desmoid tumors , also known as aggressive fibromatosis , are tumors of intermediate dignity according to the 2002 who classification . these tumors grow slowly but are locally aggressive and can be potentially life threatening when infiltrating vital structures . desmoids are very rare neoplasms with an incidence of 24 cases per 1 million inhabitants per year and make up about 5% of all soft tissue tumors . the exact pathogenesis remains unclear ; trauma and surgery as well as endocrine and genetic factors seem to be implicated in the development of desmoid tumors . the therapy strategy for aggressive fibromatosis consists of local treatment with surgery and radiation [ 3 , 4 ] , systemic therapy with non - steroidal anti - inflammatory drugs ( nsaids ) , hormonal therapy   and chemotherapy [ 6 , 7 , 8 , 9 , 10 , 11 , 14 , 15 ] . small extra - abdominal tumors are potentially curative when treated with surgery with wide local excision and histopathologic negative margins . the optimal treatment strategy for advanced , inoperable desmoid tumors consists of the use of nsaids , hormonal therapy with antiestrogen compounds or progesterone   and cytotoxic chemotherapy including ifosfamide , doxorubicin , dacarbazine , methotrexate and vinblastine [ 6 , 7 , 8 , 9 , 10 , 11 , 14 , 15 ] . due to the rarity of the disease , no randomized trials are currently available comparing different chemotherapy regimens . the tyrosine kinase inhibitor imatinib shows promising results in first trials [ 12 , 13 ] . we report on a patient with an advanced desmoid tumor on the right side of the neck who was successfully treated with polychemotherapy followed by a targeted therapy with imatinib . in october 2007 , a 40-year - old male patient presented with an advanced tumor involving the right and left supraclavicular area and the upper thorax . the patient needed constant oral analgesic therapy , and due to the tumor bleeding with severe anemia , continuing administration of blood transfusions was necessary . the patient noticed a solid painless tumor on the right side of the neck and underwent surgical excision . after tumor recurrence , no further operation or other local therapy was performed . under systemic therapy with nsaids and 10 months of antiestrogen treatment with tamoxifen the patient was treated with polychemotherapy including ifosfamide , adriablastin and dacarbazine ( ifadic ) . he received 40 mg / m adriablastin on the first day and 1,500 mg / m ifosfamide and 200 mg / m dacarbazine daily for 4 days . after a febrile neutropenia , the dose of chemotherapy was consequently reduced to 75% and a granulocyte colony - stimulating factor ( altogether , 7 cycles of polychemotherapy were administered according to the ifadic - scheme . due to a thrombosis of the right leg and the cumulative dose of adriablastin with regard to cardiotoxicity , , we started a therapy with imatinib , a selective tyrosine kinase inhibitor , 2 months after the last cycle of chemotherapy . since may 2008 , the patient has been receiving 400 mg of imatinib daily orally without any severe side effects . tumor response was evaluated clinically and by magnetic resonance imaging volumetry ( mri volumetry ) . mri volumetry was performed using t2- and t1-weighted plain sequences after administration of gadolinium - diethylenetriaminepentaacetate . tumor volume ( in cubic centimeter , cc ) was calculated by a slice - by - slice evaluation by the same radiologist . in addition , at each visit images of the tumor were taken to document the treatment response . after 2 cycles of chemotherapy , mri showed an extensive tumor at the right upper thorax aperture with 20.5 cm craniocaudal  17.5 cm transversal  13.5 cm sagittal and a total tumor volume of 2,345.09 cc ( fig . due to technical problems a comparison with the mri before start of the therapy was not possible . the mri volumetry after the fifth cycle confirmed the response ( 1,898.76 cc ) , which was observed by clinical examination . at the end of chemotherapy , the desmoid tumor decreased to 1,078.78 cc with a dramatically improvement of the clinical symptoms ( fig . 1e ) . during the targeted therapy , we observed a continuous reduction of the tumor . the last mri volumetry was performed in july 2009 and showed a tumor volume of 7,445.26 cc ( fig . 1b , c ) and clinical benefit with decline of tumor - related pain and an increased mobility in the neck . the role of chemotherapy in the treatment of advanced desmoid tumors is still under discussion . due to the rarity of these lesions and the lack of clinical trials janinis et al .   published a systematic review with overall response rates to combination chemotherapy from 17 to 100% using doxorubicin , dacarbazine , methotrexate and vinca alkaloids . methotrexate in combination with vinca alkaloids ( vincristine and vinblastine ) was used in different studies [ 6 , 10 , 15 ] . published a trial in which 30 patients with advanced aggressive fibromatosis responded to low - dose methotrexate and vinblastine weekly for a median of 1 year . combination of methotrexate with vinorelbine is mentioned as an alternative with the same outcome but potentially slightly less neurotoxicity . the most commonly used treatment regimes included the combination of doxorubicin and dacarbazine , which lead to responses in about 75% of the cases [ 7 , 8 , 9 , 11 ] . patel et al .   reported death due to cardiomyopathy in a patient who received a cumulative dose of 1,000 mg / m doxorubicin for the treatment of aggressive fibromatosis . verrill et al .   showed chemosensitivity of fibromatosis cells to 4-hydroxy - ifosfamide ( the active form of ifosfamide ) in vitro . successful treatment of 6 patients with the combination of ifosfamide and etoposide was reported by okuno et al . . table 1 shows an overview of reported chemotherapeutic regimes in the treatment of desmoid tumors . imatinib is the standard first - line therapy for chronic myeloic leukemia and gastrointestinal stroma tumors . there are 2 prospective studies investigating imatinib for the treatment of aggressive fibromatosis [ 13 , 14 ] . preliminary data was presented at the american society of clinical oncology annual meeting in 2006 and 2007 ( table 2 ) . the reported patient had a massive advanced tumor and a significant limitation of the quality of life . we decided to start combination chemotherapy with the intention to achieve a quick response and improve clinical symptoms . based on the treatment of soft tissue tumors , we combined ifosfamide to doxorubicin and dacarbazine and the patient achieved a clinical benefit after the first cycle and clinical response was observed at every further clinical visit . although the desmoid tumor is cytological benign but locally aggressive and usually slowly growing , in some cases , an aggressive combination of chemotherapy is needed to obtain a quick response . therefore the ifadic - scheme could be a possible alternative for patients with advanced desmoids and clinical symptoms . due to adequate supportive therapy side effects of chemotherapy are well manageable . as a limiting factor , the potential cardiotoxicity of doxorubicin must be mentioned . the use of combination chemotherapy including doxorubicin , ifosfamide and dacarbazine in the treatment of aggressive fibromatosis should be considered for patients who suffer from unresectable , advanced disease . for stabilization of disease , further targeted therapy with imatinib presents a valid option .
desmoid tumors , also known as aggressive fibromatosis , are tumors of intermediate dignity , which grow slowly but are locally aggressive . 
 these tumors do not metastasize but can be potentially life threatening when infiltrating vital structures . 
 the therapy strategy consists of surgery , radiation and systemic therapy with non - steroidal anti - inflammatory drugs , antiestrogen compounds and cytotoxic chemotherapy . 
 we report on a 40-year - old male patient with advanced fibromatosis of the neck who has been treated with 7 cycles of polychemotherapy ( adriablastin , ifosfamide and dacarbazine ) followed by targeted therapy with imatinib . 
 tumor response was evaluated clinically and by magnetic resonance imaging . 
 the tumor decreased significantly after the first cycle of chemotherapy and tumor - related symptoms declined . 
 the response continued after switching to targeted therapy with imatinib , which is currently ongoing . 
 the best treatment for this rare tumor remains under discussion . 
 doxorubicin and dacarbazine are frequently used agents . 
 we included ifosfamide in our therapy , which is standard in the treatment of soft tissue tumors . 
 the tyrosine kinase inhibitor imatinib seems to offer new possibilities and is currently investigated in randomized trials . 
 we conclude that combination chemotherapy including doxorubicin , ifosfamide and dacarbazine in the treatment of aggressive fibromatosis should be considered for patients suffering from unresectable , advanced disease and clinical symptoms which require a rapid response to therapy .