. | title = Utility of the immunohistochemical detection of FLI-1 expression in round cell and vascular neoplasm using a monoclonal antibody. | journal = Mod Pathol | volume = 17 | issue = 5 | pages = 547-52 | month = May | year = 2004 | doi = 10.1038/modpathol.3800065 | PMID = 15001993 }}</ref>


==Kaposiform hemangioendothelioma==
==Kaposiform hemangioendothelioma==
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==Epithelioid hemangioendothelioma==
==Epithelioid hemangioendothelioma==
*Should not be confused with ''[[epithelioid hemangioma]]''.
{{Main|Epithelioid hemangioendothelioma}}
===General===
*Malignant.<ref name=Ref_WMSP603>{{Ref WMSP|603}}</ref>
*Adults - wide age range.
*Associated with oral contraceptives, vinyl chloride.<ref name=pmid20165548/>
 
===Microscopic===
Features:<ref name=pmid20165548>{{Cite journal  | last1 = Gupta | first1 = R. | last2 = Mathur | first2 = SR. | last3 = Gupta | first3 = SD. | last4 = Durgapal | first4 = P. | last5 = Iyer | first5 = VK. | last6 = Das | first6 = CJ. | last7 = Shalimar | first7 = SK. | last8 = Acharya | first8 = . | title = Hepatic epithelioid hemangioendothelioma: A diagnostic pitfall in aspiration cytology. | journal = Cytojournal | volume = 6 | issue =  | pages = 25 | month =  | year = 2010 | doi = 10.4103/1742-6413.58951 | PMID = 20165548 | URL = http://www.biomedsearch.com/nih/Hepatic-epithelioid-hemangioendothelioma-diagnostic-pitfall/20165548.html }}</ref>
*Large epithelioid perivascular cells with:
**Abundant pale eosinophilic cytoplasm.
**Cytoplasmic vacuolation (some cells) - [[AKA]] "blister cells" - '''key feature'''.
***May form lumen and have RBC within.
**Vesicular nucleus with prominent nucleolus in some cells.
*Tuft-like projections into capillaries.


Image:
==Retiform hemangioendothelioma==
*[http://en.wikipedia.org/wiki/File:Epithelioid_hemangioendothelioma_EHE.Image5.jpg Epithelioid hemangioendothelioma (WP)].
{{Main|Retiform hemangioendothelioma}}


===IHC===
==Intimal sarcoma==
Features:<ref name=pmid20165548>{{Cite journal  | last1 = Gupta | first1 = R. | last2 = Mathur | first2 = SR. | last3 = Gupta | first3 = SD. | last4 = Durgapal | first4 = P. | last5 = Iyer | first5 = VK. | last6 = Das | first6 = CJ. | last7 = Shalimar | first7 = SK. | last8 = Acharya | first8 = . | title = Hepatic epithelioid hemangioendothelioma: A diagnostic pitfall in aspiration cytology. | journal = Cytojournal | volume = 6 | issue =  | pages = 25 | month =  | year = 2010 | doi = 10.4103/1742-6413.58951 | PMID = 20165548 | URL = http://www.biomedsearch.com/nih/Hepatic-epithelioid-hemangioendothelioma-diagnostic-pitfall/20165548.html }}</ref>
{{Main|Intimal sarcoma}}
*CD31 +ve.
*CD34 +ve.
*Factor VIII +ve.


=See also=
=See also=
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{{Reflist|2}}
{{Reflist|2}}


[[Category:Vascular tumours]]
[[Category:Soft tissue lesions]]
[[Category:Soft tissue lesions]]

Latest revision as of 17:15, 18 January 2024

Micrograph showing a capillary hemangioma, a common type of vascular tumour. H&E stain.

This article covers soft tissue vascular tumours. Vascular malformations are covered in the vascular malformations article.

Normal histology

Normal blood vessel histology is dealt with in the vascular disease article.

Mimics

Distinct entities

Hemangioma

Main article: Hemangioma
Main article: Liver hemangioma

Lymphangioma

General

Treatment:

  • Surgical excision.

Microscopic

Features:[2][1]

  • Thin-walled channels lined by endothelium.
  • +/-Eosinophilic intraluminal material.
  • +/-Clusters of intraluminal lymphocytes.
  • +/-Occasional RBCs.

DDx:

Images:

IHC

Features:[3][4]

  • D2-40 +ve.

Kaposi sarcoma

Main article: Kaposi sarcoma

Masson hemangioma

  • AKA intravascular papillary endothelial hyperplasia.[5]
  • AKA Masson tumor.[6]
Main article: intravascular papillary endothelial hyperplasia

Angiosarcoma

Main article: Angiosarcoma

Kaposiform hemangioendothelioma

General

  • Locally aggressive.[7]
  • Childhood tumour.[8]
  • Approximately half have Kasabach–Merritt phenomenon[8] = vascular tumour --> coagulopathy.

Microscopic

Features:[9]

  • Spindle cells lesions in sheets or nodules.
  • +/-Round tumour nodules - "cannon ball" appearance.

DDx:

IHC

Features:[9]

  • Vimentin +ve.
  • C31 +ve.
  • CD34 +ve.
  • UEA-1 lectin +ve.

Epithelioid hemangioendothelioma

Main article: Epithelioid hemangioendothelioma

Retiform hemangioendothelioma

Main article: Retiform hemangioendothelioma

Intimal sarcoma

Main article: Intimal sarcoma

See also

References

  1. ↑ 1.0 1.1 1.2 Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 12. ISBN 978-0781765275.
  2. ↑ Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 489. ISBN 978-0781765275.
  3. ↑ Kalof, AN.; Cooper, K. (Jan 2009). "D2-40 immunohistochemistry--so far!". Adv Anat Pathol 16 (1): 62-4. doi:10.1097/PAP.0b013e3181915e94. PMID 19098468.
  4. ↑ Kahn, HJ.; Bailey, D.; Marks, A. (Apr 2002). "Monoclonal antibody D2-40, a new marker of lymphatic endothelium, reacts with Kaposi's sarcoma and a subset of angiosarcomas.". Mod Pathol 15 (4): 434-40. doi:10.1038/modpathol.3880543. PMID 11950918.
  5. ↑ Korkolis DP, Papaevangelou M, Koulaxouzidis G, Zirganos N, Psichogiou H, Vassilopoulos PP (2005). "Intravascular papillary endothelial hyperplasia (Masson's hemangioma) presenting as a soft-tissue sarcoma". Anticancer Res. 25 (2B): 1409–12. PMID 15865098.
  6. ↑ URL: http://path.upmc.edu/cases/case544/dx.html. Accessed on: 25 January 2012.
  7. ↑ Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 603. ISBN 978-0781765275.
  8. ↑ 8.0 8.1 Lyons, LL.; North, PE.; Mac-Moune Lai, F.; Stoler, MH.; Folpe, AL.; Weiss, SW. (May 2004). "Kaposiform hemangioendothelioma: a study of 33 cases emphasizing its pathologic, immunophenotypic, and biologic uniqueness from juvenile hemangioma.". Am J Surg Pathol 28 (5): 559-68. PMID 15105642.
  9. ↑ 9.0 9.1 9.2 Miller, K. (Mar 1991). "Sister-chromatid exchange in human B- and T-lymphocytes exposed to bleomycin, cyclophosphamide, and ethyl methanesulfonate.". Mutat Res 247 (1): 175-82. PMID 1706068. http://www.nature.com/modpathol/journal/v14/n11/full/3880441a.html.
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