Pulmonary metastasis of a Dermatofibrosarcoma of Darier and Ferrand treated by IMATINIB: a case report

Pulmonary metastasis of a Dermatofibrosarcoma of Darier and Ferrand treated by IMATINIB: a case report


1Department of Medical Oncology, National Institute of Oncology, CHU Rabat, Morocco.
2Departement of Surgery, First Medical and Surgical Center, Agadir, Morocco.
3Department of Medical Oncology, FMPM, CHU Marrakech, Morocco.

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Dermatofibrosarcoma (DFS) described by Darier and Ferrand represents less than 5% of soft tissue sarcomas and metastases only very rarely.
We report the case of a 51-year-old patient followed for six years for a DFS of the right thigh root recurrent several times after non-optimal surgical resections. The occurrence of thoracic pain associated with a cough with dyspnea, motivated the realization of a thoracic computed tomography (CT) showing a right basal pulmonary mass with pulmonary nodules. CT biopsy of one of the pulmonary nodules confirmed the metastatic nature of these lesions. The search for translocation t (17,22) could not be performed. A treatment based on imatinib has been started. The evolution was marked by the disappearance of the respiratory symptomatology and a good radiological response.
The advent of targeted therapy with imatinib transformed the prognosis for this disease, which was considered incurable at the metastatic stage.

Conflict of interest: The author declares that he has no conflict of interest

Keywords: Darierand Ferrand Dermatofibrosarcoma, Imatinib, Pulmonary Metastasis, Recurrence, Translocation.

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How to cite this article:

Kamal EL BAKRAOUI, Mohamed TARCHOULI, Ibrahim ELGHISSASSI, Rhizlane BELBARAKA, Hassan ERRIHANI.Pulmonary metastasis of a Dermatofibrosarcoma of Darier and Ferrand treated by IMATINIB: a case report. International Journal of Case Reports, 2019 4:76. DOI: 10.28933/ijcr-2019-04-2705


1. Ratner D, Thomas CO, Johnson TM, Sondak VK, Hamilton TA, Nelson BR, Swanson NA, Garcia C, Clark RE, Grande DJ: 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. 1997;37:600-613.
2. McArthur GA: Molecular targeting of dermatofibrosarcoma protuberans: a new approach to a surgical disease.J Natl Compr Canc Netw. 2007;5:557-562.
3. McArthur GA, Demetri GD, van Oosterom A, Heinrich MC, Debiec-Rychter M, Corless CL, Nikolova Z, Dimitrijevic S, Fletcher JA: Molecular and clinical analysis of locally advanced dermatofibrosarcoma protuberans treated with imatinib: Imatinib Target Exploration Consortium Study B2225. J Clin Oncol. 2005;23:866-873. https://doi.org/10.1200/JCO.2005.07.088
4. Sjoblom T, Shimizu A, O’Brien KP, Pietras K, Dal Cin P, Buchdunger E, Dumanski JP, Ostman A, Heldin CH: Growth inhibition of dermatofibrosarcoma protuberans tumors by the platelet-derived growth factor receptor antagonist STI571 through induction of apoptosis. Cancer Res. 2001;61:5778-5783.
5. Kransdorf MJ: Malignant soft-tissue tumors in a large referral population: distribution of diagnoses by age, sex, and location. AJR Am J Roentgenol. 1995;164:129-134.https://doi.org/10.2214/ajr.164.1.7998525
6. Haycox CL, Odland PB, Olbricht SM, Piepkorn M: Immunohistochemical characterization of dermatofibrosarcoma protuberans with practical applications for diagnosis and treatment. J Am Acad Dermatol. 1997;37:438-444.
7. Snow SN, Gordon EM, Larson PO, Bagheri MM, Bentz ML, Sable DB: Dermatofibrosarcoma protuberans: a report on 29 patients treated by Mohs micrographic surgery with long-term follow-up and review of the literature. Cancer. 2004;101:28-38. https://doi.org/10.1002/cncr.20316
8. Paradisi A, Abeni D, Rusciani A, Cigna E, Wolter M, Scuderi N, Rusciani L, Kaufmann R, Podda M: Dermatofibrosarcoma protuberans: wide local excision vs. Mohs micrographic surgery. Cancer Treat Rev. 2008;34:728-736. https://doi.org/10.1016/j.ctrv.2008.06.002
9. Dagan R, Morris CG, Zlotecki RA, Scarborough MT, Mendenhall WM: Radiotherapy in the treatment of dermatofibrosarcoma protuberans. Am J Clin Oncol. 2005;28:537-539.
10. Rutkowski P, Debiec-Rychter M, Nowecki Z, Michej W, Symonides M, Ptaszynski K, Ruka W: Treatment of advanced dermatofibrosarcoma protuberans with imatinib mesylate with or without surgical resection. J Eur Acad Dermatol Venereol. 2011;25:264-270.https://doi.org/10.1111/j.1468-3083.2010.03774.x
11. Arifi S, El Sayadi H, Dufresne A, Ray-Coquard I, Fayette J, Meeus P, Ranchere D, Decouvelaere AV, Alberti L, Tabone-Eglinger S, Blay JY, Cassier P: [Imatinib and solid tumours].Bull Cancer. 2008;95:99-106.https://doi.org/10.1684/bdc.2008.0557
12. Labropoulos SV, Razis ED: Imatinib in the treatment of dermatofibrosarcoma protuberans. Biologics. 2007;1:347-353.
13. Rubin BP, Schuetze SM, Eary JF, Norwood TH, Mirza S, Conrad EU, Bruckner JD: Molecular targeting of platelet-derived growth factor B by imatinib mesylate in a patient with metastatic dermatofibrosarcoma protuberans. J Clin Oncol. 2002;20:3586-3591.https://doi.org/10.1200/jco.2002.01.027
14. Noujaim J, Thway K, Fisher C, Jones RL: Dermatofibrosarcoma protuberans: from translocation to targeted therapy.Cancer Biol Med. 2015;12:375-384.https://doi.org/10.7497/j.issn.2095-3941.2015.0067
15. Rutkowski P, Van Glabbeke M, Rankin CJ, Ruka W, Rubin BP, Debiec-Rychter M, Lazar A, Gelderblom H, Sciot R, Lopez-Terrada D, Hohenberger P, van Oosterom AT, Schuetze SM: Imatinib mesylate in advanced dermatofibrosarcoma protuberans: pooled analysis of two phase II clinical trials. J Clin Oncol. 2010;28:1772-1779.https://doi.org/10.1200/jco.2009.25.7899
16. El Kacemi H, Aissa A, Bazine A, Kebdani T, Bougtab A, Benjaafar N: [Dermatofibrosarcoma protuberans: report of 38 cases].Pan Afr Med J. 2014;19:274.https://doi.org/10.11604/pamj.2014.19.274.3604
17. Rabiou S, Ouadnouni Y, Efared B, Belliraj L, Issoufou I, Ammor FZ, Ghalimi J, Lakranbi M, Sani R, Oufkir A, Smahi M: [What surgery for recurrent Darier-Ferrand sarcoma of the chest wall?]. Rev Pneumol Clin. 2017;73:100-105.https://doi.org/10.1016/j.pneumo.2016.09.005

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