Establish a multi -center collaboration group to tackle the "Undead Cancer" Neurological Fibroma Diagnosis and Treatment Forum held

Type -Ⅰ neurofibroma (NF1) is a rare autonomic genetic disease. According to statistics from abroad, the incidence of newborn is about 1/3000, and 50%of patients are related to genetic. At present, there is no validity. treatment approach. Beginning in 2020, the Specialty forum of Type -Ⅰ neurofibroma has been successfully held for three sessions. This forum relies on the consensus of the first clinical diagnosis and treatment expert in type I in China, and is committed to improving the systematic and standardization of treatment. At present, the Ninth People's Hospital of Shanghai Jiaotong University School of Medicine has established the first treatment collaboration group in China with 17 well -known medical centers in China.

On July 23, as an important part of the 2022 Shanghai Plastic Surgery Technology Week activity, the Nurnic Fibroma Special Disease Forum was successfully held in Shanghai with an online+offline model. The theme of this forum with the theme of "overcoming difficulties and focusing on new medicine", inviting multiple authoritative experts and scholars in the domestic NF1 field to systematically explain the relevant content of type I neurofibroma, in order to allow domestic doctors and society to society The public understands the advanced treatment progress and scientific and scientific treatment methods.

first part:

Starting from the guide

Actively carry out multi -center treatment collaboration

In this session, Professor Wang Zhichao of the Ninth People's Hospital of Shanghai Jiaotong University Medical College and Professor Luo Xusong, Professor Luo Xusong, Ninth People's Hospital affiliated to Shanghai Jiaotong University Medical College.

Professor Wang Zhichao (left) and Professor Luo Xusong (right)

Professor Yang Jun, chairman of the conference and the Ninth People's Hospital of Shanghai Jiaotong University School of Medicine, said in his speech that when facing the pain of patients, we need to use more new medical technologies to provide this rare and complex disease with a multi -dimensional degree The treatment plan brings more hope to patients, which is exactly the value of our group.

Professor Yang Jun

Professor Happy Hua, the co -chairman of the forum and the First Affiliated Hospital of China Medical University, said that with the advent of the new targeted medicine, the ideas and ideas of this rare disease treatment and control will also change. Common discussions to actively attack and control the development and treatment of such rare diseases, and provide patients with a best clinical treatment plan.

Professor Professor

A total of more than ten experts were invited at this conference to introduce the basic research and clinical progress of type I neuroplastin disease from different aspects such as pediatrics, skin, plastic surgery, and tumors. Professor Wang Zhichao of the Ninth People's Hospital of Shanghai Jiaotong University School of Medicine first interpreted the first NF1 clinical diagnosis and treatment expert consensus (2021), led by the Ninth Hospital and published by the Ninth Hospital and published in China (2021). The evaluation diagnosis, treatment plan, clinical trials, patient follow -up and education of the disease have been introduced in a comprehensive introduction; at the same time, it also shows the clinical targeted drugs of the foreign clinical targeting drugs, the domestic Zhengda Tianqing, and Shanghai Kaizhou Pharmaceuticals, etc. Multiple innovative drugs are typical of the treatment of cluster neurofibroma, skin -shaped neurofibroma, and malignant peripheral nerve sheath tumors; and pointed out the importance of promoting the clinical trials of domestic related drugs. At present, Professor Li Qingfeng of the Ninth People's Hospital affiliated to Shanghai Jiaotong University Medical College led the development of I orphania drugs of type I neurofibroma, and clinical study of many domestic innovative drugs.

Professor Wang Zhichao

the second part:

Neurological fibroma disease diagnosis and treatment

New means to promote new development

The second part of the conference was Professor Wang Zhichao of the Ninth People's Hospital affiliated to Shanghai Jiaotong University School of Medicine, Professor Luo Xusong of the Ninth People's Hospital of Shanghai Jiaotong University School of Medicine, Professor Yin Guoqian, First Affiliated Hospital of Guangxi Medical University, and Ninth People's Affiliated to Shanghai Jiaotong University Medical College Professor Hu Xiajie of the hospital co -chaired.

Professor Wang Zhichao (left) and Professor Luo Xusong (right)

Professor Hu Xiajie (left) and Professor Yin Guo (right)

Professor Xu Xuegang, the First Affiliated Hospital of China Medical University, said that although type I neurofibroma is a rare disease, the patient group is still huge. There are about 460,000 patients with NF1 gene mutations in my country. The neurofibroma of NF1 patients is mostly multiple, and the number of people can reach thousands. Most patients will have obvious symptoms at the age of 4 to 5. Milk coffee spots are the main manifestations of type I neurofibroma. Professor Xu Xuegang introduced that surgical surgery is currently an effective means to treat type I neurofibroma, but there are still some problems.

Professor Xu Xuegang

"In the past, surgical resection was the only choice for the treatment of neurofibroma, but sometimes there were problems such as difficulty in surgery and easy recurrence after resection." Professor Yuan Xiaojun, Affiliated to Xinhua Hospital affiliated to Shanghai Jiaotong University, introduced, 2020 "New England Medical Journal" The results of the relevant clinical drugs released in the related clinical drugs showed that among the 50 children aged 10.2, the disease confirmation rate was 70%, the long -term relief rate was 56%, and the disease progress rate was 12%. It is caused by stopping or reduction. At present, under the leadership of Professor Li Qingfeng, Xinhua Hospital also conducted phase I clinical trials of NF1 children's drug treatment. At this stage, the clinical symptoms of 16 subjects have alleviated and have not had serious adverse reactions. I hope that further progress can be achieved in the future.

Professor Yuan Xiaojun

Professor Li Ming of Xinhua Hospital affiliated to Shanghai Jiaotong University School of Medicine, introduced that inlaid coffee spots are the most common clinical symptoms of inlaid type Ⅰ neuromanoma (MNF1). Compared with NF1, MNF1 usually has the characteristics of relatively mild clinical symptoms and low risk of related complications. In terms of diagnosis, Professor Li Ming stated that genetic testing confirmed that NF1 mutations were the gold standard diagnosis of MNF1, emphasizing the significance of gene diagnosis for the diagnosis of disease diagnosis, identification diagnosis, and family history of family history of such diseases. Professor Li Ming

Professor Xu Xuewen, Huaxi Hospital of Sichuan University, shared the case of pre -operating embolism combined with surgery for huge fibroma in the hip. Only 300ml of bleeding during the patient surgery. Professor Xu said that pre -operative embolism can reduce bleeding during surgery, shorten surgical time, and reduce the risk of surgery. Surgical resection within 24 hours after embolism can avoid thorous edema and new blood vessels, and can also retain the activity of tumor skin, which is conducive to the reconstruction of the tumor skin.

Professor Xu Xuewen

"During 2010 ~ 2019, our hospital surgery for 143 patients with neurofibroma." Professor Li Yang, Professor Li Yang of Xijing Hospital of the Air Force Military Medical University, said that during the diagnosis and treatment of these patients, the patient must be performed to popularize the patient The disease, inform the patient's surgery to solve and what problems can be solved, and fully respect the patient's opinions when formulating the surgical plan.

Professor Li Yang

Professor Hu Xiajie, Professor Hu Xiajie, Affiliated to the School of Medicine, Shanghai Jiaotong University, reviewed the experience of neurofibroma surgery over the years. He said that at present, there are still problems that are difficult to solve in surgery. I hope that in the future Essence

Professor Hu Xiajie

Professor Lin Zhimiao of Southern Medical University Dermatology Hospital further introduced the early diagnosis and identification diagnosis of type I neurofibroma. Professor Lin Zhimiao said that the diagnosis of the disease was made based on the overall clinical condition. It is recommended to use RNA -based NF1 mutant molecular detection to confirm the diagnosis. The disease is progressive, changing and deteriorating, and it affects multiple organ systems: skin, central and peripheral nervous systems, eyes, bones and blood vessels and endocrine systems. Children's symptoms may be mild, and regular follow -up (especially complicated cases) is recommended to discover early lesions.

Professor Lin Zhimiao

"Giant neurofibroma tumors are prone to repeated bleeding and life -threatening life. It is extremely difficult to eradicate. The surgery is mainly reduced to reducing and conservative treatment. There are fewer hospitals who are willing to receive." Although there are various difficulties, the "cure" resection is necessary. After most of the tumor body resection, the quality of life has improved significantly, the formation of the pathogen blood vessels is removed, and the risk of bleeding is significantly reduced. During the treatment of tumors, hemostasis should be fully stopped during surgery, and controlling controls are not recommended.

Professor Liu Fei

"Different from the dermatology and plastic surgery department, the patients diagnosed by the Department of Oncology are mainly malignant peripheral neurobetoma," said Professor Yang Jilong, the Cancer Hospital of Tianjin Medical University. %about. In the future, in addition to surgery, the treatment of malignant peripheral nerve sheath tumors should also be treated with multi -disciplinary collaboration if necessary, multi -pronged approach, and extend the survival of patients.

Professor Yang Jilong

The third part of the round table forum

All -round, multi -disciplinary

Help diagnosis and treatment of type I neurofibroma

Professor Li Qingfeng of the Ninth People's Hospital of Shanghai Jiaotong University Medical College presided over the round table forum in the meeting. Professor Happy Hua, the First Affiliated Hospital of China Medical University, Professor Ho Chiqi, Southern Medical University, Southern Medical University, and Renji, a affiliated to Shanghai Jiaotong University Medical College Professor Zhang Yueping, Professor Gu Jianying, Affiliated to Fudan University, Professor Yan Wangjun, Affiliated to Fudan University, Professor Zheng Shengwu, Fujian Provincial Hospital, and Professor Xu Xuewen, Huaxi Hospital of Sichuan University, expressed his views on the basis of the previous experts.

Professor Grass Professor of the First Affiliated Hospital of China Medical University believes that the diagnosis and treatment of NF1 should start from the perspective of pathogenesis and genetic perspective. In this process, multidisciplinary exchanges and collaborations are very important, helping to enlighten each other and focus on different diseases of each patient, so as to bring better help and treatment to patients.

Professor Ho Chiqi, Southern Medical University, believes that the resection of neurofibroma is one of the surgery that will bring the greatest life in plastic surgery. In this regard, he is looking forward to a certain breakthrough in drug treatment NF1 in the future. It should not be ignored that in the surgical resection of the tumor, the embolism is often faced to prevent bleeding, which causes tissue necrosis. Therefore, in the surgical resection of the tumor, on the one hand, bleeding should be prevented, and on the one hand, it is necessary to prevent embolism from causing tissue necrosis. On this basis, Professor Hu suggested to introduce experts in the intervention department to help judge the scope of the internet intervention of tumor body.

Professor Zhang Yueping of Renji Hospital affiliated to Shanghai Jiaotong University believes that in terms of single -gene NF1 rare diseases as three -generation IVF, the technology of Renji Hospital affiliated to Shanghai Jiaotong University is relatively leading in Shanghai and the success rate is very high. However, in the diagnosis and treatment of female patients in NF1, what is currently worried about ovulation promotion and bleeding during ovulation, or large doses of rapid rows, which may lead to increased tumors in patients. To this end, communicating with experts from other disciplines, providing more ideas for patients' successful fertility, and expressing their expectations.

Professor Gu Jianying, Affiliated to Fudan University, said that in the future treatment, surgeons should think about how to better cooperate with other subject doctors to better serve each patient. In addition, Professor Gu also suggested that different medical centers in China can use data networking to build disease databases nationwide, including diagnosis and genetic analysis, which facilitates the consultation of various discipline doctors in different hospitals. Professor Yan Wangjun, a Cancer Hospital of Fudan University, from the perspective of the prevention, screening, diagnosis, treatment, and rehabilitation of NF1 diseases. It is recommended to improve the entire chain link. Each link can find effective treatment methods to block the progress of the disease, which is also a great help for patients. At the same time, Professor Yan believes that on the basis of strengthening the content of NF1 diagnosis and treatment and integrating the joint participation of various discipline doctors, we must improve the national case registration system, find out the current status of the disease of this type of disease in my country, to better collect cases, promote clinical clinical clinical clinical With basic research, the opinions of Chinese doctors in the field of international NF1 are looking forward to.

Professor Zheng Shengwu, Fujian Provincial Hospital, said that the meeting has gained a lot of understanding and understanding of disease prevention and diagnosis and treatment. In future clinical work, it can better serve patients with type I nerve fibroma.

In the free speech, Professor Xu Xuewen, Huaxi Hospital of Sichuan University, believes that although the NF1 rare disease treatment cooperation group has been established nationwide, each hospital has its own diagnosis and treatment standards, including preoperative examination and diagnosis process. It is hoped that this standard can be unified in the future, and at the same time, it is hoped that a fresh specimen can be made to retain the process, which is convenient for each hospital to communicate with each other.

Finally, Professor Li Qingfeng, the Ninth People's Hospital of Shanghai Jiaotong University School of Medicine, said that we must not only collect data, but also ensure that the data must be valuable and can form available clinical evidence to guide clinicians to provide more patients with NF1 patients with more more patients with NF1 patient good service. In this regard, Professor Li Qingfeng suggested that after forming a multi -center NF1 treatment collaboration center, it is necessary to establish a good ideological intellectual property system, overall rights and responsibility segmentation system, and hardware data system management system, and do some more valuable original originals. Sexual work jointly promotes the systematic and standardized diagnosis and treatment of NF1 diseases.

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