婴儿神经内分泌细胞增生症(neuroendocrine cell hyperplasia of infancy,NEHI)是一种成熟不良的婴儿间质性肺疾病。NEHI的病因不明,临床上以呼吸急促、三凹征、爆裂音和低氧血症为特点。病理诊断主要靠蛙皮素的免疫组化,远端气道神经...婴儿神经内分泌细胞增生症(neuroendocrine cell hyperplasia of infancy,NEHI)是一种成熟不良的婴儿间质性肺疾病。NEHI的病因不明,临床上以呼吸急促、三凹征、爆裂音和低氧血症为特点。病理诊断主要靠蛙皮素的免疫组化,远端气道神经内分泌细胞(neuroendocrine cells,NECs)数目增加,NECs存在于至少75%的气道;在每一气道中NECs占10%;缺乏其他气道和间质疾病的特征,肺高分辨肺CT的特征为中心型的磨玻璃影,主要累及右中叶、左舌叶,以及其他区域的气体滞留。NEHI无特异的治疗方法,主要是氧疗和基本的营养支持治疗。NEHI是一种良性疾病,无死亡报道,病情随年龄增长可缓解。展开更多
该研究报道了12例形态学表现为类癌的肺神经内分泌肿瘤,但核分裂数>10/2 mm 2肺的神经内分泌癌。12例中男性7例和女性5例,年龄56~78岁。4例患者无吸烟史。所有肿瘤呈现出类癌的组织结构和细胞学特征,包括器官样、巢状、岛状、小梁状...该研究报道了12例形态学表现为类癌的肺神经内分泌肿瘤,但核分裂数>10/2 mm 2肺的神经内分泌癌。12例中男性7例和女性5例,年龄56~78岁。4例患者无吸烟史。所有肿瘤呈现出类癌的组织结构和细胞学特征,包括器官样、巢状、岛状、小梁状或腺泡状生长,肿瘤细胞核/质比低,具有丰富胞质,圆形、类圆形的核,胡椒盐样染色质。也可见成角或融合呈巢、岛状或小叶样的生长。局灶可见核不规则,核大小不一。核分裂数11~61/2 mm 2。8例可见点状坏死。所有肿瘤均未见细胞间变、大范围的梗死型坏死、促纤维增生、显著的炎细胞浸润。1例背景中可见弥漫性特发肺神经内分泌细胞增生。所有肿瘤均行切除术,11例随后发生转移,7例死于肿瘤。展开更多
We presented an unusual case with coexistence of carcinoid, signet-ring cell carcinoma （SRC） and heterotopic pancreatic tissue in stomach. Gastroscopic examination of this 63-year-old male patient showed multiple pr...We presented an unusual case with coexistence of carcinoid, signet-ring cell carcinoma （SRC） and heterotopic pancreatic tissue in stomach. Gastroscopic examination of this 63-year-old male patient showed multiple protrusions in gastric corpus near the greater curvature, identified by subsequent biopsy as carcinoid, Distal subtotal gastrectomy was performed, Histological and immunohistochemical examinations showed a carcinoid tumor in gastric corpus near the greater curvature, an intramucosal SRC at the lesser curvature of corpus and heterotopic pancreatic tissue in muscularis propria of the antrum at the lesser curvature with hyperplasia of peripheral endocrine cells producing multiple pancreatic hormones, We reviewed the literatures on clinicopathological characteristics and the differential diagnosis of the above three abnormalities, and concluded that the carcinoid in corpus near the greater curvature and SRC in the lesser curvature are independent lesions; the loci of endocrine cells in the muscularis propria and serosa are hyperplastic lesions from the heterotopic pancreatic tissue, rather than dissemination of carcinoid in corpus.展开更多
Pathologic hyperplasia of various pancreatic endocrine cells is rare but has been long known.β cell hyperplasia contributes to persistent hyperinsulinemic hypoglycemia of infancy,which is commonly caused by mutations...Pathologic hyperplasia of various pancreatic endocrine cells is rare but has been long known.β cell hyperplasia contributes to persistent hyperinsulinemic hypoglycemia of infancy,which is commonly caused by mutations in the islet ATP-sensitive potassium channel,and to noninsulinoma pancreatogenous hypoglycemia in adults,which may or may not be associated with bariatric surgery.α cell hyperplasia may cause glucagonoma syndrome or induce pancreatic neuroendocrine tumors.An inactivating mutation of the glucagon receptor causes α cell hyperplasia and asymptomatic hyperglucagonemia.Pancreatic polypeptide cell hyperplasia has been described without a clearly-characterized clinical syndrome and hyperplasia of other endocrine cells inside the pancreas has not been reported to our knowledge. Based on morphological evidence,the main pathogenetic mechanism for pancreatic endocrine cell hyperplasia is increased endocrine cell neogenesis from exocrine ductal epithelium.Pancreatic endocrine cell hyperplasia should be considered in the diagnosis and management of hypoglycemia,elevated islet hormone levels,and pancreatic neuroendocrine tumors.Further studies of pathologic pancreatic endocrine cell hyperplasia will likely yield insights into the pathogenesis and treatment of diabetes and pancreatic neuroendocrine tumors.展开更多
文摘该研究报道了12例形态学表现为类癌的肺神经内分泌肿瘤,但核分裂数>10/2 mm 2肺的神经内分泌癌。12例中男性7例和女性5例,年龄56~78岁。4例患者无吸烟史。所有肿瘤呈现出类癌的组织结构和细胞学特征,包括器官样、巢状、岛状、小梁状或腺泡状生长,肿瘤细胞核/质比低,具有丰富胞质,圆形、类圆形的核,胡椒盐样染色质。也可见成角或融合呈巢、岛状或小叶样的生长。局灶可见核不规则,核大小不一。核分裂数11~61/2 mm 2。8例可见点状坏死。所有肿瘤均未见细胞间变、大范围的梗死型坏死、促纤维增生、显著的炎细胞浸润。1例背景中可见弥漫性特发肺神经内分泌细胞增生。所有肿瘤均行切除术,11例随后发生转移,7例死于肿瘤。
文摘We presented an unusual case with coexistence of carcinoid, signet-ring cell carcinoma （SRC） and heterotopic pancreatic tissue in stomach. Gastroscopic examination of this 63-year-old male patient showed multiple protrusions in gastric corpus near the greater curvature, identified by subsequent biopsy as carcinoid, Distal subtotal gastrectomy was performed, Histological and immunohistochemical examinations showed a carcinoid tumor in gastric corpus near the greater curvature, an intramucosal SRC at the lesser curvature of corpus and heterotopic pancreatic tissue in muscularis propria of the antrum at the lesser curvature with hyperplasia of peripheral endocrine cells producing multiple pancreatic hormones, We reviewed the literatures on clinicopathological characteristics and the differential diagnosis of the above three abnormalities, and concluded that the carcinoid in corpus near the greater curvature and SRC in the lesser curvature are independent lesions; the loci of endocrine cells in the muscularis propria and serosa are hyperplastic lesions from the heterotopic pancreatic tissue, rather than dissemination of carcinoid in corpus.
文摘Pathologic hyperplasia of various pancreatic endocrine cells is rare but has been long known.β cell hyperplasia contributes to persistent hyperinsulinemic hypoglycemia of infancy,which is commonly caused by mutations in the islet ATP-sensitive potassium channel,and to noninsulinoma pancreatogenous hypoglycemia in adults,which may or may not be associated with bariatric surgery.α cell hyperplasia may cause glucagonoma syndrome or induce pancreatic neuroendocrine tumors.An inactivating mutation of the glucagon receptor causes α cell hyperplasia and asymptomatic hyperglucagonemia.Pancreatic polypeptide cell hyperplasia has been described without a clearly-characterized clinical syndrome and hyperplasia of other endocrine cells inside the pancreas has not been reported to our knowledge. Based on morphological evidence,the main pathogenetic mechanism for pancreatic endocrine cell hyperplasia is increased endocrine cell neogenesis from exocrine ductal epithelium.Pancreatic endocrine cell hyperplasia should be considered in the diagnosis and management of hypoglycemia,elevated islet hormone levels,and pancreatic neuroendocrine tumors.Further studies of pathologic pancreatic endocrine cell hyperplasia will likely yield insights into the pathogenesis and treatment of diabetes and pancreatic neuroendocrine tumors.
