Academic Center for Sinonasal, Ear and Skull Base tumors

Last updated: 365 days ago.

To provide "advanced patient care" for patients with sinonasal, ear and skull base tumors, by means of minimally invasive techniques (surgical and non-surgical). With the skull base team and the concept of "group surgery / therapy” , the best form of treatment can be determined in view of the specific complaints and wishes of the patient.

Advanced patient care is possible through clinical outcomes studies, in-depth anatomical study, molecular science and genetics research and an international training program.

Academic Center of Excellence

Research Activities

We are currently developing a prospective database regarding the treatment, evolution, follow-up and quality of life of the entire spectrum of sinonasal, ear and skull base tumors treated in the Erasmus MC starting 01 January 1st, 2018, the so called REPORT ( The Rotterdam Skull Base Prospective Registry).

This will facilitate the group being continuously involved in research and reporting of outcomes and quality of life of patients harboring skull base tumors. In the past years we have concentrated on our " clinical outcomes research" and this has led to 4 articles submitted to journals with high impact factors.

We are currently busy with the analysis of our clinical data on adenoid cystic carcinomas, meningiomas, juvenile angiofibroma's and esthesioneurobastomas. Late last year we started with a project wherein tumor specimen from resections and/or biopsies are stored as tissue microarrays allowing us to support our clinical data with necessary immuno and mutation studies giving us a good basis for fundamental and translational research for the coming 10-15 years. This year, we are started with a project with the Netherlands Cancer institute using the technique of Photodynamic therapy for recurrent sinonasal and skull base tumors. Two studies together with Leiden University as initiators have started namely; Understanding vestibular schwannoma treatment decision making as experienced by the patient and National inventory of patients diagnosed with NF2 tumors. Together with the Holland Proton Therapy Center, studies have been initiated to check the effectiveness of proton therapy as primary/adjuvant treatment for meningiomas, chordomas and chondrosarcomas. Ongoing is the study on the progressive hearing loss and relation to cochlea dose after LINAC based stereotactic radiotherapy for vestibular schwannomas ( not to pretretament hearing level). Another ongoing study is the measurement of the petrous bone on CT scans to clearly establish landmark relationships for middle fossa surgeries.

Type of

Collaborations

project with the Netherlands Cancer institute on the use of PDT for recurrent sinonasal and skull base tumors

ongoing research projects with Leiden and Holland Proton Therapy Center ( HPTC)

Our ACE is closely connected with the ACE for head and neck tumors and neurooncology group in terms of patient care.

Research projects are currently being drawn between our ACE and the ACE for innovative oncological surgery of the Erasmus MC aimed to improve our resection margins in sinonasal and skull base surgery and imrpove early diagnosis of neck node and distant metastases.

We are actively collaborating with EURACAN(G7) domain in the accumulation of European data on nasopharynx and salivary gland tumors of the head and neck region.

Educational

Contributions

The members of the workgroup have been very active in terms of educating medical students via the master's program and the minor programs of the college of medicine. Same members have been very active in educating residents from the department of ENT, neurosurgery, radiology, pathology and radiation oncology through daily endorsements, special lectures, anatomy courses, OR assists, ward and poli clinic supervision.

We are constantly asked by colleagues and fellows from with in and outside the Netherlands for rotations and observerships. In the past 5 years we have had 4 fellows from the Philippines and 1 from the UK who came to observe in our clinics and learn the minimal invasive techniques/ treatment for sinonasal, ear and skull base tumors.One fellow rotating at the Amsterdam Medical Center did his extra fellowship in our clinic to get more patient exposure in minimal invasive skull base surgery.

We are presently collaborating with the Erasmus University college of medicine in setting up a minor program for skull base anatomy and tumors in english to allow students from outside the Netherlands and outside the EU to participate.

During this year's skull base course of the ErasmusMC, the first live surgery was broadcasted via internet and this attracted more than 118 participants from 22 countries and 47 states.

Patient

Care Activities

More than 80 % of our patient care within the ACE is academic and tertiary owing to the rarity of these tumors and the complexity of the treatment modalities. Patient care is by nature multidisciplinary and our clinical pathways/treatment protocols have been validated and are regularly updated depending on existing evidence/new evidence and institutional experience. Most of our protocols can be found in the KIS.

We have started assessing our pathways using the Value based health care program. Quality of life issues will be one of our priorities in the coming years as discussed during the last visitation of the Royal Dutch Society for Otorhinolaryngology.

Societal Relevance to Research, Education and Patient Care

Despite the rarity of sinonasal and skull base tumors, the variability in tumor presentation and the dearth of information about the biology of these tumors lead to a great variability in treatment options of the lack of treatment protocols that re really evidence based. Oftentimes is the treatment multimodal leading to significant morbidities which can significantly affect the quality of life of patients after treatment.

To be able to come up with the necessary treatment guidelines, the first step is to bundle up clinical data together with data on how such tumors behave. Fast accrual of these data is only possible if other centers with the Netherlands and hopefully within Europe will be involved together with patient organisations.

This is one of the reasons why we were quite active in the past years to refresh our database and join one of the domains of the European reference Network for rare diseases. After a well received symposium on sinonasal tumors here at the Erasmus MC organised by our group and the head and neck tumor patient group (PVHH) a consensus day for the management of sinonasal, ear and skull base tumor is currently being organised (initiated by our group) for next year, this is again in cooperation with the patient organization (Klankbord) and the dutch workgroup for head and neck tumors.

Viability of Research, Education and Patient Care

There is still so much to learn about sinonasal, ear and skull base tumors. Due to the rarity of these tumors, their biology is not fully understood and existing treatment schemes are really not evidence based. The same is true when it comes to quality for quality of life issues. Research in these fields will continue in the coming years.

To make sure that we have enough data (clinical and tumor biology data) in the coming years, we already started building our clinical data base coupled with tissue microarrays for future immune/genetic research. This is a joint project by all departments involved with the ACE. So far we have a total of 190 patients included in our database

We have currently two PhD students working on the adenocarcinomas and the use of PDT for recurrent sinonasal tumors. In terms of continued patient care and education, we are currently training two young consultants/fellows who are also interested in skull base surgery with the plan of taking them as part of the group in the near future.

Key and relevant publications of the last five years

  • Olfactory neuroblastoma with hypernatremia. Hoorn EJ, Monserez D et al. J Clin Oncol. 2014 Mar 31.
  • The incidence of post operative thromboembolic complications following surgical resection of intracranial meningioma. A retrospective study of a large single center patient cohort. Hoefnagel D, Jwee L et al. Clin Neurol Neurosurg. 2014. Aug; 123-150-4
  • Combined modality treatment improved outcomes in sinonasal undifferentiated carcinoma: single institutional experience of 21 patients. Al Mamgani A, van Rooij P et al. Eur Arch Otorhino. 2013 Jan; 270(1):293-9
  • Postoperative value of serum Squamous Cell Carcinoma Antigen as a predictor of recurrence in sinonasal inverted papilloma.van Zijl FV, Monserez DA, Korevaar TI, Bugter O, Wieringa MH, Baatenburg de Jong RJ, Hardillo JA.Clin Otolaryngol. 2016 Sep 26.
  • Identifying at Diagnosis the Vestibular Schwannomas at Low Risk of Growth in a Long-term Retrospective Cohort.Wolbers JG, Dallenga AH, van Linge A, Te West M, Kummer EE, Méndez Romero A, Pauw BK, Wieringa MH.Clin Otolaryngol. 2016 Apr 18.
  • Hearing Loss Progresses Faster in Patients With Growing Intracanalicular Vestibular Schwannomas.van Linge A, Borsboom GJ, Wieringa MH, Goedegebure A.Otol Neurotol. 2016 Oct;37(9):1442-8.
  • Molecular profile of nasopharyngeal carcinoma: Ooft, Baatenburg R, Koljenovic S, Hardillo J et al J Clin Pathol. 2018 Apr;71(4):351-359
  • Adjuvant radiotherapy in sinonasal mucosal melanoma: A retrospective analysis. Caspers CJI, Dronkers EAC et al.Clin Otolaryngol. 2018 Apr;43(2):617-623.
  • Surgical management of inverted papilloma; a single-center analysis of 247 patients with long follow-up.BugterO, Monserez,D,hardillo J. et al.J Otolaryngol Head Neck Surg. 2017 Dec 20;46(1):67
  • Differences in cancer gene copy number alterations between Epstein-Barr virus-positive and Epstein-Barr virus-negative nasopharyngeal carcinoma.Ooft ML, van Ipenburg, Koljenovic S et al.Head Neck. 2018 Sep;40(9):1986-1998.

PhD theses of the last five years

  • Ongoing : Management of Intestinal type adenocarcinomas (Dominiek Monserez- promovendus)
  • Ongoing: Photodynamic therapy for recurrent sinonasal and skull base tumors (Tessa van Doeven- promovendus)

Non-scientific publications related to the ACE

  • https://pvhh.nl/events/informatiemiddag-over-sinonasale-tumoren/

Principal coordinator(s)