Skip to main content

Advertisement

SpringerLink
Log in

Remission with Imatinib mesylate treatment in a patient with initially unresectable dermatofibrosarcoma protuberans—a case report

  • Case Report
  • Published:
Oral and Maxillofacial Surgery Aims and scope Submit manuscript

Abstract

Background

Dermatofibrosarcoma protuberans (DFSP) is a rare, malignant dermal mesenchymal neoplasm characterized by a slow, infiltrative growth. These neoplasms have a high tendency to recur locally after surgical excision. However, metastasizing cases are exceedingly rare. Cytogenetically, DFSP is characterized by a t(17;22)(22;q13) aberration with fusion of the COL1A1 gene on chromosome 17 with the PDGFB gene on chromosome 22. Here, we report on a successful treatment of a patient with a targeted therapy using the tyrosine kinase inhibitor Imatinib mesylate in neoadjuvant intention.

Patients and methods

A patient with recurrent and initially unresectable but non-metastatic DFSP of the scalp received Imatinib over 3 months with increasing dosage from 400 mg/day to 800 mg/day orally. Due to the location of the DFSP in our patient, we intended to decrease tumor size preoperatively to allow complete surgical resection. Response to therapy was assessed by computed tomography.

Results

Preoperative treatment with Imatinib resulted in decrease of tumor size by over 60% in the greatest dimension during 3 months of therapy, enabling the complete resection of the DFSP by radical surgery with achieving an acceptable cosmetic result. Surgery was followed by adjuvant Imatinib therapy over 6 months.

Conclusions

Imatinib mesylate is effective in neoadjuvant treatment of primary unresectable dermatofibrosarcoma protuberans and can be considered as a useful option in the therapy regimen.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3
Fig. 4
Fig. 5

References

  1. 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

    Article  PubMed  CAS  Google Scholar 

  2. Barnes L, Coleman JA, Johnson JT (1984) Dermatofibrosarcoma protuberans of the head and neck. Arch Otolaryngol 1103:98–404

    PubMed  CAS  Google Scholar 

  3. Bowne WB, Antonescu CR, Leung DHY, Katz SC, Hawkins WG, Woodruff JM, Brennan MF, Lewis JJ (2000) Dermatofibrosarcoma protuberans. A clinicopathologic analysis of patients treated and followed at a single institution. Cancer 88:2711–2720

    Article  PubMed  CAS  Google Scholar 

  4. DuBay D, Cimmino V, Lowe L, Johnson TM, Sondak VK (2004) Low recurrence rate after surgery for dermatofibrosarcoma protuberans: a multidisciplinary approach from a single institution. Cancer 100:1008–1016

    Article  PubMed  Google Scholar 

  5. 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

    Article  PubMed  Google Scholar 

  6. Gloster HM Jr, Harris KR, Roenigk RK (1996) A comparison between Mohs micrographic surgery and wide surgical excision for the treatment of dermatofibrosarcoma protuberans. J Am Acad Dermatol 35:82–7

    Article  PubMed  Google Scholar 

  7. Greco A, Roccato E, Miranda C, Cleris L, Formelli F, Pierotti MA (2001) Growth-inhibitory effect of STI571 on cells transformed by the COL1A/PDGFB rearrangement. Int J Cancer 92:354–360

    Article  PubMed  CAS  Google Scholar 

  8. Kasper B, Lossignol D, Gil T, Flamen P, De Saint Aubain N, Awada A (2006) Imatinib mesylate in a patient with metastatic disease originating from a Dermatofibrosarcoma protuberans of the scalp. Anti-Cancer Drugs 17:1223–1225

    Article  PubMed  CAS  Google Scholar 

  9. Kiuru-Kuhlefelt S, El-Rifai W, Fanburg-Smith J, Kere J, Miettinen M, Knuutila S (2001) Concomitant DNA copy number amplification at 17q and 22q in dermatofibrosarcoma protuberans. Cytogenet Cell Genet 92:192–195

    Article  PubMed  CAS  Google Scholar 

  10. Loss L, Zeitouni NC (2005) Management of scalp dermatofibrosarcoma protuberans. Dermatol Surg 31:1428–1433

    PubMed  CAS  Google Scholar 

  11. McArthur GA (2006) Dermatofibrosarcoma protuberans: a surgical disease with a molecular savior. Curr Opin Oncol 18:341–346

    Article  PubMed  Google Scholar 

  12. McArthur GA, Demetri GD, van Oosterom A, Heinrich MC, Debiec-Rychter M, Corless CL, Nikolova Z, Dimitrijevic S, Fletcher JA (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

    Article  PubMed  CAS  Google Scholar 

  13. Mentzel T, Beham A, Katenkamp D, Dei Tos AP, Fletcher CD (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

    Article  PubMed  CAS  Google Scholar 

  14. O’Brien KP, Seroussi E, Dal Cin P, Sciot R, Mandahl N, Fletcher JA, Turc-Carel C, Dumanski JP (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

    Article  PubMed  CAS  Google Scholar 

  15. Shimizu A, O’Brien KP, Sjoblom T, Pietras K, Buchdunger E, Collins VP, Heldin CH, Dumanski JP, Ostman A (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-BBCancer Res 59:3719–3723

    PubMed  CAS  Google Scholar 

  16. Sirvent N, Maire G, Pedeutour F (2003) Genetics of dermatofibrosarcoma protuberans family of tumors: from ring chromosomes to tyrosine kinase inhibitor treatment. Genes Chromosom Cancer 37:1–19

    Article  PubMed  CAS  Google Scholar 

  17. Sjöblom T, Shimizu A, O’Brien KP, Pietras K, Dal Cin P, Buchdunger E, Dumanski JP, Ostman A, Heldin CH (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

    PubMed  Google Scholar 

  18. Sun LM, Wang CJ, Huang CC, Leung SW, Chen HC, Fang FM, Huang EY, Lee SP (2000) Dermatofibrosarcoma protuberans: treatment results of 35 cases. Radiother Oncol 57:175–181

    Article  PubMed  CAS  Google Scholar 

  19. Takahira T, Oda Y, Tamiya S, Higaki K, Yamamoto H, Kobayashi C, Izumi T, Tateishi N, Iwamoto Y, Tsuneyoshi M (2007) Detection of COL1A1-PDGFB fusion transcripts and PDGFB/PDGFRB mRNA expression in dermatofibrosarcoma protuberans. Mod Pathol 20:668–675

    Article  PubMed  CAS  Google Scholar 

  20. Ugurel S, Utikal J, Mohr P, Helmbold C, Pfoehler C, Schiller M, Kellner I, Schadendorf D (2006) Imatinib in locally advanced dermatofibrosarcoma protuberans (DFSP): a phase II trial of the Dermatologic Cooperative Oncology Group (DeCOG). J Clin Oncol ASCO Annual Meeting Proceedings Part I. Vol 24, No. 18S (June 20 Supplement):9561

Download references

Author information

Authors and Affiliations

  1. Department of Internal Medicine II (Oncology/Hematology), University of Jena, Erlanger Allee 101, 07740, Jena, Germany

    Doreen Lemm, Lars-Olof Muegge & Klaus Hoeffken

  2. Department of Maxillofacial and Plastic Surgery, University of Jena, Jena, Germany

    Michael Thorwarth & Stefan Schultze-Mosgau

  3. Department of Pathology, University of Jena, Jena, Germany

    Talal Aklan

  4. Dermatopathologische Gemeinschaftspraxis Friedrichshafen, Friedrichshafen, Germany

    Thomas Mentzel

Authors
  1. Doreen Lemm
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Lars-Olof Muegge
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Klaus Hoeffken
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Talal Aklan
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Thomas Mentzel
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Michael Thorwarth
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Stefan Schultze-Mosgau
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Doreen Lemm.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Lemm, D., Muegge, LO., Hoeffken, K. et al. Remission with Imatinib mesylate treatment in a patient with initially unresectable dermatofibrosarcoma protuberans—a case report. Oral Maxillofac Surg 12, 209–213 (2008). https://doi.org/10.1007/s10006-008-0130-8

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s10006-008-0130-8

Keywords

Search

Navigation