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JNCI Journal of the National Cancer Institute 2000 92(1):54-61; doi:10.1093/jnci/92.1.54
© 2000 by Oxford University Press
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Journal of the National Cancer Institute, Vol. 92, No. 1, 54-61, January 5, 2000
© 2000 Oxford University Press


REPORTS

Hematologic Disorders Associated With Primary Mediastinal Nonseminomatous Germ Cell Tumors

Jörg T. Hartmann, Craig R. Nichols, Jean-P. Droz, Alan Horwich, Arthur Gerl, Sophie D. Fossa, Jörg Beyer, Jörg Pont, Karim Fizazi, Lawrence Einhorn, Lothar Kanz, Carsten Bokemeyer

Affiliations of authors: J. T. Hartmann, L. Kanz, C. Bokemeyer, Tuebingen University Medical Center II, Germany; C. R. Nichols, Oregon Health Sciences University, Portland; J.-P. Droz, Centre Léon-Berard, Groupe d'Etude des Tumeurs Uro-geniales, Lyon, France; A. Horwich, The Royal Marsden Hospital, Sutton, U.K.; A. Gerl, Klinikum Großhadern, Munich, Germany; S. D. Fossa, The Norwegian Radium Hospital, Oslo, Norway; J. Beyer, Virchow Klinikum, Berlin, Germany; J. Pont, Kaiser-Franz-Josef Spital, Vienna, Austria; K. Fizazi, Institut Gustave-Roussy, Villejuif, France; L. Einhorn, Indiana University, Indianapolis.

Correspondence to: Carsten Bokemeyer, M.D., Department of Hematology/Oncology/Immunology, UKL-University Medical Center II, Eberhard-Karls-University, Otfried-Mueller-Str. 10, 72076 Tuebingen, Germany (e-mail: carsten.bokemeyer{at}med.uni-tuebingen.de).

BACKGROUND. The association between primary germ cell tumors of the mediastinum (the space between the lung pleura that contains the heart and other chest viscera) and hematologic malignancies has been described by retrospective analysis of patients treated at individual clinical centers. To better characterize the risk of hematologic disorders in patients with extragonadal germ cell tumors and to describe the clinical and biologic features of the disorders, we studied an unselected population in a large, international, multicenter database. METHODS. Six hundred thirty-five patients treated at 11 centers in the United States and Europe from 1975 through 1996 were evaluated retrospectively. RESULTS. A hematologic disorder was observed in 17 patients with germ cell tumors. All cases developed among the 287 patients with primary mediastinal nonseminomatous germ cell tumors, giving an incidence rate in this group of 2.0% (95% confidence interval [CI] = 1.1%-3.1%) per year over a median follow-up time of 3 years. The risk of developing hematologic disorders was statistically significantly increased in patients with primary mediastinal nonseminomatous germ cell tumors in comparison with the age-matched general population (standardized incidence ratio = 250; 95% CI = 140-405). The median time to onset of hematologic neoplasia was 6 months (range, 0-47 months), and the median survival after diagnosis of the hematologic disorder was 5 months (range, 0-16 months) (two-sided P<.0001, comparing survival from the time of diagnosis of the germ cell tumor of patients with and without hematologic disorders). CONCLUSION. In our study, approximately one in 17 patients with primary mediastinal nonseminomatous germ cell tumors was affected by a hematologic disorder, whereas no cases were seen among 334 patients with other extragonadal germ cell tumors. The hematologic disorder had a statistically significant impact on prognosis, with none of the 17 reported patients surviving for more than 2 years.



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