Prognostic Value of CTNNB1 Gene Mutation in Primary Sporadic Aggressive Fibromatosis

Broekhoven, Danique L. M. van; Verhoef, Cornelis; Grünhagen, Dirk J.; Gorp, Joost M. H. H. van; Bakker, Michael A. den; Hinrichs, John W. J.; Voijs, Carmen M. A. de; Dalen, Thijs van

doi:10.1245/s10434-014-4156-x

Cited by 106 publications

(93 citation statements)

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“…The results of the present study demonstrate that this chemotherapy regimen could be effective in patients with desmoid tumors regardless of the mutation status of CTNNB1. As reported recently, a 45F mutation status of CTNNB1 was associated with a worse outcome after surgical treatment [10][11][12]28] and a worse outcome of conservative treatment with meloxicam [23] compared with other mutation statuses (41A, 45P, and WT). However, no studies have clarified the relationship between the mutation status and the efficacy of MTX and VBL chemotherapy.…”

Section: Discussionsupporting

confidence: 53%

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Low-dose chemotherapy with methotrexate and vinblastine for patients with desmoid tumors: relationship to CTNNB1 mutation status

et al. 2015

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Section: Discussionsupporting

confidence: 53%

“…No definitive conclusion has been reached regarding the significance of the histological margin status. On the other hand, the mutation status of the catenin β-1 (CTNNB1) gene was recently reported to be a significant influence on the outcome after surgical treatment [10][11][12].…”

Section: Introductionmentioning

confidence: 99%

Low-dose chemotherapy with methotrexate and vinblastine for patients with desmoid tumors: relationship to CTNNB1 mutation status

et al. 2015

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“…Los tumores desmoides representan una proliferación fibroblástica y miofibroblástica agresiva con variable producción de colágeno que, frecuentemente, aparece en la pared abdominal anterior [17][18][19][20][21][22][23][24][25][26][27][28][29][30][31][32][33][34][35] . Estas lesiones se caracterizan por tener un crecimiento infiltrativo, ausencia de evidente atipia nuclear y bajo índice mitótico.…”

Section: Tumores De Células Granularesunclassified

“…Histológicamente, consisten en una proliferación de células fusiformes de apariencia benigna separadas por fibras gruesas de colágeno ( fig. 2C y 2D) 12,13,[17][18][19][20][21][22][23][24][25] . Las células neoplásicas tienen núcleos pequeños y de contornos regulares, donde las mitosis son muy infrecuentes y no se observa necrosis (lo que apoya la naturaleza benigna de la lesión).…”

Section: Tumores De Células Granularesunclassified

Lesiones ocupantes de espacio en pared abdominal (no herniaria). La visión del patólogo

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Carbonell

2015

Revista Hispanoamericana de Hernia

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“…In addition, molecular characteristics may also differ: many IA tumors showed CTNNB1 mutation, especially T41A, a subtype associated with a low recurrence risk. [24][25][26] Last, patient characteristics are different. IA tumors presented mostly in men with a median age of 50, whereas the extra-abdominal and AW tumors have a threefold increased incidence in women with a median age of 35 years.…”

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Abdominal Desmoid Tumors: Hands Off?

2016

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The reported incidence of desmoid tumors (DT) seems to increase from about two cases per million people in 1993 to about five cases per million people in 2013. 1 Because it lacks the ability to metastasize, a desmoid tumor is classified as a benign disorder. Nevertheless, the sequelae of this disease and the applied treatments may be underlined by the fact that patients with familial adenomatous polyposis (FAP) may die from the consequences of DT. Moreover, substantial morbidity due to invasive growth is frequently seen. Because of the poor understanding of the natural history of the disease, its tumor biology, and the lack of randomized studies comparing different treatment options, the recommended treatment options vary widely depending on tumor aggressiveness, location, patient wish, and preferences of the treating physicians.With respect to the existing treatment options, surgery has been the cornerstone in treating DT. The reported recurrence rate after resection varies greatly, from 5 to 63 %, with most studies reporting recurrence rates of approximately 20 %.2-9 The prognostic value of several characteristics has been investigated. The reported results about the importance of age, location, resection margins, and adjuvant radiotherapy are conflicting. 4,7,[10][11][12] Also, radiotherapy has been widely applied for DT, both in primary and adjuvant settings. Results have been ambiguous, especially for adjuvant radiotherapy. 4,8,11,13 A study by Keus et al. addressed the role of radiotherapy in a primary setting. They reported partial and complete response in 50 % of patients and durable stable disease in 41 % of patients, with a local control rate of 81 % at 3 years. 14 Literature on systemic treatment for DT is heterogeneous and limited. Antihormonal drugs, nonsteroid antiinflammatory drugs, chemotherapy, and tyrosine kinase inhibitors have all been applied using different regimens. Most studies consist of relatively small cohorts, rendering it difficult to put results into perspective. Prospective trials are currently running with sorafenib (NCT 02066181), pazopanib (NCT01876082), sirolimus (NCT 01265030), and PF-03084014 (NCT01981551), and their results are eagerly awaited. Isolated limb perfusion is reserved for patients with advanced disease without the possibility of limb preservation in case of surgery. This procedure is only performed in specialized centers. Three European Organization for Research and Treatment of Cancer sarcoma centers reported good results, with limb preservation rates up to 88 %. 15 Key questions in managing DT are when and how to treat. Importantly, expected benefits from therapy should be well balanced against potential treatment-induced untoward effects.Recent years have seen a growing interest in a wait-andsee approach for patients presenting with DT, an approach prompted by observations of spontaneous regressions and durable disease stabilization. 3,4,16 The study by Burtenshaw et al. published in this issue of Annals of Surgical Oncology substantially adds to our knowledge...

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Prognostic Value of CTNNB1 Gene Mutation in Primary Sporadic Aggressive Fibromatosis

Abstract: The presence of specific CTNNB1 mutations was predictive for recurrence in patients after surgical treatment for primary, sporadic extra-abdominal and abdominal AF. A S45F mutation increased the risk of recurrence significantly.

Cited by 106 publications

References 19 publications

Low-dose chemotherapy with methotrexate and vinblastine for patients with desmoid tumors: relationship to CTNNB1 mutation status

Low-dose chemotherapy with methotrexate and vinblastine for patients with desmoid tumors: relationship to CTNNB1 mutation status

Lesiones ocupantes de espacio en pared abdominal (no herniaria). La visión del patólogo

Abdominal Desmoid Tumors: Hands Off?

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