LeHavreV1, France, Analysis, bibRecord, 000026

[Desmoid tumors in an adolescent girl with familial adenomatous polyposis].

Identifieur interne : 000026 ( France/Analysis ); précédent : 000025; suivant : 000027

[Desmoid tumors in an adolescent girl with familial adenomatous polyposis].

Auteurs : M. Creuzé [France] ; P. Afchain [France] ; A. Munck [France] ; J. Viala [France] ; A. Bonnard [France] ; V. Bertrand [France]

Source :

Archives de pediatrie : organe officiel de la Societe francaise de pediatrie [ 1769-664X ] ; 2016.

RBID : pubmed:27692552

Abstract

Desmoid tumors (DT) are rare and nonmetastasizing fibroblastic neoplasms, characterized by local invasiveness. They occur sporadically or arise in the context of familial adenomatous polyposis (FAP; 5-10% of cases). Most cases develop sporadically in young adults, but some cases also occur in children. We report the case of an adolescent girl with FAP and DT, and we discuss the therapeutic strategies. An adolescent girl with FAP underwent surgery at the age of 14 years with total proctocolectomy. She had a neo-mutation in the APC gene at codon 1068, which is not usually associated with DT. Three years later, she had painful defecations. Imaging showed two abdominal DT. After a multidisciplinary team meeting, the patient was refused for surgery, and medical treatment with antihormonal agents and nonsteroidal anti-inflammatory drugs was started. Imaging 18 months later showed DT stabilization, but the patient had difficulties to control chronic pains, which required morphine treatment, hypnotic sessions, and transcutaneous electric nerve stimulation. This case highlights the importance of DT screening in patients with FAP, mainly after surgery, regardless of their age and genetic mutation. Progress remains to be made in determining DT risk factors and in developing treatment. DT are still difficult to cure because of their potential for local invasion and local recurrence, and need to be managed by a multidisciplinary team.

DOI: 10.1016/j.arcped.2016.08.009
PubMed: 27692552

Affiliations:

Links to Exploration step

pubmed:27692552

Le document en format XML

<record><TEI><teiHeader><fileDesc><titleStmt><title xml:lang="en">[Desmoid tumors in an adolescent girl with familial adenomatous polyposis].</title>
<author><name sortKey="Creuze, M" sort="Creuze, M" uniqKey="Creuze M" first="M" last="Creuzé">M. Creuzé</name>
<affiliation wicri:level="3"><nlm:affiliation>Service de pédiatrie, centre hospitalier Jacques-Monod, 29, avenue Pierre-Mendès, 76600 Le Havre, France.</nlm:affiliation>
<country xml:lang="fr">France</country>
<wicri:regionArea>Service de pédiatrie, centre hospitalier Jacques-Monod, 29, avenue Pierre-Mendès, 76600 Le Havre</wicri:regionArea>
<placeName><region type="region" nuts="2">Région Normandie</region>
<region type="old region" nuts="2">Haute-Normandie</region>
<settlement type="city">Le Havre</settlement>
</placeName>
</affiliation>
</author>
<author><name sortKey="Afchain, P" sort="Afchain, P" uniqKey="Afchain P" first="P" last="Afchain">P. Afchain</name>
<affiliation wicri:level="3"><nlm:affiliation>Service d'oncologie médecine interne, hôpital St-Antoine, 184, rue du Faubourg-St-Antoine, 75571 Paris cedex 12, France.</nlm:affiliation>
<country xml:lang="fr">France</country>
<wicri:regionArea>Service d'oncologie médecine interne, hôpital St-Antoine, 184, rue du Faubourg-St-Antoine, 75571 Paris cedex 12</wicri:regionArea>
<placeName><region type="region" nuts="2">Île-de-France</region>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
</author>
<author><name sortKey="Munck, A" sort="Munck, A" uniqKey="Munck A" first="A" last="Munck">A. Munck</name>
<affiliation wicri:level="3"><nlm:affiliation>Service de gastroentérologie et nutrition pédiatrique, hôpital Robert-Debré, 7019 université Paris 7, Assistance publique-Hôpitaux de Paris, 48, boulevard Sérurier, 75935 Paris cedex 19, France.</nlm:affiliation>
<country xml:lang="fr">France</country>
<wicri:regionArea>Service de gastroentérologie et nutrition pédiatrique, hôpital Robert-Debré, 7019 université Paris 7, Assistance publique-Hôpitaux de Paris, 48, boulevard Sérurier, 75935 Paris cedex 19</wicri:regionArea>
<placeName><region type="region" nuts="2">Île-de-France</region>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
</author>
<author><name sortKey="Viala, J" sort="Viala, J" uniqKey="Viala J" first="J" last="Viala">J. Viala</name>
<affiliation wicri:level="3"><nlm:affiliation>Service de gastroentérologie et nutrition pédiatrique, hôpital Robert-Debré, 7019 université Paris 7, Assistance publique-Hôpitaux de Paris, 48, boulevard Sérurier, 75935 Paris cedex 19, France.</nlm:affiliation>
<country xml:lang="fr">France</country>
<wicri:regionArea>Service de gastroentérologie et nutrition pédiatrique, hôpital Robert-Debré, 7019 université Paris 7, Assistance publique-Hôpitaux de Paris, 48, boulevard Sérurier, 75935 Paris cedex 19</wicri:regionArea>
<placeName><region type="region" nuts="2">Île-de-France</region>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
</author>
<author><name sortKey="Bonnard, A" sort="Bonnard, A" uniqKey="Bonnard A" first="A" last="Bonnard">A. Bonnard</name>
<affiliation wicri:level="3"><nlm:affiliation>Service de chirurgie pédiatrique, hôpital Robert-Debré, 48, boulevard Sérurier, 75935 Paris cedex 19, France.</nlm:affiliation>
<country xml:lang="fr">France</country>
<wicri:regionArea>Service de chirurgie pédiatrique, hôpital Robert-Debré, 48, boulevard Sérurier, 75935 Paris cedex 19</wicri:regionArea>
<placeName><region type="region" nuts="2">Île-de-France</region>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
</author>
<author><name sortKey="Bertrand, V" sort="Bertrand, V" uniqKey="Bertrand V" first="V" last="Bertrand">V. Bertrand</name>
<affiliation wicri:level="3"><nlm:affiliation>Service de pédiatrie, centre hospitalier Jacques-Monod, 29, avenue Pierre-Mendès, 76600 Le Havre, France. Electronic address: valerie.bertrand@ch-havre.fr.</nlm:affiliation>
<country xml:lang="fr">France</country>
<wicri:regionArea>Service de pédiatrie, centre hospitalier Jacques-Monod, 29, avenue Pierre-Mendès, 76600 Le Havre</wicri:regionArea>
<placeName><region type="region" nuts="2">Région Normandie</region>
<region type="old region" nuts="2">Haute-Normandie</region>
<settlement type="city">Le Havre</settlement>
</placeName>
</affiliation>
</author>
</titleStmt>
<publicationStmt><idno type="wicri:source">PubMed</idno>
<date when="2016">2016</date>
<idno type="RBID">pubmed:27692552</idno>
<idno type="pmid">27692552</idno>
<idno type="doi">10.1016/j.arcped.2016.08.009</idno>
<idno type="wicri:Area/PubMed/Corpus">000001</idno>
<idno type="wicri:Area/PubMed/Curation">000001</idno>
<idno type="wicri:Area/PubMed/Checkpoint">000001</idno>
<idno type="wicri:Area/Ncbi/Merge">000571</idno>
<idno type="wicri:Area/Ncbi/Curation">000571</idno>
<idno type="wicri:Area/Ncbi/Checkpoint">000571</idno>
<idno type="wicri:Area/Main/Merge">000025</idno>
<idno type="wicri:Area/Main/Curation">000026</idno>
<idno type="wicri:Area/Main/Exploration">000026</idno>
<idno type="wicri:Area/France/Extraction">000026</idno>
</publicationStmt>
<sourceDesc><biblStruct><analytic><title xml:lang="en">[Desmoid tumors in an adolescent girl with familial adenomatous polyposis].</title>
<author><name sortKey="Creuze, M" sort="Creuze, M" uniqKey="Creuze M" first="M" last="Creuzé">M. Creuzé</name>
<affiliation wicri:level="3"><nlm:affiliation>Service de pédiatrie, centre hospitalier Jacques-Monod, 29, avenue Pierre-Mendès, 76600 Le Havre, France.</nlm:affiliation>
<country xml:lang="fr">France</country>
<wicri:regionArea>Service de pédiatrie, centre hospitalier Jacques-Monod, 29, avenue Pierre-Mendès, 76600 Le Havre</wicri:regionArea>
<placeName><region type="region" nuts="2">Région Normandie</region>
<region type="old region" nuts="2">Haute-Normandie</region>
<settlement type="city">Le Havre</settlement>
</placeName>
</affiliation>
</author>
<author><name sortKey="Afchain, P" sort="Afchain, P" uniqKey="Afchain P" first="P" last="Afchain">P. Afchain</name>
<affiliation wicri:level="3"><nlm:affiliation>Service d'oncologie médecine interne, hôpital St-Antoine, 184, rue du Faubourg-St-Antoine, 75571 Paris cedex 12, France.</nlm:affiliation>
<country xml:lang="fr">France</country>
<wicri:regionArea>Service d'oncologie médecine interne, hôpital St-Antoine, 184, rue du Faubourg-St-Antoine, 75571 Paris cedex 12</wicri:regionArea>
<placeName><region type="region" nuts="2">Île-de-France</region>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
</author>
<author><name sortKey="Munck, A" sort="Munck, A" uniqKey="Munck A" first="A" last="Munck">A. Munck</name>
<affiliation wicri:level="3"><nlm:affiliation>Service de gastroentérologie et nutrition pédiatrique, hôpital Robert-Debré, 7019 université Paris 7, Assistance publique-Hôpitaux de Paris, 48, boulevard Sérurier, 75935 Paris cedex 19, France.</nlm:affiliation>
<country xml:lang="fr">France</country>
<wicri:regionArea>Service de gastroentérologie et nutrition pédiatrique, hôpital Robert-Debré, 7019 université Paris 7, Assistance publique-Hôpitaux de Paris, 48, boulevard Sérurier, 75935 Paris cedex 19</wicri:regionArea>
<placeName><region type="region" nuts="2">Île-de-France</region>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
</author>
<author><name sortKey="Viala, J" sort="Viala, J" uniqKey="Viala J" first="J" last="Viala">J. Viala</name>
<affiliation wicri:level="3"><nlm:affiliation>Service de gastroentérologie et nutrition pédiatrique, hôpital Robert-Debré, 7019 université Paris 7, Assistance publique-Hôpitaux de Paris, 48, boulevard Sérurier, 75935 Paris cedex 19, France.</nlm:affiliation>
<country xml:lang="fr">France</country>
<wicri:regionArea>Service de gastroentérologie et nutrition pédiatrique, hôpital Robert-Debré, 7019 université Paris 7, Assistance publique-Hôpitaux de Paris, 48, boulevard Sérurier, 75935 Paris cedex 19</wicri:regionArea>
<placeName><region type="region" nuts="2">Île-de-France</region>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
</author>
<author><name sortKey="Bonnard, A" sort="Bonnard, A" uniqKey="Bonnard A" first="A" last="Bonnard">A. Bonnard</name>
<affiliation wicri:level="3"><nlm:affiliation>Service de chirurgie pédiatrique, hôpital Robert-Debré, 48, boulevard Sérurier, 75935 Paris cedex 19, France.</nlm:affiliation>
<country xml:lang="fr">France</country>
<wicri:regionArea>Service de chirurgie pédiatrique, hôpital Robert-Debré, 48, boulevard Sérurier, 75935 Paris cedex 19</wicri:regionArea>
<placeName><region type="region" nuts="2">Île-de-France</region>
<settlement type="city">Paris</settlement>
</placeName>
</affiliation>
</author>
<author><name sortKey="Bertrand, V" sort="Bertrand, V" uniqKey="Bertrand V" first="V" last="Bertrand">V. Bertrand</name>
<affiliation wicri:level="3"><nlm:affiliation>Service de pédiatrie, centre hospitalier Jacques-Monod, 29, avenue Pierre-Mendès, 76600 Le Havre, France. Electronic address: valerie.bertrand@ch-havre.fr.</nlm:affiliation>
<country xml:lang="fr">France</country>
<wicri:regionArea>Service de pédiatrie, centre hospitalier Jacques-Monod, 29, avenue Pierre-Mendès, 76600 Le Havre</wicri:regionArea>
<placeName><region type="region" nuts="2">Région Normandie</region>
<region type="old region" nuts="2">Haute-Normandie</region>
<settlement type="city">Le Havre</settlement>
</placeName>
</affiliation>
</author>
</analytic>
<series><title level="j">Archives de pediatrie : organe officiel de la Societe francaise de pediatrie</title>
<idno type="eISSN">1769-664X</idno>
<imprint><date when="2016" type="published">2016</date>
</imprint>
</series>
</biblStruct>
</sourceDesc>
</fileDesc>
<profileDesc><textClass></textClass>
</profileDesc>
</teiHeader>
<front><div type="abstract" xml:lang="en">Desmoid tumors (DT) are rare and nonmetastasizing fibroblastic neoplasms, characterized by local invasiveness. They occur sporadically or arise in the context of familial adenomatous polyposis (FAP; 5-10% of cases). Most cases develop sporadically in young adults, but some cases also occur in children. We report the case of an adolescent girl with FAP and DT, and we discuss the therapeutic strategies. An adolescent girl with FAP underwent surgery at the age of 14 years with total proctocolectomy. She had a neo-mutation in the APC gene at codon 1068, which is not usually associated with DT. Three years later, she had painful defecations. Imaging showed two abdominal DT. After a multidisciplinary team meeting, the patient was refused for surgery, and medical treatment with antihormonal agents and nonsteroidal anti-inflammatory drugs was started. Imaging 18 months later showed DT stabilization, but the patient had difficulties to control chronic pains, which required morphine treatment, hypnotic sessions, and transcutaneous electric nerve stimulation. This case highlights the importance of DT screening in patients with FAP, mainly after surgery, regardless of their age and genetic mutation. Progress remains to be made in determining DT risk factors and in developing treatment. DT are still difficult to cure because of their potential for local invasion and local recurrence, and need to be managed by a multidisciplinary team.</div>
</front>
</TEI>
<affiliations><list><country><li>France</li>
</country>
<region><li>Haute-Normandie</li>
<li>Région Normandie</li>
<li>Île-de-France</li>
</region>
<settlement><li>Le Havre</li>
<li>Paris</li>
</settlement>
</list>
<tree><country name="France"><region name="Région Normandie"><name sortKey="Creuze, M" sort="Creuze, M" uniqKey="Creuze M" first="M" last="Creuzé">M. Creuzé</name>
</region>
<name sortKey="Afchain, P" sort="Afchain, P" uniqKey="Afchain P" first="P" last="Afchain">P. Afchain</name>
<name sortKey="Bertrand, V" sort="Bertrand, V" uniqKey="Bertrand V" first="V" last="Bertrand">V. Bertrand</name>
<name sortKey="Bonnard, A" sort="Bonnard, A" uniqKey="Bonnard A" first="A" last="Bonnard">A. Bonnard</name>
<name sortKey="Munck, A" sort="Munck, A" uniqKey="Munck A" first="A" last="Munck">A. Munck</name>
<name sortKey="Viala, J" sort="Viala, J" uniqKey="Viala J" first="J" last="Viala">J. Viala</name>
</country>
</tree>
</affiliations>
</record>

Pour manipuler ce document sous Unix (Dilib)

EXPLOR_STEP=$WICRI_ROOT/Wicri/France/explor/LeHavreV1/Data/France/Analysis

HfdSelect -h $EXPLOR_STEP/biblio.hfd -nk 000026 | SxmlIndent | more

HfdSelect -h $EXPLOR_AREA/Data/France/Analysis/biblio.hfd -nk 000026 | SxmlIndent | more

Pour mettre un lien sur cette page dans le réseau Wicri

{{Explor lien
   |wiki=    Wicri/France
   |area=    LeHavreV1
   |flux=    France
   |étape=   Analysis
   |type=    RBID
   |clé=     pubmed:27692552
   |texte=   [Desmoid tumors in an adolescent girl with familial adenomatous polyposis].
}}

Pour générer des pages wiki

HfdIndexSelect -h $EXPLOR_AREA/Data/France/Analysis/RBID.i   -Sk "pubmed:27692552" \
       | HfdSelect -Kh $EXPLOR_AREA/Data/France/Analysis/biblio.hfd   \
       | NlmPubMed2Wicri -a LeHavreV1

This area was generated with Dilib version V0.6.25.
Data generation: Sat Dec 3 14:37:02 2016. Site generation: Tue Mar 5 08:25:07 2024

Serveur d'exploration sur la visibilité du Havre

[Desmoid tumors in an adolescent girl with familial adenomatous polyposis].

[Desmoid tumors in an adolescent girl with familial adenomatous polyposis].

Source :

Abstract

Links toward previous steps (curation, corpus...)

Links to Exploration step

Le document en format XML

Pour manipuler ce document sous Unix (Dilib)

Pour mettre un lien sur cette page dans le réseau Wicri

Pour générer des pages wiki

	Serveur d'exploration sur la visibilité du Havre
	Attention, ce site est en cours de développement ! Attention, site généré par des moyens informatiques à partir de corpus bruts. Les informations ne sont donc pas validées.