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Dermatofibrosarcoma protuberans(DFSP)

MedGen UID:
95938
Concept ID:
C0392784
Neoplastic Process
Synonyms: DERMATOFIBROSARCOMA PROTUBERANS, SOMATIC; DFSP; Familial dermatofibrosarcoma protuberans (subtype); Giant cell fibroblastoma; Metastatic dermatofibrosarcoma protuberans (subtype)
Modes of inheritance:
not inherited
MedGen UID:
832438
Concept ID:
CN227390
Intellectual Product
Source: Orphanet
Describes a disorder that is not inherited.
not inherited (Orphanet)
SNOMED CT: [M]Dermatofibroma protuberans (76594008); Dermatofibrosarcoma protuberans (76594008); Dermatofibrosarcoma protuberans (76594008); Giant cell fibroblastoma of skin (238863004); Giant cell fibroblastoma (128742004); Giant cell fibroblastoma (238863004); DFSP - Dermatofibrosarcoma protruberans (276799004); Dermatofibrosarcoma protuberans (276799004)
 
Gene (location): PDGFB (22q13.1)
OMIM®: 607907
Orphanet: ORPHA31112

Definition

Dermatofibrosarcoma protuberans (DFSP) is an uncommon, locally aggressive, but rarely metastasizing tumor of the deep dermis and subcutaneous tissue. It typically presents during early or middle adult life and is most frequently located on the trunk and proximal extremities (Sandberg et al., 2003). [from OMIM]

Additional description

From GHR
Dermatofibrosarcoma protuberans is a rare type of cancer that causes a tumor in the deep layers of skin. This condition is a type of soft tissue sarcoma, which are cancers that affect skin, fat, muscle, and similar tissues.In dermatofibrosarcoma protuberans, the tumor most often starts as a small, firm patch of skin, usually 1 to 5 centimeters in diameter, that is usually purplish, reddish, or flesh-colored. The tumor typically grows slowly and can become a raised nodule. Occasionally, the tumor begins as a flat or depressed patch of skin (plaque). Tumors are most commonly found on the torso and can also be found on the arms, legs, head, or neck. Affected individuals usually first show signs of this condition in their thirties, but the age at which a tumor appears varies widely.In dermatofibrosarcoma protuberans, the tumor has a tendency to return after being removed. However, it does not often spread to other parts of the body (metastasize).There are several variants of dermatofibrosarcoma protuberans in which different cell types are involved in the tumor. Bednar tumors, often called pigmented dermatofibrosarcoma protuberans, contain dark-colored (pigmented) cells called melanin-containing dendritic cells. Myxoid dermatofibrosarcoma protuberans tumors contain an abnormal type of connective tissue known as myxoid stroma. Giant cell fibroblastoma, which is sometimes referred to as juvenile dermatofibrosarcoma protuberans because it typically affects children and adolescents, is characterized by giant cells in the tumor.Rarely, the tumors involved in the different types of dermatofibrosarcoma protuberans can have regions that look similar to fibrosarcoma, a more aggressive type of soft tissue sarcoma. In these cases, the condition is called fibrosarcomatous dermatofibrosarcoma protuberans or FS-DFSP. FS-DFSP tumors are more likely to metastasize than tumors in the other types of dermatofibrosarcoma protuberans.  https://ghr.nlm.nih.gov/condition/dermatofibrosarcoma-protuberans

Clinical features

Malignant Skin Neoplasm
MedGen UID:
40101
Concept ID:
C0007114
Neoplastic Process
Skin cancer is the most common form of cancer in the United States. The two most common types are basal cell cancer and squamous cell cancer. They usually form on the head, face, neck, hands, and arms. Another type of skin cancer, melanoma, is more dangerous but less common. . Anyone can get skin cancer, but it is more common in people who . - Spend a lot of time in the sun or have been sunburned. - Have light-colored skin, hair and eyes. - Have a family member with skin cancer. - Are over age 50. You should have your doctor check any suspicious skin markings and any changes in the way your skin looks. Treatment is more likely to work well when cancer is found early. If not treated, some types of skin cancer cells can spread to other tissues and organs. Treatments include surgery, radiation therapy, chemotherapy, photodynamic therapy (PDT), and biologic therapy. PDT uses a drug and a type of laser light to kill cancer cells. Biologic therapy boosts your body's own ability to fight cancer. NIH: National Cancer Institute.
Sarcoma
MedGen UID:
224714
Concept ID:
C1261473
Neoplastic Process
Your soft tissues connect, support, or surround other tissues. Examples include your muscles, tendons, fat, and blood vessels. Soft tissue sarcoma is a cancer of these soft tissues. There are many kinds, based on the type of tissue they started in. They may cause a lump or swelling in the soft tissue. Sometimes they spread and can press on nerves and organs, causing problems such as pain or trouble breathing. No one knows exactly what causes these cancers. They are not common, but you have a higher risk if you have been exposed to certain chemicals, have had radiation therapy, or have certain genetic diseases. Doctors diagnose soft tissue sarcomas with a biopsy. Treatments include surgery to remove the tumor, radiation therapy, chemotherapy, or a combination. NIH: National Cancer Institute.
Malignant Skin Neoplasm
MedGen UID:
40101
Concept ID:
C0007114
Neoplastic Process
Skin cancer is the most common form of cancer in the United States. The two most common types are basal cell cancer and squamous cell cancer. They usually form on the head, face, neck, hands, and arms. Another type of skin cancer, melanoma, is more dangerous but less common. . Anyone can get skin cancer, but it is more common in people who . - Spend a lot of time in the sun or have been sunburned. - Have light-colored skin, hair and eyes. - Have a family member with skin cancer. - Are over age 50. You should have your doctor check any suspicious skin markings and any changes in the way your skin looks. Treatment is more likely to work well when cancer is found early. If not treated, some types of skin cancer cells can spread to other tissues and organs. Treatments include surgery, radiation therapy, chemotherapy, photodynamic therapy (PDT), and biologic therapy. PDT uses a drug and a type of laser light to kill cancer cells. Biologic therapy boosts your body's own ability to fight cancer. NIH: National Cancer Institute.
Skin ulcer
MedGen UID:
52369
Concept ID:
C0037299
Disease or Syndrome
A discontinuity of the skin exhibiting complete loss of the epidermis and often portions of the dermis and even subcutaneous fat.
Pachyderma
MedGen UID:
90736
Concept ID:
C0334008
Finding
Laminar thickening of skin.

Recent clinical studies

Etiology

Kreicher KL, Kurlander DE, Gittleman HR, Barnholtz-Sloan JS, Bordeaux JS
Dermatol Surg 2016 Jan;42 Suppl 1:S24-31. doi: 10.1097/DSS.0000000000000300. PMID: 26730971
Woo KJ, Bang SI, Mun GH, Oh KS, Pyon JK, Lim SY
J Plast Reconstr Aesthet Surg 2016 Mar;69(3):395-401. Epub 2015 Oct 30 doi: 10.1016/j.bjps.2015.10.027. [Epub ahead of print] PMID: 26646402
Zheng Z, Piao J, Lee JH, Kim SE, Kim SC, Chung KY, Roh MR
Yonsei Med J 2015 Mar;56(2):440-6. doi: 10.3349/ymj.2015.56.2.440. PMID: 25683993Free PMC Article
Hoesly PM, Lowe GC, Lohse CM, Brewer JD, Lehman JS
J Am Acad Dermatol 2015 Mar;72(3):419-25. Epub 2015 Jan 10 doi: 10.1016/j.jaad.2014.11.020. [Epub ahead of print] PMID: 25582537
Liang CA, Jambusaria-Pahlajani A, Karia PS, Elenitsas R, Zhang PD, Schmults CD
J Am Acad Dermatol 2014 Oct;71(4):781-6. Epub 2014 Apr 19 doi: 10.1016/j.jaad.2014.03.018. [Epub ahead of print] PMID: 24755121

Diagnosis

Bonadies A, Elia F, Solivetti FM, Vidiri A, Muscardin L, Bucher S
Anticancer Res 2015 Nov;35(11):6121-6. PMID: 26504038
Saiag P, Grob JJ, Lebbe C, Malvehy J, del Marmol V, Pehamberger H, Peris K, Stratigos A, Middelton M, Basholt L, Testori A, Garbe C
Eur J Cancer 2015 Nov;51(17):2604-8. Epub 2015 Jul 16 doi: 10.1016/j.ejca.2015.06.108. [Epub ahead of print] PMID: 26189684
Hoesly PM, Lowe GC, Lohse CM, Brewer JD, Lehman JS
J Am Acad Dermatol 2015 Mar;72(3):419-25. Epub 2015 Jan 10 doi: 10.1016/j.jaad.2014.11.020. [Epub ahead of print] PMID: 25582537
Karanian M, Pérot G, Coindre JM, Chibon F, Pedeutour F, Neuville A
Mod Pathol 2015 Feb;28(2):230-7. Epub 2014 Aug 1 doi: 10.1038/modpathol.2014.97. [Epub ahead of print] PMID: 25081750
Millare GG, Guha-Thakurta N, Sturgis EM, El-Naggar AK, Debnam JM
AJNR Am J Neuroradiol 2014 Feb;35(2):373-8. Epub 2013 Aug 1 doi: 10.3174/ajnr.A3650. [Epub ahead of print] PMID: 23907249

Therapy

Woo KJ, Bang SI, Mun GH, Oh KS, Pyon JK, Lim SY
J Plast Reconstr Aesthet Surg 2016 Mar;69(3):395-401. Epub 2015 Oct 30 doi: 10.1016/j.bjps.2015.10.027. [Epub ahead of print] PMID: 26646402
Rutkowski P, Debiec-Rychter M
Expert Rev Anticancer Ther 2015;15(8):901-9. Epub 2015 May 31 doi: 10.1586/14737140.2015.1052799. [Epub ahead of print] PMID: 26027711
Bashir S, Tariq M, Aslam HM, Hashmi AS, Malik B, Amin A, Mumtaz S
J Med Case Rep 2015 Apr 29;9:96. doi: 10.1186/s13256-015-0561-4. [Epub ahead of print] PMID: 25924890Free PMC Article
Karanian M, Pérot G, Coindre JM, Chibon F, Pedeutour F, Neuville A
Mod Pathol 2015 Feb;28(2):230-7. Epub 2014 Aug 1 doi: 10.1038/modpathol.2014.97. [Epub ahead of print] PMID: 25081750
Loghdey MS, Varma S, Rajpara SM, Al-Rawi H, Perks G, Perkins W
J Plast Reconstr Aesthet Surg 2014 Oct;67(10):1315-21. Epub 2014 May 23 doi: 10.1016/j.bjps.2014.05.021. [Epub ahead of print] PMID: 25012249

Prognosis

Bonadies A, Elia F, Solivetti FM, Vidiri A, Muscardin L, Bucher S
Anticancer Res 2015 Nov;35(11):6121-6. PMID: 26504038
Saiag P, Grob JJ, Lebbe C, Malvehy J, del Marmol V, Pehamberger H, Peris K, Stratigos A, Middelton M, Basholt L, Testori A, Garbe C
Eur J Cancer 2015 Nov;51(17):2604-8. Epub 2015 Jul 16 doi: 10.1016/j.ejca.2015.06.108. [Epub ahead of print] PMID: 26189684
Hoesly PM, Lowe GC, Lohse CM, Brewer JD, Lehman JS
J Am Acad Dermatol 2015 Mar;72(3):419-25. Epub 2015 Jan 10 doi: 10.1016/j.jaad.2014.11.020. [Epub ahead of print] PMID: 25582537
Monteagudo C, Llombart B, Burgués O, Rubio L, Calabuig S, Sanmartín O, López-Guerrero JA
J Cutan Pathol 2015 Mar;42(3):206-12. Epub 2014 Dec 23 doi: 10.1111/cup.12456. [Epub ahead of print] PMID: 25410704
Liang CA, Jambusaria-Pahlajani A, Karia PS, Elenitsas R, Zhang PD, Schmults CD
J Am Acad Dermatol 2014 Oct;71(4):781-6. Epub 2014 Apr 19 doi: 10.1016/j.jaad.2014.03.018. [Epub ahead of print] PMID: 24755121

Clinical prediction guides

Bonadies A, Elia F, Solivetti FM, Vidiri A, Muscardin L, Bucher S
Anticancer Res 2015 Nov;35(11):6121-6. PMID: 26504038
Socoliuc C, Zurac S, Andrei R, Stăniceanu F
Rom J Intern Med 2015 Jan-Mar;53(1):79-88. PMID: 26076565
Zheng Z, Piao J, Lee JH, Kim SE, Kim SC, Chung KY, Roh MR
Yonsei Med J 2015 Mar;56(2):440-6. doi: 10.3349/ymj.2015.56.2.440. PMID: 25683993Free PMC Article
Santos-Briz A, Riveiro-Falkenbach E, Román-Curto C, Mir-Bonafé JM, Acquadro F, Mentzel T
Am J Dermatopathol 2014 Nov;36(11):920-4. doi: 10.1097/DAD.0000000000000028. PMID: 25072679
Chaput B, Filleron T, Le Guellec S, Meresse T, Courtade-Saïdi M, Grolleau JL, Chevreau C, Garrido I, Gangloff D
Ann Chir Plast Esthet 2014 Aug;59(4):219-25. Epub 2014 Jan 7 doi: 10.1016/j.anplas.2013.11.001. [Epub ahead of print] PMID: 24411817

Recent systematic reviews

Saiag P, Grob JJ, Lebbe C, Malvehy J, del Marmol V, Pehamberger H, Peris K, Stratigos A, Middelton M, Basholt L, Testori A, Garbe C
Eur J Cancer 2015 Nov;51(17):2604-8. Epub 2015 Jul 16 doi: 10.1016/j.ejca.2015.06.108. [Epub ahead of print] PMID: 26189684
Liang CA, Jambusaria-Pahlajani A, Karia PS, Elenitsas R, Zhang PD, Schmults CD
J Am Acad Dermatol 2014 Oct;71(4):781-6. Epub 2014 Apr 19 doi: 10.1016/j.jaad.2014.03.018. [Epub ahead of print] PMID: 24755121
Foroozan M, Sei JF, Amini M, Beauchet A, Saiag P
Arch Dermatol 2012 Sep;148(9):1055-63. doi: 10.1001/archdermatol.2012.1440. PMID: 22986859
Miller SJ, Alam M, Andersen JS, Berg D, Bichakjian CK, Bowen GM, Cheney RT, Glass LF, Grekin RC, Ho AL, Kessinger A, Liegeois N, Lydiatt DD, Michalski J, Morrison WH, Nehal KS, Nelson KC, Nghiem P, Olencki T, Perlis CS, Shaha AR, Tuli M, Urist MM, Wang LC, Zic JA; National Comprehensive Cancer Network
J Natl Compr Canc Netw 2012 Mar;10(3):312-8. PMID: 22393193
Voth H, Landsberg J, Hinz T, Wenzel J, Bieber T, Reinhard G, Höller T, Wendtner CM, Schmid-Wendtner MH
J Eur Acad Dermatol Venereol 2011 Dec;25(12):1385-91. Epub 2011 Jun 4 doi: 10.1111/j.1468-3083.2011.04141.x. [Epub ahead of print] PMID: 21645124

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