The American Journal Of Surgical Pathology - UserLogin multiple hamartoma syndrome. J Am Acad Dermatol 1987;173426. Context Link.4. Barr RJ, Morales RV, Graham JH. Desmoplastic nevus. http://www.ajsp.com/pt/re/ajsp/fulltext.00000478-199805000-00006.htm
[P&S Journal:Wi:97] Cowden's Syndrome: Masked Menace Instead, she suffers from a littleknown disease called Cowden s syndrome (CS;also known as multiple hamartoma syndrome)an autosomal dominant disorder http://cumc.columbia.edu/news/journal/journal-o/archives/jour_v17n1_0011.html
Extractions: W hen Mary Smith (not her real name) was 16 years old, she found a lump in her breast. "My mother was terrifiedshe thought it was cancer," says Ms. Smith, now 53. "But in those days, no one talked about cancer. So even though I was scared that I had to have surgery, I didn't know the overwhelming possibilities." Since then, she has had a seemingly endless series of cancer scares and actual bouts with cancer: four biopsies for suspicious breast lumps, a lumpectomy followed by a mastectomy, a hysterectomy, partial nephrectomy for what turned out to be a benign mass, and, in 1996, another mastectomy. Mary Smith does not have the BRCA1 or BRCA2 breast cancer genes. Instead, she suffers from a little-known disease called Cowden's syndrome (CS; also known as multiple hamartoma syndrome)an autosomal dominant disorder characterized by skin lesions and a high risk of both breast and thyroid cancer. The medical literature describes CS as a rare disease associated with marked disfigurement. But according to Dr. Monica Peacocke, associate professor of medicine and of dermatology, CS is fairly common, not generally disfiguring, easily missed by many doctors, and an under-recognized cause of many cases of familial breast cancer. "The genetic basis of many types of familial breast cancer is not yet known," says Dr. Peacocke, who is collecting the genetic pedigrees of people like Mary Smith. "CS is masquerading as sporadic breast cancer."
Extractions: Radiological Quiz - Neuroradiology SUREKHA K, AK GUPTA, SANTHOSH JOSEPH, KESAVADAS C, NKK PRABHU Ind J Radiol Imag 1999; 9:1: 25-27 A fifty-seven-years old man was well until one year prior to admission to the hospital. He developed recurrent attacks of headache and vomiting with increased frequency of symptoms about one month prior to admission. He also complained of swaying to either side. Examination revealed bilateral papilledema. In addition he also had multiple subcutaneous lipomas and papules over the body. CT and MR were performed (Figs. 1, 2). Fig 1 A RADIOLOGICAL DIAGNOSIS: Lhermitte-Duclos Disease (LDD) CT shows an ill-defined hypodense lesion of the right cerebellum with significant mass effect (Fig. 1). MR reveals a hypointense mass on the T1W images (Fig. 2A) and a laminated, striated or folial pattern of increased signal on the T2W images (Fig. 2B). The patient underwent decompression and partial resection of the lesion. On surgery, the cerebellum showed thickened folia. Pathological features demonstrated were suggestive of Lhermitte-Duclos disease (LDD). Dermatological consultation with biopsy of skin lesions was consistent with Cowden's disease.
MUMS List Of Disorders - C Costello Syndrome (7) * ; Cowden Syndrome (multiple hamartoma syndrome) (4) *;CranioFronto-Nasal Dysplasia (3); Craniodiaphyseal Dysplasia, http://www.netnet.net/mums/mum_c.htm
Extractions: Guidelines of Care for Nevi. II. Nonmelanocytic Nevi, Hamartomas, Neoplasms, and Potentially Malignant Lesions This report reflects the best data available at the time the report was prepared, but caution should be exercised in interpreting the data; the results of future studies may require alteration of the conclusions or recommendations set forth in this report. Reprint requests: American Academy of Dermatology, P.O. Box 4014, Guidelines of care for nevi. II. Nonmelanocytic nevi, hamartomas, neoplasms, and potentially malignant lesions Committee on Guidelines of Care : Lynn A. Drake, MD, Chairman, Roger I. Ceilley, MD, Raymond L. Cornelison, MD, William L. Dobes, MD, William Dorner, MD, Robert W. Goltz, MD, Gloria F. Graham, MD, Charles W. Lewis, MD, David M. Pariser, MD, Stuart J. Salasche, MD, John W.. Skouge, MD, Maria L. Chanco Turner, MD, and Barbara J. Lowery, MPH Task Force on Nevi, Part II: Robert W. Goltz, MD, Chairman, David R. Barron, MD, Alvin H. Clair, MD, Kenneth G. Gross, MD, and Charles W. Lewis, MD I. Introduction
Www.nlm.nih.gov/cgi/mesh/2K/MB_cgi?term=Hamartoma+ Annals of Surgical Oncology Wirtzfeld et al. 8 (4) 319Also referred to as the multiple hamartomaneoplasia syndrome, 31 Cowdensdisease (multiple hamartoma syndrome). Mayo Clin Proc 1981; 56 17984. http://www.nlm.nih.gov/cgi/mesh/2K/MB_cgi?term=Hamartoma Syndrome, Multiple
Irritation Fibroma cases of familial fibromatosis, fibrotic papillary hyperplasia of the palate,tuberous sclerosis, or multiple hamartoma syndrome (Cowden syndrome). http://www.maxillofacialcenter.com/BondBook/softtissue/fibroma.html
Extractions: Irritation fibromas under dentures are flat and "leaf-shaped." Quick Review for Patients The irritation fibroma is a painless, localized, mass-producing proliferation of dense fibrous "scar" tissue which can result from a single traumatic episode or from repeated, less severe traumatic episodes; chronic inflammation or infection may also produce a fibroma. The lesion can become more than 3 cm. in size but is usually less than 1 cm. It occurs at all ages and in both genders and specialized forms occur under dentures, along denture edges and on inflamed gingiva. The fibroma is best treated by conservative surgical or laser removal, with a small chance of recurrence should the originating irritation persist. There is no risk of malignant transformation. Top of This Page Introduction Note: click on underlined words for more detail or photos. Irritation fibroma
Granular Cell Tumor Malignant variants represent approximately 1% of all cases, with some representingneoplasia in Cowden syndrome (multiple hamartoma syndrome), http://www.maxillofacialcenter.com/BondBook/softtissue/grantumor.html
Extractions: Photos ... Quick Review for Patients Top of This Page Introduction Note: click on underlined words for more detail or photos. It is not clear whether or not granular cell tumor granular cell myoblastoma granular cell schwannoma ) is a true neoplasm, a developmental anomaly, or a trauma-induced proliferation. The basic cell of origin is now thought to be neural, although past reports frequently indicated an origin from striated muscle, or less frequently an origin from histiocytes, fibroblasts or pericytes. The tumor is widely distributed throughout the body, but more than half of all cases occur in the oral cavity. The other head and neck site likely to be involved is the larynx. Malignant variants represent approximately 1% of all cases, with some representing neoplasia in Cowden syndrome multiple hamartoma syndrome but some consider these malignancies to be simply variants of the distinctive and rare alveolar soft part sarcoma, a look-alike malignancy composed largely of granular epithelioid cells organized in alveolar-like nests. Once considered an organoid variant of malignant granular cell tumor or a malignant nonchromaffin paraganglioma, it also has immunohistochemical features of skeletal muscle. Its true histogenesis, however, remains debatable and it is included here only for purposes of convenience. Approximately one in four lesions occur in the head and neck region, usually the oral cavity, pharynx and orbit.
GeneCard For PTEN The predominant phenotype for CD is multiple hamartoma syndrome, in many organsystems including the breast (70% of CD patients), thyroid http://bioinfo.cnio.es/cgi-bin/genecards/carddisp?PTEN
UniProtKB Entry - UniProt [the Universal Protein Resource] The predominant phenotype for CD is multiple hamartoma syndrome, in many organsystems including the breast (70% of CD patients), thyroid (4060%), skin, http://www.pir.uniprot.org/cgi-bin/upEntry?id=PTEN_HUMAN
PhosphaBase 158350 COWDEN DISEASE; CD;;COWDEN SYNDROME; CS;;multiple hamartoma syndrome;MHAM;DYSPLASTIC GANGLIOCYTOMA OF THE CEREBELLUM, http://bioinf.man.ac.uk/phosphabase/tyrdisease.html
Cancers - Genetics Home Reference MHAM see Cowden syndrome multiple endocrine neoplasia type 1 multipleendocrine neoplasia type 2; multiple hamartoma syndrome see Cowden syndrome http://ghr.nlm.nih.gov/ghr/conditionsByCategory/show/cancers
Extractions: Home What's New Browse Handbook ... Search Browse Browse Genes Browse Chromosomes Cancer is a condition in which abnormal cells divide without control. Cancer cells can invade nearby tissues and can spread through the bloodstream and lymphatic system to other parts of the body. There are many types of cancer that affect various parts of the body. Adenomatous Polyposis Coli see familial adenomatous polyposis Adenomatous Polyposis of the Colon see familial adenomatous polyposis Angiomatosis retinae see von Hippel-Lindau syndrome BANF see neurofibromatosis 2 BHD see Bilateral Acoustic Neurofibromatosis see neurofibromatosis 2 bladder cancer breast cancer Broad Thumb-Hallux Syndrome see Rubinstein-Taybi syndrome Cancer Family Syndrome see hereditary nonpolyposis colorectal cancer Cancer of breast see breast cancer Cancer of the bladder see bladder cancer Cerebelloretinal Angiomatosis, Familial see von Hippel-Lindau syndrome Colon cancer, familial see familial adenomatous polyposis Colon Cancer, Familial Nonpolyposis see hereditary nonpolyposis colorectal cancer Cowden syndrome CS see Cowden syndrome Familial Acoustic Neuromas see neurofibromatosis 2 familial adenomatous polyposis Familial nonpolyposis colon cancer see hereditary nonpolyposis colorectal cancer FAP see familial adenomatous polyposis Fibrofolliculomas with trichodiscomas and acrochordons see Glioblastoma, retinal
A Case Of Familial Angiolipomatosis With Lisch Nodules multiple hamartoma syndrome (Cowden s disease). Arch Dermatol 1972;106682690.PubMed Citation 15. Enzinger FM, Weiss SW. Soft Tissue Tumors. 3rd ed. http://arpa.allenpress.com/arpaonline/?request=get-document&doi=10.1043/0003-998
J Med Genet -- Sign In Page multiple hamartoma syndrome. Cowdens disease. Arch Dermatol 1978;1147436.Abstract;Weary PE, Gorlin RJ, Gentry WC, Comer JE, Greer KE. http://jmg.bmjjournals.com/cgi/content/full/42/8/e50
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Extractions: Vol Page [Advanced] This Article Abstract Full Text (PDF) Submit a response ... Citation Map Services Email this link to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal ... Cited by other online articles PubMed PubMed Citation Articles by Peacocke, M. Related Collections Other Oncology Genetics J Med Genet 360-364 ( May ) a Hui C Tsou a Fei Fei Chen a Hong Zhang a Xiao Li Ping a Mark G Lebwohl b Jeffrey Kezis a Monica Peacocke a a Department of Dermatology, Columbia Presbyterian Hospital, Columbia University, College of Physicians and Surgeons, 630 West 168th Street, New York, New York 10032, USA, b Department of Dermatology, The Mount Sinai School of Medicine, The Mount Sinai Medical Center, One Gustave L Levy Place, Box 1130, New York, New York 10029, USA
Anales De Medicina Interna - Translate this page multiple hamartoma syndrome presenting with oral lesions. Early diagnosis ofmultiple hamartoma nad neoplasia syndrome (Cowden disease). http://scielo.isciii.es/scielo.php?pid=S0212-71992001000800006&script=sci_arttex
Information Center For Rare Diseases And Orphan Drugs - Sections multiple fibrofolliculoma familial multiple hamartoma syndrome multiple jointdislocations metaphyseal dysplasia multiple pterygium syndrome http://www.raredis.org/modules/sections/index.php?op=viewarticle&artid=27
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