Hemangioma
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Diffuse, multinodular cavernous hemangioma in Sturge-Weber syndrome.

 

 

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Introduction

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The most likely vasoformative tumor to be submitted for biopsy from the oral/pharyngeal region is the hemangioma. Hemangioma of the head and neck region is relatively common, representing at least a third of all hemangiomas in humans. Oral hemangioma represents 14% of all human hemangiomas and is found in 5.5 of every 1,000 U.S. adults (Table 1). It was first reported in 1841 as bluish excrescence and erectile tissue.

Composed of a proliferation or excess of vascular channels, it is usually present at birth but may arise in young adults or older individuals. Most lesions appear to be developmental anomalies or hamartomas, seen in 2% of newborns (all anatomic sites). Some result from abnormal vessel proliferation after trauma, and a few appear to be true benign neoplasms. In this light, it should be mentioned that some authorities reserve the term hemangioma exclusively for the neoplastic variant.

Many maxillofacial entities of a nonvascular nature have vascular subtypes, such as angioleiomyoma and angiolipoma. Likewise, a paraoral hemangioma may rarely be associated with an overlying reactive hyperkeratosis and papillomatosis (verrucous hemangioma). The cherry angioma (senile angioma, De Morgan's spots) so common to the skin of older adults is not seen on the mucosa of the mouth and throat.

The oral or pharyngeal hemangioma has an older age at diagnosis than lesions from other sites. In adults, the mucosal hemangioma most often arises from the frequently traumatized mucosal sites: the lip mucosa (63% of oral cases), the buccal mucosa (14% of cases) and the lateral borders of the tongue (14% of cases), but it may occur at any oral or pharyngeal location. In population studies there is a strong (2:1) male predilection, although there is minimal gender predilection in hospital-based studies. Congenital and neonatal lesions do occur, especially in the lips and parotid glands.

The mucosal hemangioma is typically a soft, moderately well circumscribed, painless mass which is red or blue in coloration (Figures 1 & 2). The more superficial ones are often lobulated and will blanch under finger pressure. Deeper lesions tend to be dome-shaped with normal or blue surface coloration; they seldom blanch. A lesion with a thrill or bruit, or with an obviously warmer surface, is most likely a special vascular malformation, called arteriovenous hemangioma (arteriovenous aneurysm, A-V shunt, arteriovenous malformation), with direct flow of blood from the venous to the arterial system, bypassing the capillary beds.

The lesion is usually less than 2 cm. in greatest dimension, but may be so extensive as to encompass much of the oral/pharyngeal tissues. Congenital lesions tend to keep pace with body growth, while adult-onset lesions tend to slowly enlarge over a period of months or years. Extension into underlying muscle or pressure atrophy of underlying bone may occur and, of course, the hemangioma may be part of a syndrome (Table 2).

The hemangioma is characterized by an excess of blood vessels, usually veins and capillaries, in a focal area of submucosal connective tissue. It is almost never encapsulated. Lesions are subdivided into several categories. Capillary hemangioma is the most common type and is comprised of numerous intertwining capillary-sized vessels lined by endothelium with relatively flat or plump nuclei, depending on the duration of the lesion (Figures 3 & 4). Those with plump endothelial nuclei are younger and often demonstrate mitotic activity, a feature not present in older lesions.

While lacking a capsule, the capillary hemangioma is often well circumscribed and there is typically a central feeder vessel with radiating, lobular extensions or vascular proliferations, leading some to prefer the diagnosis of lobular hemangioma (Figure 5). Lobular vascular architecture is used to confirm the benign nature of such lesions and is characteristic of several vascular subtypes, such as cellular hemangioma of infancy (strawberry nevus, benign hemangioendothelioma of infancy, juvenile hemangioma) and epithelioid hemangioma. The relationship between the lobular capillary hemangioma and the pyogenic granuloma is somewhat unclear at this time.

The lumina in capillary hemangioma are typically small, perhaps to the point of masking the vascular nature of the lesion. Reticulin stains will more easily demonstrate the vessels and factor XIII-positive interstitial cells are consistently seen in this lesion.

Endothelial proliferation which takes place completely or almost completely within a venous lumen, typically with tufts and papillary projections, may also be seen. This is usually termed papillary endothelial hyperplasia (intravascular hemangioendothelioma, intravascular angiomatosis) and is presumed to arise from abnormal organization of a thrombus.

The epithelioid hemangioma deserves special mention. Often called angiolymphoid hyperplasia with eosinophilia, histiocytoid hemangioma or, mistakenly, Kimura's disease, this superficial, often multifocal lesion has a strong predilection for the head and neck region and may be associated with an unidentified infectious agent. The vessels in this lesion are lined by epithelioid or histiocytoid endothelial cells which extend considerably into the lumen, imparting a "tombstone" effect to the vessel walls. The lesional cells have rounded nuclei and abundant eosinophilic cytoplasm with occasional vacuoles. Electron microscopy will reveal excess mitochondria, endoplasmic reticulum and cytofilaments in the cytoplasm. Another epithelioid change is seen: there are gaps between endothelial cells, with desmosome-like attachments between them.

A mixed chronic inflammatory cell infiltrate, including eosinophils and occasional germinal centers, is seen to surround the lesional vessels of epithelioid hemangioma. It should be mentioned that Kimura's disease is a chronic inflammatory condition endemic to Asian populations and presents with lesions bearing superficial resemblance to epithelioid hemangioma. This disease presents with peripheral eosinophilia, increased serum IgE, proteinuria, and nephrotic syndrome, and has a strong predilection for males. Submucosal or subcutaneous lesions are characterized by abundant, aggregated lymphoid tissue with prominent germinal centers, but the associated vessels are thin walled and the number of eosinophils is often much more marked ("eosinophilic abscess") than in epithelioid hemangioma.

Intramuscular hemangioma shows capillaries in a loose fibrous stroma interspersed between striated muscle bundles in a pseudo-infiltrative fashion that may mimic malignancy. Vessel lumina are usually well developed in this tumor, but occasional cases show a more solidly cellular appearance. Mitotic activity is usually not pronounced and intraluminal tufts of endothelial cells may be seen to project into vessel lumina. Capillaries may be seen to proliferate within perineural sheaths.

When lesional vascular channels are considerably enlarged, the term cavernous hemangioma has traditionally been applied (Figure 6). This differs from capillary hemangioma in that it is less well circumscribed, is larger and is usually deeper in submucosal tissues. Sluggish blood flow may result in organized or dystrophically calcified thrombi within dilated vessels. The vessels may be arranged in a haphazard or a somewhat lobular pattern and there may be areas with fibrosis of the background stroma. Occasional vascular lesions, in fact, are dominated so much by a dense fibrous stroma that they are called sclerosing hemangioma. Chronic inflammatory cells may be scattered in multiple foci.

Some examples of hemangioma have minimal stroma and demonstrate excess anastomosis of adjacent cavernous channels, often with a papillary infolding of the endothelium at points of contact between vessels. The term sinusoidal hemangioma has been used for such lesions. The arteriovenous hemangioma, on the other hand, presents as a nonencapsulated aggregation of intertwining, tortuous medium-sized or larger arteries and veins in the submucosal tissues. This may show excessive or dilated lymphatic channels in addition to the blood vessels.

The hemangioma is usually treated by conservative surgical excision, but it is known to respond well to:

• intralesional injections of sclerosing chemicals, such as sodium morrhuate
• implanted or external irradiation
• cryosurgery
• laser ablation
• strangulation of the feeder vessel
• intralesional injections of corticosteroid

The lack of encapsulation and the infiltrating nature of the lesional border, especially in intramuscular hemangioma, often forces the surgeon to perform a simple debulking procedure, with remnants of tumor deliberately left behind in order to preserve the maximum amount of surrounding normal tissues. Recurrence is not unusual unless the tumor is completely excised. Epithelioid hemangioma responds to low-dosage radiotherapy, but not to cryotherapy or intralesional steroids.

Most congenital capillary hemangiomas will spontaneously regress or disappear by the fifth or sixth years of life, but cavernous types tend less to do this, as do those associated with Sturge-Weber syndrome. When hemangioma admixes with lymphatic proliferations, i.e. hemangiolymphangioma, the lesion tends to behave more like a lymphangioma than a hemangioma.

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