Hemangioma and Lymphangioma

Hemangioma and lymphangioma

Hemangioma

The term "hemangioma" has commonly been used as a generic term for both neoplastic and hamartomatous lesions. In this text, we will divide these vascular lesions into two main groups: congenital hemangiomas and vascular malformations.

Congenital hemangiomas are vascular neoplasms characterized by the proliferation of endothelial cells. These lesions occur at birth and may regress with age, disappearing around early childhood. Vascular malformations represent lesions related to the morphology of blood vessels, meaning they are vessels that have an altered structure and often exhibit dilation. These lesions are persistent and accompany the individual's growth, with no regression as seen in congenital hemangiomas.

Vascular lesions can be classified according to the type of vessel (arterial, venous, or mixed) and according to blood flow (low or high flow). The vast majority of oral "hemangiomas" are low-flow lesions. Overall, these lesions are more prevalent in females and occur more commonly in white patients.

Strawberry Mark - Hemangioma

https://drnumb.com/blog/know-about-strawberry-marks-birthmarks

Clinical Features

Congenital hemangiomas appear at birth, usually as single lesions, and initially present as flat telangiectatic lesions. During the first few weeks of life, hemangiomas tend to develop quite rapidly, and then go through a period of remission until complete disappearance of the lesion, which usually occurs around 5 (50%) or 9 (90%) years of age. Few patients are left with any remnants of the lesion. This lesion is also called a "strawberry hemangioma" because, on the skin, it exhibits a similar color. The lesions have a smooth or irregular surface, fibroelastic consistency, and are usually painless. Hemangiomas located deeper in the tissues may exhibit a purplish color. Ulcerations, secondary infections, and hemorrhages are possible complications associated with this pathology.

Patients who present with multiple segmented hemangiomas on the face or intraorally should be evaluated for PHACE syndrome, which is characterized by the following symptoms: Posterior fossa malformations (P), hemangioma (H), arterial anomalies (A), cardiac defects (C), and eye abnormalities (E).

Vascular malformations, unlike the previous lesions, persist throughout the patient's life, present similar clinical characteristics, can be flat or nodular lesions, and are more common in the facial region. "Port wine stains" or "nevus flammeus" are lesions that often affect the face (usually unilateral, following the regions of the trigeminal nerve). This macule is an important sign for the diagnosis of Sturge-Weber syndrome. Mental retardation and the presence of intracranial calcifications are important characteristics.

Intraosseous lesions are rare and usually represent vascular malformations, and can produce painful symptoms, volume increase, spontaneous bleeding, and dental mobility, although most of these lesions are asymptomatic. The mandible is commonly more affected than the maxilla.

Radiographically, "intraosseous hemangiomas" are characterized by a radiolucent image, usually multilocular, which can resemble the radiographic features of ameloblastoma ("honeycomb" and/or "soap bubble") or odontogenic myxoma. Some lesions may present as unilocular images or exhibit a "sunburst" appearance, which is commonly seen in osteosarcoma.

In Sturge-Weber syndrome, the presence of "tram-track" calcifications is quite common in skull radiographs.

Other lesions such as aneurysmal bone cyst and fibrous dysplasia are mentioned in the literature as important differential diagnoses.

Capillary Hemangioma

https://www.doccheck.com/en/detail/photos/5993-capillary-hemangioma-of-the-oral-mucous-membrane-structure

Oral Hemangioma on Tongue

http://www.ghorayeb.com/TongueHemangioma.htm

Stuge-Weber Syndrome - Port-Wine Stain

https://aapos.org/glossary/sturge-weber-syndrome

Sunburst Appearance 

http://www.amhsjournal.org/article.asp?issn=2321-4848;year=2014;volume=2;issue=1;spage=67;epage=70;aulast=Balan

Histopathological Characteristics

Microscopically, congenital hemangiomas are characterized by the presence of a proliferation of endothelial cells, which in the initial phase is called "juvenile or capillary hemangioma", after this phase, the lesion cells tend to form evident capillary vessels. As already mentioned, these lesions usually regress, and histopathologically, the vascular tissue is replaced by fibrous tissue.

Vascular malformations present tissue with less proliferation of endothelial cells and we can observe dilated and tortuous vascular spaces. The vessels can be of small caliber (very similar to capillary hemangioma) or large caliber.

"Port wine stains" or "flame nevi" present many dilated blood vessels located in the connective tissue of the skin. Hereditary hemorrhagic telangiectasia (or Osler-Weber-Rendu syndrome) presents vascular lesions in the mucosa and skin as the main characteristic. Histopathologically, these lesions are similar to congenital hemangiomas and vascular malformations mentioned here.

Capillary hemangioma - observe the presence of multiple blood vessels filled with red blood cells.

http://en.wikipedia.org/wiki/Hemangioma

Cavernous hemangioma - although the image is not of an oral lesion, it represents well the microscopic characteristics of the pathology.

Diagnosis and Treatment

These lesions rarely cause complications for the individual, have low morbidity, and usually do not affect the patient's quality of life. Diagnosis is made based on clinical examination. In intraosseous lesions, surgical maneuvers such as aspiration biopsy and incisional biopsy can help in differential diagnosis.

Congenital hemangiomas often do not require treatment, as most of these lesions regress. Sclerotherapy followed by surgical excision is the treatment of choice for vascular malformations. Sclerosing agents such as propranolol and monoethanolamine oleate act by inducing necrosis of the blood vessels in the lesion and subsequent fibrosis, reducing or eliminating a large part of the lesion.

For lesions composed of large blood vessels, embolization is the most recommended technique, especially when the pathology affects vital regions. The risk of complications such as bleeding is higher in intraosseous lesions, so whenever possible, aspiration of the lesion should be performed before any procedure when there is a radiolucent image suggestive of a vascular lesion. There is no specific treatment for Sturge-Weber syndrome, but facial lesions can be alleviated with laser therapy.

Lymphangioma

Lymphangiomas are hamartomatous lesions that usually occur at birth. It is believed that the lesion develops from "ectopic" lymphatic tissue, which means it is not related to larger lymphatic channels. These lesions have a high prevalence in the head and neck region, accounting for about 75% of cases. As mentioned earlier, the lesions typically begin at birth and develop around two years of age, and males are more commonly affected.

Clinical Features

Lymphangiomas can be divided into three groups:

• Capillary Lymphangioma - When it is formed by small lymphatic vessels, it is the most common in the oral cavity.
• Cavernous Lymphangioma - They are formed by larger vessels and are lesions that present greater morbidity.
• Cystic Hygroma (cervical lymphangioma) - They are cavernous lymphangiomas that occur more commonly in the cervical region, although other locations, such as the armpits, can be affected.

Cervical lymphangiomas present as soft, painless swellings that exhibit accelerated growth and can reach large dimensions. Complications such as intralesional bleeding, difficulty swallowing and breathing may be associated. When palpated, these lesions usually emit a characteristic crackling sound, which is the result of fluid (lymph) movement inside the lesion.

In the oral cavity, the most affected site is the tongue, and deeper and diffuse lymphangiomas often produce macroglossia or localized volume increases. Superficial lesions are characterized by the presence of multiple translucent or red-purple "vesicles" (when associated with hemangiomas) on the affected surface, producing an aspect that the literature calls "frog eggs" or "tapioca pudding."

Other conditions that cause macroglossia should always be included in the differential diagnosis. Among the main ones, we can highlight lesions such as amyloidosis, vascular malformations, and angioedema. Down syndrome and Beckwith-Wiedemann syndrome often present macroglossia as an important component of the symptomatology.

Clinical Aspect of Oral Lymphangioma

Lymphangioma definition, types, causes, diagnosis & lymphangioma treatment (healthjade.net)

Histopathological characteristics

Histologically, lymphangiomas exhibit an increase in lymphatic vascular spaces (dilation). These spaces are lined by a thin layer of endothelial cells and within them, a pale eosinophilic material can be observed. Communication between the dilated lymphatic vessels and the surface epithelium is an important factor for microscopic diagnosis.

Vascular malformations may exhibit similar characteristics, although communication between the lesion vessels and the surface tissue is not common.

https://www.dovemed.com/diseases-conditions/oral-lymphangioma/?trendmd-shared=0

Diagnosis and Treatment

Lymphangiomas are diagnosed through clinical examination, and the preferred treatment for superficial lesions is surgical excision. Deeper lesions can pose greater problems for the patient, as they often involve important structures, making complete removal of the lesion impossible and increasing the risk of recurrence.

Sclerosing agents such as propranolol and monoethanolamine oleate do not yield good results in treating lymphangiomas. OK-432 is a sclerosing agent composed of lyophilized strains of low-virulence streptococcus pyogenes and potassium penicillin G. It acts by inducing the migration of inflammatory cells into the lesion, damaging the endothelium of the vessels and increasing permeability, thereby reducing the size of the lesion. The use of this agent has achieved better results compared to other sclerosing agents.