There has been only one report available that focuses on the treatment with imatinib mesylate of two individual persons with aggressive fibromatosis. The authors concluded that after long-term treatment, for 9 and 11 months, with imatinib mesylate, both patients demonstrated radiographic and clinical responses. The novel therapy should be considered as salvage in patients with aggressive fibromatosis expressed platelet-derived growth factor receptor-alfa, beta (PDGFR-alfa, PDGFR-beta), and/or c-kit, whose tumors are uncontrollable by the standard management. On the other hand, the number of kinases blocked by imatinib mesylate is notching up, for instance the tyrosine kinase, which is associated with macrophage-colony stimulating factor receptor (M-CSFR).
The patient was suffering from aggressive fibromatosis after prior therapy including surgery (R2), radiotherapy, and systemic treatment with combination of tamoxifen and sulindac. The tumor specimen was immunostained for PDGFR-beta and c-kit (CD117), and PDGFR-alfa and cytokines platelet-derived growth factor-alfa and beta were not assessed. The tests for both assessed molecules revealed negative results. In spite of this, the patient underwent a unique treatment with imatinib mesylate at the dose of 400 mg orally once daily for 3 years and 2 months.
After three months of the therapy, radiographic (met criteria of SD but small decrease of the tumor was noted) and clinical responses were recorded for the first time. The same was seen after 6 and 13 months of therapy continuation with imatinib mesylate. Currently, the patient is treated with imatinib mesylate (400 mg orally once daily) without any toxicity effects. The last MRI revealed readily a smaller tumor (35 x 20 mm) after such a therapy lasted more than 3 years.
Treatment with imatinib mesylate has been a well-accepted therapy for chronic myelogenous leukemia (CML) and gastrointestinal stromal tumors (GIST). There have been established four kinases (p210(bcr/abl), c-kit, PDGFR-alfa, PDGFR-beta) suggested as the target for imatinib mesylate. Other potential targets will be discovered as it has lately been determined that M-CSFR kinase activity was blocked by imatinib mesylate. The salvage therapy for aggressive fibromatosis with imatinib mesylate seems to be an attractive opportunity for patients with the advanced disease, whose prior therapy failed.
"More recent data on molecular-based targets for systemic treatments aim to elucidate a biological rationale for these therapies  . Interestingly, Wcislo et al.  reported a case of tumor response to imatinib mesylate despite negative histologic staining for c-KIT and PDGF-beta expression, hypothesizing that the effect of this agent may be related to other unidentified kinase receptor targets. Similarly, Leithner et al.  conducted an immunohistochemical analysis of desmoid tumors, demonstrating that most tumors were estrogen receptor alpha negative and c-KIT detectable in only one out of 180 cases. "
[Show abstract][Hide abstract] ABSTRACT: To describe a difficult case of a large extra-abdominal desmoid fibroma of the posterior neck and back; to discuss the pathologic findings and treatment options of this case and to review the current literature for a rare presentation of this disease.
A case report and review of the current relevant English literature, carried out using PubMed Medline, are presented.
We present a challenging case in which a locally invasive desmoid of the posterior neck and back had grown to such an extent that complete surgical excision in one procedure was not possible.
Extra-abdominal desmoid fibromas are rare tumors with multiple treatment options. The literature supports incomplete surgical resection when necessary to reduce postoperative morbidity. Further options described for residual or recurrent disease include repeat surgical excision, radiation therapy, and possible chemotherapy. For particularly large tumors, close observation and a planned second stage procedure are an appropriate choice.
American journal of otolaryngology 09/2013; 34(6). DOI:10.1016/j.amjoto.2013.08.013 · 0.98 Impact Factor
"Most chemotherapeutic protocols use doxorubicin.9 Recently, a successful therapy of a desmoid tumour resistant to traditional chemotherapeutic regimens was reported with imatinib, a tyrosine kinase inhibitor that is successfully used in advanced gastrointestinal stromal tumours.10,11 "
[Show abstract][Hide abstract] ABSTRACT: Introduction
Mesenteric fibromatosis or intra-abdominal desmoid tumour is a rare proliferative disease affecting the mesentery. It is a locally aggressive tumour that lacks metastatic potential, but the local recurrence is common. Mesenteric fibromatosis with the intestinal involvement can be easily confused with other primary gastrointestinal tumours, especially with that of the mesenchymal origin.
We report a case of a 44-year-old female who presented with an abdominal mass that radiologically and pathologically mimicked a gastrointestinal stromal tumour.
The diagnosis of mesenteric fibromatosis should always be considered in the case of mesenchymal tumours apparently originating from the bowel wall that diffusely infiltrate the mesentery.
Radiology and Oncology 02/2011; 45(1):59-63. DOI:10.2478/v10019-010-0051-7 · 1.91 Impact Factor
"Our findings and those of others suggest that (pegylated liposomal) doxorubicin-based chemotherapeutic regimens are effective for patients with progressive, symptomatic desmoid tumours. Inspite of previous promising reports (Heinrich et al, 2006; Wcisio et al, 2007), imatinib treatment had no evident positive effects in our patients. Longterm effects of targeted therapies are yet to be evaluated. "
[Show abstract][Hide abstract] ABSTRACT: The optimal treatment of desmoid tumours is controversial. We evaluated desmoid management in Dutch familial adenomatous polyposis (FAP) patients.
Seventy-eight FAP patients with desmoids were identified from the Dutch Polyposis Registry. Data on desmoid morphology, management, and outcome were analysed retrospectively. Progression-free survival (PFS) rates and final outcome were compared for surgical vs non-surgical treatment, for intra-abdominal and extra-abdominal desmoids separately. Also, pharmacological treatment was evaluated for all desmoids.
Median follow-up was 8 years. For intra-abdominal desmoids (n=62), PFS rates at 10 years of follow-up were comparable after surgical and non-surgical treatment (33% and 49%, respectively, P=0.163). None of these desmoids could be removed entirely. Eventually, one fifth died from desmoid disease. Most extra-abdominal and abdominal wall desmoids were treated surgically with a PFS rate of 63% and no deaths from desmoid disease. Comparison between NSAID and anti-estrogen treatment showed comparable outcomes. Four of the 10 patients who received chemotherapy had stabilisation of tumour growth, all after doxorubicin combination therapy.
For intra-abdominal desmoids, a conservative approach and surgery showed comparable outcomes. For extra-abdominal and abdominal wall desmoids, surgery seemed appropriate. Different pharmacological therapies showed comparable outcomes. If chemotherapy was given for progressively growing intra-abdominal desmoids, most favourable outcomes occurred after combinations including doxorubicin.
British Journal of Cancer 11/2010; 104(1):37-42. DOI:10.1038/sj.bjc.6605997 · 4.84 Impact Factor
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