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Objective:To analyze the clinical features and mutation of myeloid differentiation factor 88 (MYD88) L265P in patients with diffuse large B-cell lymphoma (DLBCL) of central nervous system (CNS).Methods:The clinicopathological materials of 45 cases of DLBCL of CNS were retrospectively collected in Xuanwu Hospital, Capital Medical University from September 2014 to February 2017. The clinicopathological data were retrospectively analyzed, combined with immunohistochemistry, EB virus in situ hybridization, imaging and medical history. The mutation of MYD88 L265P gene was detected by pyrosequencing and its clinical significance was analyzed. Results:The age of the patients ranged from 42 to 82 years [(57.6±8.8) years], including 24 males and 21 females. Totally 93.3% (42/45) of the patients had supratentorial tumours, which were single or multiple. The cerebral hemisphere (31/45, 68.9%) was the most common involved site, and 21 cases (21/45, 46.7%) had multiple lesions. Histologically, DLBCL in the CNS showed diffuse infiltration of tumor tissue, some of which grew around blood vessels in a "sleeve" arrangement. CD 20 and CD 79a were diffusely and strongly positive. Thirty-nine cases (39/45, 86.7%) were non-germinal center B cell (non-GCB) subtype and 6 cases (6/45, 13.3%) were germinal center B cell (GCB) subtype. MYD88 L265P mutation was found in 64.4% (29/45) patients. There was statistically significant difference between non-GCB type (71.8%, 28/39) and GCB type DLBCL (1/6, P=0.017). Compared with the operation/biopsy group without chemotherapy, operation+chemotherapy, biopsy+chemotherapy, operation/biopsy+chemotherapy+stem cell transplantation can improve the survival and prognosis ( HR=0.05, 95% CI 0.01-0.33 , P=0.002; HR=0.04, 95% CI 0.01-0.36 , P=0.004; HR=0.01, 95% CI 0.00-0.17 , P=0.001; respectively). Conclusions:DLBCL of the CNS is aggressive tumor with poor prognosis, the clinical manifestations are complex and diverse, and the diagnosis is challenging. MYD88 L265P is a common and specific gene mutation in primary CNS lymphoma(PCNSL), which is of great significance in the diagnosis and treatment of lymphoma. The MYD88 L265P mutation was more frequently detected in non-GCB than GCB subtype. Chemotherapy can improve the survival rate of PCNSL patients. If chemotherapy achieves complete remission and autologous hematopoietic stem cell transplantation is performed, there may be a chance of long-term survival.
Résumé
Objective To summarize the clinicopathological features of cystic meningioangiomatosis.Methods The clinical manifestations,imaging characteristics and pathological features of a case of cystic meningioangiomatosis were analysed,and the relevant literature was reviewed.Results A 16-year-old male patient from Xuanwu Hospital,Capital Medical University had a history of epileptic seizures for more than three months.Magnetic resonance imaging (MRI) demonstrated a cystic mass in the left frontal lobe with long T1 and long T2 signals.Extensive resection of the upper frontal gyms was performed.The excised lesion presented with a cystic shape after incision and contained colorless translucent liquid.Microscopic examination of the lesion showed that the number of blood vessels in the local cortex of the brain tissue was increased and the vessels appeared to be branching.The blood vessel walls were surrounded by proliferative spindle cells,which were arranged in concentric circles.Immunohistochemical study revealed that those spindle cells and the cyst wall were vimentin positive.These cells had a rich reticular fibers.Ten months after the operation,the general condition of the patient was good,no epileptic seizure was observed,and the follow-up MRI did not reveal any residual lesion.Conclusions MRI of cystic meningioangiomatosis shows cystic space occupying.Pathological findings show typical features of meningioangiomatosis and cystic space formation.Cystic meningioangiomatosis has good prognosis after surgical resection.
Résumé
Objective@#To summarize the clinicopathological features of cystic meningioangiomatosis.@*Methods@#The clinical manifestations, imaging characteristics and pathological features of a case of cystic meningioangiomatosis were analysed, and the relevant literature was reviewed.@*Results@#A 16-year-old male patient from Xuanwu Hospital, Capital Medical University had a history of epileptic seizures for more than three months. Magnetic resonance imaging (MRI) demonstrated a cystic mass in the left frontal lobe with long T1 and long T2 signals. Extensive resection of the upper frontal gyrus was performed. The excised lesion presented with a cystic shape after incision and contained colorless translucent liquid. Microscopic examination of the lesion showed that the number of blood vessels in the local cortex of the brain tissue was increased and the vessels appeared to be branching. The blood vessel walls were surrounded by proliferative spindle cells, which were arranged in concentric circles. Immunohistochemical study revealed that those spindle cells and the cyst wall were vimentin positive. These cells had a rich reticular fibers. Ten months after the operation, the general condition of the patient was good, no epileptic seiƶure was observed, and the follow-up MRI did not reveal any residual lesion.@*Conclusions@#MRI of cystic meningioangiomatosis shows cystic space occupying. Pathological findings show typical features of meningioangiomatosis and cystic space formation. Cystic meningioangiomatosis has good prognosis after surgical resection.