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Conservative management of endolymphatic sac tumors in von Hippel–Lindau disease: case report

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Abstract

In endolymphatic sac tumors associated with von Hippel–Lindau disease, early detection and surgery have been warranted to avoid associated neurological morbidity. However, in lately discovered tumors, hearing preserving surgery is often impossible and timing of surgical resection is difficult to define. We report two cases of tumors revealed by a sudden and profound hearing loss and managed conservatively for more than 15 years without worsening of the neurological symptoms associated with the endolymphatic sac tumor. Tumor size remained stable for the first patient and a stuttering growth pattern was observed for the second patient. Initial observation may be considered a not unreasonable management paradigm in these cases.

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Acknowledgments

This work was supported by the Institut National du Cancer (INCa) and the Ligue Nationale contre le Cancer (Comités du Cher et de l’Indre).

Conflicts of interest

None.

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Authors and Affiliations

  1. Service de Neurochirurgie, Hôpital Beaujon, AP-HP, Clichy, France

    Matthieu Peyre

  2. Service de Neurochirurgie, Hôpital Foch, Suresnes, France

    Stephan Gaillard

  3. Service de Neurochirurgie, Hôpital de la Pitié-Salpêtrière, AP-HP, Paris, France

    Remy van Effenterre

  4. Laboratoire de Génétique, Hôpital Edouard Herriot, Lyon, France

    Sophie Giraud

  5. Centre de Références Cancers Rares PREDIR de l’Institut National du Cancer, Service d’Urologie, AP-HP, Hôpital de Bicêtre, Le Kremlin-Bicêtre, France

    Sophie Giraud & Stéphane Richard

  6. Laboratoire de Génétique Oncologique EPHE, Faculté de Médecine Paris-Sud, Le Kremlin-Bicêtre and INSERM U753, Institut de cancérologie Gustave Roussy, Villejuif, France

    Stéphane Richard

  7. Department of Neurosurgery, Hopital Beaujon, 100 bvd du Général Leclerc, 92110, Clichy Cedex, France

    Matthieu Peyre

Authors
  1. Matthieu Peyre
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  2. Stephan Gaillard
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  3. Remy van Effenterre
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  4. Sophie Giraud
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  5. Stéphane Richard
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Corresponding author

Correspondence to Matthieu Peyre.

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Comment

In this paper, the authors put together some interesting observations regarding the treatment options for von Hippel–Lindau-associated endolymphatic sac tumors. These tumors, which make part of the multiple array of tumors in this hereditary cancer syndrome, are found in 0–11% of vHL patients. They are usually associated with some degree of auditory function impairment. Despite surgery provides better results if the tumor is relatively small and confined in the temporal bone, the conservative management adopted in the present cases, with appropriate clinical and radiological surveillance, may be an option since the natural history of these lesion seems characterized by long periods of quiescence. Moreover, their management must be considered in a peculiar context of multiorgan tumors, where aggressive surgical therapy should be reserved for clinically significant lesions. In our experience, we recommend surgery for endolymphatic sac tumors, when their growth extends beyond the temporal bone borders with a correlated clinical progression. The question whether to treat an incidental endolymphatic sac tumor in vHL patients becomes more troublesome to answer when these tumors are bilateral; the necessity to preserve a serviceable hearing may suggest an early surgery in these circumstances.

Domenico d'Avella

Giacomo Pavesi

Padova, Italy

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Peyre, M., Gaillard, S., van Effenterre, R. et al. Conservative management of endolymphatic sac tumors in von Hippel–Lindau disease: case report. Acta Neurochir 153, 42–47 (2011). https://doi.org/10.1007/s00701-010-0873-5

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  • DOI: https://doi.org/10.1007/s00701-010-0873-5

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