Neurenteric cysts are extremely rare congenital anomalies, often presenting in the first 5 years of life, and are caused by an incomplete separation of the notochord from the foregut during the third week of embryogen...Neurenteric cysts are extremely rare congenital anomalies, often presenting in the first 5 years of life, and are caused by an incomplete separation of the notochord from the foregut during the third week of embryogenesis. They are frequently accompanied with spinal or gastrointestinal abnormalities, but the latter may be absent in adults. Although usually located in the thorax, neurenteric cysts may be found along the entire spine. We present a 24-year-old woman admitted for epigastric pain, nausea, vomiting, low grade fever and leucocytosis. She underwent cystgastrostomy for a Ioculated cyst of the distal pancreas at the age of 4 years, which recurred when she was at the age of 11 years. Ultrasound and computer tomograghy （CT） scan revealed a 16 cmx 15 cm cystic mass in the body and tail of pancreas, with a 6-7 mm thickened wall. Laboratory data and chest X-ray were normal and spinal radiographs did not show any structural abnormalities. The patient underwent a complete cyst excision, and after an uneventful recovery, remained symptom-free without recurrence during the 5-year follow-up. The cyst was found to contain 1200 mL of pale viscous fluid. It was covered by a primitive singlelayered cuboidal epithelium, along with specialized antral glandular parenchyma and hypoplastic primitive gastric mucosa. Focal glandular groups resembling those of the body of the stomach were also seen. In addition, ciliary respiratory epithelium, foci of squamous metaplasia and mucinous glands were present. The wall of the cyst contained a muscular layer, neuroglial tissue with plexogenic nerve fascicles, Paccini corpuscle-like structures, hyperplastic neuroganglionar elements and occasional psammomatous bodies, as well as fibroblast-like areas of surrounding stroma. Cartilagenous tissue was not found in any part of the cyst. Immunohistochemistry confirmed the presence of neurogenic elements marked by S-100, GFAP, NF and NSE. The gastric epithelium showed mostly CK7 and EMA immunoexpression, and the respiratory epithelium revealed a CK8 and CK18 immunoprofile without CK 10/13 positive elements, though neither CEA or AFP positive cells were found. To our knowledge, this is the first reported case of an abdominally located neurenteric cyst with no associated spinal anomalies.展开更多
Somatostatinomas are extremely rare neuroendocrine tumors of the gastrointestinal tract,f irst described in the pancreas in 1977 and in the duodenum in 1979.They may be functional and cause somatostatinoma or inhibi-t...Somatostatinomas are extremely rare neuroendocrine tumors of the gastrointestinal tract,f irst described in the pancreas in 1977 and in the duodenum in 1979.They may be functional and cause somatostatinoma or inhibi-tory syndrome,but more frequently are non-functioning pancreatic endocrine tumors that produce somatostatin alone.They are usually single,malignant,large lesions,frequently associated with metastases,and generally with poor prognosis.We present the unique case of a 57-year-old woman with two synchronous non-function-ing somatostatinomas,one solid duodenal lesion and one cystic lesion within the head of the pancreas,that were successfully resected with a pylorus-preserving Whipple's procedure.No secondaries were found in the liver,or in any of the removed regional lymph nodes.The patient had an uneventful recovery,and remains well and symptom-free at 18 mo postoperatively.This is an extremely rare case of a patient with two synchro-nous somatostatinomas of the duodenum and the pancreas.The condition is discussed with reference to the literature.展开更多
In the past four decades,skull base surgery has undergone dramatic paradigm shifts.Evolution in understanding of physiology and anatomy coupled with advances in technology and training have completely revolutionized m...In the past four decades,skull base surgery has undergone dramatic paradigm shifts.Evolution in understanding of physiology and anatomy coupled with advances in technology and training have completely revolutionized management of patients with benign and malignant conditions of the anterior skull base.The“Skull Base Surgeon”requires broad and deep knowledge related to pathology,surgical approach and reconstruction with open and endoscopic techniques,perioperative management,and adjuvant and neoadjuvant indications and techniques.展开更多
文摘Neurenteric cysts are extremely rare congenital anomalies, often presenting in the first 5 years of life, and are caused by an incomplete separation of the notochord from the foregut during the third week of embryogenesis. They are frequently accompanied with spinal or gastrointestinal abnormalities, but the latter may be absent in adults. Although usually located in the thorax, neurenteric cysts may be found along the entire spine. We present a 24-year-old woman admitted for epigastric pain, nausea, vomiting, low grade fever and leucocytosis. She underwent cystgastrostomy for a Ioculated cyst of the distal pancreas at the age of 4 years, which recurred when she was at the age of 11 years. Ultrasound and computer tomograghy （CT） scan revealed a 16 cmx 15 cm cystic mass in the body and tail of pancreas, with a 6-7 mm thickened wall. Laboratory data and chest X-ray were normal and spinal radiographs did not show any structural abnormalities. The patient underwent a complete cyst excision, and after an uneventful recovery, remained symptom-free without recurrence during the 5-year follow-up. The cyst was found to contain 1200 mL of pale viscous fluid. It was covered by a primitive singlelayered cuboidal epithelium, along with specialized antral glandular parenchyma and hypoplastic primitive gastric mucosa. Focal glandular groups resembling those of the body of the stomach were also seen. In addition, ciliary respiratory epithelium, foci of squamous metaplasia and mucinous glands were present. The wall of the cyst contained a muscular layer, neuroglial tissue with plexogenic nerve fascicles, Paccini corpuscle-like structures, hyperplastic neuroganglionar elements and occasional psammomatous bodies, as well as fibroblast-like areas of surrounding stroma. Cartilagenous tissue was not found in any part of the cyst. Immunohistochemistry confirmed the presence of neurogenic elements marked by S-100, GFAP, NF and NSE. The gastric epithelium showed mostly CK7 and EMA immunoexpression, and the respiratory epithelium revealed a CK8 and CK18 immunoprofile without CK 10/13 positive elements, though neither CEA or AFP positive cells were found. To our knowledge, this is the first reported case of an abdominally located neurenteric cyst with no associated spinal anomalies.
文摘Somatostatinomas are extremely rare neuroendocrine tumors of the gastrointestinal tract,f irst described in the pancreas in 1977 and in the duodenum in 1979.They may be functional and cause somatostatinoma or inhibi-tory syndrome,but more frequently are non-functioning pancreatic endocrine tumors that produce somatostatin alone.They are usually single,malignant,large lesions,frequently associated with metastases,and generally with poor prognosis.We present the unique case of a 57-year-old woman with two synchronous non-function-ing somatostatinomas,one solid duodenal lesion and one cystic lesion within the head of the pancreas,that were successfully resected with a pylorus-preserving Whipple's procedure.No secondaries were found in the liver,or in any of the removed regional lymph nodes.The patient had an uneventful recovery,and remains well and symptom-free at 18 mo postoperatively.This is an extremely rare case of a patient with two synchro-nous somatostatinomas of the duodenum and the pancreas.The condition is discussed with reference to the literature.
文摘In the past four decades,skull base surgery has undergone dramatic paradigm shifts.Evolution in understanding of physiology and anatomy coupled with advances in technology and training have completely revolutionized management of patients with benign and malignant conditions of the anterior skull base.The“Skull Base Surgeon”requires broad and deep knowledge related to pathology,surgical approach and reconstruction with open and endoscopic techniques,perioperative management,and adjuvant and neoadjuvant indications and techniques.
