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Table of Contents
EDITORIAL
Year : 2019  |  Volume : 2  |  Issue : 4  |  Page : 85-87

Hemangioma and venous malformation are as different as an apple and orange!


Department of Vascular Surgery, George Washington University, Washington, DC, USA

Date of Submission12-Dec-2019
Date of Decision20-Dec-2019
Date of Acceptance26-Dec-2019
Date of Web Publication25-Feb-2020

Correspondence Address:
B B Lee
Department of Vascular Surgery, George Washington University, Washington, DC
USA
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/VIT.VIT_2_20

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How to cite this article:
Lee B B. Hemangioma and venous malformation are as different as an apple and orange!. Vasc Invest Ther 2019;2:85-7

How to cite this URL:
Lee B B. Hemangioma and venous malformation are as different as an apple and orange!. Vasc Invest Ther [serial online] 2019 [cited 2020 Sep 26];2:85-7. Available from: http://www.vitonline.org/text.asp?2019/2/4/85/279228



Venous malformation (VM) is the most common type of congenital vascular malformations (CVMs); it represents a group of birth defect limited to the venous system among the circulation system. However, the VM has been called so erroneously as “hemangioma” based on misunderstanding of its nature and is still infrequently called by mistake although the VM is NOT a hemangioma.[1],[2],[3],[4],[5],[6],[7],[8]

The CVMs, representing extremely variable conditions of the birth defects affecting the entire vascular system, have remained a symbol of confusion due to such improper terminology/nomenclature based on limited knowledge through the last century. Moreover, the term of “hemangioma” in particular, is one example of how such confusion has become a frequent source of serious mistakes through decades.

Indeed, the terms of “cavernous/capillary” hemangioma are the most abused term for the VM to become a constant source of confusion. Such confusion with a genuine hemangioma which is not a vascular malformation, but a vascular tumor only increases the risk of mismanagement of the VM to warrant a proper understanding of these two fundamentally different types of vascular anomalies.

All the CVMs, including the VM present at birth as inborn vascular defects and continue to grow at a rate that is proportional to the growth rate of the body regardless of its type. However, the hemangioma is not as a vascular tumor that originates from endothelial cells, usually appearing in the early neonatal period.

Unlike CVMs, the hemangioma, mostly of neonatal/infantile type, has a distinctive growth cycle, characterized by the initial proliferation phase of early rapid growth and subsequent involutional phase of slow regression to follow, that usually completes before the age of 5–10 years in the majority.

In other words, a genuine “hemangioma” is not a vascular malformation but the vascular tumor with the unique characteristic of a “self-limited” growth while the venous/vascular malformations are embryologic tissue remnants with “self-perpetuating” growth characteristic.

Although ISSVA classification classified vascular malformations and vascular tumors/hemangiomas as one group of “Vascular Anomalies” together, both conditions are fundamentally different, not only in their anatomical, histological, and pathophysiological findings but also in their clinical courses.

CVMs represent the outcome of birth defects developed during various stages of embryogenesis (vasculogenesis/angiogenesis) and hence they can develop throughout the entire peripheral circulation systems, often affecting more than one vascular system: arterial, venous, and lymphatic and capillary systems.

CVMs are, therefore, a group of various vascular defects with mixture of different histo-pathological characteristics and clinical/hemodynamic behaviors; their form would depend on the location, extent and severity either as one independent/predominant lesion (e.g., VM) or a mixed lesion with two or three different types of CVMs (e.g., hemo-lymphatic malformation).

The VM is one of these CVMs affecting the venous system, either alone or combined with other types of the CVMs (e.g., lymphatic malformation; AV-shunting malformation). Hence, the VM remains one of the most difficult and confusing diagnostic and therapeutic enigmas together with the rest of CVMs due to the wide range of clinical presentation, unpredictable clinical course, erratic response to the treatment with high recurrence, and high morbidity by currently available conventional treatment.

To clarify such confusing terminology without proper information on etiology, anatomy, and pathophysiology, the Hamburg classification of the CVMs was proposed to improve their management through the Hamburg workshop in 1988 as a newer classification system for contemporary concept of the CVMs, for the first time based on anatomical, pathological, and embryological criteria.

The Hamburg classification has become the standard classification system to provide a new definition on various vascular malformations as well as embryonic subclassification of the CVMs for their advanced management. The ISSVA classification was also offered for the classification of vascular malformations and vascular tumors/hemangiomas together under the group of vascular anomalies.

“Modified” Hamburg classification has further subclassified all the CVM lesions on the basis of their embryological status of the lesion, when/which stage of the embryonal life the developmental arrest has occurred. The CVM lesion originated from the “earlier” stage of embryogenesis was defined as an “extra-truncular” lesion, whereas the lesion from the “later” stage as a “truncular” lesion.

Extratruncular lesion is the outcome of the developmental arrest that occurres in the “earlier” reticular stage of embryonic life. Hence, this embryonic tissue remnant of mesodermal origin retains the mesenchymal cells (angioblasts) characteristics of the evolutional potential to grow when stimulated (e.g., trauma, surgery, hormone, and pregnancy). Therefore, it always accompanies the risk of a recurrence after suboptimum management.

Extratruncular lesions remain as amorphous vascular structures since further development was arrested before the vascular system forms vascular trunks; it presents as clinically diffuse infiltrating mass lesion to giving a mechanical compression impact to the surrounding tissues/organs besides the hemodynamic impact to its belonging vascular system.

Truncular lesion is the outcome of defective development during the “latter” stage of fetal development following the reticular stage of vascular development. Therefore, as embryonic tissue remnant originated from the vascular trunk formation (truncal) stage, does no longer retain the embryonic characteristics of the mesenchymal cells (angioblasts) with no more risk of recurrence/growth.

However, the truncular lesion has much more serious hemodynamic impact to the involved vascular system in general (e.g., suprahepatic IVC occlusion to cause primary Budd-Chiari syndrome, extracranial jugular/azygos vein stenosis to cause CCSVI) since the lesion is directly involved to the vein trunk.

For example, the truncular VM lesion with defective vessel trunk formation is the group of obstruction/dilatation of the formed vessel (e.g., vein web and venous aneurysm), or aplasia, hypoplasia, and/or hyperplasia of the vessel development (e.g., rudimentary iliac vein and the absence of the femoral vein).

Another form of truncular VM lesions is the fetal (truncal) vessel which remains without completion of normal involution (e.g., sciatic vein, marginal vein); it often exists with additional defective vessel condition to make the lesion more difficult to handle (e.g., Klippel-Trenaunay syndrome).

Finally, both truncular and extratruncular VM lesions may exist together; may exist with other vascular malformations (e.g., capillary, AV-shunting, and/or lymphatic malformation) in addition, they may exist even with the (genuine) hemangioma as well.

Therefore, the confirmation of this embryological subtype: “extratruncular versus truncular” is essential for the proper management of every VM lesion because “extratruncular” type of VM lesion is a primitive CVM lesion so that it has a high tendency to progress/grow when the condition is met and also it has high recurrence following the treatment due to this unique embryologic characteristic. Moreover, improper treatment strategy will only stimulate dormant lesion to respond in an erratic way.

Most of all, these “extratruncular” lesions are often described as “cavernous/capillary hemangioma” by misunderstanding. Therefore, proper identification of genuine “hemangioma” is extremely important to avoid unwarranted mismanagement of the VM lesions.


  Summary Top


VM is not a hemangioma. VM and hemangioma are two fundamentally different conditions with different anatomical, histological, and pathophysiological findings and their clinical courses as well.

VM is one of the vascular malformations with “self-perpetuating” growth characteristic as an embryonic tissue remnant, while hemangioma is a vascular tumor originated from endothelial cells with “self-limited” growth characteristic.

Hence, proper confirmation of the VM lesion versus “hemangioma” is essential as the first step for its proper management and the identification of its embryological subtype: “extratruncular” and “truncular” is warranted for every VM lesion because “extratruncular” lesions possess the evolutional potential to grow when stimulated!

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Lee AB, Laredo J, Neville R. Embryological background of truncular venous malformation in the extracranial venous pathways as the cause of chronic cerebro spinal venous insufficiency. Int Angiol 2010;29:95-108.  Back to cited text no. 1
    
2.
Lee BB, Baumgartner I, Berlien HP, Bianchini G, Burrows P, Do YS, et al. Consensus Document of the International Union of Angiology (IUA)-2013. Current concept on the management of arterio-venous management. Int Angiol 2013;32:9-36.  Back to cited text no. 2
    
3.
Lee BB. Venous malformation and haemangioma: Differential diagnosis, diagnosis, natural history and consequences. Phlebology 2013;28 Suppl 1:176-87.  Back to cited text no. 3
    
4.
Lee B, Laredo J. Venous malformation: Treatment needs a bird's-eye view. Phlebology 2013;28:62-3.  Back to cited text no. 4
    
5.
Lee BB, Baumgartner BB. Contemporary diagnosis of venous malformation. J Vasc Diagn 2013;1:25-34.  Back to cited text no. 5
    
6.
Lee BB, Baumgartner I, Berlien P, Bianchini G, Burrows P, Gloviczki P, et al. Diagnosis and treatment of venous malformations. Consensus document of the international union of phlebology (IUP): Updated 2013. Int Angiol 2015;34:97-149.  Back to cited text no. 6
    
7.
Lee BB, Antignani PL, Baraldini V, Baumgartner I, Berlien P, Blei F, et al. ISVI-IUA consensus document diagnostic guidelines of vascular anomalies: Vascular malformations and hemangiomas. Int Angiol 2015;34:333-74.  Back to cited text no. 7
    
8.
Lee BB. All congenital vascular malformations should belong to one of two types: “truncular” or “extratruncular“, as different as apples and oranges! Editorial. Phlebol Rev 2015;1:1-3.  Back to cited text no. 8
    




 

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