A team of specialists at Shanghai Jiao Tong University School of Medicine has developed a novel method for removing enormous and complex tumours known as giant plexiform neurofibromas (PNF), providing hope to those suffering from a severe form of the genetic illness neurofibromatosis Type 1 (NF1). 

Their method, known as FENCY ligation, was tried along with preoperative embolisation (blocking blood vessels before surgery to minimise bleeding). The results, published in the Chinese Journal of Plastic and Reconstructive Surgery and reported by other reliable sources like Wiley's Clinical Genetics journal and Orphanet Journal of Rare Diseases, confirm how serious NF1 is and how challenging PNF surgery can be. 

PNFs are benign (non-cancerous) tumours that develop along nerves. According to the National Institutes of Health (NIH), they occur in approximately 25% of patients with NF1, a disorder that affects one in every 3,000 births worldwide. These tumours can become enormous, causing severe deformity, agony, and trouble moving. 

"It has been challenging to resect giant PNFs, particularly when the tumours are highly vascular and located in critical anatomical regions where bleeding and irreversible nerve damage are likely to occur," noted Dr. Zhichao Wang, the study's principal author. 

The FENCY method consists of tying up blood arteries surrounding the tumour in a "fence-like" design. This method prevents bleeding (known medically as haemostasis) during surgery. This dual method, along with preoperative embolisation, which prevents blood supply to the tumour before it is removed, lowers surgical risks. 

In their research, the team operated on 11 patients. The average tumour size was 30.4 cm, with the largest measuring 55.6 cm (about the size of a huge watermelon). Despite the difficulties, most patients reported improved mobility and less discomfort following surgery. 

"Most patients reported significant functional improvement and high levels of satisfaction," Wang told reporters. "It's gratifying to see how these patients, some of whom had been dealing with these tumours for years, regained mobility and improved their quality of life after surgery." 

Experts believe that surgery for these tumours is difficult because they develop around nerves and blood arteries. Cutting them out can cause severe bleeding or irreversible nerve damage. This situation has reduced surgical choices for many persons with NF1. 

The team's findings are particularly significant because they demonstrated that the procedure can be used even on the face and neck, which are generally too dangerous for standard surgery. 

Nonetheless, the researchers remain concerned. "We hope that larger patient groups will be used to fully understand the long-term impacts and refine the procedure further," Wang told me. 

While FENCY is not yet a global standard, the findings indicate that it may become a safer alternative for those with large PNF. The work contributes to a growing body of research into new strategies to support patients with NF1, who frequently experience lifelong medical issues. 

The study also emphasises the necessity of worldwide research collaboration and innovation for treating uncommon diseases. According to the NIH, despite its rarity, NF1 can be catastrophic to patients if not treated effectively.