Evidence map›Paper›PMID 27891388›Full record

ArticleJournal of clinical and diagnostic research : JCDR2016

Desmoid Tumours in Familial Adenomatous Polyposis: Review of 17 Patients from a Portuguese Tertiary Center.

Marco Santos, Anabela Rocha, Vilma Martins, Marisa Santos

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Article in Journal of clinical and diagnostic research : JCDR, 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 6 papers.

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6citing papers in PubMed
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1 · What the graph read from it

What it found

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2 · The registry

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3 · Its place in the literature

Who cites it

6 citing papers in PubMed.

  1. Article
  2. Article
  3. Article
  4. The Management of Desmoid Tumors: A Retrospective Study of 30 Cases.International journal of surgical oncology · 2020
    Article
  5. Review
  6. Article
4 · The record

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5 · Who and what money

Authors and funding

4 authors.

Marco SantosGeneral Surgeons, Department of Surgery, General Surgery Service, Centro Hospitalar do Porto , Largo Prof. Abel Salazar, 4099-001, Porto, Portugal .
Anabela RochaGeneral Surgeons, Department of Surgery, General Surgery Service, Centro Hospitalar do Porto , Largo Prof. Abel Salazar, 4099-001, Porto, Portugal .
Vilma MartinsGeneral Surgeons, Department of Surgery, General Surgery Service, Centro Hospitalar do Porto , Largo Prof. Abel Salazar, 4099-001, Porto, Portugal .
Marisa SantosGeneral Surgeons, Department of Surgery, General Surgery Service, Centro Hospitalar do Porto , Largo Prof. Abel Salazar, 4099-001, Porto, Portugal .

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

introductionDesmoid Tumours (DT) are benign tumours with an estimated incidence of 2-4 per million per year. Between 7-16% of them are associated with Familial Adenomatous Polyposis (FAP) and are mostly parietal or intra-abdominal. They are a challenge in relation to their unpredictable natural course, associated complications and difficult treatment.

aimThe aim of the present study was to review the occurrence, management and follow-up of DT on FAP patients treated consecutively at a tertiary care center. MATERIALS AND

methodsA retrospective review of clinical data from patients treated consecutively between 1993 and 2014. Patients' data was gathered from clinical records. Data collection included the following variables: demographic data, genotype, FAP phenotype, data on FAP related surgery, DT diagnosis, location, size and number, DT treatment, patients' status and follow-up data.

resultsThe study population consisted of 17 patients from 9 families; with a mean age of 41 years, mostly women (59%) and most with a mutation either on codon 232 or 554. Most tumours had an intra-abdominal component (59%) with a mean size of 5cm. Fifteen patients were first treated with pharmacotherapy (Non-steroidal Anti-inflammatory Drugs (NSAIDs) and Tamoxifen). Five patients (29%) underwent surgery, 4 of them for complications of intra-abdominal tumours and 1 patient for abdominal wall tumours. Two patients underwent chemotherapy in relation to aggressive intra-abdominal disease. The mean follow-up time since diagnosis of DT was 123 months. Overall, 2 patients had remission, 11 patients had regression or stabilized disease, and 2 patients had progression. One patient died due to surgical complications.

conclusionDiagnosis of DT is based on clinical symptoms, without the need for screening, although imaging plays an important role once diagnosis is suspected. The treatment approach is conservative on most patients, leaving surgery for DT related complications. The follow-up of patients with DT is also based on clinical symptoms.

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Aggressive fibromatosisFibroproliferative diseasesInfiltrative nature

Identifiers

PMID27891388
PMCPMC5121726

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