ABSTRACT
Purpose To investigate the clinicopthologic features of dysplastic cerebellar gongliocytoma/Lhermitte-Duclos disease (LDD) and to discuss the diagnosis and differential diagnosis of the tumor.Methods Histopathological characteristics and immunohistochemical of SP results of 6 cases of LDD were studied and the relevant literatures were reviewed.Results 6 cases ranged in age from 23 to 56 years old,with an average of 34 years.The clinical manifestations were intracranial hypertension with or without cerebellar signs.MRI manifestations were characteristic by "tiger spots".Histology showed local cerebellar structure disorders,granulocytes and Purkinje cells decreased and replaced by a large number of abnormal ganglion cells.2 cases were followed up for 5 to 8 years recurrence,the remaining 4 cases recovered well.Conclusion LDD is a rare primary benign lesions of the cerebellum.Diagnosis depends on imaging and histopathological examination.The gross total resection is the best treatment choice.
ABSTRACT
Purpose To investigate the pathological morphology and diagnostic parameters in the high-grade primary neuroendocrine carcinoma (NECa) of the uterus.Methods The clinical manifestations,pathological morphology,and immunohistochemical features of 25 uterine high-grade primary NECa cases were analyzed,and the relevant literatures were reviewed.Results The age of the 25 patients ranged from 24 to 72 years (mean age,44 years).Clinically,most of them complained of the vaginal bleeding or occupying lesions.84％ cases displayed typical neuroendocrine architectures histologically,and neurosecretory granules were observed in the electron microscopy examination.The nucleus of the small-cell type and large-cell type NECa were typical.Additionally,the mitosis number was more than 20/10 HPF in 64％ cases,10 to 20/10 HPF in 28％ cases,and less than 10/10 HPF in 8％ cases.Furthermore,highgrade NECa was often accompanied with apoptosis (72％) and necrosis (92％).The diagnosis included 13 cases of small-cell carcinoma,5 cases of combined small-cell carcinoma (1 with cervical intraepithelial neoplasia 3,1 with squamous cell carcinoma,and 1 with adenocarcinoma),4 cases of large-cell NECa,and 3 cases of combined large-cell NECa (2 with squamous cell carcinoma,and 1 with adenocarcinoma).The neuroendocrine markers were positive with Syn (96％),CgA (70.5％) and CD56 (78.6％).76.9％ cases demonstrated CKpan positivity,with small foci or linetype of cytoplasmic immunostaining.The proliferation index of Ki-67 was ranged from 60％ to 90％.In this study,13 patients received the uterus excision with adjuvant chemotherapy,and 5 cases died within 2 years.Conclusion High-grade NECa of the uterus is rare,and the terminology is similar to that of the digestive tract and lung.Yet,the diagnostic parameters are slightly different to some extent.The therapy is the surgical resection followed by adjuvant chemotherapy.