Abstract
Dermatofibrosarcoma protuberans (DFSP) is a rare malignant dermal neoplasm characterized by slow infiltrative growth, little metastatic potential but a high tendency to recur locally after surgical excision. DFSP is associated with a high cure rate. The optimal therapy is complete surgical resection. The recurrence potential of DFSP is directly related to the extent of resection. The need for wide excision margins has been amply documented. Wide local excision is a frequently used practice. Mohs micrographic surgery with continuous histological margin control is further propagated to reduce local recurrence rates. In more than 90% of DFSP, a specific chromosomal aberration is described, involving Chromosomes 17 and 22. It leads to a constitutive activation of the platelet-derived growth factor receptor (PDGFR) followed by continuous stimulation of the tumor cell growth. The use of targeted inhibitors of PDGFR is a good therapeutic option in the treatment strategy of unresectable locally advanced, recurrent or metastatic disease. With Imatinib, a selective PDGFR tyrosin kinase inhibitor, partial and complete remissions of DFSP could be achieved. This article reviews the current opinion and literature about DFSP and resulting therapy strategies.
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References
Abbott JJ, Oliveira AM, Nascimento AG (2006) The prognostic significance of fibrosarcomatous transformation in dermatofibrosarcoma protuberans. Am J Surg Pathol 30:436–443. doi:10.1097/00000478-200604000-00002
Abenoza P, Lillemoe T (1993) CD34 and Factor XIIIa in the differential diagnosis of dermatofibroma and dermatofibrosarcoma protuberans. Am J Dermatopathol 15:429–434. doi:10.1097/00000372-199310000-00003
Abrams TA, Schuetze SM (2006) Targeted therapy for dermatofibrosarcoma protuberans. Curr Oncol Rep 8:291–296. doi:10.1007/s11912-006-0035-3
Ah-Weng A, Marsden JR, Sanders DS, Waters R (2002) Dermatofibrosarcoma protuberans treated by Mohs micrographic surgery. Br J Cancer 87:1386–1389. doi:10.1038/sj.bjc.6600643
Aiba S, Tabata N, Ishii H et al (1992) Dermatofibrosarcoma protuberans is a unique fibrohistiocytic tumour expressing CD34. Br J Dermatol 127:79–84. doi:10.1111/j.1365-2133.1992.tb08036.x
Ballo MT, Zagars GK, Pisters P, Pollack A (1998) The role of radiation therapy in the management of dermatofibrosarcoma protuberans. Int J Radiat Oncol Biol Phys 40:823–827. doi:10.1016/S0360-3016(97)00895-X
Barnes L, Coleman JA, Johnson JT (1984) Dermatofibrosarcoma protuberans of the head and neck. Arch Otolaryngol 111:132–138
Bednar B (1957) Storiform neurofibromas of the skin, pigmented and nonpigmented. Cancer 10:368. doi: 10.1002/1097-0142(195703/04)10:2<368::AID-CNCR2820100218>3.0.CO;2-3
Behbahani R, Patenotre P, Capon N et al (2005) Vers une réduction des marges latérales dans les dermatofibrosarcomes de Darier et Ferrand? Étude rétrospective de 34 cas. Ann Chir Plast Esthet 50:179–185. doi: 10.1016/j.anplas.2005.03.006
Bowne WB, Antonescu CR, Leung DHY et al (2000) Dermatofibrosarcoma protuberans. A clinicopathologic analysis of patients treated and followed at a single institution. Cancer 88:2711–2720. doi:10.1002/1097-0142(20000615)88:12<2711::AID-CNCR9>3.0.CO;2-M
Brenner W, Schaefler K, Chabra H, Postel A (1975) Dermatofibrosarcoma protuberans metastatic to a regional lymph node: report of a case and review. Cancer 36:1897–1902. doi:10.1002/1097-0142(197511)36:5<1897::AID-CNCR2820360547>3.0.CO;2-X
Breuninger H, Schaumburg-Lever G (1988) Control of excisional margins by conventional histopathological techniques in the treatment of skin tumours. An alternative to Moh’s technique. J Pathol 154:16–71. doi:10.1002/path.1711540209
Breuninger H, Thaller A, Schippert W (1994) Die subklinische Ausbreitung des Dermatofibrosarcoma protuberans (DFSP) und daraus resultierende Behandlungsmodalitäten. Hautarzt 45:541–545. doi:10.1007/s001050050123
Buchdunger E, Cioffi CL, Law N et al (2000) Abl protein-tyrosine kinase inhibitor STI 571 inhibits in vitro signal transduction mediated by c-kit and platelet-derived growth factor receptors. J Pharmacol Exp Ther 295:139–145
Burkhardt BR, Soule EH, Winkelman RK (1966) Dermatofibrosarcoma protuberans: study of fifty-six cases. Am J Surg 166:638–644. doi:10.1016/0002-9610(66)90031-6
Calonje E, Fletcher CDM (1996) Myoid differentiation in dermatofibrosarcoma protuberans and its fibrosarcomatous variant: clinicopathologic analysis of 5 cases. J Cutan Pathol 23:30–36. doi:10.1111/j.1600-0560.1996.tb00774.x
Chacon M, Gonzalez A, Urrutia G et al (2004) Dermatofibrosarcoma protuberans (DFSP): Mohs surgery technique and prognostic factors. J Clin Oncol, 2004 ASCO annual meeting proceedings (post-meeting edition). Vol 22, No 14S (July 15 Supplement), 2004:9066. http://meeting.ascopubs.org/cgi/content/abstract/22/14_suppl/9066
Chang CK, Jacobs IA, Salti GI (2004) Outcomes of surgery for dermatofibrosarcoma protuberans. Eur J Surg Oncol 30:341–345. doi:10.1016/j.ejso.2003.12.005
Connelly JH, Evans HL (1992) Dermatofibrosarcoma protuberans. A clinicopathologic review with emphasis on fibrosarcomatous areas. Am J Surg Pathol 16:921–925. doi:10.1097/00000478-199210000-00001
Criscione VD, Weinstock MA (2007) Descriptive epidemiology of dermatofibrosarcoma protuberans in the United States, 1973 to 2002. J Am Acad Dermatol 56:968–973. doi:10.1016/j.jaad.2006.09.006
Dagan R, Morris CG, Ra Zloteki et al (2005) Radiotherapy in the treatment of dermatofibrosarcoma protuberans. Am J Clin Oncol 28:537–539. doi:10.1097/01.coc.0000171278.69291.64
D’Andrea F, Vozza A, Brongo S, Di Girolamo F, Vozza G (2001) Dermatofibrosarcoma protuberans: experience with 14 cases. J Eur Acad Dermatol Venereol 15:427–429. doi:10.1046/j.0926-9959.2001.00307.x
Darier J, Ferrand M (1924) Dermatofibromes progressifs et recidivants ou fibrosarcomes de la peu. Ann Dermatol Syphiligr (Paris) 5:542–562
Dawes HK, Hanke CW (1996) Dermatotibrosarcoma protuberans treated with Mohs micrographic surgery—cure rates and surgical margins. J Dermatol Surg Oncol 22:530–534. doi:10.1016/1076-0512(95)00171-9
Ding J, Hashimoto H, Enjoji M (1989) Dermatofibrosarcoma protuberans with fibrosarcomatous areas. A clinicopathologic study of nine cases and a comparison with allied tumors. Cancer 64:721–729. doi:10.1002/1097-0142(19890801)64:3<721::AID-CNCR2820640326>3.0.CO;2-Q
Diwan AH, Skelton HGIII, Horenstein MG, Kelly DR, Barrett TL, Bussian AH, Sanders DY, Lazar AJ, Prieto VG, Smith KJ (2008) Dermatofibrosarcoma protuberans and giant cell fibroblastoma exhibit CD99 positivity. J Cutan Pathol 35(7):647–650. doi:10.1111/j.1600-0560.2007.00872.x
DuBay D, Cimmino V, Lowe L et al (2004) Low recurrence rate after surgery for dermatofibrosarcoma protuberans: a multidisciplinary approach from a single institution. Cancer 100:1008–1016. doi:10.1002/cncr.20051
Dupree WB, Langloss JM, Weiss SW (1985) Pigmented dermatofibrosarcoma protuberans (Bednar tumor). A pathologic, ultrastructural, and immunohistochemical study. Am J Surg Pathol 9:630–639. doi:10.1097/00000478-198509000-00002
Elgart GW, Hanly A, Busso M, Spencer JM (1999) Bednar tumor (pigmented dermatofibrosarcoma protuberans) occurring in a site of prior immunization: immunochemical findings and therapy. J Am Acad Dermatol 40:315. doi:10.1016/S0190-9622(99)70474-3
Fiore M, Miceli R, Mussi C (2005) Dermatofibrosarcoma protuberans treated at a single institution: a surgical disease with a high cure rate. J Clin Oncol 23:7669–7675. doi:10.1200/JCO.2005.02.5122
Fletcher CDM, Theaker JM, Flanagan A, Krausz T (1988) Pigmented dermatofibrosarcoma protuberans (Bednar tumour): melanocytic colonization or neuroectodermal differentiation? A clinicopathological and immunohistochemical study. Histopathology 13:631–643
Frierson HF, Cooper PH (1983) Myxoid variant of dermatofibrosarcoma protuberans. Am J Surg Pathol 7:445–450. doi:10.1097/00000478-198307000-00007
Garcia C, Clark RE, Buchanen M (1996a) Dermatofibrosarcoma protuberans. Int J Dermatol 35(12):867–871. doi:10.1111/j.1365-4362.1996.tb05053.x
Garcia C, Viehman G, Hitchcock M, Clark RE (1996b) Dermatofibrosarcoma protuberans treated with Mohs surgery. A case with CD34 immunostaining variability. Dermatol Surg 22:177–179. doi:10.1016/1076-0512(95)00499-8
George S, Demetri GD (2007) Dermatofibrosarcoma protuberans case report: when drug therapy can obviate the need for surgery. Commun Oncol 4:462–463
Gloster HM Jr (1996) Dermatofibrosarcoma protuberans. J Am Acad Dermatol 35:355–374. doi:10.1016/S0190-9622(96)90597-6
Gloster HM Jr, Harris KR, Roenigk RK et al (1996) A comparison between Mohs micrographic surgery and wide surgical excision for the treatment of dermatofibrosarcoma protuberans. J Am Acad Dermatol 35:82–87. doi:10.1016/S0190-9622(96)90597-6
Goldblum JR, Reith JD, Weiss SW (2000) Sarcomas arising in dermatofibrosarcoma protuberans: a reappraisal of biologic behavior in eighteen cases treated by wide local excision with extended clinical follow up. Am J Surg Pathol 24:1125–1130. doi:10.1097/00000478-200008000-00010
Greco A, Roccato E, Miranda C et al (2001) Growth-inhibitory effect of STI571 on cells transformed by the COL1A/PDGFB rearrangement. Int J Cancer 92:354–360. doi:10.1002/ijc.1190
Guillen DR, Cockerell CJ (2001) Cutaneous and subcutaneous sarcomas. Clin Dermatol 19:262–268. doi:10.1016/S0738-081X(01)00177-8
Haas RL, Keus RB, Loftus BM et al (1997) The role of radiotherapy in the local management of dermatofibrosarcoma protuberans. Soft tissue Tumours Working Group. Eur J Cancer 33:1055–1060. doi:10.1016/S0959-8049(97)00026-9
Har-Shai Y, Govrin-Yehudain J, Ullmann Y et al (1993) Dermatofibrosarcoma protuberans appearing during pregnancy. Ann Plast Surg 31:91–93
Haycox CL, Odland PB, Olbricht SM, Casey B (1997a) Dermatofibrosarcoma protuberans (DFSP): growth characteristics based on tumor modelling and a review of cases treated with Mohs micrographic surgery. Ann Plast Surg 38:246–251. doi:10.1097/00000637-199703000-00010
Haycox CL, Odland PB, Olbricht SM, Piepkorn M (1997b) Immunhistochemical characterization of dermatofibrosarcoma protuberans with practical applications for diagnosis and treatment. J Am Acad Dermatol 37:438–444. doi:10.1016/S0190-9622(97)70146-4
Horta M, Caetano M, Rocha N, Calhim I, Costa V, Massa A (2003) Dermatofibrosarcoma protuberans. Review of 16 cases. Skin Cancer 18:21–28
Huether MJ, Zitelli JA, Brodland DG (2001) Mohs micrographic surgery for the treatment of spindle cell tumors of the skin. J Am Acad Dermatol 44(4):656–659. doi:10.1067/mjd.2001.112381
Jimenez FJ, Grichnik JM, Buchanan MD, Clark RE (1994) Immunohistochemical margin control applied to Mohs micrographic surgical excision of dermatofibrosarcoma protuberans. J Dermatol Surg Oncol 20:687–689
Joensuu H, Roberts PJ, Sarlomo-Rikala M et al (2001) Effect of the tyrosine kinase inhibitor STI571 in a patient with a metastatic gastrointestinal stromal tumor. N Engl J Med 344:1052–1056. doi:10.1056/NEJM200104053441404
Kasper B, Lossignol D, Gil T et al (2006) Imatinib mesylate in a patient with metastatic disease originating from a dermatofibrosarcoma protuberans of the scalp. Anticancer Drugs 17:1223–1225. doi:10.1097/01.cad.0000231486.94191.65
Khatri VP, Galante JM, Bold RJ, Schneider PD, Ramsamooj R, Goodnight JE Jr (2003) Dermatofibrosarcoma protuberans: reappraisal of wide local excision and impact of inadequate initial treatment. Ann Surg Oncol 10:1118–1122. doi:10.1245/ASO.2003.03.581
Kiuru-Kuhlefelt S, El-Rifai W, Fanburg-Smith J et al (2001) Concomitant DNA copy number amplification at 17q and 22q in dermatofibrosarcoma protuberans. Cytogenet Cell Genet 92:192–195. doi:10.1159/000056901
Koh CK, Ko CB, Bury HP, Wyatt EH (1995) Dermatofibrosarcoma protuberans. Int J Dermatol 34:256–260. doi:10.1111/j.1365-4362.1995.tb01591.x
Kransdorf MJ (1995) Malignant soft-tissue tumors in a large referral population: distribution of diagnoses by age, sex and location. AJR Am J Roentgenol 164:129–134
Kricorian GJ, Schanbacher CF, Kelly AP, Bennett RG (2000) Dermatofibrosarcoma protuberans growing around plantar aponeurosis: excision by Mohs micrographic surgery. Dermatol Surg 26:941–945. doi:10.1046/j.1524-4725.2000.026010941.x
Kutzner H (1993) Expression of the human progenitor cell antigen (CD34 (HPCA-1) distinguishes dermatofibrosarcoma protuberans from fibrous histiocytoma in formalin-fixed, paraffin-embedded tissue. J Am Acad Dermatol 28:613–617
Labropoulos SV, Fletcher JA, Oliveira AM et al (2005) Sustained complete remission of metastatic dermatofibrosarcoma protuberans with imatinib mesylate. Anticancer Drugs 16:461–466. doi:10.1097/00001813-200504000-00014
Lal P, Sharma R, Mohan H, Sekhon MS (1999) Dermatofibrosarcoma protuberans metastasizing to lymph nodes: a case report and review of literature. J Surg Oncol 72:178–180. doi:10.1002/(SICI)1096-9098(199911)72:3<178::AID-JSO12>3.0.CO;2-7
Lemm D, Muegge LO, Hoeffken K, Talal A, Mentzel T, Thorwarth M, Schultze-Mosgau S (2008) Remission with Imatinib mesylate treatment in a patient with initially unresectable dermatofibrosarcoma protuberans—a case report. Oral Maxillofac Surg 12(4):209–213. doi:10.1007/s10006-008-0130-8
Lindner NJ, Scarborough MT, Powell GJ et al (1999) Revision surgery in dermatofibrosarcoma protuberans of the trunk and extremities. Eur J Surg Oncol 25:392–397. doi:10.1053/ejso.1999.0663
Loss L, Zeitouni NC (2005) Management of scalp dermatofibrosarcoma protuberans. Dermatol Surg 31:1428–1433
Macapinlac M, Elrafei T, Cunningham I, et al (2004) Dermatofibrosarcoma protuberans: neoadjuvant therapy with imatinib mesylate and use of plasma PDGF-B levels to monitor clinical response. J Clin Oncol 2004 ASCO annual meeting proceedings, vol 22, No. 14S (July 15 Supplement), p 9049
Macarenco RS, Zamolyi R, Franco MF, Nascimento AG, Abott JJ, Wang X, Erickson-Johnson MR, Oliveira AM (2008) Genomic gains of COL1A1-PDFGB occur in the histologic evolution of giant cell fibroblastoma into dermatofibrosarcoma protuberans. Genes Chromosomes Cancer 47(3):260–265. doi:10.1002/gcc.20530
Maki RG, Awan RA, Dixon RH et al (2002) Differential sensitivity to imatinib of 2 patients with metastatic sarcoma arising from dermatofibrosarcoma protuberans. Int J Cancer 100:623–626. doi:10.1002/ijc.10535
Marcus JR, Few JW, Senger C, Reynolds M (1998) Dermatofibrosarcoma protuberans and the Bednar tumor: treatment in the pediatric population. J Pediatr Surg 33:1811–1814. doi:10.1016/S0022-3468(98)90292-5
Mark RJ, Bailet JW, Tran LM, Poen J, Fu YS, Calcaterra TC (1993) Dermatofibrosarcoma protuberans of the head and neck. A report of 16 cases. Arch Otolaryngol Head Neck Surg 119(8):891–896
Marks LB, Suit H, Rosenberg AE, Wood WC (1989) Dermatofibrosarcoma protuberans treated with radiation therapy. Int J Radiat Oncol Biol Phys 17:379–384
Martin L, Piette F, Blanc P, Mortier L et al (2005) Clinical variants of the preprotuberant stage of dermatofibrosarcoma protuberans. Br J Dermatol 153:932–936. doi:10.1111/j.1365-2133.2005.06823.x
Massey RA, Tok J, Strippoli BA, Szabolcs MJ, Silvers DN, Eliezri YD (1998) A comparison of frozen and paraffin sections in dermatofibrosarcoma protuberans. Dermatol Surg 24:995–998. doi:10.1016/S1076-0512(98)00055-7
Mauro MJ, Druker BJ (2001) STI571: targeting BCR-ABL as therapy for CML. Oncologist 6:233–238. doi:10.1634/theoncologist.6-3-233
Mavili ME, Gursu KG, Gokoz A (1994) Dermatofibrosarcoma with lymph node involvement. Ann Plast Surg 32:438–440. doi:10.1097/00000637-199404000-00022
McArthur GA (2006) Dermatofibrosarcoma protuberans: a surgical disease with a molecular savior. Curr Opin Oncol 18:341–346. doi:10.1097/01.cco.0000228739.62756.df
McArthur GA (2007) Molecular targeting of dermatofibrosarcoma protuberans: a new approach to a surgical disease. J Natl Compr Canc Netw 5(5):557–562
McArthur GA, Demetri GD, van Oosterom A et al (2005) Molecular and clinical analysis of locally advanced dermatofibrosarcoma protuberans treated with Imatinib: Imatinib target exploration consortium study B2225. J Clin Oncol 23:866–873. doi:10.1200/JCO.2005.07.088
McPeak CJ, Cruz T, Nicastri AD (1967) Dermatofibrosarcoma protuberans: an analysis of 86 cases-five with metastasis. Ann Surg 166:803–816. doi:10.1097/00000658-196711000-00011
Mehrany K, Swanson NA, Heinrich MD et al (2006) Dermatofibrosarcoma protuberans: a partial response to Imatinib therapy. Dermatol Surg 32:456–459
Mendenhall WM, Zlotecki RA, Scarborough MT (2004) Dermatofibrosarcoma protuberans. Cancer 101:2503–2508. doi:10.1002/cncr.20678
Mendoza CB Jr, Gerwig WH Jr, Watne AL (1970) Dermatofibrosarcoma protuberans with metastases treated with methotrexate. Am J Surg 120:119–121. doi:10.1016/S0002-9610(70)80162-3
Mentzel T, Beham A, Katenkamp D et al (1998) Fibrosarcomatous (“high-grade”) dermatofibrosarcoma protuberans: clinicopathologic and immunohistochemical study of a series of 41 cases with emphasis on prognostic significance. Am J Surg Pathol 22:576–587. doi:10.1097/00000478-199805000-00009
Mentzel T, Scharer L, Kazakov D, Michal M (2007) Myxoid Dermatofibrosarcoma protuberans: clinicopathologic, immunohistochemical, and molecular analysis of eight cases. Am J Dermatopathol 29(5):443–448
Misset J, Kerob D, Porcher R et al (2007) Imatinib mesylate as a preoperative therapy in dermatofibrosarcoma: result of a multicentric phase II study on 25 patients. J Clin Oncol 2007, ASCO annual meeting proceedings, vol 25, No 18S (June 20 Supplement), p 10032
Mizutani K, Tamada Y, Hara K et al (2004) Imatinib mesylate inhibits the growth of metastatic lung lesions in a patient with dermatofibrosarcoma protuberans. Br J Dermatol 151:235–236. doi:10.1111/j.1365-2133.2004.06024.x
Mohs FE (1980) Chemosurgery. Clin Plast Surg 7:349–360
Naeem R, Lux ML, Huang SF, Naber SP, Corson JM, Fletcher JA (1995) Ring chromosomes in dermatofibrosarcoma protuberans are composed of interspersed sequences from chromosomes 17 and 22. Am J Pathol 147:1553–1558
Nakra T, Cook T, Douglas RS, Goldberg RA (2004) Dermatofibrosarcoma protuberans metastatic to the orbit. Arch Ophthalmol 122:1240–1241. doi:10.1001/archopht.122.8.1240
Ng A, Nishikawa H, Lander A, Grundy R (2005) Chemosensitivity in pediatric dermatofibrosarcoma protuberans. J Pediatr Hematol Oncol 27:100–102. doi:10.1097/01.mph.0000152861.05622.99
Nishio J, Iwasaki H, Ishiguro M et al (2001) Supernumerary ring chromosome in a Bednar tumor (pigmented dermatofibrosarcoma protuberans) is composed of interspersed sequences from chromosomes 17 and 22: a fluorescence in situ hybridization and comparative genomic hybridization analysis. Genes Chromosomes Cancer 30:305–309. doi:10.1002/1098-2264(2000)9999:9999<::AID-GCC1091>3.0.CO;2-R
Nouri K, Lodha R, Jimenez G, Robins P (2002) Mohs micrographic surgery for dermatofibrosarcoma protuberans: University of Miami and NYU experience. Dermatol Surg 28:1060–1064. doi:10.1046/j.1524-4725.2002.02084.x
O’Brien KP, Seroussi E, Dal Cin P et al (1998) Various regions within the alpha-helical domain of the COL1A1 gene are fused to the second exon of the PDGFB gene in dermatofibrosarcomas and giant-cell fibroblastomas. Genes Chromosomes Cancer 23:187–193. doi:10.1002/(SICI)1098-2264(199810)23:2<187::AID-GCC13>3.0.CO;2-L
Pack GT, Tabah EJ (1951) Dermatofibrosarcoma protuberans: a report of thirty-nine cases. Arch Surg 62:391–411
Pappo AS, Rao BN, Cain A et al (1997) Dermatofibrosarcoma protuberans: the pediatric experience at St. Jude Children’s Research Hospital. Pediatr Hematol Oncol 14:563–568
Parker TL, Zitelli JA (1995) Surgical margins for excision of dermatofibrosarcoma protuberans. J Am Acad Dermatol 32:233–236. doi:10.1016/0190-9622(95)90132-9
Parlette LE, Smith CK, Germain LM et al (1999) Accelerated growth of dermatofibrosarcoma protuberans during pregnancy. J Am Acad Dermatol 41:778–783. doi:10.1016/S0190-9622(99)70023-X
Patel KU, Szabo SS, Hernandez VS, Prieto VG, Abruzzo LV, Lazar AJ, López-Terrada D (2008) Dermatofibrosarcoma protuberans COL1A1-PDGFB fusion is identified in virtually all dermatofibrosarcoma protuberans cases when investigated by newly developed multiplex reverse transcription polymerase chain reaction and fluorescence in situ hybridization assays. Hum Pathol 39(2):184–193. doi:10.1016/j.humpath.2007.06.009
Popov P, Böhling T, Asko-Seljavaara S, Tukiainen E (2007) Microscopic margins and results of surgery for dermatofibrosarcoma protuberans. Plast Reconstr Surg 119:1779–1784. doi:10.1097/01.prs.0000246491.79337.25
Price VE, Fletcher JA, Zielenska M et al (2005) Imatinib mesylate: an attractive alternative in young children with large, surgically challenging dermatofibrosarcoma protuberans. Pediatr Blood Cancer 44:1–5. doi:10.1002/pbc.20249
Rademaker B, Angelillo M, Reiman HM, Jackson IT (1990) Dermatofibrosarcoma protuberans: a review of 73 cases. Eur J Plast Surg 13:251–255. doi:10.1007/BF00208325
Ratner D, Thomas CO, Johnson TM et al (1997) Mohs micrographic surgery for the treatment of dermatofibrosarcoma protuberans. Results of a multiinstitutional series with an analysis of the extent of microscopic spread. J Am Acad Dermatol 37:600–613. doi:10.1016/S0190-9622(97)70179-8
Reimann JD, Fletcher CD (2007) Myxoid dermatofibrosarcoma protuberans: a rare variant analyzed in a series of 23 cases. Am J Surg Pathol 31(9):1371–1377. doi:10.1097/PAS.0b013e31802ff7e7
Robinson JK (1985) Dermatofibrosarcoma protuberans resected by Mohs` surgery (chemosurgery). A five-year prospective study. J Am Acad Dermatol 12:1093–1098. doi:10.1016/S0190-9622(85)80193-6
Roses DF, Valensi Q, LaTrenta G, Harris MN (1986) Surgical treatment of dermatofibrosarcoma protuberans. Surg Gynecol Obstet 162(5):449–452
Rubin BP, Schuetze SM, Eary JF et al (2002) Molecular targeting of platelet-derived growth factor B by imatinib mesylate in a patient with metastatic dermatofibrosarcoma protuberans. J Clin Oncol 20:3586–3591. doi:10.1200/JCO.2002.01.027
Rutgers EJ, Kroon BB, Albus-Lutter CE, Gortzak E (1992) Dermatofibrosarcoma protuberans: treatment and prognosis. Eur J Surg Oncol 18:241–248
Sandberg AA, Bridge JA (2003) Updates on the cytogenetics and molecular genetics of bone and soft tissue tumors. Dermatofibrosarcoma protuberans and giant cell fibroblastoma. Cancer Genet Cytogenet 140:1–12. doi:10.1016/S0165-4608(02)00848-8
Savoia P, Ortoncelli M, Quaglioni P, Bernengo MG (2006) Imatinib mesylate in the treatment of a large unresectable dermatofibrosarcoma protuberans: a case study. Dermatol Surg 32:1097–1102. doi:10.1111/j.1524-4725.2006.32236.x
Shimizu A, O’Brien KP, Sjoblom T et al (1999) The dermatofibrosarcoma protuberans associated collagen type Ia1/plateletderived growth factor (PDGF) B-chain fusion gene generates a transforming protein that is processed to functional PDGF-BB. Cancer Res 59:3719–3723
Simon MP, Pedeutour F, Sirvent N et al (1997) Deregulation of the platelet-derived growth factor B-chain gene via fusion with collagen gene COL1A1 in dermatofibrosarcoma protuberans and giant-cell fibroblastoma. Nat Genet 15:95–98. doi:10.1038/ng0197-95
Sirvent N, Maire G, Pedeutour F (2003) Genetics of dermatofibrosarcoma protuberans family of tumors: from ring chromosomes to tyrosine kinase inhibitor treatment. Genes Chromosomes Cancer 37:1–19. doi:10.1002/gcc.10202
Sjöblom T, Shimizu A, O’Brien KP et al (2001) Growth inhibition of dermatofibrosarcoma protuberans tumors by the platelet-derived growth factor receptor antagonist ST1571 through induction of apoptosis. Cancer Res 61:5778–5783
Smola MG, Soyer HP, Scharnagl E (1991) Surgical treatment of dermatofibrosarcoma protuberans. A retrospective study of 20 cases with review of literature. Eur J Surg Oncol 17:447–453
Snow SN, Gordon EM, Larson PO et al (2004) Dermatofibrosarcoma protuberans: a report of 29 patients treated by Mohs micrographic surgery with long-term follow-up and review of the literature. Cancer 101:28–38. doi:10.1002/cncr.20316
Stojadinovic A, Karpoff HM, Antonescu CR et al (2000) Dermatofibrosarcoma protuberans of the head and neck. Ann Surg Oncol 7:696–704. doi:10.1007/s10434-000-0696-3
Stojadinovic A, Hoos A, Karpoff HM et al (2001) Soft tissue tumors of the abdominal wall. Analysis of disease patterns and treatment. Arch Surg 136:70–79. doi:10.1001/archsurg.136.1.70
Suit H, Spiro I, Mankin HJ et al (1996) Radiation in management of patients with dermatofibrosarcoma protuberans. J Clin Oncol 14:2365–2369
Sun LM, Wang CJ, Huang CC et al (2000) Dermatofibrosarcoma protuberans: Treatment results of 35 cases. Radiother Oncol 57:175–181. doi:10.1016/S0167-8140(00)00228-0
Szollosi Z, Nemes Z (2005) Transformed dermatofibrosarcoma protuberans: a clinicopathological study of eight cases. J Clin Pathol 58:751–756. doi:10.1136/jcp.2004.019349
Takahira T, Oda Y, Tamiya S et al (2007) Detection of COL1A1-PDGFB fusion transcripts and PDGFB/PDGFRB mRNA expression in dermatofibrosarcoma protuberans. Mod Pathol 20:668–675. doi:10.1038/modpathol.3800783
Taylor RW (1890) Sarcomatous tumors resembling in some respects keloids. Arch Dermatol 8:384–387
Taylor HB, Helwig EB (1962) Dermatofibrosarcoma protuberans: a study of 115 cases. Cancer 15:717–725. doi:10.1002/1097-0142(196207/08)15:4<717::AID-CNCR2820150405>3.0.CO;2-2
Terrier-Lacombe MJ, Guillou L, Maire G, Terrier P, Vince DR, de Saint Aubain Somerhausen N, Collin F, Pedeutour F, Coindre JM (2003) Dermatofibrosarcoma protuberans, giant cell fibroblastoma, and hybrid lesions in children: clinicopathologic comparative analysis of 28 cases with molecular data—a study from the French Federation of Cancer Centers Sarcoma Group. Am J Surg Pathol 27(1):27–39. doi:10.1097/00000478-200301000-00004
Thornton SL, Reid J, Papay FA, Vidimos AT (2005) Childhood dermatofibrosarcoma protuberans: role of preoperative imaging. J Am Acad Dermatol 53:76–83. doi:10.1016/j.jaad.2004.11.071
Tom WD, Hybarger CP, Rasgon BM (2003) Dermatofibrosarcoma protuberans of the head and neck: treatment with Mohs surgery using inverted horizontal paraffin sectioning. Laryngoscope 113:1289–1293. doi:10.1097/00005537-200308000-00004
Ugurel S (2007) Targeted therapy of dermatofibrosarcoma with imatinib. JDDG 5(3):261. doi:10.1111/j.1610-0387.2007.07009.x
Ugurel S, Utikal J, Mohr P et al (2006) Imatinib in locally advanced dermatofibrosarcoma protuberans (DFSP): a phase II trial of the Dermatologic Cooperative Oncology Group (DeCOG). 2006 ASCO annual meeting proceedings part I, vol 24, No. 18S (June 20 Supplement), p 9561
Ugurel S, Kortmann RD, Mohr P, Mentzel T, Garbe C, Breuninger H (2008) Short German guidelines: dermatofibrosarcoma protuberans. J Dtsch Dermatol Ges 6(Suppl 1):17–18
Vandeweyer E, De Saint A, Gebhart M (2002) Dermatofibrosarcoma protuberans: how wide is wide in surgical excision? Acta Chir Belg 102:455–458
Wacker J, Khan-Durani B, Hartschuh W (2004) Modified Mohs micrographic surgery in the therapy of dermatofibrosarcoma protuberans: analysis of 22 patients. Ann Surg Oncol 11:438–444. doi:10.1245/ASO.2004.06.014
Wang J, Hisaoka M, Shimajiri S, Morimitsu Y, Hashimoto H (1999) Detection of COL1A1-PDGFB fusion transcripts in dermatofibrosarcoma protuberans by reverse transcription-polymerase chain reaction using archival formalin-fixed, paraffinembedded tissues. Diagn Mol Pathol 8:113–119. doi:10.1097/00019606-199909000-00002
Weinstein JM, Drolet BA, Esterly NB, Rogers M, Bauer BS, Wagner AM, Mancini AJ (2003) Congenital dermatofibrosarcoma protuberans: variability in presentation. Arch Dermatol 139:207–211. doi:10.1001/archderm.139.2.207
West RB, Harvell J, Linn SC et al (2004) Apo D in soft tissue tumors: a novel marker for dermatofibrosarcoma protuberans. Am J Surg Pathol 28:1063–1069. doi:10.1097/01.pas.0000126857.86186.4c
Westermann GW, Buerger H, Kappes U et al (2002) Dermatofibrosarcoma protuberans with lung metastasis in a patient with progressive systemic sclerosis. South Med J 95:363–365
Wright TI, Petersen JE (2007) Treatment of recurrent dermatofibrosarcoma protuberans with imatinib mesylate, followed by Mohs micrographic surgery. Dermatol Surg 33:741–744. doi:10.1111/j.1524-4725.2007.33154.x
Wrotnowski U, Cooper PH, Shmookler BJ (1988) Fibrosarcomatous change in dermatofibrosarcoma protuberans. Am J Surg Pathol 12:287–293. doi:10.1097/00000478-198804000-00005
Yagi Y, Ueda K, Maruyama S, Noborio R (2004) Bednar tumor: a report of two cases. J Dermatol 31:484–487
Yokoyama Y, Murakami Y, Sasaki M et al (2004) Pancreatic metastasis of dermatofibrosarcoma protuberans. J Gastroenterol 39:798–800. doi:10.1007/s00535-004-1376-z
Zelger B, Sidoroff A, Stanzl U et al (1994) Deep penetrating dermatofibroma versus dermatofibrosarcoma protuberans. A clinicopathologic comparison. Am J Surg Pathol 18:677–686
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Lemm, D., Mügge, LO., Mentzel, T. et al. Current treatment options in dermatofibrosarcoma protuberans. J Cancer Res Clin Oncol 135, 653–665 (2009). https://doi.org/10.1007/s00432-009-0550-3
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DOI: https://doi.org/10.1007/s00432-009-0550-3