Zusammenfassung
Ein primäres pulmonales Rhabdomyosarkom ist eine Rarität. Die histologische Differenzialdiagnose
kann schwierig sein. Bei einer 43-jährigen Patientin, Raucherin (25 pack-years), wurde
eine solitäre Hirnmetastase diagnostiziert und enukleiert, die histologisch als typisches
kleinzelliges Karzinom imponierte. Biopsien aus einem Tumor im linken Lungenoberlappen
bestätigten ein kleinzelliges Karzinom, das trotz Chemotherapie jedoch rasch progredient
war. Erneute Biopsien zeigten wieder das Bild eines kleinzelligen malignen Tumors.
Aufgrund der immunhistochemischen Befunde wurde ein pleomorphes Rhabdomyosarkom diagnostiziert.
Bei superinfiziertem Tumorzerfall und Fehlen weiterer Metastasen erfolgte die Lobektomie
des linken Lungenoberlappens. Ein postoperatives Pleuraempyem limitierte die Möglichkeiten
einer adjuvanten Therapie. Ein frühes Rezidiv mit pleuralen, pulmonalen, Thoraxwand-
und spinalen Metastasen erforderte eine Laminektomie und Exstirpation der spinalen
Metastasen, sowie eine Strahlentherapie und eine Chemotherapie mit Iphosphamid und
Doxorubicin, was zu einer partiellen Remission für wenige Monate führte. Die Patientin
verstarb an dem metastasierenden primären pulmonalen Rhabdomyosarkom der Lunge. Diese
seltene Tumorentität wurde initial als kleinzelliges Karzinom fehlgedeutet. Eine Würdigung
des atypischen klinischen Verlaufs und ein enger Dialog zwischen Pathologen und Klinikern
führten zur korrekten Diagnose.
Abstract
Primary pulmonary rhabdomyosarcoma is a rare entity and the histological differential
diagnosis can be difficult. We report on a 43-year old female patient, smoker (25
pack-years), in whom a large solitary brain metastasis was diagnosed and enucleated.
Histological examination revealed a typical small cell carcinoma and histological
examination of biopsies obtained from a tumor in the left upper lobe of the lung was
compatible with a small cell carcinoma. Despite chemotherapy there was a progressive
tumor growth. Bronchial biopsies again showed a small cell tumor, although immunohistochemistry
proved it to be a pleomorphic rhabdomyosarcoma. Due to the progressive tumor growth
with necrosis and superinfection and a lack of further metastases lobectomy of the
left upper lobe was performed, complicated by postoperative pleural empyema, limiting
the possibilities of adjuvant therapy. Early relapse occurred with pleural, pulmonary,
chest wall and spinal metastases. Laminectomy and extirpation of the spinal metastases,
local radiotherapy and chemotherapy with iphosphamide and doxorubicine led to partial
remission and clinical improvement for few months only. The patient died from metastatic
primary rhabdomyosarcoma of the lung. This rare tumor mimicked small cell lung cancer.
Appraisal of the atypical clinical course and a close dialogue between pathologists
and clinicians enabled the correct diagnosis.
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PD Dr. med. Jens Schreiber
Städtisches Klinikum Dessau · Klinik für Innere Medizin · Abteilung für Pneumologie
06847 Dessau
Email: jens.schreiber@klinikum-dessau.de