Hamartoma

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It is a local malformation of cells that resembles a neoplasm of local tissue.

It is usually due to an overgrowth of multiple ab2242ant cells, with a basis in a systemic genetic condition, rather than a growth descended from a single mutated cell as would typically define a benign neoplasm/tumor.

A hamartoma is a mostly benign.

However, many hamartomas are found to have clonal chromosomal ab2242ations that are acquired through somatic mutations, and on this basis the term hamartoma is sometimes considered synonymous with neoplasm.

Hamartomas are by definition benign, slow-growing or self-limiting.

Its underlying condition may still predispose the individual towards malignancies.

Usually caused by a genetic syndrome that affects the development cycle of all or at least multiple cells.

Hamartomas occur in many different parts of the body and are most often asymptomatic incidentalomas.

The definition of hamartoma versus benign neoplasm is often unclear, since both lesions can be clonal.

Adenomas, developmental cysts, hemangiomas, lymphangiomas and rhabdomyomas within the kidneys, lungs or pancreas are interpreted by some experts as hamartomas while others consider them true neoplasms.

Hamartomas show a benign histology.

Some rare but life-threatening complications occur with neurofibromatosis type I and tuberous sclerosis.

Hamartoma is an excess of normal tissue in a normal situation.

They result from an abnormal formation of normal tissue, and grow along with, and at the same rate as, the organ from whose tissue they are made, and, unlike cancerous tumors, only rarely invade or compress surrounding structures significantly.

Hamartoma are the most common benign tumors of the lung.

The majority of lung tumors are found periphery, with an endobronchial location reported in 1.4-10% of cases.

The most common hamartomas occur in the lungs.

About 5–8% of all solitary lung nodules, about 75% of all benign lung tumors, are hamartomas.

Hamartomas almost always arise from connective tissue.

Hamartomas are generally formed of cartilage, connective tissue, and fat cells, although they may include many other types of cells.

The great majority of lung hamartomas form in the connective tissue on the outside of the lungs.

About 10% form deep in the linings of the bronchi.

It is sometimes difficult to make the important distinction between a hamartoma and a lung malignancy.

X-rays will often not provide a definitive diagnosis

CT scan may be insufficient for diagnosis if the hamartoma lacks the cartilage and fat tissue.

Lung hamartomas may have suggestive popcorn-like calcifications on chest xray or computed tomography (CT scan).

Lung hamartomas are more common in men than in women.

Lung hamartomas, if treated, are
surgically resection, with an excellent prognosis.

Cardiac rhabdomyomas are hamartomas
that are the most common tumor of the heart in children and infants

Cardiac rhabdomyomas are composed of altered cardiac myocytes that contain large vacuoles and glycogen.

A strong relationship exists between cardiac rhabdomyomas and tuberous sclerosis.

25-50% of patients with cardiac rhabdomyomas will have tuberous sclerosis, and up to 100% of patients with tuberous sclerosis will have cardiac masses by echocardiography.

Symptoms of cardiac hamartomas depend on the size of the tumor, its location relative to the conduction system, and whether or not it obstructs cardiac blood flow.

A hypothalamic hamartoma is symptomatic, and most often causes
seizures, and can cause visual problems, rage disorders associated with hypothalamic diseases, and early onset of puberty.

Hamartomas may impinge into blood vessels, resulting in a risk of serious bleeding.

It typically lacks elastic tissue, and may lead to the formation of aneurysms and thus possible hemorrhage.

Angiomyolipoma of the kidney was previously considered to be a hamartoma.or choristoma.

A pancreatic rest, is ectopic pancreatic tissue found in the stomach, duodenum, or proximal jejunum.

Hamartomas of the spleen are associated with abdominal pain in 50% of such cases and they are often associated with hematologic abnormalities and spontaneous rupture.

Cowden syndrome is a genetic disorder characterized by multiple hamartomas.

In Cowden syndrome skin hamartomas exist, and in about 66% of cases hamartoma of the thyroid gland exists.

In Cowden syndrome additional growths can form in bones, CNS, the eyes, the genitourinary tract, the GI tract, and mucosa.

They are generally benign, can cause problems due to their location, and can obstruct practically any organ in the body.

They can cause major health issues when located in the hypothalamus, kidneys, lips, or spleen.

After being removed surgically they are not likely to recur.

Prognosis will depend upon the location and size of the lesion, as well as the overall health of the patient.

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