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         Multiple Hamartoma Syndrome:     more detail

81. Bannayan-Riley-Ruvalcaba Syndrome,BRRS,Bannayan-Zonana Syndrome (BZS),Riley-Smit
This group of syndromes includes PeutzJeghers, multiple hamartoma, juvenile polyposis, Cronkhite-Canada, and Bannayan-Riley-Ruvalcaba.
http://www.icomm.ca/geneinfo/brrs.htm
Bannayan-Riley-Ruvalcaba syndrome,BRRS,Bannayan-Zonana syndrome (BZS),Riley-Smith syndrome,Ruvalcaba-Myhre-Smith syndrome (RMSS),Macrocephaly with multiple lipomas and hemangiomata,Macrocephaly with pseudopapilledema and multiple hemangiomata For Information on Workshops and Seminars for Special Needs Children click here The GAPS INDEX
to Information on the Internet about Genetic Disorders and Birth Defects

Genetic Information and Patient Services, Inc. (GAPS)
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DISORDERS GLOSSARY Bannayan-Riley-Ruvalcaba syndrome
also known as:
BRRS
Bannayan-Zonana syndrome (BZS)
Riley-Smith syndrome
Ruvalcaba-Myhre-Smith syndrome (RMSS)
Macrocephaly, multiple lipomas, and hemangiomata
Macrocephaly, pseudopapilledema, and multiple hemangiomata (as defined by the National Organization for Rare Disorders
Bannayan-Riley-Ruvalcaba syndrome is a rare inherited disorder characterized by excessive growth before and after birth; an abnormally large head (macrocephaly) that is often long and narrow (scaphocephaly); normal intelligence or mild mental retardation; and/or benign tumor-like growths (hamartomas) that, in most cases, occur below the surface of the skin (subcutaneously). The symptoms of this disorder vary greatly from case to case. In most cases, infants with Bannayan-Riley-Ruvalcaba syndrome exhibit increased birth weight and length. As affected infants age, the growth rate slows and adults with this disorder often attain a height that is within the normal range.

82. Mafucci's Syndrome (www.whonamedit.com)
dyschondroplasia with haemangioma, dyschondrodysplasiahaemangiomas syndrome; multiple enchondromatosis syndrome; vascular hamartoma-dyschondroplasia syndrome.
http://www.whonamedit.com/synd.cfm/585.html

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This survey of medical eponyms and the persons behind them is meant as a general interest site only. No information found here must under any circumstances be used for medical purposes, diagnostically, therapeutically or otherwise. If you, or anybody close to you, is affected, or believe to be affected, by any condition mentioned here: see a doctor.
Mafucci's syndrome Also known as:
Kast’s disease
Kast’s syndrome Maffucci-Kast syndrome Synonyms: Achondromatosis with haemangiomata, chondrodysplasia angiomatosis syndrome, chondrodystrophy-haemangiomas syndrome; chondrodystrophy and vascular hamartoma syndrome, chondrodystrophy with angiomatosis, chondrodystrophy with vascular hamartoma, cutaneous dyschondroplasia-dyschromia syndrome, dyschondroplasia-angiomatosis syndrome, dyschondroplasia with haemangioma, dyschondrodysplasia-haemangiomas syndrome; multiple enchondromatosis syndrome; vascular hamartoma-dyschondroplasia syndrome. Associated persons: Alfred Kast Angelo Maffucci Description: Syndrome of enchondromas (benign tumours of cartilage), associated with multiple cavernous haemangiomas. Sometimes the patients show pigmentation. Normal at birth; bone and cartilage deformities appear during childhood in the years before puberty and the deformities increase during the period of growth. Complications are pathological fractures and other disorders of nonossified cartilage in the metaphyses and diaphyses of the long bones, chondrosarcoma and angiosarcoma. The skin and bony elsions are asymmetrical and do not coincide anatomically. Usually, no history of pain; orthostatic hypotension in sitting or standing position. Normal intelligence. Males are more frequently affected. Both sexes affected. Most cases are sporadic, but some instances of familial occurrence have been reported.

83. Diagnosis And Discussion Follicular Hamartoma
of the BirtHogg-Dube syndrome are characterized by multiple papular lesions Some of the lesions of the follicular hamartoma syndromes are also characterized by
http://www.xmission.com/~bweems/bhddxdis.html
DIAGNOSIS AND DISCUSSION case 5 of the Ochsner Clinicopathologic Skin Conference presented and discussed by R.J. Reed, M.D.
Diagnosis: Branching or arborizing, trichogenic, compound stromal and epithelial (catagen-like, anagen-like, and mantle-like patterns) hamartoma (Trent’s follicular hamartoma)
DISCUSSION
The stromal component of this unusual lesion is modified perifollicular connective tissue. The hair papilla is a special modification of the same mesenchyme. A natural representation of such mesenchyme might be found in a site in which a hair bulb has undergone cytolysis and the immediately adjacent tissue, including the papilla and the connective tissue sheath, has responded by becoming sclerotic ( Fig 10 At least a part of the lesion in question has features of a fibrofolliculoma as seen in the Birt-Hogg-Dube syndrome. Patterns of a characteristic example of the papular and polypoid lesions of the follicular hamartoma syndrome are represented in figs 11- 16 . In Fig 11 , three follicles are represented in cross section. The red arrow points to a follicle showing the pattern of fibrofolliculoma with mantle-like patterns and perifollicular fibrosis. The green arrows identify follicles that are not significantly distorted in the plane of the section but there is perifollicular fibrosis (perifolliculoma pattern). A sebaceous gland is associated with the follicle on the right. The lesion in fig 12 is slightly polypoid. The pattern is mantle-like with perifollicular fibrosis and sebaceous gland lobules (fibrofolliculoma pattern). The lesion in

84. Syndrome Publications Nevoid Basal Cell Carcinoma Syndrome / Gorlin Goltz Syndro
hamartoma syndrome, multiple. A hereditary disease characterised by multiple ectodermal, mesodermal, and endodermal nevoid and neoplastic anomalies.
http://www.nbccs.net/SyndromePublications.asp
Unfortunately we have collected way too many articles and too much information to affordably publish on one website. Eventually we hope to make all of these available for download from this site. We would like to get this information to you now if you desire. Here is an example of a collection of over 1200 articles and abstracts that have been organized and sorted by manifestation and date. Each category contains a summary of the information along with links to websites with articles and information, definitions of the pertinent terms. Many are full text articles. PDF available for many as well. Dozens of quality photos. Main points of the articles and summary's are highlighted in yellow. This is a great reference tool or resource for you and your health care providers. The entire collection is available on CD Rom. For more information contact the Nevoid Basal Cell Carcinoma Syndrome / Gorlin Syndrome Patient Advocate or you may request a copy of the CD from our Registration Page with articles in either Microsoft Word or soon in Microsoft Works Format. You will be notified of updates if you are a registered member.

85. MeSH-D Terms Associated To MeSH-C Term Hamartoma Syndrome
MeSHD terms associated to MeSH-C term hamartoma syndrome, multiple, G2D Home. The number indicates the strength of the association
http://www.bork.embl-heidelberg.de/g2d/c2d.pl?Hamartoma_Syndrome,_Multiple:unkno

86. Genetic Catalog - NEOPLASMS
Gardner syndrome. hamartoma syndrome, multiple. LiFraumeni syndrome. multiple Endocrine Neoplasia. multiple Endocrine Neoplasia Type 1.
http://www.rusmedserv.com/genetics/catalog/links61.htm
NEOPLASMS
General Aspects
Russian Resources
NEOPLASMS BY SITE
Abdominal Neoplasms
Breast Neoplasms
Breast Neoplasms, Male Mammary Neoplasms
Digestive System Neoplasms
Endocrine Gland Neoplasms
Eye Neoplasms
Hematologic Neoplasms
Nervous System Neoplasms

87. GeneCard For PTEN
cds (GDB); MMAC1 phosphatase and tension homolog deleted on chromosome 10 (LL); multiple hamartoma (Cowden syndrome) (GDB); mutated in
http://genecards.bcgsc.ca/cgi-bin/carddisp?PTEN

88. Entrez PubMed
GermLine Mutation; hamartoma syndrome, multiple/genetics*; Human; Male; Mammary Neoplasms, Animal/genetics*; Mammary Neoplasms, Animal
http://www.biomedcentral.com/pubmed/10910075
Entrez PubMed Nucleotide Protein ... Books Search PubMed Protein Nucleotide Structure Genome Books CancerChromosomes 3D Domains Domains Gene GEO GEO DataSets HomoloGene Journals MeSH NCBI Web Site OMIM PMC PopSet SNP Taxonomy UniGene UniSTS for Limits Preview/Index History Clipboard ...
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Cancer Res. 2000 Jul 1;60(13):3605-11. Related Articles, Links
High incidence of breast and endometrial neoplasia resembling human Cowden syndrome in pten+/- mice. Stambolic V, Tsao MS, Macpherson D, Suzuki A, Chapman WB, Mak TW.

89. Entrez PubMed
Reports; Letter. MeSH Terms Adult; hamartoma syndrome, multiple/microbiology*; hamartoma syndrome, multiple/pathology; Helicobacter
http://www.biomedcentral.com/pubmed/15093765
Entrez PubMed Nucleotide Protein ... Books Search PubMed Protein Nucleotide Structure Genome Books CancerChromosomes 3D Domains Domains Gene GEO GEO DataSets HomoloGene Journals MeSH NCBI Web Site OMIM PMC PopSet SNP Taxonomy UniGene UniSTS for Limits Preview/Index History Clipboard ...
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Entrez PubMed
Overview

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Single Citation Matcher Batch Citation Matcher ... Cubby Related Resources Order Documents NLM Gateway TOXNET Consumer Health ... PubMed Central Summary Brief Abstract Citation ASN.1 MEDLINE XML UI List LinkOut Related Articles Cited in Books CancerChrom Links Domain Links 3D Domain Links GEO DataSet Links Gene Links Genome Links GEO Links HomoloGene Links Nucleotide Links OMIM Links PMC Links Cited in PMC PopSet Links Protein Links SNP Links Structure Links UniSTS Links Show: Sort Author Journal Pub Date Text File Clipboard E-mail Order
Am J Med. 2004 May 1;116(9):642-4. Related Articles, Links
Gastric hamartomatous polyposis, Cowden syndrome, and H. pylori. Isomoto H, Urata M, Mizuta Y, Kohno S, Sawada T, Okada T. Publication Types:
  • Case Reports Letter
PMID: 15093765 [PubMed - indexed for MEDLINE]
Summary Brief Abstract Citation ASN.1

90. Cowden's Disease.
hamartoma syndrome, multiple/GE; hamartoma syndrome, multiple/PA; hamartoma syndrome, multiple/DI; Meningeal Neoplasms/PA; Neoplasms/ET; Skin Neoplasms/GE
http://medind.nic.in/imvw/imvw12897.html
Extracted from IndMED Khatri ML; Shafi M; Sen NK Departments of Dermatology and Pathology, Faculty of Medicine, A1-Fateh University of Medical Sciences, Tripoli, Libya. Cowden's disease. Indian Journal of Dermatology, Venereology and Leprology. 1996 Sep-Oct; 62(5): 322-4 ABSTRACT: A 24-year-old male patient developed multiple lesions of keratoacanthoma in the epidermal verrucous naevus. He also had multiple papillomatous lesions on the lips, buccal mucosa, gingiva and tongue, with positive family history of similar lesions. He also had multiple skin tags and patchy palmoplantar keratoderma and minimal kyphoscoliosis. KEYWORDS: Hamartoma Syndrome, Multiple/GE; Hamartoma Syndrome, Multiple/PA; Hamartoma Syndrome, Multiple/DI; Meningeal Neoplasms/PA; Neoplasms/ET; Skin Neoplasms/GE; Skin Neoplasms/DI; Skin/PA; Keratoacanthoma/DI; Keratoacanthoma/GE; Neoplasms, Multiple Primary/GE; Papilloma/DI; Adult; Human; Male; Case Report OTHER KEYWORDS: Histopathology References: 6 Record Identifier: TB3765

91. Clinica De Ortodoncia Dr. Arthur Nouel
oral diseases. Cowden’s syndrome. Is also known as multiple hamartoma and neoplasia syndrome. It is characterized orally by the
http://www.infocompu.com/adolfo_arthur/ingles/s_cowden.htm
Portal de ortodoncia y enfermedades de la boca Home Virtual visit Clinic profile Our procedures Services Orthodontics Radiographs Diagnosis Educational Clinical cases Oral diseases Maxillofacial surgery Oral surgery Radiology Index News Products we use Te invitamos a visitar la sección de Cirugía Bucal, con las técnicas quirúrgicas más comunes de la especialidad. oral diseases Is also known as multiple hamartoma and neoplasia syndrome. see other photographs Home Virtual visit Clinic profile ... News

92. New Page 1
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93. M From Linkspider UK Health Directory
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