{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,11,2]],"date-time":"2025-11-02T06:53:12Z","timestamp":1762066392592,"version":"build-2065373602"},"reference-count":14,"publisher":"Georg Thieme Verlag KG","issue":"09","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Pneumologie"],"published-print":{"date-parts":[[2022,9]]},"abstract":"<jats:title>Zusammenfassung<\/jats:title><jats:p>Das Angiosarkom ist eine seltene, maligne Erkrankung, die von den Blutgef\u00e4\u00dfen ausgeht. Das pulmonale Angiosarkom kann sich diagnostisch initial wie eine chronisch thromboembolische pulmonale Hypertonie (CTEPH) darstellen. Die CTEPH ist eine Erh\u00f6hung des pulmonal-arteriellen Drucks und Widerstands als Folge einer persistierenden Obstruktion der Lungenstrombahn nach pulmonalen Thromboembolien trotz ad\u00e4quater Antikoagulation.<\/jats:p><jats:p>In unserer Ambulanz f\u00fcr pulmonale Hypertonie stellte sich ein 76-j\u00e4hriger Patient mit\n      anamnestisch vor 7 Monaten erlittener subakuter, links zentraler Lungenarterienembolie (LAE)\n      vor, die seitdem mit Apixaban therapiert wurde. Bei persistierender Belastungsdyspnoe wurde\n      der Verdacht auf eine CTEPH gestellt und es erfolgte weitere Diagnostik. Passend zur CTEPH\n      zeigte sich spiroergometrisch eine ineffektive Ventilation und echokardiografisch eine\n      Rechtsherzbelastung. Eine Verlaufs-CT des Thorax ergab einen persistierenden und\n      gr\u00f6\u00dfenkonstanten F\u00fcllungsdefekt der linken Pulmonalarterie mit szintigrafischem Nachweis einer\n      prim\u00e4ren Perfusionsst\u00f6rung des gesamten linken Lungenfl\u00fcgels. Mit dem Verdacht auf ein\n      malignes Geschehen veranlassten wir eine PET-CT, in welcher sich die L\u00e4sion der\n      Pulmonalarterie stoffwechselaktiv darstellte. Eine unter endobronchialer Ultraschallkontrolle\n      durchgef\u00fchrte Punktion der L\u00e4sion ergab histologisch den Nachweis eines sarkomatoiden Tumors\n      mit Amplifikation des MDM2-Gens. Wir stellten die Diagnose eines Intimasarkoms der linken\n      Pulmonalarterie und f\u00fchrten den Patienten einer Pneumektomie zu.<\/jats:p><jats:p>Die Intimasarkome der Pulmonalarterie sind selten und f\u00fchren durch Wachstum und\n      Thrombusneigung zur Stenose des Gef\u00e4\u00dfes. Die damit einhergehende klinische und\n      echokardiografische Pr\u00e4sentation kann eine CTEPH imitieren und stellt somit eine wichtige\n      Differenzialdiagnose zu dieser dar. 2015 wurden weniger als 300 Fallberichte\n      beschrieben.<\/jats:p><jats:p>Hinweise auf ein pulmonales Angiosarkom: Kontrastmittel aufnehmende, intraluminale\n      Gewebsformation mit Dilatation der Pulmonalarterie und Infiltration der Gef\u00e4\u00dfwand, erh\u00f6hte\n      Stoffwechselaktivit\u00e4t des Befundes in der FDG-PET CT, Gr\u00f6\u00dfenprogredienz unter Antikoagulation,\n      anamnestisch keine stattgehabte LAE, B-Symptome.<\/jats:p><jats:p>Die Diagnosesicherung erfolgt durch Biopsie. Das mediane \u00dcberleben wird mit 1,5\u201317 Monaten\n      beschrieben, welches mit therapeutischen Interventionen um einige Monate mit Symptomreduktion\n      verl\u00e4ngert werden kann. Als therapeutischer Standard gilt die Resektion des Tumors mit ggf.\n      Metastasenentfernung. Der Nutzen von Radio- und Chemotherapie ist Bestandteil aktueller\n      Forschung. Eine Behandlung ist in erfahrenen Zentren anzustreben und es bedarf weiterer\n      Forschung zur Verbesserung von Diagnostik und Therapie.<\/jats:p>","DOI":"10.1055\/a-1883-8890","type":"journal-article","created":{"date-parts":[[2022,9,15]],"date-time":"2022-09-15T00:27:49Z","timestamp":1663201669000},"page":"633-638","source":"Crossref","is-referenced-by-count":2,"title":["Das intimale Sarkom der Pulmonalarterie \u2013 Fallbericht einer seltenen Differenzialdiagnose der chronisch thromboembolischen pulmonalen Hypertonie"],"prefix":"10.1055","volume":"76","author":[{"given":"Julian","family":"Pott","sequence":"additional","affiliation":[{"name":"Abteilung f\u00fcr Pneumologie, II. Medizinische Klinik und Poliklinik, Zentrum f\u00fcr Onkologie, Universit\u00e4tsklinikum Hamburg-Eppendorf, Hamburg, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Paulina","family":"Gr\u00e4fin zu Reventlow","sequence":"additional","affiliation":[{"name":"Zentrum f\u00fcr Radiologie und Endoskopie, Klinik und Poliklinik f\u00fcr Diagnostische und Interventionelle Radiologie und Nuklearmedizin, Universit\u00e4tsklinikum Hamburg-Eppendorf, Hamburg, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Marcel","family":"Simon","sequence":"additional","affiliation":[{"name":"Abteilung f\u00fcr Pneumologie, II. Medizinische Klinik und Poliklinik, Zentrum f\u00fcr Onkologie, Universit\u00e4tsklinikum Hamburg-Eppendorf, Hamburg, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Stefan","family":"Steurer","sequence":"additional","affiliation":[{"name":"Institut f\u00fcr Pathologie mit den Sektionen Molekularpathologie und Zytopathologie, Zentrum f\u00fcr Diagnostik, Universit\u00e4tsklinikum Hamburg-Eppendorf, Hamburg, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Lars","family":"Harbaum","sequence":"additional","affiliation":[{"name":"Abteilung f\u00fcr Pneumologie, II. Medizinische Klinik und Poliklinik, Zentrum f\u00fcr Onkologie, Universit\u00e4tsklinikum Hamburg-Eppendorf, Hamburg, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Frank Oliver","family":"Henes","sequence":"additional","affiliation":[{"name":"Zentrum f\u00fcr Radiologie und Endoskopie, Klinik und Poliklinik f\u00fcr Diagnostische und Interventionelle Radiologie und Nuklearmedizin, Universit\u00e4tsklinikum Hamburg-Eppendorf, Hamburg, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hans","family":"Klose","sequence":"additional","affiliation":[{"name":"Abteilung f\u00fcr Pneumologie, II. 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