A skilled multidisciplinary team worked successfully at Macquarie University Hospital to remove a large, uncommon and benign – but locally aggressive and highly vascular – nasal tumour from a 37-year-old male patient.

When Khador Farhat experienced four to six months of an increasingly blocked right nostril and recurrent nasal bleeding that were not responding to standard therapies, he sought ENT specialist advice.

In April 2013, Khador’s specialist ordered an MRI of the head, which showed a large vascular tumour in the right posterior nasal cavity and nasopharynx, extending to the nose and pterygopalatine fossa. The mass was also located close to the skull base and major nerves and vessels.

Diagnosed with juvenile nasopharyngeal angiofibroma (JNA), Khador was referred to Associate Professor Ray Sacks, Head of the Department of Otorhinolaryngology and Head and Neck Surgery at Macquarie University Hospital, and one of only a handful of surgeons specially trained in the endoscopic management of JNA.

“JNA is most commonly seen in adolescent males between the ages of 9 and 25 – more narrowly, between the ages of 14 and 18,” said Associate Professor Sacks. “Khador, at 37, was the oldest patient I’ve treated with JNA.”

JNA makes up only 0.5 per cent of all head and neck tumours, and is classed as a hamartoma – a benign vascular tumour caused by incomplete regression of a branchial artery. Blood vessels in and around the tumour express the protein laminim alpha 2, which serves as an early angiogenesis marker.

This type of tumour is locally aggressive and requires complete removal to ensure that residual tumour does not remain. Because of the very vascular nature of the mass, JNA removal requires a highly skilled team.

Associate Professor Sacks was able to assemble a multidisciplinary team at Macquarie University Hospital who could also access some of the best equipment for JNA surgery – including specialised pre-operative selective angiographic embolisation of arteries, intra-operative computer image-guided surgery and coblation technology.

“Because JNA tumours are very vascular, issues around blood supply during surgery are crucial,” said Associate Professor Sacks. “The patient needs to be prepared for removal of the tumour in a way that ensures control of that blood supply.

“This is done using the pre-surgical embolisation of the feeding arteries and vessels, within 24 to 48 hours pre-operatively. It is very important to ensuring successful surgery.”

Macquarie Medical Imaging (MMI) interventional radiologist Dr Geoffrey Parker – who specialises in neuroradiology and head and neck radiology, including advanced MRI neuroimaging, performed the pre-embolisation.

During both the embolisation and the surgery, the team used MMI’s superior intra-operative image guidance system. This allowed them to accurately and safely determine the full extent of the tumour and to remove it from regions surrounded by vital neural structures by minimising any trauma and damage to those vital structures such as the eye, the optic nerve, the brain and the cranial nerves in the vicinity, which supply both sensation to the head and neck as well as movement to the muscles in the region.

Associate Professor Sacks performed an endoscopic resection of the entire tumour through the nostrils without the need for any skin incisions and resultant facial scars and deformities.

“It’s invaluable to have this type of procedure done under the advanced image guidance system at MMI,” said Associate Professor Sacks. “The endoscopic approach allows us to have massive magnification and to be able to accurately identify vital structures and to preserve them. It also enables safe and complete removal of the tumour and thus dramatically reduces any risk of leaving behind tumour cells and having a subsequent recurrence of the tumour.

“The coblation technology available at Macquarie University Hospital also allows us to be able to reduce the tumour size without the risks of massive intra-operative bleeding.”

Khador spent three nights in hospital and returned to work after two weeks.

“When I walked out of hospital, I asked my wife to pass me a mirror so that I could look at my face,” said Khador. “It looked as if no operation had been done. I was amazed.”

Khador returned to work after two weeks. He says that it’s a comfort to be able to breathe normally again. Repeat endoscopies show no sign of any residual or recurrent tumour and he will return to MMI for a follow-up MRI scan at about one year post-operatively to ensure that all the tumour has been fully removed.

About Professor Raymond Sacks

Associate Professor Sacks has authored multiple book chapters and published more than 50 peer-reviewed papers, including a manuscript on the endoscopic management of JNAs. He has been the keynote speaker at multiple conferences and sinus surgical workshops throughout Australasia, Asia, Europe and North America. During 2013, he was the main guest speaker for the Japanese Rhinology Society annual meeting and has been invited to be part of the faculty of the internationally prestigious University of Pennsylvania Annual Sinus Surgery Course in Philadelphia in 2014.

Associate Professor Sacks is currently the Senior Examiner in Otolaryngology/Head and Neck Surgery for the Royal Australasian College of Surgeons and is the immediate past-president of the Australian and New Zealand Rhinologic Society.