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A case of recurrent respiratory papillomatosis with malignant transformation, HPV11 DNAemia, high L1 antibody titre and a fatal papillary endocardial lesion

Paul-Stefan Mauz1, Manola Zago2, Ralf Kurth3, Michael Pawlita4, Martin Holderried1, John Thiericke1, Angelika Iftner2, Frank Stubenrauch2, Karl Sotlar5 and Thomas Iftner2*

Author Affiliations

1 Department of Otolaryngology, Head and Neck Surgery, University Hospital Tuebingen, Tuebingen, Germany

2 Division of Experimental Virology, Institute for Medical Virology, University Hospital Tuebingen, Elfriede-Aulhorn-Str. 6, 72076 Tuebingen, Germany

3 Institute of Pathology, University Hospital Tuebingen, Tuebingen, Germany

4 Department of Genome Modifications and Carcinogenesis, Research Program Infection and Cancer, German Cancer Research Centre (DKFZ), Heidelberg, Germany

5 Institute of Pathology, University of Munich, Munich, Germany

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Virology Journal 2014, 11:114  doi:10.1186/1743-422X-11-114

Published: 18 June 2014



Recurrent respiratory papillomatosis (RRP) is a rare disease, which is characterised by the growth of papillomavirus-induced papillomas within the respiratory tract. Malignant transformation occurs in less than 1% of the cases.

Case presentation

We report a case of human papillomavirus (HPV) type 11-associated juvenile-onset RRP (JORRP) initially diagnosed at the age of two years. Remarkably high copy numbers of HPV11 DNA and antibody titres targeting the capsid protein L1 were detected in the patient’s serum. The patient developed squamous cell carcinomas in both lungs and extraordinarily an HPV11 DNA-positive papillary endocardial lesion in the left atrium of the heart, which caused thromboembolic events leading to the patient’s death at 19 years old.


We here report a severe case of JORRP hallmarked by HPV11 DNAemia and very high antibody titres directed against the major viral capsid protein L1. Furthermore, the extent of malignant transformation and the discovery of a very rare fatal endocardial lesion highlight the unpredictability of JORRP and the complexity of its clinical management.