2. Cavernous hemangioma
B. Stage 2 (retiform plexus)
1. Diffuse microfistula
2. Localized macrofistula
C. Stage 3 (mature vascular malformation)
1. Venous hemangioma
2. Venous hypoplasia (Klippel-Trénaunay syndrome)
3. Hemangiolymphangioma (vascular hamartoma)
Spira (1983)
A. Benign hemangiomas
1. Typical
a. Capillary hemangioma
b. Cavernous hemangioma
c. Mixed-combined hemangioma
d. Port-wine stain – nevus flammeus
e. Angioma racemosum
f. Angiokeratoma (Mibelli)
2. Atypical
a. Sclerosing hemangioma
b. Pyogenic granuloma
c. Spider telangiectasia (nevus araneus)
d. Glomus tumor
e. Hemangiopericytoma
f. Juvenile nasopharyngeal angiofibroma
g. Venous lakes
B. Syndromes – diseases
1. Rendu-Osler-Weber syndrome
2. Sturge-Weber-Dimitri syndrome
3. von Hippel-Lindau disease
4. Maffucci syndrome
5. Blue Rubber Bleb syndrome
6. Kasabach-Merritt syndrome
7. Klippel-Trenaunay syndrome
C. Malignant hemangiomas
1. Angiosarcoma
2. Kaposi sarcoma
3. Dermatofibrosarcoma protuberans
Classification of Neuroradiologists Merland et al. (1983)
1. Pure arterial dysplasia (2 cases)
2. A-V dysplasia (macroscopic shunt)
a. Simple direct A-V fistulavertebro-vertebral, vertebro-jugularcarotido-cavernous, carotido-jugular
b. A-V malformation (60 cases)
3. Capillary and capillary-venous malformation (26 cases)
a. Pure capillary (Rendu-Osler)
b. Capillary-venous malformation
4. Venous and cavernous ectasias (100 + 4 cases)
5. Additional types
a. Unmature angioma of the newborn
b. Portwine stain angioma
c. Unusual angiomas
Hemodynamic Classification
1. Active (large blood flow, direct A-V fistula) high flow
2. Inactive vascular
Cushing and Bailey (1928) were the first to separate two groups:
I. Angioblastoma (true neoplasm),
II. Angiomatous malformation.
They did not consider cavernous angioma as a separate entity and listed it under angioblastoma. Their venous angiomas would be called AVMs today.
Bergstrand et al. (1936) added to the neoplastic group the angioglioma of Roussy and Oberling. These are to a large degree still not accepted, yet appear to have been occasionally identified (Bonnin et al. 1983). Bergstrand doubted the existence of a true arterial aneurysm as described and illustrated by Simmonds (1905) (Figs 3.5, 3.6).
Fig 3.6 Coloured artistic drawing of Simmonds (1905) unique case of a pure arterial malformation in a 53 year old man who died of an acute intracerebral hematoma. At autopsy a medium-sized arterial convolution was found. It was histologically verified to be purely arterial.
Historical review shows that 4 or 5 different vascular diseases (angioblastoma, telangiectasia, venous, cavernous and arteriovenous angiomas) were recognized in the very earliest studies and then gradually identified as being separate entities.
McCormick’s classification (Table 3.6b) is similar to earlier works and he no longer appears to regard “varix” as a special entity. His contribution was important in that he provided statistics regarding the numbers of different types of malformation and reopened the discussion on the relationship of transitional types of angiomas to vascular tumors as originally mentioned by Virchow.
Huang et al. (1984) noted the wide acceptance of Russel and Rubinstein’s (1963) classification. However, they pointed out the disadvantages of attempting to differentiate histologically between many cavernous venous malformations and venous angiomas and showed that some areas within venous angiomas may be similar to capillary malformations or even an AVM. The classification of Huang et al. has put forward new and important elements for consideration.
We include the classification of Merland et al. (1983) as a very stimulating view of the external angiomas, seen from the perspective of the interventional neuroradiologist.
The classification of Kaplan (1983), Spira (1983) show up the similar problems experienced by plastic surgeons in describing cutaneous malformations.
The Author’s Classification
Our own classification is based on the relative preponderance and contribution of the various vascular elements, arteries, veins, capillaries, and abnormal channels (Table 3.6c). There may run a spectrum from theoretically completely arterial lesions to completely venous lesions and from large fistulae to extensive convoluted vessels. While the lesions can conveniently be grouped into four primary headings, there are at each level examples of transitional lesions, e. g. AVMs with slow flow or venous malformations with increased flow. Part of the definition of the lesion must rest with dynamic properties related to flow and shunting, which cannot be examined by the pathologist in the resected specimen or at autopsy. We propose the following classification more for practical use in neuroradiology, neurology and neurosurgery but hope that neuropathologists will be stimulated to undertake further investigation of these lesions.
Table 3.6c Authors classification
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I. Vascular neoplasms
1. Hemangioblastoma
a. Cystic
b. Solid
2. Angioglioma (mixed hemangioblastoma and glioma)
3. Angioblastic meningioma
4. Hemangiopericytic meningioma (hemangiopericytoma of the meninges)
5. Angiosarcoma
II. Malformations
1. Telangiectasia
2. Cavernous malformation
a. Intrinsic
b. Extrinsic
3. Venous malformation
a. Cortical
b. Subcortical (medullary)
α superficial
β deep
4. Arteriovenous malformation
a. Plexiform (dilated, tortuous pathological vessels with thickened or thinned (or combined) walls, arteriectasia, aneurysms, phlebectasia, varices; they can be cryptic, occult, micro, moderate, large or giant in size.They may be uni- or multilocular.They may have a mono-nidus with mono- or multi-compartments)
b. A-V Fistula (direct communication between arteries and venous channels (veins and sinuses) without the interposition of a convolue.
α simple: – carotid = cavernous, carotid = jugular,
– MCA = v. Labbé or v. Trolard or Sylvii,
– ACA = inferior sagittal sinus,
– pericallosal artery = v. Galen,
– PCA
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