Dermatofibrosarcoma protuberans

Genetic and Rare Diseases Information Center (GARD)

DFSP
Giant cell fibroblastoma
Familial dermatofibrosarcoma protuberans (subtype)
Metastatic dermatofibrosarcoma protuberans (subtype)

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Dermatofibrosarcoma protuberans
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Dermatofibrosarcoma protuberans is an uncommon tumor that arises in the skin. The tumor is firm and often flesh-colored although it can be reddish, bluish, or purplish. The tumor is often found on the chest or shoulders, but it can be found on other parts of the body. Dermatofibrosarcoma protuberans may cause no symptoms, and the initial size of the tumor tends to be around 1 to 5 centimeters. This tumor has a low potential to spread to other tissues (metastasize). Treatment often involves surgery to remove the tumor, such as by Mohs’ micrographic surgery. ^[1]

References

Wood GS. et al. Clinical Oncology, 4th ed. In: . Nonmelanoma Skin Cancers: Basal Cell and Squamous Cell Carcinomas. Philadelphia, PA:Churchill Livingstone; 2008:

Questions & Answers (Found 3 Questions)

A list of questions from the public on rare and/or genetic diseases that have been answered by the Genetic and Rare Disease Information Center. Click on each question to find the answer.

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For more information about Dermatofibrosarcoma protuberans click on the boxes below:

More Detailed Information (Found 7 resources)
Links where you can find more general information, comprehensive resources, selected full text journal articles, and news updates. General DermNet NZ is an online resource about skin diseases developed by the New Zealand Dermatological Society Incorporated. DermNet NZ provides information about this condition. Click on the link to view the information. Genetics Home Reference (GHR) contains information on Dermatofibrosarcoma protuberans. Click on the link to go to GHR and review the information. Medscape Reference provides information on this topic. Click on the link to view this information. You may need to register to view the medical textbook, but registration is free. MeSH® (Medical Subject Headings) is a terminology tool used by the National Library of Medicine. Click on the link to view information on this topic. The Online Mendelian Inheritance in Man (OMIM) database contains genetics resources that discuss Dermatofibrosarcoma protuberans. Click on the link to go to OMIM and review these resources. Orphanet is a database dedicated to information on rare diseases and orphan drugs. Access to this database is free of charge. Click on the link to read information on this topic. PubMed is a searchable database of medical literature and lists journal articles that discuss Dermatofibrosarcoma protuberans. Click on the link to view a sample search on this topic.

More Detailed Information (Found 7 resources)

Links where you can find more general information, comprehensive resources, selected full text journal articles, and news updates.

General
- DermNet NZ is an online resource about skin diseases developed by the New Zealand Dermatological Society Incorporated. DermNet NZ provides information about this condition. Click on the link to view the information.
- Genetics Home Reference (GHR) contains information on Dermatofibrosarcoma protuberans. Click on the link to go to GHR and review the information.
- Medscape Reference provides information on this topic. Click on the link to view this information. You may need to register to view the medical textbook, but registration is free.
- MeSH® (Medical Subject Headings) is a terminology tool used by the National Library of Medicine. Click on the link to view information on this topic.
- The Online Mendelian Inheritance in Man (OMIM) database contains genetics resources that discuss Dermatofibrosarcoma protuberans. Click on the link to go to OMIM and review these resources.
- Orphanet is a database dedicated to information on rare diseases and orphan drugs. Access to this database is free of charge. Click on the link to read information on this topic.
- PubMed is a searchable database of medical literature and lists journal articles that discuss Dermatofibrosarcoma protuberans. Click on the link to view a sample search on this topic.

Organizations (Found 10 resources) Disease-Specific Organizations \| Umbrella Organizations \| Social Networking Websites
Groups providing a wide range of services, supportive resources, and information. Disease-Specific Organizations CancerCare 275 Seventh Ave, Floor 22 New York, NY 10001 Toll-free: 1-800-813-HOPE Fax: 212-712-8495 E-mail: info@cancercare.org Web site: http://www.cancercare.org/ Find help by topic: http://www.cancercare.org/diagnosis The Skin Cancer Foundation 149 Madison Avenue Suite 901 New York, New York 10016 Phone: 212-725-5176 Web site: http://www.skincancer.org/ American Cancer Society 1599 Clifton Road NE Atlanta GA 30329 Toll-free: 1-800-227-2345 E-mail: http://www.cancer.org/asp/contactUs/cus_global.asp Web site: http://www.cancer.org American Skin Association, Inc. 346 Park Avenue South, 4th floor New York, NY 10010 Toll-free: 800-499-SKIN Telephone: 212-889-4858 Fax: 212-889-4959 E-mail: info@skinassn.org Web site: http://www.americanskin.org Umbrella Organizations Genetic Alliance 4301 Connecticut Avenue NW Suite 404 Washington, DC 20008-2369 Telephone: 202-966-5557 Fax: 202-966-8553 E-mail: info@geneticalliance.org Web site: http://www.geneticalliance.org National Organization for Rare Disorders (NORD) 55 Kenosia Avenue PO Box 1968 Danbury, CT 06813-1968 Toll-free: 1-800-999-6673 (voicemail only) Telephone: 203-744-0100 TTY: 203-797-9590 Fax: 203-798-2291 E-mail: orphan@rarediseases.org Web site: http://www.rarediseases.org/ Social Networking Websites DNAandU.org is a Web site and blog that collects firsthand stories from people facing issues, making tough decisions, and using genomic (DNA) information in their own healthcare. Click on the link to learn from their experiences and/or submit your own story. Madisons Foundation P.O. Box 241956 Los Angeles, CA 90024 Telephone: 310-264-0826 Fax: 310-264-4766 E-mail: getinfo@madisonsfoundation.org Web site: http://www.madisonsfoundation.org/ The National Organization of Rare Disorders (NORD) has partnered with Inspire.com to launch an online community for people with rare diseases called The NORD Rare Disease Community. This community connects medical patients, family members, caregivers, and professionals. Click on The NORD Rare Disease Community to learn more. RareShare is an online social hub dedicated to patients, families and healthcare professionals affected by rare medical disorders. Click on RareShare to learn more.

Organizations (Found 10 resources)
Disease-Specific Organizations | Umbrella Organizations | Social Networking Websites

Groups providing a wide range of services, supportive resources, and information.

Disease-Specific Organizations
- CancerCare
  275 Seventh Ave, Floor 22
  New York, NY 10001
  Toll-free: 1-800-813-HOPE
  Fax: 212-712-8495
  E-mail: info@cancercare.org
  Web site: http://www.cancercare.org/
  Find help by topic: http://www.cancercare.org/diagnosis
- The Skin Cancer Foundation
  149 Madison Avenue Suite 901
  New York, New York 10016
  Phone: 212-725-5176
  Web site: http://www.skincancer.org/
- American Cancer Society
  1599 Clifton Road NE
  Atlanta GA 30329
  Toll-free: 1-800-227-2345
  E-mail: http://www.cancer.org/asp/contactUs/cus_global.asp
  Web site: http://www.cancer.org
- American Skin Association, Inc.
  346 Park Avenue South, 4th floor
  New York, NY 10010
  Toll-free: 800-499-SKIN
  Telephone: 212-889-4858
  Fax: 212-889-4959
  E-mail: info@skinassn.org
  Web site: http://www.americanskin.org
Umbrella Organizations
- Genetic Alliance
  4301 Connecticut Avenue NW
  Suite 404
  Washington, DC 20008-2369
  Telephone: 202-966-5557
  Fax: 202-966-8553
  E-mail: info@geneticalliance.org
  Web site: http://www.geneticalliance.org
- National Organization for Rare Disorders (NORD)
  55 Kenosia Avenue
  PO Box 1968
  Danbury, CT 06813-1968
  Toll-free: 1-800-999-6673 (voicemail only)
  Telephone: 203-744-0100
  TTY: 203-797-9590
  Fax: 203-798-2291
  E-mail: orphan@rarediseases.org
  Web site: http://www.rarediseases.org/
Social Networking Websites
- DNAandU.org is a Web site and blog that collects firsthand stories from people facing issues, making tough decisions, and using genomic (DNA) information in their own healthcare. Click on the link to learn from their experiences and/or submit your own story.
- Madisons Foundation
  P.O. Box 241956
  Los Angeles, CA 90024
  Telephone: 310-264-0826
  Fax: 310-264-4766
  E-mail: getinfo@madisonsfoundation.org
  Web site: http://www.madisonsfoundation.org/
- The National Organization of Rare Disorders (NORD) has partnered with Inspire.com to launch an online community for people with rare diseases called The NORD Rare Disease Community. This community connects medical patients, family members, caregivers, and professionals. Click on The NORD Rare Disease Community to learn more.
- RareShare is an online social hub dedicated to patients, families and healthcare professionals affected by rare medical disorders. Click on RareShare to learn more.

Medical Products (Found 1 resource)

U.S. Food and Drug Administration

The Office of Orphan Products Development (OOPD)

The medications listed in the table below have been approved by the Food and Drug Administration (FDA) for treatment of this condition. The FDA Office of Orphan Products Development designates "orphan products" for those that treat rare diseases affecting fewer than 200,000 Americans. The table below may not be an exhaustive list of drugs or products used to treat this condition. There may be other products available that are not considered orphan products. To search all FDA approved drugs, visit Drugs@FDA You can find orphan products used to treat other conditions by searching the Orphan Drug Product Designation database.

Generic Name	Trade Name (Manufacturer Name)	Indication	Resources
Imatinib mesylate	Gleevec® (Novartis Pharmaceuticals Corp.)	Treatment of adult patients with unresectable, recurrent and/or metastatic dermatofibrosarcoma protuberans (DFSP)	Drug Information Portal

Services (Found 2 resources)
Links to Web sites that offer services, such as tools to locate specialists, specialty clinics, genetic services, and genetic testing laboratories. Many individuals want to know about healthcare professionals or researchers who have knowledge of their conditions. When a condition is rare, it can be difficult to find someone who has seen many cases. Although there is no list of experts in rare diseases, GARD's How to Find an Expert fact sheet provides several ways to identify healthcare professionals who have experience with a particular condition. Click on the link to access this resource and learn more. Parent Resources The Parent Technical Assistance Center Network provides a list of the Parent Training and Information Centers in each state. These centers are funded by the United States Department of Education to provide early intervention and special education information and training to parents of children with disabilities from birth to age 26. Click on the link to find the Parent Center in your state.

Services (Found 2 resources)

Links to Web sites that offer services, such as tools to locate specialists, specialty clinics, genetic services, and genetic testing laboratories.

- Many individuals want to know about healthcare professionals or researchers who have knowledge of their conditions. When a condition is rare, it can be difficult to find someone who has seen many cases. Although there is no list of experts in rare diseases, GARD's How to Find an Expert fact sheet provides several ways to identify healthcare professionals who have experience with a particular condition. Click on the link to access this resource and learn more.
Parent Resources
- The Parent Technical Assistance Center Network provides a list of the Parent Training and Information Centers in each state. These centers are funded by the United States Department of Education to provide early intervention and special education information and training to parents of children with disabilities from birth to age 26. Click on the link to find the Parent Center in your state.

Scientific Conferences (Found 3 resources) Upcoming Conferences \| Past Conferences
Resources where you can find information on completed and upcoming scientific conferences and programs. Upcoming Conferences RDCRN 3rd Conference on Clinical Research for Rare Diseases, October 2, 2012 Location: Hilton Washington DC/Rockville Hotel & Executive Meeting Center, Rockville, MD Description: Clinical research in rare diseases presents a number of challenges and unique issues that are not usually considered in the training of clinical investigators through existing training programs. Goals of the conference include: Direct instruction of trainees and new investigators in rare disease research methodology; Development of a reusable curriculum/syllabus on rare disease research methodology; Stimulation of ideas regarding the unique issues facing investigators engaged in the study of rare diseases Past Conferences Overcoming Barriers to International Clinical Trials for Rare Cancers , December 10, 2010 Description: The goals of this conference were to introduce key institutional players to the topic of international clinical trials in rare cancers and to establish an ongoing dialogue. Participants left the meeting with a set of specific priorities that need to be enacted to promote these trials. The meeting promoted consensus on the way that resources are prioritized to address rare cancers. Participants were asked to convey the content of the meeting to their constituencies and to follow up with pilot concepts. VI International Conference on Rare Diseases and Orphan Drugs (ICORD 2010): Global Approach to Accessibility in Rare Diseases, Orphan Drugs and Neglected Diseases , March 18, 2010 - March 20, 2010 Location: Palais Rouge Convention Center, Palermo, Buenos Aires Description: For the first time, the VI International Conference on Rare Diseases and Orphan Drugs (ICORD 2010) was convened in the southern hemisphere in agreement with its aim of globalization of rare diseases research and orphan products development activities.

Scientific Conferences (Found 3 resources)
Upcoming Conferences | Past Conferences

Resources where you can find information on completed and upcoming scientific conferences and programs.

Upcoming Conferences
- RDCRN 3rd Conference on Clinical Research for Rare Diseases, October 2, 2012
  Location: Hilton Washington DC/Rockville Hotel & Executive Meeting Center, Rockville, MD
  Description: Clinical research in rare diseases presents a number of challenges and unique issues that are not usually considered in the training of clinical investigators through existing training programs. Goals of the conference include: Direct instruction of trainees and new investigators in rare disease research methodology; Development of a reusable curriculum/syllabus on rare disease research methodology; Stimulation of ideas regarding the unique issues facing investigators engaged in the study of rare diseases
Past Conferences
- Overcoming Barriers to International Clinical Trials for Rare Cancers , December 10, 2010
  Description: The goals of this conference were to introduce key institutional players to the topic of international clinical trials in rare cancers and to establish an ongoing dialogue. Participants left the meeting with a set of specific priorities that need to be enacted to promote these trials. The meeting promoted consensus on the way that resources are prioritized to address rare cancers. Participants were asked to convey the content of the meeting to their constituencies and to follow up with pilot concepts.
- VI International Conference on Rare Diseases and Orphan Drugs (ICORD 2010): Global Approach to Accessibility in Rare Diseases, Orphan Drugs and Neglected Diseases , March 18, 2010 - March 20, 2010
  Location: Palais Rouge Convention Center, Palermo, Buenos Aires
  Description: For the first time, the VI International Conference on Rare Diseases and Orphan Drugs (ICORD 2010) was convened in the southern hemisphere in agreement with its aim of globalization of rare diseases research and orphan products development activities.

Clinical Trials & Research (Found 5 resources)
Resources where you may find research studies and clinical trials. ClinicalTrials.gov lists trials that are studying or have studied Dermatofibrosarcoma protuberans. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies. The U.S. National Institutes of Health, through the National Library of Medicine, developed ClinicalTrials.gov to provide patients, family members, and members of the public with current information on clinical research studies. There is a study titled Evaluation and Treatment of Patients with Dermatologic Diseases which may be of interest to you. To find this trial, click on the link above. NIH Clinical Trials and You is a website developed by the National Institutes of Health (NIH) to help people learn more about clinical trials, why they matter, and how to participate. The Research Portfolio Online Reporting Tool (RePORT) provides access to reports, data, and analyses of research activities at the National Institutes of Health (NIH), including information on NIH expenditures and the results of NIH-supported research. There is a study titled Dermatology Consultation Clinic and Clinical Research that may be of interest to you. You may want to contact the investigator, Maria Turner (maria.turner@nih.gov) to learn more. Patient Registry ResearchMatch is a free national research registry designed to bring together patients, healthy volunteers and researchers. Anyone from the United States can register with ResearchMatch, and a parent, legal guardian, or caretaker may register on behalf of a volunteer. Researchers from participating institutions use the ResearchMatch database to search for patients or healthy volunteers who meet the study criteria. Many studies are looking for healthy people of all ages, while some are looking for people with specific illnesses. ResearchMatch was developed by major academic institutions across the country and is funded by the National Center for Research Resources (NCRR), a center of the National Institutes of Health (NIH), the primary Federal agency for conducting and supporting medical research. Click on the link to learn more about ResearchMatch.

Clinical Trials & Research (Found 5 resources)

Resources where you may find research studies and clinical trials.

- ClinicalTrials.gov lists trials that are studying or have studied Dermatofibrosarcoma protuberans. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.
- The U.S. National Institutes of Health, through the National Library of Medicine, developed ClinicalTrials.gov to provide patients, family members, and members of the public with current information on clinical research studies. There is a study titled Evaluation and Treatment of Patients with Dermatologic Diseases which may be of interest to you. To find this trial, click on the link above.
- NIH Clinical Trials and You is a website developed by the National Institutes of Health (NIH) to help people learn more about clinical trials, why they matter, and how to participate.
- The Research Portfolio Online Reporting Tool (RePORT) provides access to reports, data, and analyses of research activities at the National Institutes of Health (NIH), including information on NIH expenditures and the results of NIH-supported research. There is a study titled Dermatology Consultation Clinic and Clinical Research that may be of interest to you. You may want to contact the investigator, Maria Turner (maria.turner@nih.gov) to learn more.
Patient Registry
- ResearchMatch is a free national research registry designed to bring together patients, healthy volunteers and researchers. Anyone from the United States can register with ResearchMatch, and a parent, legal guardian, or caretaker may register on behalf of a volunteer. Researchers from participating institutions use the ResearchMatch database to search for patients or healthy volunteers who meet the study criteria. Many studies are looking for healthy people of all ages, while some are looking for people with specific illnesses. ResearchMatch was developed by major academic institutions across the country and is funded by the National Center for Research Resources (NCRR), a center of the National Institutes of Health (NIH), the primary Federal agency for conducting and supporting medical research. Click on the link to learn more about ResearchMatch.