Successful Resection of a Re-Occurred Pulmonary Myosarcoma in a Patient with Turner Syndrome Mosaic
Identifieur interne : 003F36 ( Pmc/Checkpoint ); précédent : 003F35; suivant : 003F37Successful Resection of a Re-Occurred Pulmonary Myosarcoma in a Patient with Turner Syndrome Mosaic
Auteurs : Volker F. H. Brauer [Allemagne] ; Frank Reichenberger [Allemagne] ; Anke Müller [Allemagne] ; Matthias Steinert [Allemagne] ; Ursula G. Froster [Allemagne] ; Hubert R. W. Wirtz [Allemagne] ; Joachim Schauer [Allemagne]Source :
- Sarcoma [ 1357-714X ] ; 2002.
Abstract
We describe a patient who underwent thoracic radiation therapy for biopsy-proven pulmonary spindle cell sarcoma in the left lower lobe, 15 months after birth. At the age of 37 she developed shoulder pain, fatigue, and progressive exertion dyspnoea. Chest X-ray revealed a pulmonary mass in the left lower lobe due to a cytology-proven malignant tumour.The patient underwent left pneumonectomy. Histology revealed a myosarcoma of the lung, similar to the previous sarcoma. Furthermore, the patient was diagnosed to have Turner syndrome mosaic and chromosomal analysis revealed a translocation t(1;13) in 3/50 metaphases. However a germline mutation of the p53 tumour suppressor gene was excluded. After 2 years of follow-up the patient is stable and there are no signs of recurrence of the tumour.We conclude a re-occurrence of this very rare malignant disorder of the lung after a 36-year interval in a patient with Turner syndrome mosaic. Following initial curative radiation therapy, with a remission over 36 years, lung resection was now successfully performed.
Url:
DOI: 10.1080/1357714021000066395
PubMed: 18521351
PubMed Central: 2395496
Affiliations:
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<front><div type="abstract" xml:lang="en"><p>We describe a patient who underwent thoracic radiation therapy for biopsy-proven pulmonary spindle cell sarcoma in the
left lower lobe, 15 months after birth. At the age of 37 she developed shoulder pain, fatigue, and progressive exertion dyspnoea.
Chest X-ray revealed a pulmonary mass in the left lower lobe due to a cytology-proven malignant tumour.The patient
underwent left pneumonectomy. Histology revealed a myosarcoma of the lung, similar to the previous sarcoma.
Furthermore, the patient was diagnosed to have Turner syndrome mosaic and chromosomal analysis revealed a translocation
t(1;13) in 3/50 metaphases. However a germline mutation of the p53 tumour suppressor gene was excluded. After 2
years of follow-up the patient is stable and there are no signs of recurrence of the tumour.We conclude a re-occurrence of
this very rare malignant disorder of the lung after a 36-year interval in a patient with Turner syndrome mosaic. Following
initial curative radiation therapy, with a remission over 36 years, lung resection was now successfully performed.</p>
</div>
</front>
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<issn pub-type="ppub">1357-714X</issn>
<issn pub-type="epub">1369-1643</issn>
<publisher><publisher-name>Hindawi Publishing Corporation</publisher-name>
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<article-id pub-id-type="pmc">2395496</article-id>
<article-id pub-id-type="pii">S1357714X02000336</article-id>
<article-id pub-id-type="doi">10.1080/1357714021000066395</article-id>
<article-categories><subj-group subj-group-type="heading"><subject>Research Article</subject>
</subj-group>
</article-categories>
<title-group><article-title>Successful Resection of a Re-Occurred Pulmonary Myosarcoma in a
Patient with Turner Syndrome Mosaic</article-title>
</title-group>
<contrib-group><contrib contrib-type="author" corresp="yes"><name><surname>Brauer</surname>
<given-names>Volker F. H.</given-names>
</name>
<address><email>vbrauer@web.de</email>
</address>
<xref ref-type="aff" rid="aff1">1</xref>
<xref ref-type="aff" rid="aff5">5</xref>
</contrib>
<contrib contrib-type="author"><name><surname>Reichenberger</surname>
<given-names>Frank</given-names>
</name>
<xref ref-type="aff" rid="aff1">1</xref>
</contrib>
<contrib contrib-type="author"><name><surname>Müller</surname>
<given-names>Anke</given-names>
</name>
<xref ref-type="aff" rid="aff2">2</xref>
</contrib>
<contrib contrib-type="author"><name><surname>Steinert</surname>
<given-names>Matthias</given-names>
</name>
<xref ref-type="aff" rid="aff3">3</xref>
</contrib>
<contrib contrib-type="author"><name><surname>Froster</surname>
<given-names>Ursula G.</given-names>
</name>
<xref ref-type="aff" rid="aff4">4</xref>
</contrib>
<contrib contrib-type="author"><name><surname>Wirtz</surname>
<given-names>Hubert R. W.</given-names>
</name>
<xref ref-type="aff" rid="aff1">1</xref>
</contrib>
<contrib contrib-type="author"><name><surname>Schauer</surname>
<given-names>Joachim</given-names>
</name>
<xref ref-type="aff" rid="aff1">1</xref>
</contrib>
</contrib-group>
<aff id="aff1"><label>1</label>
<addr-line>Division of Pneumology</addr-line>
<addr-line>Department of Medicine</addr-line>
<addr-line>University Hospital Leipzig</addr-line>
<addr-line>Leipzig</addr-line>
<country>Germany</country>
</aff>
<aff id="aff2"><label>2</label>
<addr-line>Institute of Pathology</addr-line>
<addr-line>University Hospital Leipzig</addr-line>
<addr-line>Leipzig</addr-line>
<country>Germany</country>
</aff>
<aff id="aff3"><label>3</label>
<addr-line>Division of Thoracic Surgery</addr-line>
<addr-line>Department of Surgery</addr-line>
<addr-line>University Hospital Leipzig</addr-line>
<addr-line>Leipzig</addr-line>
<country>Germany</country>
</aff>
<aff id="aff4"><label>4</label>
<addr-line>Institute of Human Genetics</addr-line>
<addr-line>University Hospital Leipzig</addr-line>
<addr-line>Leipzig</addr-line>
<country>Germany</country>
</aff>
<aff id="aff5"><label>5</label>
<addr-line>Department of Internal Medicine III</addr-line>
<addr-line>University Hospital Leipzig</addr-line>
<addr-line>Philipp-Rosenthal-Str. 27</addr-line>
<addr-line>Leipzig</addr-line>
<addr-line>04103</addr-line>
<country>Germany</country>
</aff>
<pub-date pub-type="ppub"><month>12</month>
<year>2002</year>
</pub-date>
<volume>6</volume>
<issue>4</issue>
<fpage>141</fpage>
<lpage>143</lpage>
<permissions><copyright-statement>Copyright © 2002 Hindawi Publishing Corporation.</copyright-statement>
<copyright-year>2002</copyright-year>
<copyright-holder>Hindawi Publishing Corporation</copyright-holder>
<license license-type="open-access" xlink:href="http://creativecommons.org/licenses/by/"><p>This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.</p>
</license>
</permissions>
<abstract><p>We describe a patient who underwent thoracic radiation therapy for biopsy-proven pulmonary spindle cell sarcoma in the
left lower lobe, 15 months after birth. At the age of 37 she developed shoulder pain, fatigue, and progressive exertion dyspnoea.
Chest X-ray revealed a pulmonary mass in the left lower lobe due to a cytology-proven malignant tumour.The patient
underwent left pneumonectomy. Histology revealed a myosarcoma of the lung, similar to the previous sarcoma.
Furthermore, the patient was diagnosed to have Turner syndrome mosaic and chromosomal analysis revealed a translocation
t(1;13) in 3/50 metaphases. However a germline mutation of the p53 tumour suppressor gene was excluded. After 2
years of follow-up the patient is stable and there are no signs of recurrence of the tumour.We conclude a re-occurrence of
this very rare malignant disorder of the lung after a 36-year interval in a patient with Turner syndrome mosaic. Following
initial curative radiation therapy, with a remission over 36 years, lung resection was now successfully performed.</p>
</abstract>
</article-meta>
</front>
</pmc>
<affiliations><list><country><li>Allemagne</li>
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<tree><country name="Allemagne"><noRegion><name sortKey="Brauer, Volker F H" sort="Brauer, Volker F H" uniqKey="Brauer V" first="Volker F. H." last="Brauer">Volker F. H. Brauer</name>
</noRegion>
<name sortKey="Brauer, Volker F H" sort="Brauer, Volker F H" uniqKey="Brauer V" first="Volker F. H." last="Brauer">Volker F. H. Brauer</name>
<name sortKey="Froster, Ursula G" sort="Froster, Ursula G" uniqKey="Froster U" first="Ursula G." last="Froster">Ursula G. Froster</name>
<name sortKey="Muller, Anke" sort="Muller, Anke" uniqKey="Muller A" first="Anke" last="Müller">Anke Müller</name>
<name sortKey="Reichenberger, Frank" sort="Reichenberger, Frank" uniqKey="Reichenberger F" first="Frank" last="Reichenberger">Frank Reichenberger</name>
<name sortKey="Schauer, Joachim" sort="Schauer, Joachim" uniqKey="Schauer J" first="Joachim" last="Schauer">Joachim Schauer</name>
<name sortKey="Steinert, Matthias" sort="Steinert, Matthias" uniqKey="Steinert M" first="Matthias" last="Steinert">Matthias Steinert</name>
<name sortKey="Wirtz, Hubert R W" sort="Wirtz, Hubert R W" uniqKey="Wirtz H" first="Hubert R. W." last="Wirtz">Hubert R. W. Wirtz</name>
</country>
</tree>
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