ObjectiveTo review the clinical records of patients with central nervous system (CNS) embryonal tumors, not otherwise specified (NOS); and summarize their clinical features, diagnosis, and treatment.MethodWe reviewed the data of patients with intracranial tumors admitted to Department of Neurosurgery of West China Hospital, Sichuan University from January 2014 to December 2016, and retrospectively analyzed the clinical features, diagnosis, and treatment of seven patients with CNS embryonal tumors, NOS.ResultsThere were 4 males and 3 females, and the mean age was 25.4 years old. The tumor was located in cerebral hemisphere in 5 patients, and in third ventricle in 2. Clinical presentation included headache, nausea, and vomiting due to intracranial hypertension, and focal neurological signs. All patients underwent craniotomy for tumor resection and postoperative pathology confirmed CNS embryonal tumor, NOS. The patients were followed up for 6 months to 3 years, and 2 patients died during follow-up.ConclusionsCNS embryonal tumor, NOS is malignant intracranial lesion, and has been enlisted as a separate entity under classification of CNS embryonal tumors. It has its unique radiological features, including rare occurrence of perilesional edema, cystic changes, and clear demarcation. Through comprehensive treatment including surgical resection, chemotherapy, and radiation therapy, patients can enjoy prolonged survival and improved quality of life.
Objective To investigate the expression and clinicopathological features of fumarate hydratase (FH) in multiple leiomyoma of the uterus. Methods Relevant clinicopathological data of patients with multiple leiomyoma of the uterus diagnosed pathologically between September 1, 2018 and August 31, 2019 were collected from the Department of Pathology of West China Second University Hospital. The morphology, FH protein expression and prognosis of FH negative patients were observed. Results A total of 900 patients were included. Among them, there were 825 cases of nonatypical leiomyomas, 36 cases of leiomyoma with bizarre nuclei, 34 cases of cellular leiomyoma and 5 cases of mitotically active leiomyoma. A total of 69 patients with negative FH expression were included, with an average age of (37.28±8.22) years. Among them, there were 43 cases of nonatypical leiomyomas, 19 cases of leiomyoma with bizarre nuclei, 6 cases of cellular leiomyoma, and 1 cases of mitotically active leiomyoma. At low magnification, staghorn shaped blood vessels, alveolar pattern edema, palisade distribution of smooth muscle cells and bizarre nuclei smooth muscle cells could be seen in patients with negative FH expression. At high magnification, cytoplasmic eosinophilicglobules andeosinophilic nucleolus surrounded by a halo could be seen in patients with negative FH expression. Among the 69 cases with negative FH expression, 60 cases (86.96%, 60/69) were followed up. All patients survived, of which 24 cases (40%, 24/60) recurred. Conclusion Immunohistochemically, the marker FH for multiple leiomyoma of the uterus is helpful to improve the identification of FH deficient leiomyoma in clinical work as well as early diagnosis in hereditary leiomyomatosis renal cell cancer syndrome.