Dermatofibrosarcoma protuberans with metastases treated with methotrexate

Mendoza, Catalino B.; Gerwig, Walter H.; Watne, Alvin L.

doi:10.1016/s0002-9610(70)80162-3

Cited by 31 publications

(17 citation statements)

References 6 publications

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“…The response of metastatic DFSP to conventional chemotherapy has classically been poor, [1, 3, 27] as is supported by our small experience. Reports from Rubin, et.…”

Section: Discussionsupporting

confidence: 63%

Dermatofibrosarcoma protuberans (DFSP): Predictors of Recurrence and the Use of Systemic Therapy

et al. 2010

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BACKGROUND DFSP is an uncommon soft tissue malignancy that typically presents with local invasion but rarely metastasizes. We examine clinicopathologic factors associated with disease-free survival (DFS) in patients with primary and recurrent DFSP and evaluate responses to multimodality therapy. METHODS Patients treated for DFSP were identified in a prospectively maintained database. Clinicopathologic factors associated with DFS were analyzed using uni- and multi-variate analysis. RESULTS 244 patients with DFSP were identified. Median follow-up was 50 months. 14 patients had LR and two patients had distant recurrence (DR), with a median time to recurrence of 35 months. At time of last follow-up, 70% and 47% of patients were NED in the primary (n=197) and recurrent groups (n=47), respectively. On univariate analysis, tumor location and depth were associated with DFS in the primary group, while margin status (R1 v. R0) was associated with DFS in the LR group. On multivariate analysis, only depth (primary group) and margin status (LR group), remained significant. 22 patients had therapy other than surgical resection: 14 radiotherapy, 4 tyrosine kinase inhibitor (TKI) only, 2 conventional chemotherapy only, and 2 chemotherapy plus TKI. Responses to other therapies were variable. CONCLUSIONS DFS after treatment for DFSP is strongly predicted by tumor depth in the primary setting and margin status in recurrent tumors. The treatment for DFSP in the primary or recurrent setting is excision with negative margins, resulting in low recurrence rates and infrequent metastatic spread. Multimodality treatment, especially TKI use, can be effective, but is not curative.

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“…The response of metastatic DFSP to conventional chemotherapy has classically been poor, [1, 3, 27] as is supported by our small experience. Reports from Rubin, et.…”

Section: Discussionsupporting

confidence: 63%

Dermatofibrosarcoma protuberans (DFSP): Predictors of Recurrence and the Use of Systemic Therapy

et al. 2010

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“…An eYcient conventional chemotherapy is not known until now (Mendoza et al 1970) and only few data are available. One case report suggests responsiveness of advanced DFSP to low-dose-Methotrexate (20 mg/m 2 ) and weekly Vinblastine (6 mg/m 2 ) in an attempt to achieve disease control, a similar regimen used for desmoid tumors (Ng et al 2005).…”

Section: Conventional Chemotherapymentioning

confidence: 99%

Current treatment options in dermatofibrosarcoma protuberans

Lemm

Mügge

Mentzel

et al. 2009

J Cancer Res Clin Oncol

222

220

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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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“…Methotrexate appears to have activity in advanced, unresectable DFSP, but treatment with this agent is largely a historical footnote [36]. Molecularly targeted therapies, which produce better results with less toxicity, have revolutionized the treatment of malignancies like chronic myelogenous leukemia (CML) and gastrointestinal stromal tumor (GIST).…”

Section: Treatmentmentioning

confidence: 99%

Targeted therapy for dermatofibrosarcoma protuberans

Abrams

Schuetze

2006

Curr Oncol Rep

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Dermatofibrosarcoma protuberans (DFSP) is a rare, cutaneous tumor characterized by aggressive local invasion. Local recurrence after excision is common, especially when negative margins are not achieved. DFSP frequently exhibits translocation of chromosomes 17 and 22, t(17;22). This rearrangement fuses the collagen type Ialpha1 (COL1A1) gene to the platelet-derived growth factor B-chain (PDGFB) gene. The resultant chimeric gene causes unregulated expression of platelet-derived growth factor leading to abnormal activation of the platelet-derived growth factor receptor (PDGFR) beta tyrosine kinase through an autocrine loop. This is believed to be the critical event in DFSP tumorigenesis. Imatinib mesylate is a potent inhibitor of several protein tyrosine kinases, including the PDGFRs. Clinical evidence suggests that imatinib mesylate is a safe and effective treatment in DFSP, especially in cases of recurrent or metastatic disease. Three phase II, multicenter clinical trials are open to further investigate the role of imatinib mesylate in DFSP.

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Dermatofibrosarcoma protuberans with metastases treated with methotrexate

Cited by 31 publications

References 6 publications

Dermatofibrosarcoma protuberans (DFSP): Predictors of Recurrence and the Use of Systemic Therapy

Dermatofibrosarcoma protuberans (DFSP): Predictors of Recurrence and the Use of Systemic Therapy

Current treatment options in dermatofibrosarcoma protuberans

Targeted therapy for dermatofibrosarcoma protuberans

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