Sporadic Abdominal Wall Desmoid type Fibromatosis: treatment paradigm after thirty two years

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dc.contributor Sistema FMUSP-HC: Faculdade de Medicina da Universidade de São Paulo (FMUSP) e Hospital das Clínicas da FMUSP
dc.contributor.author COUTO NETTO, S. D. FMUSP-HC
TEIXEIRA, F. FMUSP-HC
MENEGOZZO, C. A. M. FMUSP-HC
LEAO-FILHO, H. M. FMUSP-HC
ALBERTINI, A.
FERREIRA, F. O. FMUSP-HC
AKAISHI, E. H. FMUSP-HC
UTIYAMA, E. M. FMUSP-HC
dc.date.issued 2018
dc.identifier.citation BMC SURGERY, v.18, article ID 37, 7p, 2018
dc.identifier.issn 1471-2482
dc.identifier.uri http://observatorio.fm.usp.br/handle/OPI/27116
dc.description.abstract Background: Desmoid-type fibromatosis is a benign mesenchymal neoplastic process. It exhibits an uncertain growth pattern and high recurrence rate. Previously radical surgical resection was the mainstay of treatment but recently more surgeons are opting for conservative management with observation (""wait and see"" policy). The authors intend to evaluate different therapeutic modalities and ontological outcomes for abdominal wall desmoid tumors. Methods: We performed a retrospective study of patients who underwent surgical, hormonal or chemotherapy treatment for abdominal wall desmoid tumors between 1982 to 2014 at two institutions affiliated with the University of Sao Paulo, Brazil. Results: In the study period, 32 patients were included. Twenty-seven patients had surgery upfront. Of those, 89% were women with a median age of 33 years. Mean tumor size was 10 cm. Pathology confirmed free margins in 92% of resections. Tumor recurrence rate was 11%, with median relapse-free survival being 24 months. Multivariate analysis showed that positive final margins (p < 0.001) and positive frozen section (p = 0.001) were independent predictors of recurrence. For the 5 patients who underwent pharmacological therapy, median age was 33 years and median tumor diameter before treatment was 13 cm. Four patients exhibited partial response by Response Evaluation Criteria in Solid Tumors (RECIST). The single patient who did not respond to RECIST underwent radiotherapy. Conclusion: Desmoid tumor treatment has been evolving over the past decade towards a more conservative approach. Pharmacological treatment may result in tumor size regression. When surgical excision is indicated, positive margins represent an important prognostic factor for local tumor recurrence.
dc.language.iso eng
dc.publisher BIOMED CENTRAL LTD
dc.relation.ispartof BMC Surgery
dc.rights openAccess
dc.subject Abdominal wall desmoid; Desmoid tumor; Non aggressive management; Watch and wait strategy
dc.subject.other aggressive fibromatosis; prognostic-factors; single institution; radiation-therapy; tumors; management; surgery; recurrence; resection; outcomes
dc.title Sporadic Abdominal Wall Desmoid type Fibromatosis: treatment paradigm after thirty two years
dc.type article
dc.rights.holder Copyright BIOMED CENTRAL LTD
dc.description.group LIM/62
dc.identifier.doi 10.1186/s12893-018-0367-6
dc.identifier.pmid 29879959
dc.type.category original article
dc.type.version publishedVersion
hcfmusp.author COUTO NETTO, S. D.:HC:ICHC
hcfmusp.author TEIXEIRA, F.:HC:ICESP
hcfmusp.author MENEGOZZO, C. A. M.:HC:PAHC
hcfmusp.author LEAO-FILHO, H. M.:HC:INRAD
hcfmusp.author FERREIRA, F. O.:HC:ICESP
hcfmusp.author AKAISHI, E. H.:FM:
hcfmusp.author UTIYAMA, E. M.:FM:MCG
hcfmusp.author.external · ALBERTINI, A.:Univ Sao Paulo, Hosp Clin, Div Gen Surg & Trauma, Dept Surg, Av Dr Eneas Carvalho Aguiar 255, BR-05403010 Sao Paulo, Brazil
hcfmusp.origem.id WOS:000434455000001
hcfmusp.origem.id 2-s2.0-85048171228
hcfmusp.publisher.city LONDON
hcfmusp.publisher.country ENGLAND
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dc.description.index MEDLINE
hcfmusp.citation.scopus 2
hcfmusp.citation.wos 2
hcfmusp.affiliation.country Brasil


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