The Gruppo Otologico experience of endolymphatic sac tumor.

OBJECTIVE: Endolymphatic sac tumor (ELST) is a rare low grade adenocarcinoma of the skull base. During the past decade the number of the reported cases has increased. This study exposes our experience in the management of ELST with a review of the literature. STUDY DESIGN: Retrospective study of patients with ELST at a quaternary referral otology and skull base center. METHODS: A review of the records from the Gruppo Otologico revealed 7 patients treated for ELST. All papers containing series of three or more cases of ELST published in the English literature were selected for analysis. RESULTS: Hearing loss and tinnitus were present in almost all our cases. All of them were evaluated with audiometric tests, computed tomography and magnetic resonance imaging. All the patients were treated surgically with preservation of the facial nerve and preoperative embolization was performed in 5 patients. Genetic study was performed on all our cases and revealed the presence of von Hippel-Lindau syndrome in one patient who had the tumor as the initial manifestation of his syndrome. None of the patients received postoperative radiotherapy and one of them had recurrence of the tumor 13 years following surgery. CONCLUSIONS: Complete surgical resection with preoperative embolization of large tumors is the mainstay treatment for ELST. The facial nerve should not be sacrificed unless it is totally invaded by the tumor. A long term follow up is recommended and the role of postoperative adjunctive radiotherapy is still controversial.

Salvage surgery of vestibular schwannoma after failed radiotherapy: the Gruppo Otologico experience and review of the literature.

OBJECTIVES: The use of radiation therapy has largely widespread and becomes in many centers the preference modality of treatment for symptomatic patients who are old, medically unfit for surgical therapy, those who refuse surgery and in some recurrent or residual growing tumors. The risk of radiotherapy failure in the treatment of vestibular schwannoma might be underestimated in the literature. The purpose of this study is to show the Gruppo Otologico experience with salvage surgery to better understand the surgical outcomes and difficulties in treating vestibular schwannoma after failed radiotherapy. STUDY DESIGN: Retrospective chart review of patients who required salvage surgery of vestibular schwannoma after failed radiotherapy. SETTINGS: Quaternary referral otology and skull base center. RESULTS: Between 1987 and 2010, 2500 cases of VS underwent surgical treatment at the Gruppo Otologico. Nineteen patients had received stereotactic radiation therapy before the surgical treatment. The interval time between radiotherapy and surgical salvage ranged from 1 to 10 years. In all the cases decision of surgery was taken following an increase in tumor size with or without new onset of symptoms. Complete tumor removal was achieved in 86.6% of the cases through a transotic, transcochlear or enlarged translabyrinthine approach with trans-apical extension. Difficult dissection of the tumor was encountered in 93.3% the cases. The facial nerve was anatomically preserved in 93.3% but its function was worsened in 73.3% of patients after at least 6 months of follow up. Malignant transformation of the vestibular schwannoma was encountered in one patient. CONCLUSION: Complete surgical resection of VS is more difficult after radiotherapy with relatively poor facial nerve outcomes and nearly impossible hearing preservation. Patients who receive radiation therapy for the treatment of vestibular schwannoma should be made aware of its potential complications and risk of failure, especially in young patients and NF2 cases.

Facial reanimation after facial nerve injury using hypoglossal to facial nerve anastomosis: the gruppo otologico experience.

To evaluate the results of facial nerve reanimation after facial nerve injury by means of hypoglossal to facial nerve anastomosis. Retrospective case review. Private neuro-otologic and cranial base quaternary referral center. Sixty patients underwent hypoglossal to facial nerve anastomosis for facial nerve reanimation between April 1987 and December 2010. Only forty patients completed a minimal follow up of 24 months at the time of evaluation and were included in the study population. Facial nerve paralysis was present for a mean duration of 11.3 months (range 2-42 months) and all the patients had a HB grade VI prior their surgery. Final facial nerve motor function. The most common cause of facial paralysis was vestibular Schwannoma surgery. All the patients achieved a postoperative HB grade III or IV after a mean follow-up time of 20 months. The facial movements were detected after a period that ranged from ranged from 5 to 9 months. Only 4 patients suffered from difficulties during eating and drinking and three of them had associated lower cranial nerve deficit. Despite the various techniques in facial reanimation following total facial nerve paralysis, the end to end of hypoglossal to facial nerve anastomosis remains one of the best treatments in cases of viable distal facial stump and nonatrophic musculature.