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Encyclopedia > Hamartoma
Hamartoma
Classifications and external resources
ICD-10 Q85.9
ICD-9 757.32, 759.6

A hamartoma is a common benign tumor in an organ composed of tissue elements normally found at that site but that are growing in a disorganized mass. They occur in many different parts of the body and are most often asymptomatic and undetected unless seen on an image taken for another reason. Hamartomas result from an abnormal formation of normal tissue, although the underlying reasons for the abnormality are not fully understood. They 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. The International Statistical Classification of Diseases and Related Health Problems (commonly known by the abbreviation ICD) is a detailed description of known diseases and injuries. ... The following codes are used with International Statistical Classification of Diseases and Related Health Problems. ... The International Statistical Classification of Diseases and Related Health Problems (commonly known by the abbreviation ICD) is a detailed description of known diseases and injuries. ... The following is a list of codes for International Statistical Classification of Diseases and Related Health Problems. ... Tumor (American English) or tumour (British English) originally means swelling, and is sometimes still used with that meaning. ...


A hamartoma, while generally benign, can cause problems due to their location. When located on the skin, especially the face or neck, they can be extremely disfiguring, as in the case of a man with a hamartoma the size of a small orange on his eyelid.[1] They may obstruct practically any organ in the body, such as the eye, the colon, etc. They are particularly likely to cause major health issues when located in the hypothalamus, spleen or kidneys.

Contents

Lung

The most common hamartomas occur in the lungs. About 5-8% of all solitary lung tumors, about 75% of all benign lung tumors are hamartomas. They almost always arise from connective tissue and are generally formed of cartilage, fat, and connective tissue cells, although they may include many other types of cells. The great majority of them form in the connective tissue on the outside of the lungs, although about 10% form deep in the linings of the bronchii. They can be worrisome, especially if situated deep in the lung, as it is important and sometimes difficult to distinguish them from malignancies. An x-ray will often not provide definitive diagnosis, and even a CAT scan may be insufficient if the hamartoma atypically lacks cartilage and fat cells. Lung hamartomas are more common in men than in women and may present additional difficulties in smokers. Benign can refer to any medical condition which, untreated or with symptomatic therapy, will not become life-threatening. ...


Some lung hamartomas can compress surrounding lung tissue to a degree, but this is generally not debilitative or even noticed by the patient, especially for the more common peripheral growths. They are treated, if at all, by surgical resection, with an excellent prognosis: generally, the only real danger is the inherent possibility of surgical complications.


Heart

Cardiac rhabdomyomas are hamartomas comprised of altered cardiac myocytes that contain large vacuoles and glycogen. They are the second most common tumor of the heart in children and infants (after fibromas). There is a strong association between cardiac rhabdomyomas and tuberous sclerosis (characterized by hamartomas of the central nervous system, kidneys and skin, as well as pancreatic cysts); 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 depend on the size of the tumor, its location relative to the conduction system, and whether it obstructs blood flow. Symptoms are usually from congestive heart failure (in utero heart failure may occur). If patients survive infancy, their tumors regress spontaneously; resection in symptomatic patients has good results.


Hypothalamus

One of the most troublesome hamartomas occurs on the hypthalamus. Unlike most such growths, a hypothalamic hamartoma is symptomatic; it most often causes gelastic seizures, and can cause visual problems, other seizures, rage disorders associated with hypothalamic diseases, and early onset of puberty. The symptoms typically begin in early infancy and are progressive, often into general cognitive and/or fuctional disability. Moreover, resection is usually difficult, as the growths are generally adjacent to, or even intertwined with, the optic nerve; however, the symptoms are resistant to medical control. Luckily, surgical techniques are improving and can result in immense improvement of prognosis. [2] In the anatomy of mammals, the hypothalamus is a region of the brain located below the thalamus, forming the major portion of the ventral region of the diencephalon and functioning to regulate certain metabolic processes and other autonomic activities. ... Hypothalamic hamartoma (abbreviated as HH) is a congenital condition consisting of a mass of disorganized neuronal or glial tissue on or near the hypothalamus. ... Gelastic is a term used to refer to a certain type of epilepsy or epileptic seizure. ...


Kidneys, spleen, and other vascular organs

Hamartoma of the spleen. The hamartoma is the dark circular object on the left that dominates the image. This is a cross-section, the growth being about 9cm in diameter, while the spleen is actually about 11cm.
Hamartoma of the spleen. The hamartoma is the dark circular object on the left that dominates the image. This is a cross-section, the growth being about 9cm in diameter, while the spleen is actually about 11cm.[3]

One general danger of hamartoma is that they may impinge into blood vessels, resulting in a risk of serious bleeding. Because hamartoma typically lacks elastic tissue, it may lead to the formation of aneurysms and thus possible hemorrhage. Where a hamartoma impinges into a major blood vessel, such as the renal artery, hemorrhage must be considered life-threatening. Image File history File links Spleen. ...


Hamartoma of the kidney is also called angiomyolipoma and can be associated with tuberous sclerosis. It is one of the more frequently seen hamartomas. The condition is more prevalent in women than men, and generally occurs in the right kidney. Hamartomas of the spleen are uncommon, but can be dangerous. About 50% of such cases manifest abdominal pain and they are often associated with hematologic abnormalities and spontaneous rupture. Angiomyolipoma is a benign renal lesion. ... Tuberous sclerosis, (meaning hard potatoes), is a rare genetic disorder primarily characterized by a triad of seizures, mental retardation, and skin lesions (called adenoma sebaceum). ...



Angiomyolipoma is not a hamartoma by definition, because fat and smooth muscles are not normal constituents of renal parenchyma. It is a Choristoma (microscopically normal cells or tissues in abnormal locations).


Cowden syndrome

Cowden Syndrome or Cowden Disease is a serious genetic disorder [4] characterized by multiple hamartomas. Usually skin hamartomas exist, and commonly (about 66% of cases) hamartoma of the thyroid gland exists. Additional growths can form in many parts of the body, especially in mucosa, the GI tract, bones, CNS, the eyes, and the genourinary tract. The hamartomas themselves may cause symptoms or even death, but morbidity is more often associated with increased occurrence of malignancies, usually in the breast or thyroid. Cowden syndrome is an inherited disorder characterized by multiple tumor-like growths called hamartomas, and an increased risk of certain cancers. ...


Notes

  1. ^ http://dermatlas.med.jhmi.edu/derm/IndexDisplay.cfm?ImageID=2040682463
  2. ^ http://www.emergemd.com/bniq2/article.asp?article_ref_id=20-1-1 Barrow Neurological Institute
  3. ^ Many thanks to Dr. Ed Uthman for the public domain photograph.
  4. ^ Mutation of PTEN gene on arm 10q or rarely germline mutation in BMPR1A

External links

  • eMedicine - "Lung Hamartoma"
  • eMedicine - "Angiomyolipoma"
  • humpath.com - "Hamartomas"
Tumors (and related structures), Cancer, and Oncology edit
Benign - Premalignant - Carcinoma in situ - Malignant

Topography: Anus - Bladder - Bone - Brain - Breast - Cervix - Colon/rectum - Duodenum - Endometrium - Esophagus - Eye - Gallbladder - Head/Neck - Liver - Larynx - Lung - Mouth - Pancreas - Penis - Prostate - Kidney - Ovaries - Skin - Stomach - Testicles - Thyroid Tumor (American English) or tumour (British English) originally means swelling, and is sometimes still used with that meaning. ... Cancer is a class of diseases or disorders characterized by uncontrolled division of cells and the ability of these cells to invade other tissues, either by direct growth into adjacent tissue through invasion or by implantation into distant sites by metastasis. ... Oncology is the medical subspecialty dealing with the study and treatment of cancer. ... Benign can refer to any medical condition which, untreated or with symptomatic therapy, will not become life-threatening. ... A premalignant condition is a disease, syndrome, or finding that, if left untreated, may lead to cancer. ... Carcinoma in situ is present when a tumor has been detected that has the characteristics of malignancy but has not invaded other tissues. ... In medicine, malignant is a clinical term that is used to describe a clinical course that progresses rapidly to death. ... Anal cancer is a distinct entity from the more common colorectal cancer. ... Bladder cancer refers to any of several types of malignant growths of the urinary bladder. ... Bone tumor is an inexact term, which can be used for both benign and malignant abnormal growths found in bone, but is most commonly used for primary tumors of bone, such as osteosarcoma (or osteoma). ... A brain tumor is any intracranial tumor created by abnormal and uncontrolled cell division, normally either found in the brain itself (neurons, glial cells (astrocytes, oligodendrocytes, ependymal cells), lymphatic tissue, blood vessels), in the cranial nerves (myelin-producing Schwann cells), in the brain envelopes (meninges), skull, pituitary and pineal gland... Breast cancer is cancer of breast tissue. ... Cervical cancer is a malignancy of the cervix. ... Colorectal cancer, also called colon cancer or bowel cancer, includes cancerous growths in the colon, rectum and appendix. ... This article needs more context around or a better explanation of technical details to make it more accessible to general readers and technical readers outside the specialty, without removing technical details. ... Endometrial cancer involves cancerous growth of the endometrium (lining of the uterus). ... Esophageal cancer is malignancy of the esophagus. ... Ocular oncology is branch of medicine dealing with tumors relating to the eye and its adnexa. ... Bold textA more uncommon cancer predominate in females, if found early on before symptoms, can be cured by removing Gallbladder, most often it is found after symptoms occur (abdominal pain, Jaundice) and has spread to other organs such as liver and the outlook at this point is poor. ... Head and neck cancers are malignant growths originating in the lip and oral cavity (mouth), nasal cavity, pharynx, larynx, thyroid, paranasal sinuses, salivary glands and cervical lymph nodes of the neck. ... Hepatocellular carcinoma (HCC, also called hepatoma or liver cancer) is a primary malignancy (cancer) of the liver. ... Cancer of the larynx also may be called laryngeal cancer. ... Lung cancer is a cancer of the lungs characterized by the presence of malignant tumours. ... Oral cancer is any cancerous tissue growth located in the mouth. ... Pancreatic cancer (also called cancer of the pancreas) is a malignant tumour within the pancreatic gland. ... Penile cancer is a malignant growth found on the skin or in the tissues of the penis, usually originating in the glans and/or foreskin. ... Prostate cancer is a disease in which cancer develops in the prostate, a gland in the male reproductive system. ... Renal cell carcinoma, also known by a gurnistical tumor, is the most common form of kidney cancer arising from the renal tubule. ... Ovarian cancer is a malignant ovarian neoplasm (an abnormal growth located on the ovaries). ... Skin cancer is a malignant growth on the skin, which can have many causes, including repeated severe sunburn or long-term exposure to the sun. ... Stomach cancer (also called gastric cancer) can develop in any part of the stomach and may spread throughout the stomach and to other organs, particularly the esophagus, small intestine. ... Testicular cancer is a type of cancer that develops in the testicles, a part of the male reproductive system. ...


Morphology: Papilloma/carcinoma - Choriocarcinoma - Adenoma/adenocarcinoma - Soft tissue sarcoma - Melanoma - Fibroma/fibrosarcoma - Metastasis - Lipoma/liposarcoma - Leiomyoma/leiomyosarcoma - Rhabdomyoma/rhabdomyosarcoma - Mesothelioma - Angioma/angiosarcoma - Osteoma/osteosarcoma - Chondroma/chondrosarcoma - Glioma - Lymphoma/leukemia Papilloma refers to a benign epithelial tumor. ... In medicine, carcinoma is any cancer that arises from epithelial cells. ... Choriocarcinoma is a rare cancer of the placenta, curable by chemotherapy. ... Adenoma refers to a collection of growths (-oma) of glandular origin. ... In medicine, carcinoma is any cancer that arises from epithelial cells. ... Malignant (cancerous) tumors that develop in soft tissue are called sarcomas, a term that comes from a Greek word meaning fleshy growth. ... Melanoma is a malignant tumor of melanocytes and, less frequently, of retinal pigment epithelial cells (of the eye, see uveal melanoma). ... Soft Fibroma (fibroma molle). ... Fibrosarcoma (fibroblastic sarcoma) is a malignant tumor derived from fibrous connective tissue and characterized by immature proliferating fibroblasts or undifferentiated anaplastic spindle cells. ... Metastasis (Greek: change of the state) is the spread of cancer from its primary site to other places in the body. ... A lipoma is a common, benign tumor composed of fatty tissue. ... This article needs to be cleaned up to conform to a higher standard of quality. ... A leiomyoma (plural is leiomyomata) is a benign smooth muscle neoplasm that is not premalignant. ... A sarcoma is a cancer of the bone, cartilage, fat, muscle, blood vessels, or other connective or supportive tissue. ... A rhabdomyoma is a benign tumor of muscle. ... A rhabdomyosarcoma is a type of cancer, specifically a sarcoma (cancer of connective tissues), in which the cancer cells arise from skeletal muscle. ... Angiomas are benign tumors that are made up of small blood vessels. ... Angiosarcoma is a rare, malignant tumor consisting of endothelial and fibroblastic tissue that proliferates and eventually surrounds vascular channels. ... An osteoma is a new piece of bone growing on another piece, typically the skull. ... Osteosarcoma is a common primary bone cancer. ... A chondroma is a benign cartilaginous tumor, which is encapsulated with a lobular growing pattern. ... A chondrosarcoma is a cancer of the cartilage. ... A glioma is a type of primary central nervous system (CNS) tumor that arises from glial cells. ... To meet Wikipedias quality standards, this article may require cleanup. ... Leukemia (or leukaemia; see spelling differences) is a cancer of the blood or bone marrow characterized by an abnormal proliferation of blood cells, usually white blood cells (leukocytes). ...


Treatment: Surgery - Chemotherapy - Radiation therapy - Immunotherapy - Experimental cancer treatment Intraoperative X-Ray of a Humerus fixated by Kirschner wires Surgery (from the Greek meaning hand work) is the medical specialty that treats diseases or injuries by operative manual and instrumental treatment. ... Chemotherapy is the use of chemical substances to treat disease. ... Clinac 2100 C accelerator Radiation therapy (or radiotherapy) is the medical use of ionizing radiation as part of cancer treatment to control malignant cells (not to be confused with radiology, the use of radiation in medical imaging and diagnosis). ... Cancer Immunotherapy is the use of monoclonal antibodies (-mab) to specifically target cells. ... Experimental cancer treatments are medical therapies intended or claimed to treat cancer (see also tumor) by improving on, supplementing or replacing conventional methods (surgery, chemotherapy, radiation, and immunotherapy). ...


Related structures: Cyst - Dysplasia - Hamartoma - Neoplasia - Nodule - Polyp - Pseudocyst This is an article about cysts in the body. ... Dysplasia (latin for bad form) is an abnormality in the appearance of cells indicative of an early step towards transformation into a neoplasia. ... Neoplasia (literally: new growth) is abnormal, disorganized growth in a tissue or organ, usually forming a distinct mass. ... In medicine, a nodule refers to a small aggregation of cells. ... Polyp of sigmoid colon as revealed by colonoscopy. ... A pseudocyst is a pathological collection of fluid. ...


Misc: Tumor suppressor genes/oncogenes - Staging/grading - Carcinogenesis/metastasis - Carcinogen - Research - Paraneoplastic phenomenon - ICD-O - List of oncology-related terms A tumor suppressor gene is a gene that reduces the probability that a cell in a multicellular organism will turn into a tumor cell. ... An oncogene is a modified gene that increases the malignancy of a tumor cell. ... The stage of a cancer is a descriptor (usually numbers I to IV) of how much the cancer has spread. ... In pathology, Grading is a measure of the progress of tumors. ... Cancers are caused by a series of mutations. ... Metastasis (Greek: change of the state) is the spread of cancer from its primary site to other places in the body. ... In pathology, a carcinogen is any substance or agent that promotes cancer. ... Cancer research is the intense scientific effort to understand the development of cancer and identify potential therapies. ... A paraneoplastic phenomenon is a disease or symptom that is the consequence of the presence of cancer in the body, but is not due to the local presence of cancer cells. ... The International Classification of Diseases for Oncology (ICD-O) is a domain specific extension of the International Statistical Classification of Diseases and Related Health Problems for tumor diseases. ... This is a list of terms related to oncology. ...


  Results from FactBites:
 
Case 21--hamartoma (970 words)
Hamartomas of the lung are benign lesions composed of an abnormal mixture of epithelial and mesenchymal elements.
Prevalence of peripheral hamartomas at autopsy in African miners was found to increase gradually from 1/1000 in the third decade to 12/1000 in the eighth decade [8].
Bateson E. So-called hamartoma of the lung--a true neoplasm of fibrous connective tissue of the bronchi.
Hamartoma of the hypothalamus and tuber cinereum : a brief review of the literature. Sharma RR J Postgrad Med (5168 words)
Hamartoma of the hypothalamus and tuber cinereum represents a midline dysraphic syndrome and presents as an ectopic cerebral grey matter,[19] comprising a mass of normal neuronal tissue,[7],[60] or redundant brain tissue.
Hamartoma of the hypothalamus and tuber cinereum may be regarded as a midline dysraphism like callosal defects, prosencephaly or septal dysplasia, and they may be associated with various other malformations of cerebral hemisphere.
Absence of precocious puberty in cases of hamartoma of the hypothalamus and tuber cinereum is attributed to the young age at death, or pressure atrophy of hypothalamus (medial eminence),[49] or a lack of neuronal connection between hamartoma and brain.
  More results at FactBites »

 
 

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