Rapidly involuting congenital hemangioma
   HOME

TheInfoList



OR:

A vascular tumor is a
tumor A neoplasm () is a type of abnormal and excessive growth of tissue. The process that occurs to form or produce a neoplasm is called neoplasia. The growth of a neoplasm is uncoordinated with that of the normal surrounding tissue, and persists ...
of vascular origin; a soft tissue growth that can be either
benign Malignancy () is the tendency of a medical condition to become progressively worse. Malignancy is most familiar as a characterization of cancer. A ''malignant'' tumor contrasts with a non-cancerous ''benign'' tumor in that a malignancy is not s ...
or
malignant Malignancy () is the tendency of a medical condition to become progressively worse. Malignancy is most familiar as a characterization of cancer. A ''malignant'' tumor contrasts with a non-cancerous ''benign'' tumor in that a malignancy is not s ...
, formed from
blood vessel The blood vessels are the components of the circulatory system that transport blood throughout the human body. These vessels transport blood cells, nutrients, and oxygen to the tissues of the body. They also take waste and carbon dioxide away ...
s or lymph vessels. Examples of vascular tumors include
hemangioma A hemangioma or haemangioma is a usually benign vascular tumor derived from blood vessel cell types. The most common form, seen in infants, is an infantile hemangioma, known colloquially as a "strawberry mark", most commonly presenting on the ski ...
s, lymphangiomas,
hemangioendothelioma Hemangioendotheliomas are a family of vascular neoplasms of intermediate malignancy. Signs and symptoms They have been described as masses that fall between a hemangioma and angiosarcoma. They are vascular tumors that commonly present with an en ...
s,
Kaposi's sarcoma Kaposi's sarcoma (KS) is a type of cancer that can form masses in the skin, in lymph nodes, in the mouth, or in other organs. The skin lesions are usually painless, purple and may be flat or raised. Lesions can occur singly, multiply in a limit ...
s,
angiosarcoma Angiosarcoma is a rare and aggressive cancer that starts in the endothelial cells that line the walls of blood vessels or lymphatic vessels. Since they are made from vascular lining, they can appear anywhere and at any age, but older people are ...
s, and
hemangioblastoma Hemangioblastomas, or haemangioblastomas, are vascular tumors of the central nervous system that originate from the vascular system, usually during middle age. Sometimes, these tumors occur in other sites such as the spinal cord and retina. They ma ...
s. An
angioma Angiomas are benign tumors derived from cells of the vascular or lymphatic vessel walls (endothelium) or derived from cells of the tissues surrounding these vessels. Angiomas are a frequent occurrence as patients age, but they might be an indicat ...
refers to any type of benign vascular tumor. Some vascular tumors can be associated with serious blood-clotting disorders, making correct diagnosis critical. A vascular tumor may be described in terms of being ''highly vascularized'', or ''poorly vascularized'', referring to the degree of blood supply to the tumor.


Classification

Vascular tumors make up one of the classifications of
vascular anomalies A vascular anomaly is any of a range of lesions from a simple birthmark to a large tumor that may be disfiguring. They are caused by a disorder of the vascular system. A vascular anomaly is a localized defect in blood or lymph vessels. These defects ...
. The other grouping is vascular malformations. Vascular tumors can be further subclassified as being benign, borderline or aggressive, and malignant. Vascular tumors are described as ''proliferative'', and vascular malformations as ''nonproliferative''.


Types

A vascular tumor typically grows quickly by the proliferation of endothelial cells. Most are not
birth defect A birth defect, also known as a congenital disorder, is an abnormal condition that is present at birth regardless of its cause. Birth defects may result in disabilities that may be physical, intellectual, or developmental. The disabilities ca ...
s.


Benign

The most common type of benign vascular tumors are
hemangioma A hemangioma or haemangioma is a usually benign vascular tumor derived from blood vessel cell types. The most common form, seen in infants, is an infantile hemangioma, known colloquially as a "strawberry mark", most commonly presenting on the ski ...
s, most commonly infantile hemangiomas, and less commonly congenital hemangiomas.


Infantile hemangioma

Infantile hemangioma An infantile hemangioma (IH), sometimes called a strawberry mark due to appearance, is a type of benign vascular tumor or anomaly that affects babies. Other names include capillary hemangioma, strawberry hemangioma, strawberry birthmark and str ...
s are the most common type of vascular tumor to affect babies, accounting for 90% of hemangiomas. They are characterised by the abnormal proliferation of endothelial cells and of deviant blood vessel formation or architecture. Hypoxic stress seems to be a major trigger for this. Infantile hemangiomas are easily diagnosed, and little if any aggressive treatment is needed. They are characterised by rapid growth in the first few months, followed by spontaneous regression in early childhood.


Congenital hemangioma

Congenital hemangiomas are present and fully formed at birth, and only account for 2% of the hemangiomas. They do not have the postnatal phase of proliferation common to infantile hemangiomas. There are two main variants of congenital hemangioma: non-involuting, and rapidly involuting (beginning in the first year of life). A third variant is also recognised as ''partially involuting''. Congenital hemangiomas can also be distinguished from infantile hemangiomas in that neither variant of congenital hemangioma expresses the glucose transporter
GLUT 1 Glucose transporter 1 (or GLUT1), also known as solute carrier family 2, facilitated glucose transporter member 1 (SLC2A1), is a uniporter protein that in humans is encoded by the ''SLC2A1'' gene. GLUT1 facilitates the transport of glucose acros ...
. Some cases have been associated with a mild form of
thrombocytopenia Thrombocytopenia is a condition characterized by abnormally low levels of platelets, also known as thrombocytes, in the blood. It is the most common coagulation disorder among intensive care patients and is seen in a fifth of medical patients a ...
. Rare cases have been associated with heart failure.


Hemangioblastoma

Hemangioblastoma Hemangioblastomas, or haemangioblastomas, are vascular tumors of the central nervous system that originate from the vascular system, usually during middle age. Sometimes, these tumors occur in other sites such as the spinal cord and retina. They ma ...
s are vascular tumors of the
central nervous system The central nervous system (CNS) is the part of the nervous system consisting primarily of the brain and spinal cord. The CNS is so named because the brain integrates the received information and coordinates and influences the activity of all p ...
.


Pyogenic granuloma

A range of benign vascular tumors are described as ''reactive proliferative lesions'' that grow in response to a stimulus, such as trauma, or a local
thrombosis Thrombosis (from Ancient Greek "clotting") is the formation of a blood clot inside a blood vessel, obstructing the flow of blood through the circulatory system. When a blood vessel (a vein or an artery) is injured, the body uses platelets (t ...
. They can also form infrequently during
pregnancy Pregnancy is the time during which one or more offspring develops (gestation, gestates) inside a woman, woman's uterus (womb). A multiple birth, multiple pregnancy involves more than one offspring, such as with twins. Pregnancy usually occur ...
as a hormonal reaction affecting the
gums The gums or gingiva (plural: ''gingivae'') consist of the mucosal tissue that lies over the mandible and maxilla inside the mouth. Gum health and disease can have an effect on general health. Structure The gums are part of the soft tissue lin ...
. The most common type of reactive proliferative tumors are
pyogenic granuloma A pyogenic granuloma or lobular capillary hemangioma is a vascular tumor that occurs on both mucosa and skin, and appears as an overgrowth of tissue due to irritation, physical trauma, or hormonal factors. It is often found to involve the gums ...
s also known as ''lobular capillary hemangiomas'', that are more often found in children and young adults. These granulomas are well defined growths of less than a centimetre across. They are bright red due to being highly vascularised, and bleed and ulcerate easily. Their colouring fades with age.


Tufted angioma

Tufted angioma A tufted angioma (also known as an "Acquired tufted angioma," "Angioblastoma," "Angioblastoma of Nakagawa," "Hypertrophic hemangioma," "Progressive capillary hemangioma," and "Tufted hemangioma") usually develops in infancy or early childhood on th ...
s are hereditary hemangiomas found in infants from birth to five years of age, however they may occur in adults. They are found on the neck, shoulders, and trunk as rounded nodules. Tufted angiomas are usually poorly defined lesions of purple colouration. The tumors are of tufts of capillary-sized vessels in lobules that are scattered in the skin, and that sometimes reach into the subcutaneous tissue, and have lymph vessels on the periphery. Their growth is slow to begin with, and progresses to a stable size. They show a high rate of spontaneous regression, particularly in congenital and early-onset cases. They typically have a deep nodular component sometimes extending into the subcutaneous tissue, fascia, and muscle, and can sometimes be painful. Tufted angiomas are associated with arteriovenous malformations. The origin of tufted angiomas is not clear but markers on the cells suggest a possible derivation from the endothelial cells of lymph vessels. They are also associated with the local secretion of
growth factor A growth factor is a naturally occurring substance capable of stimulating cell proliferation, wound healing, and occasionally cellular differentiation. Usually it is a secreted protein or a steroid hormone. Growth factors are important for regul ...
s that affect angiogenesis and promote the development of vascular lobules.


Borderline

Kaposiform hemangioendotheliomas (KHEs) are borderline, locally destructive vascular tumors. They are named after their resemblance to the lesions of
Kaposi's sarcoma Kaposi's sarcoma (KS) is a type of cancer that can form masses in the skin, in lymph nodes, in the mouth, or in other organs. The skin lesions are usually painless, purple and may be flat or raised. Lesions can occur singly, multiply in a limit ...
. KHEs are described as locally destructive because they can infiltrate underlying muscle and fat. They are often seen to overlap with tufted angiomas (TAs) but TAs may be a milder, benign counterpart. KHEs show as a red or purple expanding mass of soft tissue, found mostly in infants. Under the microscope KHE is characterised by nodules of tumor-like spindled endothelial cells. Unlike infantile hemangiomas, KHEs have a high mortality rate. Both KHEs and TAs are unique in that they carry the risk of the development of
Kasabach–Merritt syndrome Kasabach–Merritt syndrome, also known as hemangioma with thrombocytopenia, is a rare disease, usually of infants, in which a vascular tumor leads to decreased platelet counts and sometimes other bleeding problems, which can be life-threatening. ...
.


Malignant

Malignant vascular tumors are rare, and include
angiosarcoma Angiosarcoma is a rare and aggressive cancer that starts in the endothelial cells that line the walls of blood vessels or lymphatic vessels. Since they are made from vascular lining, they can appear anywhere and at any age, but older people are ...
s, and epithelioid hemangioendotheliomas. Other types are hemangiopericytomas, and
lymphangiosarcoma Lymphangiosarcoma is a rare cancer which occurs in long-standing cases of primary or secondary lymphedema. It involves either the upper or lower lymphedematous extremities but is most common in upper extremities. Although its name implies lymphat ...
s.


References

{{Vascular tumors Neoplasms Anatomical pathology