Malignant neoplasms of the appendix are rare and represent less than 1% of gastrointestinal cancers.Goblet cell carcinoids(GCC) tumors are a distinctive group of heterogeneous appendiceal neoplasm that exhibit unique ...Malignant neoplasms of the appendix are rare and represent less than 1% of gastrointestinal cancers.Goblet cell carcinoids(GCC) tumors are a distinctive group of heterogeneous appendiceal neoplasm that exhibit unique clinical and pathologic features.This review focuses on the current diagnostic procedures,pathogenesis,possible signaling mechanisms and treatment options for GCC.Perspectives for future research are discussed.The tumor likely arises from pluripotent intestinal epithelial crypt base stem cells.Previous findings of Notch signaling as a tumor suppressor in Neuroendocrine tumors may have a similar role in this tumor too.Loss of Notch signaling may be the driver mutation with other successive downstream mutations likely favors them into progressing and behavior similar to poorly differentiated adenocarcinoma with minimal neuroendocrine differentiation.A multidisciplinary approach is suggested for optimal outcomes.Surgery remains the main treatment modality.Simple appendectomy may be sufficient in early stages while right hemicolectomy is recommended for advanced tumors.Cytoreductive surgery with heated intraperitoneal chemotherapy may improve survival in a select few with metastatic peritoneal disease.These tumors have an unpredictable behavior even in early stages and local recurrence and delayed metastases may be seen.Lifelong surveillance is warranted.展开更多
Neuroendocrine neoplasms are the most common epithelial tumors among appendix tumors. Appendix tumors that are completely or partially composed of neuroendocrine cells are divided into two categories: Classic carcinoi...Neuroendocrine neoplasms are the most common epithelial tumors among appendix tumors. Appendix tumors that are completely or partially composed of neuroendocrine cells are divided into two categories: Classic carcinoid tumors and goblet cell carcinoid tumors(GCCT). They are known to progress more aggressively than classic(neuro) endocrine tumors. In this study, three cases with acute appendicitis symptoms are presented, including their clinical and histopathological findings. Microscopic examination detected GCCT in two cases and mixed adenoneuroendocrine carcinoma in one case, in addition to acute appendicitis.展开更多
文摘Malignant neoplasms of the appendix are rare and represent less than 1% of gastrointestinal cancers.Goblet cell carcinoids(GCC) tumors are a distinctive group of heterogeneous appendiceal neoplasm that exhibit unique clinical and pathologic features.This review focuses on the current diagnostic procedures,pathogenesis,possible signaling mechanisms and treatment options for GCC.Perspectives for future research are discussed.The tumor likely arises from pluripotent intestinal epithelial crypt base stem cells.Previous findings of Notch signaling as a tumor suppressor in Neuroendocrine tumors may have a similar role in this tumor too.Loss of Notch signaling may be the driver mutation with other successive downstream mutations likely favors them into progressing and behavior similar to poorly differentiated adenocarcinoma with minimal neuroendocrine differentiation.A multidisciplinary approach is suggested for optimal outcomes.Surgery remains the main treatment modality.Simple appendectomy may be sufficient in early stages while right hemicolectomy is recommended for advanced tumors.Cytoreductive surgery with heated intraperitoneal chemotherapy may improve survival in a select few with metastatic peritoneal disease.These tumors have an unpredictable behavior even in early stages and local recurrence and delayed metastases may be seen.Lifelong surveillance is warranted.
文摘Neuroendocrine neoplasms are the most common epithelial tumors among appendix tumors. Appendix tumors that are completely or partially composed of neuroendocrine cells are divided into two categories: Classic carcinoid tumors and goblet cell carcinoid tumors(GCCT). They are known to progress more aggressively than classic(neuro) endocrine tumors. In this study, three cases with acute appendicitis symptoms are presented, including their clinical and histopathological findings. Microscopic examination detected GCCT in two cases and mixed adenoneuroendocrine carcinoma in one case, in addition to acute appendicitis.
