2019年 · 第99卷第19期

Regarding the diagnosis of pituitary adenoma recurrence after surgery, there are currently several different criteria that have not been completely unified. Some scholars believe that as long as postoperative imaging examination finds that the tumor increases again, it can be diagnosed as recurrence; Some scholars also believe that after completely removing pituitary adenoma and reaching the level of clinical cure and remission, the tumor grows again, which is called recurrence. The residual tumor tissue continues to grow after operation, which belongs to residual tumor regrowth (regrowth) and should not be diagnosed as recurrence. In the "Chinese Expert Consensus on the Diagnosis and Treatment of Recurrent Pituitary Adenoma" (hereinafter referred to as this consensus), recurrent pituitary adenoma is defined as: the reappearance of symptoms and signs that have disappeared after pituitary adenoma resection; The endocrine index increased again after reaching the remission standard; Imaging examination showed tumor growth again. It is precisely because of the different understanding of the concept of recurrence, the different length of postoperative follow-up and the lack of bulk case reports, and the recurrence rates of various types of pituitary adenomas are quite different, so the recurrence rates of pituitary adenomas reported in the literature are quite different at present, ranging from 5.4% to 44%.

Pituitary adenoma (PA) is one of the most common intracranial tumors. Most of them show the growth characteristics of benign tumors. Most patients can be cured by surgery and/or drug treatment. However, a small number of pituitary adenomas grow aggressively in imaging, grow faster than general tumors, are resistant to conventional treatments such as surgery, drug therapy and radiotherapy, and often relapse or regrow early after surgery. Such tumors are called aggressive PA or refractory PA^[1,2]。 Pituitary adenoma with intracranial spinal canal metastasis or other systemic metastasis is pituitary adenocarcinoma (PC)^[3]。 The diagnosis and treatment of refractory pituitary adenoma and pituitary adenocarcinoma are very difficult. The tumor can seriously affect the quality of life of patients, even endanger their survival, and the prognosis of patients is poor. Standardized diagnosis and treatment measures are urgently needed. In January 2018, the European Endocrinology Society issued the Guidelines for the Diagnosis and Treatment of Refractory Pituitary Adenoma/Pituitary Adenocarcinoma^[4]The aim is to provide standardized clinical guidance for the diagnosis, treatment and follow-up of refractory pituitary adenoma and pituitary adenocarcinoma. However, there are shortcomings such as unclear diagnostic criteria, and the treatment of this disease is still a worldwide problem. Therefore, the cooperative group formulated the Expert Consensus on the Diagnosis and Treatment of Refractory Pituitary Adenoma in China (hereinafter referred to as the Consensus) to standardize the diagnosis and treatment of refractory pituitary adenoma.

spinal muscular atrophy (SMA) is a neurodegenerative disease caused by the degeneration of motor neurons in the anterior horn of the spinal cord and the medulla oblongata, resulting in progressive and symmetrical muscular weakness and muscular atrophy of the proximal limbs and trunk. Although SMA can be caused by a variety of gene mutations, it generally refers to autosomal recessive diseases caused by mutations in the survival motor neuron 1 (SMN1)^[1]。 The incidence of the disease among surviving newborns in European and American populations is about 1/10 000, and the carrier frequency is 1/40 to 1/50^[2], ranking first among fatal genetic diseases in children under 2 years of age. At present, there is no definite data on the incidence rate in China, and the frequency of carriers in the Chinese population is about 1/42^[3,4]。 As the disease progresses, muscle weakness can further lead to skeletal system, respiratory system, digestive system and other system abnormalities, among which respiratory failure is the most common cause of death. Therefore, the treatment of SMA often requires multidisciplinary evaluation and integrated management. Although international colleagues have developed at least two versions of the SMA multidisciplinary diagnosis and treatment consensus^[5,6,7]However, due to the large number of patients, wide distribution and unbalanced development of economic conditions in China, a standardized opinion is needed for the diagnosis, treatment and treatment of this disease. To this end, experts in pediatric and adult neurology, genetics, respiratory medicine, orthopedics/spine surgery, rehabilitation medicine, clinical nutrition and other specialties have jointly formulated a consensus on the multidisciplinary management of SMA in China to assist clinicians, laboratory diagnosis and prenatal diagnosis related personnel to standardize the diagnosis and treatment of SMA.