Sporadic desmoid-type fibromatosis: a stepwise approach to a non-metastasising neoplasm—a position paper from the Italian and the French Sarcoma Group

Gronchi, Alessandro; Colombo, Chiara; Péchoux, C. Le; Tos, Angelo Paolo Dei; Cesne, Axel Le; Marrari, Andrea; Penel, Nicolas; Grignani, Giovanni; Blay, Jean‐Yves; Casali, Paolo G.; Stoeckle, Eberhard; Gherlinzoni, F.; Méeus, Pierre; Mussi, Chiara; Gouin, F.; Duffaud, Florence; Fiore, Marco; Bonvalot, Sylvie

doi:10.1093/annonc/mdt485

Cited by 150 publications

(115 citation statements)

References 54 publications

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“…2 This series confirms recent data 3 indicating the strong predominance (96 %) of females, particularly those at reproductive age, with tumors in this location. During the study period, the authors systematically performed surgical resection following a biopsy.…”

supporting

confidence: 90%

Sporadic Abdominal Wall Desmoid: Is it Time to Change Our First-Line Approach?

Bonvalot

2014

Ann Surg Oncol

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supporting

confidence: 90%

Sporadic Abdominal Wall Desmoid: Is it Time to Change Our First-Line Approach?

Bonvalot

2014

Ann Surg Oncol

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“…This shows that there is an unpredictable clinical course in desmoid tumors and justifies a "wait-and-see" policy as initial strategy. It is therefore necessary to identify patients who are at a higher risk of progression and the search for molecular markers may be a way forward (1,2).…”

Section: Discussionmentioning

confidence: 99%

“…The major obstacle in the management of desmoid tumors is their high propensity for local recurrence even after complete surgical removal. Currently, an initial "wait-and-see" policy is explored as a possible standard of care (1,2), but because desmoid tumors exhibit an unpredictable clinical course and an indistinguishable morphology, translational research is crucial to identify the predictive factors of progression in addition to the clinical parameters. A significant improvement would be to be able to detect patients who are at a higher risk of progression and those with no risk of progression.…”

Section: Introductionmentioning

confidence: 99%

Gene Expression Profiling of Desmoid Tumors by cDNA Microarrays and Correlation with Progression-Free Survival

Salas

Brulard

Terrier

et al. 2015

Clinical Cancer Research

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Purpose: Because desmoid tumors exhibit an unpredictable clinical course, translational research is crucial to identify the predictive factors of progression in addition to the clinical parameters. The main issue is to detect patients who are at a higher risk of progression. The aim of this work was to identify molecular markers that can predict progression-free survival (PFS).Experimental Design: Gene-expression screening was conducted on 115 available independent untreated primary desmoid tumors using cDNA microarray. We established a prognostic gene-expression signature composed of 36 genes. To test robustness, we randomly generated 1,000 36-gene signatures and compared their outcome association to our define 36-genes molecular signature and we calculated positive predictive value (PPV) and negative predictive value (NPV).Results: Multivariate analysis showed that our molecular signature had a significant impact on PFS while no clinical factor had any prognostic value. Among the 1,000 random signatures generated, 56.7% were significant and none was more significant than our 36-gene molecular signature. PPV and NPV were high (75.58% and 81.82%, respectively). Finally, the top two genes downregulated in no-recurrence were FECH and STOML2 and the top gene upregulated in no-recurrence was TRIP6.Conclusions: By analyzing expression profiles, we have identified a gene-expression signature that is able to predict PFS. This tool may be useful for prospective clinical studies.

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“…Although surgery remains the standard and only potentially curative therapy in the management of localized STSs and gastrointestinal stromal tumors (GISTs), 6 it is now postponed to a later line in desmoid-type fibromatosis (DF) if or until other available options fail. 7 Although they belong to the same overarching family of tumors, the natural histories, surgical principles, and sensitivity to locoregional and systemic therapies differ completely among these 3 entities; therefore, they are discussed separately in this review. The treatment of pediatric sarcomas (including primitive peripheral neuroectodermal tumors, alveolar/embryonal rhabdomyosarcomas, and desmoplastic small round cell tumors) is very different, and systemic chemotherapy (CT) is the primary therapy.…”

Section: Introductionmentioning

confidence: 99%

“…7 Prospective observational studies are currently underway to validate these results and possibly shed more light on the biologic background of this intriguing disease (ClinicalTrials.gov Identifier NCT01801176).…”

Section: Introductionmentioning

confidence: 99%

Surgical management of localized soft tissue tumors

Gronchi

Colombo

Raut

2014

Cancer

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Soft tissue tumors are rare and can arise from a variety of soft tissues and viscera in a variety of body sites. Their management requires a thorough understanding of the biology of the different histologies and molecular subtypes as well as the constraints of specific anatomic sites. The surgical approach has undergone significant changes thanks to a better understanding of the natural history of the different histologic subtypes, the importance of site, and the different sensitivity to available drugs. The soft tissue tumor family includes 3 major, distinct categories: desmoid-type fibromatosis, soft tissue sarcoma, and gastrointestinal stromal tumor. In general, limb-sparing and function-sparing approaches should be used when feasible for tumors located in the extremities and girdles. Resections of adherent structures/viscera, which may be close but not invaded, may be needed in tumors arising in the retroperitoneum/abdomen to minimize microscopic intralesional margins, maximize local tumor control, and possibly improve survival. Margins of resection and the use of adjuvant/neadjuvant radiation therapy and/or chemotherapy are contingent on an accurate histologic diagnosis. Treatment planning should include multidisciplinary consultation to determine optimal therapy, taking into consideration tumor histology, site and extent of the disease, its natural history and sensitivity to available treatments, surgical challenges, and-of course-quality of life. Cancer 2014;120:2638-48.

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Sporadic desmoid-type fibromatosis: a stepwise approach to a non-metastasising neoplasm—a position paper from the Italian and the French Sarcoma Group

Cited by 150 publications

References 54 publications

Sporadic Abdominal Wall Desmoid: Is it Time to Change Our First-Line Approach?

Sporadic Abdominal Wall Desmoid: Is it Time to Change Our First-Line Approach?

Gene Expression Profiling of Desmoid Tumors by cDNA Microarrays and Correlation with Progression-Free Survival

Surgical management of localized soft tissue tumors

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