Bilateral orbital Erdheim-Chester disease treated with 12 weekly administrations of VNCOP-B chemotherapy: a case report and a review of literature
Identifieur interne : 001578 ( Main/Exploration ); précédent : 001577; suivant : 001579Bilateral orbital Erdheim-Chester disease treated with 12 weekly administrations of VNCOP-B chemotherapy: a case report and a review of literature
Auteurs : Alessandro Broccoli [Italie] ; Vittorio Stefoni [Italie] ; Luca Faccioli [Italie] ; Claudio Agostinelli [Italie] ; Luca Spinardi [Italie] ; Marco Pastore Trossello [Italie] ; Pier Luigi Zinzani [Italie]Source :
- Rheumatology International [ 0172-8172 ] ; 2012-07-01.
English descriptors
- KwdEn :
Abstract
Abstract: Erdheim-Chester disease (ECD) is a non-Langerhans’ cells histiocytosis of unknown etiology, which generally presents with long bones involvement, even if extraskeletal lesions may be frequently recognized. As a consequence of its rarity, there is no consensus concerning the best standard of care for affected patients. We present the case of a 53-year-old woman with bilateral orbital histologically documented ECD, presenting with an important thickening and swelling of the periorbital tissue and massive involvement of lateral rectal muscles, as documented by magnetic resonance. The patient was successfully addressed to 12 cycles of a weekly lymphoma-designed chemotherapy regimen, including etoposide, mitoxantrone, cyclophosphamide, vincristine, bleomycin, and prednisone (VNCOP-B regimen). Periorbital lesions reduced during the courses of chemotherapy, along with a regression to normal appearance of the extrinsic ocular musculature. This appears as an effective and well-tolerated first-line treatment option for ECD patients, due to the possibility of maintaining an adequate dose intensity, with also a concomitant continuous steroid administration.
Url:
DOI: 10.1007/s00296-011-1998-4
Affiliations:
Links toward previous steps (curation, corpus...)
- to stream Istex, to step Corpus: 001F38
- to stream Istex, to step Curation: 001F38
- to stream Istex, to step Checkpoint: 000554
- to stream Main, to step Merge: 001580
- to stream Main, to step Curation: 001578
Le document en format XML
<record><TEI wicri:istexFullTextTei="biblStruct"><teiHeader><fileDesc><titleStmt><title xml:lang="en">Bilateral orbital Erdheim-Chester disease treated with 12 weekly administrations of VNCOP-B chemotherapy: a case report and a review of literature</title><author><name sortKey="Broccoli, Alessandro" sort="Broccoli, Alessandro" uniqKey="Broccoli A" first="Alessandro" last="Broccoli">Alessandro Broccoli</name></author><author><name sortKey="Stefoni, Vittorio" sort="Stefoni, Vittorio" uniqKey="Stefoni V" first="Vittorio" last="Stefoni">Vittorio Stefoni</name></author><author><name sortKey="Faccioli, Luca" sort="Faccioli, Luca" uniqKey="Faccioli L" first="Luca" last="Faccioli">Luca Faccioli</name></author><author><name sortKey="Agostinelli, Claudio" sort="Agostinelli, Claudio" uniqKey="Agostinelli C" first="Claudio" last="Agostinelli">Claudio Agostinelli</name></author><author><name sortKey="Spinardi, Luca" sort="Spinardi, Luca" uniqKey="Spinardi L" first="Luca" last="Spinardi">Luca Spinardi</name></author><author><name sortKey="Pastore Trossello, Marco" sort="Pastore Trossello, Marco" uniqKey="Pastore Trossello M" first="Marco" last="Pastore Trossello">Marco Pastore Trossello</name></author><author><name sortKey="Zinzani, Pier Luigi" sort="Zinzani, Pier Luigi" uniqKey="Zinzani P" first="Pier Luigi" last="Zinzani">Pier Luigi Zinzani</name></author></titleStmt><publicationStmt><idno type="wicri:source">ISTEX</idno><idno type="RBID">ISTEX:64B359DE6C57506BFDED0F70F3F93BCE0EDEE829</idno><date when="2011" year="2011">2011</date><idno type="doi">10.1007/s00296-011-1998-4</idno><idno type="url">https://api.istex.fr/ark:/67375/VQC-XMMXN6RB-6/fulltext.pdf</idno><idno type="wicri:Area/Istex/Corpus">001F38</idno><idno type="wicri:explorRef" wicri:stream="Istex" wicri:step="Corpus" wicri:corpus="ISTEX">001F38</idno><idno type="wicri:Area/Istex/Curation">001F38</idno><idno type="wicri:Area/Istex/Checkpoint">000554</idno><idno type="wicri:explorRef" wicri:stream="Istex" wicri:step="Checkpoint">000554</idno><idno type="wicri:doubleKey">0172-8172:2011:Broccoli A:bilateral:orbital:erdheim</idno><idno type="wicri:Area/Main/Merge">001580</idno><idno type="wicri:Area/Main/Curation">001578</idno><idno type="wicri:Area/Main/Exploration">001578</idno></publicationStmt><sourceDesc><biblStruct><analytic><title level="a" type="main" xml:lang="en">Bilateral orbital Erdheim-Chester disease treated with 12 weekly administrations of VNCOP-B chemotherapy: a case report and a review of literature</title><author><name sortKey="Broccoli, Alessandro" sort="Broccoli, Alessandro" uniqKey="Broccoli A" first="Alessandro" last="Broccoli">Alessandro Broccoli</name><affiliation wicri:level="1"><country xml:lang="fr">Italie</country><wicri:regionArea>Institute of Hematology and Medical Oncology “L. e A. Seràgnoli”, Policlinico “Sant’Orsola-Malpighi”, University of Bologna, Via Massarenti, 9, 40138, Bologna</wicri:regionArea><wicri:noRegion>Bologna</wicri:noRegion></affiliation><affiliation wicri:level="1"><country wicri:rule="url">Italie</country></affiliation></author><author><name sortKey="Stefoni, Vittorio" sort="Stefoni, Vittorio" uniqKey="Stefoni V" first="Vittorio" last="Stefoni">Vittorio Stefoni</name><affiliation wicri:level="1"><country xml:lang="fr">Italie</country><wicri:regionArea>Institute of Hematology and Medical Oncology “L. e A. Seràgnoli”, Policlinico “Sant’Orsola-Malpighi”, University of Bologna, Via Massarenti, 9, 40138, Bologna</wicri:regionArea><wicri:noRegion>Bologna</wicri:noRegion></affiliation></author><author><name sortKey="Faccioli, Luca" sort="Faccioli, Luca" uniqKey="Faccioli L" first="Luca" last="Faccioli">Luca Faccioli</name><affiliation wicri:level="1"><country xml:lang="fr">Italie</country><wicri:regionArea>Neuroradiology Unit, Policlinico “Sant’Orsola-Malpighi”, Bologna</wicri:regionArea><wicri:noRegion>Bologna</wicri:noRegion></affiliation></author><author><name sortKey="Agostinelli, Claudio" sort="Agostinelli, Claudio" uniqKey="Agostinelli C" first="Claudio" last="Agostinelli">Claudio Agostinelli</name><affiliation wicri:level="1"><country xml:lang="fr">Italie</country><wicri:regionArea>Institute of Hematology and Medical Oncology “L. e A. Seràgnoli”, Policlinico “Sant’Orsola-Malpighi”, University of Bologna, Via Massarenti, 9, 40138, Bologna</wicri:regionArea><wicri:noRegion>Bologna</wicri:noRegion></affiliation></author><author><name sortKey="Spinardi, Luca" sort="Spinardi, Luca" uniqKey="Spinardi L" first="Luca" last="Spinardi">Luca Spinardi</name><affiliation wicri:level="1"><country xml:lang="fr">Italie</country><wicri:regionArea>Neuroradiology Unit, Policlinico “Sant’Orsola-Malpighi”, Bologna</wicri:regionArea><wicri:noRegion>Bologna</wicri:noRegion></affiliation></author><author><name sortKey="Pastore Trossello, Marco" sort="Pastore Trossello, Marco" uniqKey="Pastore Trossello M" first="Marco" last="Pastore Trossello">Marco Pastore Trossello</name><affiliation wicri:level="1"><country xml:lang="fr">Italie</country><wicri:regionArea>Neuroradiology Unit, Policlinico “Sant’Orsola-Malpighi”, Bologna</wicri:regionArea><wicri:noRegion>Bologna</wicri:noRegion></affiliation></author><author><name sortKey="Zinzani, Pier Luigi" sort="Zinzani, Pier Luigi" uniqKey="Zinzani P" first="Pier Luigi" last="Zinzani">Pier Luigi Zinzani</name><affiliation wicri:level="1"><country xml:lang="fr">Italie</country><wicri:regionArea>Institute of Hematology and Medical Oncology “L. e A. Seràgnoli”, Policlinico “Sant’Orsola-Malpighi”, University of Bologna, Via Massarenti, 9, 40138, Bologna</wicri:regionArea><wicri:noRegion>Bologna</wicri:noRegion></affiliation></author></analytic><monogr></monogr><series><title level="j">Rheumatology International</title><title level="j" type="sub">Clinical and Experimental Investigations</title><title level="j" type="abbrev">Rheumatol Int</title><idno type="ISSN">0172-8172</idno><idno type="eISSN">1437-160X</idno><imprint><publisher>Springer-Verlag</publisher><pubPlace>Berlin/Heidelberg</pubPlace><date type="published" when="2012-07-01">2012-07-01</date><biblScope unit="volume">32</biblScope><biblScope unit="issue">7</biblScope><biblScope unit="page" from="2209">2209</biblScope><biblScope unit="page" to="2213">2213</biblScope></imprint><idno type="ISSN">0172-8172</idno></series></biblStruct></sourceDesc><seriesStmt><idno type="ISSN">0172-8172</idno></seriesStmt></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Chemotherapy</term><term>Erdheim-Chester disease</term><term>Non-Langerhans’ cells histiocytosis</term><term>Orbital involvement</term><term>VNCOP-B</term><term>Xantoma-like lesions</term></keywords></textClass><langUsage><language ident="en">en</language></langUsage></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">Abstract: Erdheim-Chester disease (ECD) is a non-Langerhans’ cells histiocytosis of unknown etiology, which generally presents with long bones involvement, even if extraskeletal lesions may be frequently recognized. As a consequence of its rarity, there is no consensus concerning the best standard of care for affected patients. We present the case of a 53-year-old woman with bilateral orbital histologically documented ECD, presenting with an important thickening and swelling of the periorbital tissue and massive involvement of lateral rectal muscles, as documented by magnetic resonance. The patient was successfully addressed to 12 cycles of a weekly lymphoma-designed chemotherapy regimen, including etoposide, mitoxantrone, cyclophosphamide, vincristine, bleomycin, and prednisone (VNCOP-B regimen). Periorbital lesions reduced during the courses of chemotherapy, along with a regression to normal appearance of the extrinsic ocular musculature. This appears as an effective and well-tolerated first-line treatment option for ECD patients, due to the possibility of maintaining an adequate dose intensity, with also a concomitant continuous steroid administration.</div></front></TEI><affiliations><list><country><li>Italie</li></country></list><tree><country name="Italie"><noRegion><name sortKey="Broccoli, Alessandro" sort="Broccoli, Alessandro" uniqKey="Broccoli A" first="Alessandro" last="Broccoli">Alessandro Broccoli</name></noRegion><name sortKey="Agostinelli, Claudio" sort="Agostinelli, Claudio" uniqKey="Agostinelli C" first="Claudio" last="Agostinelli">Claudio Agostinelli</name><name sortKey="Broccoli, Alessandro" sort="Broccoli, Alessandro" uniqKey="Broccoli A" first="Alessandro" last="Broccoli">Alessandro Broccoli</name><name sortKey="Faccioli, Luca" sort="Faccioli, Luca" uniqKey="Faccioli L" first="Luca" last="Faccioli">Luca Faccioli</name><name sortKey="Pastore Trossello, Marco" sort="Pastore Trossello, Marco" uniqKey="Pastore Trossello M" first="Marco" last="Pastore Trossello">Marco Pastore Trossello</name><name sortKey="Spinardi, Luca" sort="Spinardi, Luca" uniqKey="Spinardi L" first="Luca" last="Spinardi">Luca Spinardi</name><name sortKey="Stefoni, Vittorio" sort="Stefoni, Vittorio" uniqKey="Stefoni V" first="Vittorio" last="Stefoni">Vittorio Stefoni</name><name sortKey="Zinzani, Pier Luigi" sort="Zinzani, Pier Luigi" uniqKey="Zinzani P" first="Pier Luigi" last="Zinzani">Pier Luigi Zinzani</name></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
EXPLOR_STEP=$WICRI_ROOT/Sante/explor/ChloroquineV1/Data/Main/Exploration
HfdSelect -h $EXPLOR_STEP/biblio.hfd -nk 001578 | SxmlIndent | more
Ou
HfdSelect -h $EXPLOR_AREA/Data/Main/Exploration/biblio.hfd -nk 001578 | SxmlIndent | more
Pour mettre un lien sur cette page dans le réseau Wicri
{{Explor lien
|wiki= Sante
|area= ChloroquineV1
|flux= Main
|étape= Exploration
|type= RBID
|clé= ISTEX:64B359DE6C57506BFDED0F70F3F93BCE0EDEE829
|texte= Bilateral orbital Erdheim-Chester disease treated with 12 weekly administrations of VNCOP-B chemotherapy: a case report and a review of literature
}}
| This area was generated with Dilib version V0.6.33. |  |