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Aggressive Fibromatosis Response to Tamoxifen

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Aggressive Fibromatosis Response to Tamoxifen Libertini et al. Clin Sarcoma Res (2018) 8:13 https://doi.org/10.1186/s13569-018-0100-3 Clinical Sarcoma Research RESEARCH Open Access Aggressive fbromatosis response to tamoxifen: lack of correlation between MRI and symptomatic response M. Libertini1 , I. Mitra1,2, W. T. A. van der Graaf1,3, A. B. Miah1,3, I. Judson1,3, R. L. Jones1,3, K. Thomas2, E. Moskovic1,3, Z. Szucs1, C. Benson1 and C. Messiou1,2,3* Abstract Background: One of the commonly used systemic agents for the treatment of aggressive fbromatosis is the anti- oestrogen drug tamoxifen. However, data on efcacy and optimum methods of response assessment are limited, consisting mainly of small case series and reports. Methods: A retrospective database was used to identify consecutive patients diagnosed with aggressive fbroma- tosis (AF) and treated with tamoxifen plus/minus non-steroidal anti-infammatory drugs at our tertiary referral centre between 2007 and 2014. MRI and symptom changes were recorded. Results: Thirty-two patients (13 male 19 female, median age 41 years) were included. Median duration of treatment with tamoxifen was 316 days. Of 9 patients with progressive disease by RECIST 1.1 (28%): 4 patients experienced wors- ening symptoms; 3 patients had improved symptoms and 2 had no change in symptoms. Of 22 patients with stable disease (69%): 11 had no change in symptoms; 6 had improved symptoms and 5 patients had worsening symptoms. One patient achieved a partial response with improved symptoms. Conclusions: No relationship was identifed between symptomatic beneft and response by RECIST 1.1 on MRI. Pro- spective studies in AF should incorporate endpoints focusing on patient symptoms. Keywords: Aggressive fbromatosis, Anti-oestrogen therapy, Symptoms, MRI features, T2 weighted signal Background from any anatomical site, commonly the extremities, Aggressive fbromatosis (AF), also named desmoid-type abdominal and chest wall and paravertebral tissues [6, 7]. fbromatosis, is characterised by monoclonal myofbro- Although AF is slow growing without metastatic poten- blastic proliferation in soft tissues. It is a rare disease tial, its unpredictable behaviour, propensity for pro- accounting for 3% of all soft tissue neoplasms, with an gressive infltration and local invasion makes treatment incidence of 2–5 people per million per year [1]. It has challenging. Currently there is no established evidence- a female predominance and a peak incidence in the based approach to treatment [8], although a consensus third to fourth decades [2]. AF is often sporadic, how- approach based on wide consultation with physicians ever, there is a reported increased incidence of 3.5–32% and patient groups has been published [9]. Active surveil- in patients with familial adenomatous polyposis (FAP) lance is now often used in asymptomatic cases [10]. High or Gardner’s variant [3, 4]. AF is usually solitary but local recurrence rates of 15–50% [11–14] up to 87% [15] multifocal tumours have been reported [5]. It may arise in younger patients, despite apparently complete resec- tion, have reduced the popularity of surgical resection as initial management. Radiotherapy can help improve local *Correspondence: [email protected] control [16] however, side efects, including radiation- 2 Department of Radiology, The Royal Marsden NHS Foundation Trust, 203 induced malignancies have to be considered especially in Fulham Road, London SW3 6JJ, UK Full list of author information is available at the end of the article young patients [17]. © The Author(s) 2018. This article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/ publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated. Libertini et al. Clin Sarcoma Res (2018) 8:13 Page 2 of 7 Drugs used in the treatment of AF, include hormonal particular, the highly cellular, actively growing lesions tend therapy (e.g. tamoxifen and toremifene) [18], Non ste- to be of high signal on T2-weighted MR images [6, 36, 37]. roidal antinfammatory drugs (NSAIDs) and cytotoxic Interspersed low signal bands correspond with the colla- chemotherapeutic agents, such as anthracycline-based gen bundles. As the lesion matures, the increase in collagen regimens [19] (including pegylated liposomal doxoru- deposition and decreased cellularity result in a decrease in bicin [20]) and vinblastine plus methotrexate [21]. Tyros- T2 signal [38, 39]. However to date it has not been possible ine kinase inhibitors, including imatinib [22], sorafenib to predict behaviour based on MRI signal [40, 41]. [23] and pazopanib [24] can also play a role in the treat- Te main aims of this study were to assess MRI ment of AF. A recent phase I study demonstrated dem- response and symptom control in patients with AF onstrated promising efcacy of a γ-secretase inhibitor in treated with tamoxifen with or without NSAIDs. desmoid tumours [25]. For many centres, frst line systemic treatment of non- Methods resectable or symptomatic desmoid-type fbromatosis Patient selection consists of hormonal manipulation, with or without a Te prospectively collected Royal Marsden Hospital sar- NSAID. Particularly in centres within the United King- coma database was used to identify consecutive patients dom, this is heavily infuenced by lack of reimbursed diagnosed with AF and treated with tamoxifen at our ter- alternatives, availability and the low side efect profle. tiary referral centre between 2007 and 2014. Institutional Immunohistochemical studies have demonstrated the approval was obtained. Inclusion criteria were patients presence of oestrogen receptor-beta in 90% of desmoid- aged 18 years and over, treated with tamoxifen, with a base- type tumours [26]. Tis is supported by the tendency of line and at least one follow-up MRI scan. Demographic fbromatosis to occur more often in women, particularly data, disease characteristics, previous treatments, date of during pregnancy/within 1 year post partum [27], or on starting and stopping tamoxifen, toxicity and clinical symp- oral contraception, and there are reports of spontane- toms were collected from clinical notes. Descriptive statis- ous regression during menopause and post-partum [2, tical analysis was applied: progression-free survival (PFS) 28]. Several publications have documented the efective- was calculated using the Kaplan–Meier method. ness of hormonal manipulation in AF treatment [29–32]. Despite the lack of randomised prospective data, it has Imaging data been reported that antioestrogen therapy can be efec- Baseline MRI images, defned as the last MRI performed tive in about half of patients [18]. Tamoxifen is a non- prior to tamoxifen treatment, and subsequent follow steroidal triphenylethylene derivative that binds to up MRI images were identifed for each patient. Where oestrogen receptors. One suggested mechanism for the available a pre-baseline MRI was also collected. On aver- anti-proliferative action of tamoxifen is regulation of the age 3 (range 2–6) follow up MRIs were assessed for each synthesis of the cytokine transforming growth factor-β patient. Te minimum MRI protocol for inclusion in the (TGF-β) [32] and its receptors, which are also involved in study was axial T1W, T2W, STIR and coronal T2W and AF pathogenesis. STIR images. All images were re-reviewed by a special- Tere are several means of monitoring treatment ist soft tissue radiologist (CM). Tumour size, RECIST 1.1 response, including clinical evaluation of tumour size and assessment and T2-weighted signal changes were docu- symptoms as well as radiological. Te Response Evalu- mented at each time point. ation Criteria in Solid Tumours (RECIST 1.1) [33] are currently employed within clinical trials. Magnetic reso- Results nance imaging (MRI) has become the imaging modality Between 2007 and 2014 a total of 35 patients were treated of choice for soft tissue lesions, due to better evaluation with tamoxifen at the Royal Marsden Hospital. Baseline of the tumour and its relationship with surrounding imaging was not available for 3 patients, and they were structures such as nerves and vessels. Given the chronic therefore not eligible for this study. Of the remaining 32 nature of the disease, the lack of radiation exposure cases, the median age at the time of commencing tamox- makes MRI ideal for follow up studies. Furthermore, in ifen was 41 years (range 19–68 years). Tere was a 3:2 lesions undergoing radiation or drug therapy, MR sur- female to male ratio [19 (60%): 13 (40%)]. One patient veillance has been used to assess response to treatment (3%) had a diagnosis of FAP. Te most common site of with a decrease in T2-weighted signal and lesion size origin was limb and limb girdle (18; 56%), followed by being suggested as indicators of treatment response [34]. chest wall (5; 15%), pelvis (3; 10%), abdominal wall (3; Te variable content of spindle cells, collagen and myx- 10%), paravertebral tissues (1; 3%) and head/neck (2; 6%). oid tissue of AF correlates with the observation that these Fourteen patients (44%) received tamoxifen as frst-line lesions often show heterogeneous signals on MRI [35]. In treatment. Eighteen of 32 patients (56%) had been treated Libertini et al. Clin Sarcoma Res (2018) 8:13 Page 3 of 7 previously, with surgery, radiotherapy, steroid injections, tamoxifen because of pregnancy. Two patients died for NSAIDs or doxorubicin chemotherapy. Patient and dis- reasons unrelated to AF. One patient stopped tamoxifen ease characteristics are summarised in Table 1. due to side efects, and subsequently received an anti- Tamoxifen treatment was started due to tumour tumour necrosis factor (anti-TNF)-α agent, adalimumab growth and worsening symptoms, mostly characterised [43], for his rheumatoid arthritis and the AF decreased in by pain, including neuropathic and somatic pain, and size.
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