Zusammenfassung
Lokalisierte, solitäre fibröse Tumoren (SFT) finden sich meist im Thorax, können aber auch in Leber, Haut, Meningen, am Peritoneum und Perikard auftreten. Die Sicherung der Diagnose ist oftmals schwierig. Entscheidend ist die histopathologische Begutachtung mit differenzierter Immunhistochemie. SFT zeichnen sich durch positive Markerkonstellation für CD34 und negative Markerkonstellation für epitheliale Marker aus. Somit ist eine Differenzialdiagnose zu Pleuramesotheliomen und Fibrosarkomen möglich. Die chirurgische Resektion der lokalisierten SFT ist die Therapie der Wahl. Chemo- und Strahlentherapie erwiesen sich als wenig erfolgreich. Die Rezidiv- und Metastasenrate ist abhängig von der histologischen Dignität. Bei benignen Befunden zeigen sich in nur 2% Rezidive, die ebenfalls überwiegend benigne sind. Unter den malignen Tumoren kann nur in 50% eine Heilung erreicht werden. Deshalb sind kontinuierliche Nachsorgeuntersuchungen indiziert. Wir berichten über zwei 65- und 75-jährige Patienten, bei denen im Rahmen der präoperativen Routinediagnostik für andere chirurgische Eingriffe intrathorakale Raumforderungen erheblicher Größe als Zufallsbefund erhoben wurden. Die histologische Begutachtung nach primärer chirurgischer Resektion ergab die seltene Diagnose eines SFT der Pleura.
Abstract
The solitary fibrous tumor (SFT) is a very rare and usually benign neoplasm. This tumor is mostly located in the thoracic cavity, but it has also been reported in numerous sites including liver, skin, and meninges. Due to its rarity diagnosis is often difficult. Accordingly, histopathological differentiation is of major importance. Solitary fibrous tumors consistently express CD 34 and react negatively to epithelial markers. This absence of expression of epithelial markers, especially cytokeratin, is also useful to distinguish these rare entities from sarcomatous mesotheliomas. The treatment of choice for solitary fibrous tumors is extensive surgical resection. Up to now there is no evidence that radiation and chemotherapy are effective. The local recurrence or onset of metastases depends on histologic parameters. In cases of benign classification (60–80%), the recurrence rate was only 2% after surgical excision. One-half of the patients with malignant tumors can be cured; the rest develop recurrences and metastases more often. Especially in those cases, emphasis should be placed on continual follow-up examination. We report two cases of a 65- and a 75-year-old male patient with huge intrathoracic solitary fibrous subpleural tumors, discovered incidentally on routine chest X-ray.
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Schwab, R., Schneider, C., Junge, K. et al. Große solitäre fibröse Tumoren der Pleura als Zufallsbefund. Chirurg 75, 200–205 (2004). https://doi.org/10.1007/s00104-003-0764-x
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DOI: https://doi.org/10.1007/s00104-003-0764-x