Endolymphatic Sac Tumors in Von Hippel Lindau Disease: Personal Experience

Objective: Endolymphatic sac tumor (ELST) is a low grade adenocarcinoma arising sporadically or associated with Von-Hippel-Lindau (VHL) disease. The tumor is locally aggressive to adjacent structures. Clinical manifestations depend on the extent of the disease. Treatment is surgical radical removal trough transpetrous approaches. When part of VHL the tumor is more aggressive and has higher incidence. We discuss the surgical therapy, the outcome of the VHL related to ELST tumors, with the aim of investigating the best option in the frame of a multi-organ disease.

Methods: The treatment of patients (6) affected from both ELST and VHL disease managed at ENT department of Padova University Hospital between 2012 and 2014 is reviewed. We analyzed time intervals between clinical manifestation and diagnosis and between diagnosis and surgical treatment.

Results: This series is consistent compared with the literature over several decades of sporadic ELST. The disease extension dictates the type of surgery and the related functional outcome. Early surgery has very low surgical morbidity, whereas observation and growing of tumors involve more extensive, and more aggressive, surgery.

Conclusion: An early tumor radiologic diagnosis, which should be a screening in all the patients affected by VHL disease, prevents diagnostic and therapeutic delay. Curative treatment is complete surgical excision of the neoplasm. Surgical morbidity is strictly related to tumor size and the need of extensive surgery. The problem of setting an early treatment in VHL patients is the choice of the surgery time, since these patients are affected by multiorgan disease which also requires repeated treatments.