U.S. ResidentsUNRESECTABLE, RECURRENT, AND/OR METASTATIC DERMATOFIBROSARCOMA PROTUBERANS (DFSP)
DFSP is a cutaneous soft tissue sarcoma that is characterized by intermediate malignancy.1
DFSP arises in the dermis layer of the skin, invades the deeper subcutaneous tissue, and ultimately becomes nodular or protuberant.1,2
- Trunk: 50%-60%
- Proximal extremities: 20%-30%
- Head and neck: 10%-15%
Accurate diagnosis is often delayed, so large tumor size at diagnosis is common.5
The initial treatment for DFSP is surgical. The risk of recurrence, however, can be as high as 60% after excision.6
Epidemiology
DFSP is rare, with an estimated annual incidence of approximately 0.8 cases per million persons.3,5
It typically affects young to middle-aged adults (20-50 years old), and it occurs with almost equal frequency in men and women.3
Presenting symptoms
(A)
- DFSP initially presents as an asymptomatic, indurated plaque(s) or firm, irregular, cutaneous nodule(s)1,4
Images reproduced with permission of Richard Anderson, MD.7
(B)
- There is a slow increase in size of the sarcoma. After several years, protuberant nodules develop within the plaque, and the growth rate increases1
- Approximately 25% of patients may complain of ulceration, bleeding, or pain at lesion site1
Negative prognostic factors
Prescribing GLIVEC
PRESCRIBING GLIVEC FOR DERMATOFIBROSARCOMA PROTUBERANS (DFSP)
GLIVEC is indicated for the treatment of adult patients with unresectable DFSP and adult patients with recurrent and/or metastatic DFSP who are not eligible for surgery.1*
The recommended dose of GLIVEC is 800 mg/day for patients with DFSP.1
| Dose adjustments for neutropenia and thrombocytopenia in DFSP1 | ||
|---|---|---|
| DFSP (starting dose 800 mg) | ANC <1.0 x 109/L and/or Platelets <50 x 109/L |
|
- For patients with severe nonhematologic adverse events, hepatic insufficiency or renal insufficiency, a dose adjustment may be necessary if treatment is not tolerated1
Response rates seen in patients with DFSP receiving GLIVEC 800 mg/day in clinical trials and published case reports†
| Response in DFSP2 | |||
|---|---|---|---|
| Number of patients (N=18) | % | ||
| Complete response | 7 | 39 | |
| Partial response‡ | 8 | 44 | |
| Total responders | 15 | 83 | |
* The efficacy of GLIVEC is based on objective response rates in adult patients with DFSP. There are no controlled trials demonstrating a clinical benefit or increased survival for this disease.1
†Twelve patients aged 23 to 75 years with DFSP were studied in an open-label, multicenter, phase 2 clinical trial that was conducted testing GLIVEC in diverse populations of patients with life-threatening diseases involving Abl, KIT, or PDGFR protein tyrosine kinases. GLIVEC was dosed 800 mg daily, and median duration of response was 6.2 months. A further 6 patients aged 18 months to 49 years were reported in 5 published case reports. Adult patients received either 400 mg or 800 mg daily, and 1 pediatric patient received 400 mg/m2/daily increased to 520 mg/m2/daily. Response durations for these patients were between 4 weeks and more than 20 months.2
‡Twelve of these 18 patients either achieved a complete response (7 patients) or were made disease free by surgery after a partial response (5 patients, including 1 child), for a total complete response rate of 67%. A further 3 patients achieved a partial response, for an overall response rate of 83%. Of the 8 patients with metastatic disease, 5 responded (62%), 3 of them completely (37%). For the 10 study patients with the PDGF B gene rearrangement, there were 4 complete and 6 partial responses.2
References
References
- McArthur G. Molecularly targeted treatment for dermatofibrosarcoma protuberans. Semin Oncol. 2004;31(2)(suppl 6):30-36.
- Chen C-SJ, Siegel DM. Dermatofibrosarcoma protuberans. http://www.emedicine.com/derm/topic97.htm. Updated July 23,2010. Accessed August 17, 2010.
- Gloster HM Jr. Dermatofibrosarcoma protuberans. J Am Acad Dermatol. 1996;35(3, pt 1):355-374;quiz375-376.
- Stojadinovic A, Karpoff HM, Antonescu CR, et al. Dermatofibrosarcoma protuberans of the head and neck. Ann Surg Oncol. 2000;7(9):696-704.
- The NCCN Dermatofibrosarcoma Protuberans Clinical Practice Guidelines in Oncology (Version 1.2010). ©2010 National Comprehensive Cancer Network, Inc. http://www.nccn.org. Accessed August 27, 2010. To view the most recent and complete version of the guidelines, go online to www.nccn.org.
- Bowne WB, Antonescu CR, Leung DHY, et al. Dermatofibrosarcoma protuberans: a clinicopathologic analysis of patients treated and followed at a single institution. Cancer. 2000;88(12):2711-2720.
- Anderson R. Soft tissue pathology. Powerpoint presentation at University of Illinois College of Medicine. UIC M2 Pathology. 2004.
