Long-term outcomes of 47 patients with aggressive fibromatosis of the chest treated with surgery

Ma, Dongjie; Li, S.; Fu, Rochelle; Zhang, Zhi‐Qian; Cui, Yuming; Liu, H.; Meng, Yunxiao; Wang, W.; Bi, Yalan; Xiao, Yu

doi:10.1016/j.ejso.2016.06.396

Cited by 14 publications

(12 citation statements)

References 17 publications

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“…In some cases radiation therapy can be useful 97,103,112,[122][123][124][125][126][127] ; although radiation therapy has been reported to decrease local recurrence after marginal surgery in several uncontrolled studies, other retrospective studies have found no benefit. 128 The role of radiation therapy among the various treatment options remains controversial because of long-term sequelae, including edema, pain, and second malignant neoplasm. 4,19,94,97,103,112,116,121,123,[129][130][131] In 1 study of 6 radiation-induced sarcomas in patients with DTF whose original tumor had a mutation in CTNNB1, 3 had the same CTNNB1 mutation as the original DTF, and 3 had no CTNNB1 mutation, suggesting that some cases of DTF were not derived from the original DTF tumor clone.…”

Section: Evidence For Clinical Treatments Of Dtfmentioning

confidence: 99%

Biology and Treatment of Aggressive Fibromatosis or Desmoid Tumor

Skubitz

2017

Mayo Clinic Proceedings

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Aggressive fibromatosis, also known as desmoid-type fibromatosis (DTF) or desmoid tumor, is an uncommon locally invasive tumor. Because of its low incidence and variable behavior, DTF is often first seen by physicians who are not familiar with it, and recent advances in understanding this disease have led to changes in treatment approaches. The Wnt (β-catenin) pathway appears to play a key role in DTF pathogenesis, and recent studies of DTF biology suggest a possible model of DTF pathogenesis. Histologically, DTF shows a poorly circumscribed proliferation of myofibroblast-like cells with variable collagen deposition, similar to the proliferative phase of wound healing, and DTF has been associated with trauma and pregnancy. Desmoid-type fibromatosis may be a useful model of the tumor stroma in carcinomas as well as other fibrosing diseases such as progressive pulmonary fibrosis. The clinical course of DTF can vary greatly among patients, complicating the determination of the optimal treatment approach. Treatment options include surgery, nonsteroidal anti-inflammatory drugs with or without hormonal manipulation, chemotherapy, radiation therapy, and other forms of local therapy. Many treatments have been used, but these are not without toxicities. Because of the variable nature of the disease and the potential morbidity of treatment, some cases of DTF may do better without treatment; simple observation is often the best initial treatment. This review used a PubMed search from January 1, 1980, through October 31, 2016, using the terms fibromatosis and desmoid and discusses DTF disease characteristics, pathophysiology, and treatment options as well as examines several cases illustrating key points in the biology and treatment of this heterogeneous disease.

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Section: Evidence For Clinical Treatments Of Dtfmentioning

confidence: 99%

Biology and Treatment of Aggressive Fibromatosis or Desmoid Tumor

Skubitz

2017

Mayo Clinic Proceedings

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“…The overall recurrence rate after surgery ranges from 18 to 39% [7–11]. Local recurrence rate after surgery with complete resection is 7–28% [7, 8, 10, 12–16] and 26–100% with incomplete resection [7, 8, 10, 13, 14]. Moreover, surgery may have functional and esthetic consequences.…”

Section: Introductionmentioning

confidence: 99%

Medical treatment of mammary desmoid-type fibromatosis: which benefit?

et al. 2017

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BackgroundBreast fibromatosis is a rare disease characterized by monoclonal fibroblast proliferation. It has no ability to metastasize but has a high local recurrence rate and often infiltrates surrounding tissues. Surgical treatment is the reference, but recently, new targeted therapies have emerged. We report an original case of a patient with breast fibromatosis who received exclusive medical treatment. Our aim was to analyze these treatments based on the clinical and radiological outcome, iatrogenic effects, and pharmacological action.Case presentationWe report the case of a 19-year-old woman who developed a desmoid-type fibromatosis of the lower inner quadrant of the right breast, measuring 50 × 25 mm (i.e., a volume of 27.4 cm3). Initial surgery was not possible because of potential esthetic and functional prejudice. Thus, she had an exclusive medical treatment including several lines: NSAIDs with tamoxifen and triptorelin, followed by sorafenib, then interferon α2b, and finally sunitinib. With tyrosine-kinase inhibitors (TKIs) (sunitinib), a significant partial response was observed (57% reduction of the maximal tumoral volume). For each treatment, we provided the clinical and radiological outcome in association with known pharmacological action.ConclusionsTKI had been an interesting alternative option to initial surgery, providing at least a partial response and potentially allowing less mutilating surgery. However, no pharmacological mechanism can unequivocally explain TKI efficacy. In general, breast fibromatosis should be treated along with oncologist and interventional radiologists in a trans-disciplinary modality, thus offering an adapted treatment for this particular desmoid-type fibromatosis localization.

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“…This may also be the reason why we did not obtain the conclusion that adjuvant radiotherapy reduces recurrence in our study, as all of these patients had a negative margin status (R0) in our study. For the Chinese population, most studies still report that postoperative adjuvant radiotherapy cannot reduce recurrence (33,34). Among them, the study of Huang et al included 214 patients (24).…”

Section: Discussionmentioning

confidence: 99%

Development, Validation, and Visualization of A Web-Based Nomogram for Predicting the Recurrence-Free Survival Rate of Patients With Desmoid Tumors

Liu

Huang

et al. 2021

Front. Oncol.

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BackgroundSurgery is an important treatment option for desmoid tumor (DT) patients, but how to decrease and predict the high recurrence rate remains a major challenge.MethodsDesmoid tumor patients diagnosed and treated at Tianjin Cancer Institute & Hospital were included, and a web-based nomogram was constructed by screening the recurrence-related risk factors using Cox regression analysis. External validation was conducted with data from the Fudan University Shanghai Cancer Center.ResultsA total of 385 patients were identified. Finally, after excluding patients without surgery, patients who were lost to follow-up, and patients without complete resection, a total of 267 patients were included in the nomogram construction. Among these patients, 53 experienced recurrence, with a recurrence rate of 20.15%. The 3-year and 5-year recurrence-free survival (RFS) rates were 82.5% and 78%, respectively. Age, tumor diameter, admission status, location, and tumor number were correlated with recurrence in univariate Cox analysis. In multivariate Cox analysis, only age, tumor diameter and tumor number were independent risk factors for recurrence and were then used to construct a web-based nomogram to predict recurrence. The concordance index (C-index) of the nomogram was 0.718, and the areas under the curves (AUCs) of the 3-year and 5-year receiver operating characteristic (ROC) curves were 0.751 and 0.761, respectively. In the external validation set, the C-index was 0.706, and the AUCs of the 3-year and 5-year ROC curves are 0.788 and 0.794, respectively.ConclusionsAge, tumor diameter, and tumor number were independent predictors of recurrence for DTs, and a web-based nomogram containing these three predictors could accurately predict RFS (https://stepforward.shinyapps.io/Desmoidtumor/).

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Long-term outcomes of 47 patients with aggressive fibromatosis of the chest treated with surgery

Cited by 14 publications

References 17 publications

Biology and Treatment of Aggressive Fibromatosis or Desmoid Tumor

Biology and Treatment of Aggressive Fibromatosis or Desmoid Tumor

Medical treatment of mammary desmoid-type fibromatosis: which benefit?

Development, Validation, and Visualization of A Web-Based Nomogram for Predicting the Recurrence-Free Survival Rate of Patients With Desmoid Tumors

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