Huge facial tumor linked to rare disease removed by city surgeons

Local medical experts have removed a huge tumor from the face of a man suffering from a rare disease. The patient received 4,000 milliliters of blood during the 9-hour surgery, a quantity equivalent to his body's blood volume.

In addition to removing the tumor, which was bigger than an adult male's fist, doctors also reconstructed the patient's facial features to largely reduce the effects of the operation.

The patient had endured the lump on his face since childhood. It grew as he aged and further deformed his face despite repeated surgeries. The lump had increased quickly in size in the past three months and the pain became greater so he went to Shanghai No. 9 People's Hospital.

Dr Li Qingfeng diagnosed the patient suffered from neurofibromatosis type 1, a rare disease affecting one in every 3,000 people.

About 10 percent of patients can experience cancerous changes. However this man's huge tumor had been seen only rarely in clinical practice.

"The tumor was cancerous and it grew rapidly, invading the skull and part of the man's air tube and esophagus. The surgery was a huge risk," Li said.

As the tumor hadn't spread to other parts of the man's body, surgery was still the best solution and doctors developed a detailed plan to ensure the operation's success and the patient's safety.

After the surgery, the man remains in a stable condition and will receive chemotherapy and targeted medication as the next steps, doctors said.

Li said his team had been focused on rare disease research for many years. Experts have developed assessment protocols for neurofibromatosis type 1 to boost proper diagnosis and treatment of the affliction. The team is also involved in clinical research into innovative drugs to treat such rare diseases.