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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.  http://ghr.nlm.nih.gov/condition/dermatofibrosarcoma-protuberans

Clinical features

Neoplasm of the skin
MedGen UID:
428780
Concept ID:
CN007095
Finding
A tumor (abnormal growth of tissue) of the skin.
Sarcoma
MedGen UID:
506452
Concept ID:
CN117138
Finding
A connective tissue neoplasm formed by proliferation of mesodermal cells. Bone and soft tissue sarcomas are the main types of sarcoma. Sarcoma is usually highly malignant.
Thickened skin
MedGen UID:
504710
Concept ID:
CN001007
Finding
Laminar thickening of skin.
Neoplasm of the skin
MedGen UID:
428780
Concept ID:
CN007095
Finding
A tumor (abnormal growth of tissue) of the skin.
Skin ulcer
MedGen UID:
506561
Concept ID:
CN117783
Finding
A discontinuity of the skin exhibiting complete loss of the epidermis and often portions of the dermis and even subcutaneous fat.

Recent clinical studies

Etiology

Wang C, Luo Z, Chen J, Zheng B, Zhang R, Chen Y, Shi Y
Medicine (Baltimore) 2015 May;94(17):e773. doi: 10.1097/MD.0000000000000773. PMID: 25929918
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
Socoliuc C, Zurac S, Andrei R, Stăniceanu F
Rom J Intern Med 2014 Oct-Dec;52(4):239-50. PMID: 25726626
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

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
Socoliuc C, Zurac S, Andrei R, Stăniceanu F
Rom J Intern Med 2014 Oct-Dec;52(4):239-50. PMID: 25726626
Tsai YJ, Lin PY, Chew KY, Chiang YC
J Plast Reconstr Aesthet Surg 2014 Sep;67(9):1222-9. Epub 2014 Jun 2 doi: 10.1016/j.bjps.2014.05.031. [Epub ahead of print] PMID: 24973861
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

Wang C, Luo Z, Chen J, Zheng B, Zhang R, Chen Y, Shi Y
Medicine (Baltimore) 2015 May;94(17):e773. doi: 10.1097/MD.0000000000000773. PMID: 25929918
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
Ugurel S, Mentzel T, Utikal J, Helmbold P, Mohr P, Pföhler C, Schiller M, Hauschild A, Hein R, Kämpgen E, Kellner I, Leverkus M, Becker JC, Ströbel P, Schadendorf D
Clin Cancer Res 2014 Jan 15;20(2):499-510. Epub 2013 Oct 30 doi: 10.1158/1078-0432.CCR-13-1411. [Epub ahead of print] PMID: 24173542
Akram J, Wooler G, Lock-Andersen J
J Plast Surg Hand Surg 2014 Feb;48(1):67-73. Epub 2013 Jul 9 doi: 10.3109/2000656X.2013.812969. [Epub ahead of print] PMID: 23837507
Castle KO, Guadagnolo BA, Tsai CJ, Feig BW, Zagars GK
Int J Radiat Oncol Biol Phys 2013 Jul 1;86(3):585-90. Epub 2013 Apr 26 doi: 10.1016/j.ijrobp.2013.02.024. [Epub ahead of print] PMID: 23628134

Prognosis

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
Tsai YJ, Lin PY, Chew KY, Chiang YC
J Plast Reconstr Aesthet Surg 2014 Sep;67(9):1222-9. Epub 2014 Jun 2 doi: 10.1016/j.bjps.2014.05.031. [Epub ahead of print] PMID: 24973861
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
Xu J, Li J, Zhou X, Zeng J, Yao F, Wang Y, Mu F, Niu L, Chen J, Liu J, Xu K
Cryobiology 2014 Feb;68(1):134-8. Epub 2014 Jan 21 doi: 10.1016/j.cryobiol.2014.01.009. [Epub ahead of print] PMID: 24463091
Agostini T, Dini M, Quattrini Li A, Grassetti L, Mori A, Spinelli G, Russo GL, Lazzeri D
J Craniomaxillofac Surg 2013 Oct;41(7):681-5. Epub 2013 Feb 26 doi: 10.1016/j.jcms.2013.01.009. [Epub ahead of print] PMID: 23485484

Clinical prediction guides

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
Park HJ, Nguyen JV, Miller CJ, Klein WM, Rubin AI, Elenitsas R
Am J Dermatopathol 2014 Feb;36(2):186-8. doi: 10.1097/DAD.0b013e3182a64a02. PMID: 24394304
Serra-Guillén C, Llombart B, Nagore E, Requena C, Traves V, Llorca D, Kindem S, Alcalá R, Guillén C, Sanmartín O
J Eur Acad Dermatol Venereol 2014 Aug;28(8):1012-5. Epub 2013 Aug 12 doi: 10.1111/jdv.12235. [Epub ahead of print] PMID: 23931335

Recent systematic reviews

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
McArthur GA
Curr Opin Oncol 2006 Jul;18(4):341-6. doi: 10.1097/01.cco.0000228739.62756.df. PMID: 16721128

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