BACKGROUND Low-grade appendiceal neoplasms(LAMN)are characterized by low incidence and atypical clinical presentations,often leading to misdiagnosis as acute or chronic appendicitis before surgery.The primary diagnost...BACKGROUND Low-grade appendiceal neoplasms(LAMN)are characterized by low incidence and atypical clinical presentations,often leading to misdiagnosis as acute or chronic appendicitis before surgery.The primary diagnostic tool for LAMN is abdominal computed tomography(CT)imaging.Surgical resection remains the cornerstone of LAMN management,necessitating en bloc tumor excision to minimize the risk of iatrogenic rupture.Laparoscopy,known for its minimal invasiveness,reduced postoperative discomfort,and expedited recovery,is a safe and reliable approach for LAMN treatment.Despite the possibility of pseudomyxoma peritonei development,appendectomy and partial appendectomy generally result in negative tumor margins and favorable outcomes,which can be attributed to the disease’s slow growth and lower malignancy.CASE SUMMARY A 71-year-old male patient was admitted to our hospital with a pelvic spaceoccupying lesion detected 1 mo prior.Physical examination showed a soft abdomen without tenderness or rebound and no palpable masses.No shifting dullness was noted,and digital rectal examination revealed no palpable mass.Enteroscopy revealed a raised,smooth-surfaced mass measuring 3.0 cm in the cecum.Abdominal contrast-enhanced CT showed a markedly thickened and dilated appendix with visible cystic shadows.Laparoscopic surgery was performed and revealed a significantly dilated appendix,leading to laparoscopic resection of the appendix and part of the cecum.Post-surgical pathologic analysis confirmed LAMN.The patient received symptomatic and supportive post-operative care and was discharged on postoperative day 4 without complications such as abdominal bleeding,intestinal obstruction,or incision infection.No tumor recurrence was observed during a 7-mo follow-up period.CONCLUSION LAMN is a rare disease that lacks specific clinical manifestations.Abdominal CT plays a crucial role in diagnosing LAMN,and laparoscopic surgery is a safe and effective diagnostic and therapeutic approach.展开更多
We present a rare case of synchronous mucinous borderline tumor of the ovary and low-grade appendiceal mucinous neoplasm (LAMN). We performed a left adnexectomy to diagnose the left ovarian borderline malignancy and a...We present a rare case of synchronous mucinous borderline tumor of the ovary and low-grade appendiceal mucinous neoplasm (LAMN). We performed a left adnexectomy to diagnose the left ovarian borderline malignancy and an ileostomy because of the swollen appendix during the operation. It was diagnosed as left ovarian mucinous borderline malignancy and LAMN. LAMN causes peritoneal dissemination, ovarian metastasis, and peritoneal pseudomyxoma. The appendix and ovary are close to each other anatomically and can metastasize if there is a tumor in either. For ovarian mucinous tumors, it is necessary to search the gastrointestinal tract, especially the appendix, as the primary lesion. For appendix tumors, it is necessary to search for the ovary. Since LAMN may be associated with borderline ovarian malignancies, as in this case, there is a possibility of the duplication of tumors when searching for ovarian mucinous tumors as the primary tumor and if ovarian tumors are found. Since LAMN and mucinous ovarian tumors have similar histological features, immunohistochemical staining is useful for their differentiation because they show different immunostaining patterns.展开更多
We here present a rare case of appendiceal tumor mimicking ovarian tumor in menopause woman. The patient was a 56-year-old woman, G1P1, who presented to our hospital with a right adnexal cyst diagnosed at another hosp...We here present a rare case of appendiceal tumor mimicking ovarian tumor in menopause woman. The patient was a 56-year-old woman, G1P1, who presented to our hospital with a right adnexal cyst diagnosed at another hospital. Transvaginal echocardiography showed a cyst in the right adnexal region, and pelvic contrast-enhanced MRI revealed a small cyst in the same region. The left ovary was atrophic and identifiable. It was unclear whether the cyst was contiguous with the gastrointestinal tract. Blood tests showed no elevation of tumor markers. We considered its possibility of a gastrointestinal origin, but since right normal ovary was not found, we thought the tumor was of ovarian origin and decided on a laparoscopic resection of the right adnexa. Intraoperatively, we observed atrophied bilateral normal ovaries, and the pelvic tumor was contiguous to the appendix. Surgeons performed a laparoscopic appendectomy after consultation with us. After resection we searched the abdominal and pelvic cavities, but found no obvious disseminated lesions. The histological diagnosis was low-grade appendiceal mucinous neoplasm (LAMN), a rare benign tumor of the appendix. Appendiceal tumors can be difficult to differentiate from right ovarian tumors due to their close anatomic location in the pelvis. It is possible to determine whether the tumor is of ovarian or appendiceal origin by identifying normal ovaries and the location of the feeding vessels into the tumors. In our case, there were no lesions other than the appendix, but LAMN can metastasize to the ovary, cause pseudomyoxoma peritonei, or be an overlapping tumor with an ovarian tumor. If an appendiceal tumor is diagnosed after surgery for ovarian tumor, the intra-abdominal cavity should be searched for metastasis or dissemination, and a thorough search for ovarian lesions should be performed with the possibility of an overlapping tumor in mind.展开更多
BACKGROUND Despite the rarity of appendiceal tumors,research in this field has intensified,resulting in a growing number of studies and published papers.Surprisingly,no comprehensive bibliometric analysis has specific...BACKGROUND Despite the rarity of appendiceal tumors,research in this field has intensified,resulting in a growing number of studies and published papers.Surprisingly,no comprehensive bibliometric analysis has specifically addressed appendiceal tumors.AIM To offer a thorough analysis of the current landscape and future trends in appendiceal tumor research.METHODS In our bibliometric analysis studies,we explored the Web of Science Core Collection database.The bibliographic details of the chosen publications were automatically converted and analyzed using the bibliometric package in the R environment.Additionally,we employed VoSviewer to create cooperation network maps for countries,institutions,and authors,as well as clustering maps for keywords.Furthermore,CiteSpace,another software tool,was utilized to build dual-map overlays of journals and analyze references with citation bursts.RESULTS Our study included 780 English-language articles published after 2010.The number of related publications and citations has increased in the past decade.The United States leads in this area,but there is a need to improve cooperation and communication among countries and institutions.Co-occurrence analysis also revealed close collaboration among different authors.Annals of Surgical Oncology was the most influential journal in this field.Analysis of references with high cocitations and references with citation bursts,consistent with analysis of keywords and hotspots,indicated that current research primarily centers on the classification and management of appendiceal mucinous neoplasms and consequent pseudomyxoma peritonei.Despite the abundance of clinical studies,a greater number of in-depth basic research studies should be conducted.CONCLUSION Current research on appendiceal tumors focuses on classification and management of appendiceal mucinous neoplasms and pseudomyxoma peritonei.Enhanced collaboration and basic research are vital for further advancement.展开更多
BACKGROUND Appendiceal mucinous neoplasm (AMN) is extremely rare. Since the disease does not manifest a characteristic profile of clinical symptoms, it is easy to misdiagnose and still difficult to diagnose without op...BACKGROUND Appendiceal mucinous neoplasm (AMN) is extremely rare. Since the disease does not manifest a characteristic profile of clinical symptoms, it is easy to misdiagnose and still difficult to diagnose without operation. Here, we report a case of low-grade AMN (LAMN) and summarize its clinical features, diagnosis, and treatment. CASE SUMMARY A 63-year-old postmenopausal woman presented with a history of right lower abdominal mass. The patient underwent laparotomy, which showed an appendiceal mucocele originating from the apex of the appendix, and a simple appendectomy was performed. The subsequent histological assessment identified an LAMN with no lymph node involvement and negative surgical margin. The patient received six cycles of chemotherapy after surgery, and to date, more than a year after the surgery, the patient remains in good health. CONCLUSION A unified, standardized, detailed, and accurate pathological diagnosis is needed for LAMN, to facilitate selection of an appropriate surgical plan. In addition, the surgeon should record the details of the tumors in the surgical records in order to facilitate follow-up after surgery.展开更多
BACKGROUND Appendiceal mucinous neoplasms(AMNs),although not classified as rare,are relatively uncommon tumors most often discovered incidentally during colorectal surgery.Accurate identification of AMNs is difficult ...BACKGROUND Appendiceal mucinous neoplasms(AMNs),although not classified as rare,are relatively uncommon tumors most often discovered incidentally during colorectal surgery.Accurate identification of AMNs is difficult due to non-specific sym-ptoms,overlapping tumor markers with other conditions,and the potential for misdiagnosis.This underscores the urgent need for precision in diagnosis to pre-vent severe complications.CASE SUMMARY This case report describes the unexpected discovery and treatment of a low-grade AMN(LAMN)in a 74-year-old man undergoing laparoscopic hemicolectomy for transverse colon adenocarcinoma(AC).Preoperatively,non-specific gastroin-testinal symptoms and elevated tumor markers masked the presence of AMN.The tumor,presumed to be an AMN peritoneal cyst intraoperatively,was con-firmed as LAMN through histopathological examination.The neoplasm exhibited mucin accumulation and a distinct immunohistochemical profile:Positive for Ho-meobox protein CDX-2,Cytokeratin 20,special AT-rich sequence-binding protein 2,and Mucin 2 but negative for cytokeratin 7 and Paired box gene 8.This profile aids in distinguishing appendiceal and ovarian mucinous tumors.Postoperative recovery was uncomplicated,and the patient initiated adjuvant chemotherapy for the colon AC.CONCLUSION This case highlights the diagnostic complexity of AMNs,emphasizing the need for vigilant identification to avert potential complications,such as pseudomyxoma peritonei.展开更多
BACKGROUND Primary appendiceal tumors are histologically diverse and have an insidious onset and few specific clinical manifestations.In the majority of cases,these tumors are discovered after appendectomy during path...BACKGROUND Primary appendiceal tumors are histologically diverse and have an insidious onset and few specific clinical manifestations.In the majority of cases,these tumors are discovered after appendectomy during pathological exam of the resected tissue.Treatment may include appendectomy(simple or radical)and right hemicolectomy depending on factors such as histological type,tumor size and lymph node/organ involvement.The aim of this case study is to describe a rare case of a giant appendicular mucocele and raise awareness of this condition and its management options and follow-up protocol.CASE SUMMARY We present the case of a 43-year-old patient who presented to our emergency department with mild right lower quadrant pain.After the initial check-up and imaging exams,he underwent surgery,where a giant 20 cm×13 cm appendicular tumor was found and resected.Appendicular mucocele was suspected due to the macroscopic appearance and was later confirmed by the pathological exam.The patient’s postoperative evolution was uneventful,and after discharge,he was included in our follow-up program.CONCLUSION In conclusion,mucinous appendiceal neoplasms embody a rare pathology;they are asymptomatic or have few,unspecific clinical signs and in many cases are discovered after appendectomy.展开更多
Objective: To report a case of pseudomyxoma peritonei accidentally discovered during preoperative examination and review relevant literature to improve the early diagnosis of the origin of pseudomyxoma peritonei. Meth...Objective: To report a case of pseudomyxoma peritonei accidentally discovered during preoperative examination and review relevant literature to improve the early diagnosis of the origin of pseudomyxoma peritonei. Methods: To analyze a case accidentally discovered due to abdominal distension in our hospital in October 2023. Combined with domestic and foreign literature, the etiology, clinical manifestations, diagnosis, differential diagnosis and treatment of PMP were discussed. Results: The patient was admitted due to abdominal distension with no other specific discomfort. Mucinous tumors were found during imaging examinations. Conclusion: PMP is a rare disease, and clinical manifestations often include abdominal distension. It is usually first discovered through imaging examinations such as ultrasound, CT or MRI. CRS and HIPEC are still recommended as the standard treatment for PMP.展开更多
Goblet cell carcinoid is an enigmatic and rare tumor involving the appendix almost exclusively. Since its identification in 1969, understanding of this disease has evolved greatly, but issues regarding its histogenesi...Goblet cell carcinoid is an enigmatic and rare tumor involving the appendix almost exclusively. Since its identification in 1969, understanding of this disease has evolved greatly, but issues regarding its histogenesis, nomenclature and management are still conjectural. The published English language literature from 1966 to 2009 was retrieved via PubMed and reviewed. Various other names have been used for this entity such as adenocarcinoid, mucinous carcinoid, crypt cell carcinoma, and mucin-producing neuroendocrine tumor, although none have been found to be completely satisfactory or universally accepted. The tumor is thought to arise from pluripotent intestinal epithelial crypt-base stem cells by dual neuroendocrine and mucinous differentiation. GCCs present in the fifth to sixth decade and show no definite sex predominance. The most common clinical presentation is acute appendicitis, followed by abdominal pain and a mass. Fifty percent of the female patients present with ovarian metastases. The histologic hallmark of this entity is the presence of clusters of goblet cells in the lamina propria or submucosa stain for various neuroendocrine markers, though the intensity is often patchy. Atypia is usually minimal, but carcinomatous growth patterns may be seen. These may be of signet ring cell type or poorly differentiated adenocarcinoma. Recently molecular studies have shown these tumors to lack the signatures of adenocarcinoma but they have some changes similar to that of ileal carcinoids (allelic loss of chromosome 11q, 16q and 18q). The natural history of GCC is intermediate between carcinoids and adenocarcinomas of the appendix. The 5-year overall survival is 76%. The most important prognostic factor is the stage of disease. Appendectomy and right hemicolectomy are the main modalities of treatment, followed by adjuvant chemotherapy in select cases. There is some debate about the surgical approach for these tumors, and a summary of published series and recommendations are provided.展开更多
基金Scientific Research Fund of National Health Commission of China,Key Health Science and Technology Program of Zhejiang Province,No.WKJ-ZJ-2201Key Project of Social Welfare Program of Zhejiang Science and Technology Department,“Lingyan”Program,No.2022C03099and Clinical Research Fund Project of Zhejiang Medical Association,No.2021ZYC-A173.
文摘BACKGROUND Low-grade appendiceal neoplasms(LAMN)are characterized by low incidence and atypical clinical presentations,often leading to misdiagnosis as acute or chronic appendicitis before surgery.The primary diagnostic tool for LAMN is abdominal computed tomography(CT)imaging.Surgical resection remains the cornerstone of LAMN management,necessitating en bloc tumor excision to minimize the risk of iatrogenic rupture.Laparoscopy,known for its minimal invasiveness,reduced postoperative discomfort,and expedited recovery,is a safe and reliable approach for LAMN treatment.Despite the possibility of pseudomyxoma peritonei development,appendectomy and partial appendectomy generally result in negative tumor margins and favorable outcomes,which can be attributed to the disease’s slow growth and lower malignancy.CASE SUMMARY A 71-year-old male patient was admitted to our hospital with a pelvic spaceoccupying lesion detected 1 mo prior.Physical examination showed a soft abdomen without tenderness or rebound and no palpable masses.No shifting dullness was noted,and digital rectal examination revealed no palpable mass.Enteroscopy revealed a raised,smooth-surfaced mass measuring 3.0 cm in the cecum.Abdominal contrast-enhanced CT showed a markedly thickened and dilated appendix with visible cystic shadows.Laparoscopic surgery was performed and revealed a significantly dilated appendix,leading to laparoscopic resection of the appendix and part of the cecum.Post-surgical pathologic analysis confirmed LAMN.The patient received symptomatic and supportive post-operative care and was discharged on postoperative day 4 without complications such as abdominal bleeding,intestinal obstruction,or incision infection.No tumor recurrence was observed during a 7-mo follow-up period.CONCLUSION LAMN is a rare disease that lacks specific clinical manifestations.Abdominal CT plays a crucial role in diagnosing LAMN,and laparoscopic surgery is a safe and effective diagnostic and therapeutic approach.
文摘We present a rare case of synchronous mucinous borderline tumor of the ovary and low-grade appendiceal mucinous neoplasm (LAMN). We performed a left adnexectomy to diagnose the left ovarian borderline malignancy and an ileostomy because of the swollen appendix during the operation. It was diagnosed as left ovarian mucinous borderline malignancy and LAMN. LAMN causes peritoneal dissemination, ovarian metastasis, and peritoneal pseudomyxoma. The appendix and ovary are close to each other anatomically and can metastasize if there is a tumor in either. For ovarian mucinous tumors, it is necessary to search the gastrointestinal tract, especially the appendix, as the primary lesion. For appendix tumors, it is necessary to search for the ovary. Since LAMN may be associated with borderline ovarian malignancies, as in this case, there is a possibility of the duplication of tumors when searching for ovarian mucinous tumors as the primary tumor and if ovarian tumors are found. Since LAMN and mucinous ovarian tumors have similar histological features, immunohistochemical staining is useful for their differentiation because they show different immunostaining patterns.
文摘We here present a rare case of appendiceal tumor mimicking ovarian tumor in menopause woman. The patient was a 56-year-old woman, G1P1, who presented to our hospital with a right adnexal cyst diagnosed at another hospital. Transvaginal echocardiography showed a cyst in the right adnexal region, and pelvic contrast-enhanced MRI revealed a small cyst in the same region. The left ovary was atrophic and identifiable. It was unclear whether the cyst was contiguous with the gastrointestinal tract. Blood tests showed no elevation of tumor markers. We considered its possibility of a gastrointestinal origin, but since right normal ovary was not found, we thought the tumor was of ovarian origin and decided on a laparoscopic resection of the right adnexa. Intraoperatively, we observed atrophied bilateral normal ovaries, and the pelvic tumor was contiguous to the appendix. Surgeons performed a laparoscopic appendectomy after consultation with us. After resection we searched the abdominal and pelvic cavities, but found no obvious disseminated lesions. The histological diagnosis was low-grade appendiceal mucinous neoplasm (LAMN), a rare benign tumor of the appendix. Appendiceal tumors can be difficult to differentiate from right ovarian tumors due to their close anatomic location in the pelvis. It is possible to determine whether the tumor is of ovarian or appendiceal origin by identifying normal ovaries and the location of the feeding vessels into the tumors. In our case, there were no lesions other than the appendix, but LAMN can metastasize to the ovary, cause pseudomyoxoma peritonei, or be an overlapping tumor with an ovarian tumor. If an appendiceal tumor is diagnosed after surgery for ovarian tumor, the intra-abdominal cavity should be searched for metastasis or dissemination, and a thorough search for ovarian lesions should be performed with the possibility of an overlapping tumor in mind.
文摘BACKGROUND Despite the rarity of appendiceal tumors,research in this field has intensified,resulting in a growing number of studies and published papers.Surprisingly,no comprehensive bibliometric analysis has specifically addressed appendiceal tumors.AIM To offer a thorough analysis of the current landscape and future trends in appendiceal tumor research.METHODS In our bibliometric analysis studies,we explored the Web of Science Core Collection database.The bibliographic details of the chosen publications were automatically converted and analyzed using the bibliometric package in the R environment.Additionally,we employed VoSviewer to create cooperation network maps for countries,institutions,and authors,as well as clustering maps for keywords.Furthermore,CiteSpace,another software tool,was utilized to build dual-map overlays of journals and analyze references with citation bursts.RESULTS Our study included 780 English-language articles published after 2010.The number of related publications and citations has increased in the past decade.The United States leads in this area,but there is a need to improve cooperation and communication among countries and institutions.Co-occurrence analysis also revealed close collaboration among different authors.Annals of Surgical Oncology was the most influential journal in this field.Analysis of references with high cocitations and references with citation bursts,consistent with analysis of keywords and hotspots,indicated that current research primarily centers on the classification and management of appendiceal mucinous neoplasms and consequent pseudomyxoma peritonei.Despite the abundance of clinical studies,a greater number of in-depth basic research studies should be conducted.CONCLUSION Current research on appendiceal tumors focuses on classification and management of appendiceal mucinous neoplasms and pseudomyxoma peritonei.Enhanced collaboration and basic research are vital for further advancement.
基金Supported by Wu Jieping Medical Foundation of China,No.320.6750.18492the Innovative Talents Fund of Harbin Science and Technology Bureau of China,No.2015RAQYJ103
文摘BACKGROUND Appendiceal mucinous neoplasm (AMN) is extremely rare. Since the disease does not manifest a characteristic profile of clinical symptoms, it is easy to misdiagnose and still difficult to diagnose without operation. Here, we report a case of low-grade AMN (LAMN) and summarize its clinical features, diagnosis, and treatment. CASE SUMMARY A 63-year-old postmenopausal woman presented with a history of right lower abdominal mass. The patient underwent laparotomy, which showed an appendiceal mucocele originating from the apex of the appendix, and a simple appendectomy was performed. The subsequent histological assessment identified an LAMN with no lymph node involvement and negative surgical margin. The patient received six cycles of chemotherapy after surgery, and to date, more than a year after the surgery, the patient remains in good health. CONCLUSION A unified, standardized, detailed, and accurate pathological diagnosis is needed for LAMN, to facilitate selection of an appropriate surgical plan. In addition, the surgeon should record the details of the tumors in the surgical records in order to facilitate follow-up after surgery.
文摘BACKGROUND Appendiceal mucinous neoplasms(AMNs),although not classified as rare,are relatively uncommon tumors most often discovered incidentally during colorectal surgery.Accurate identification of AMNs is difficult due to non-specific sym-ptoms,overlapping tumor markers with other conditions,and the potential for misdiagnosis.This underscores the urgent need for precision in diagnosis to pre-vent severe complications.CASE SUMMARY This case report describes the unexpected discovery and treatment of a low-grade AMN(LAMN)in a 74-year-old man undergoing laparoscopic hemicolectomy for transverse colon adenocarcinoma(AC).Preoperatively,non-specific gastroin-testinal symptoms and elevated tumor markers masked the presence of AMN.The tumor,presumed to be an AMN peritoneal cyst intraoperatively,was con-firmed as LAMN through histopathological examination.The neoplasm exhibited mucin accumulation and a distinct immunohistochemical profile:Positive for Ho-meobox protein CDX-2,Cytokeratin 20,special AT-rich sequence-binding protein 2,and Mucin 2 but negative for cytokeratin 7 and Paired box gene 8.This profile aids in distinguishing appendiceal and ovarian mucinous tumors.Postoperative recovery was uncomplicated,and the patient initiated adjuvant chemotherapy for the colon AC.CONCLUSION This case highlights the diagnostic complexity of AMNs,emphasizing the need for vigilant identification to avert potential complications,such as pseudomyxoma peritonei.
文摘BACKGROUND Primary appendiceal tumors are histologically diverse and have an insidious onset and few specific clinical manifestations.In the majority of cases,these tumors are discovered after appendectomy during pathological exam of the resected tissue.Treatment may include appendectomy(simple or radical)and right hemicolectomy depending on factors such as histological type,tumor size and lymph node/organ involvement.The aim of this case study is to describe a rare case of a giant appendicular mucocele and raise awareness of this condition and its management options and follow-up protocol.CASE SUMMARY We present the case of a 43-year-old patient who presented to our emergency department with mild right lower quadrant pain.After the initial check-up and imaging exams,he underwent surgery,where a giant 20 cm×13 cm appendicular tumor was found and resected.Appendicular mucocele was suspected due to the macroscopic appearance and was later confirmed by the pathological exam.The patient’s postoperative evolution was uneventful,and after discharge,he was included in our follow-up program.CONCLUSION In conclusion,mucinous appendiceal neoplasms embody a rare pathology;they are asymptomatic or have few,unspecific clinical signs and in many cases are discovered after appendectomy.
文摘Objective: To report a case of pseudomyxoma peritonei accidentally discovered during preoperative examination and review relevant literature to improve the early diagnosis of the origin of pseudomyxoma peritonei. Methods: To analyze a case accidentally discovered due to abdominal distension in our hospital in October 2023. Combined with domestic and foreign literature, the etiology, clinical manifestations, diagnosis, differential diagnosis and treatment of PMP were discussed. Results: The patient was admitted due to abdominal distension with no other specific discomfort. Mucinous tumors were found during imaging examinations. Conclusion: PMP is a rare disease, and clinical manifestations often include abdominal distension. It is usually first discovered through imaging examinations such as ultrasound, CT or MRI. CRS and HIPEC are still recommended as the standard treatment for PMP.
文摘Goblet cell carcinoid is an enigmatic and rare tumor involving the appendix almost exclusively. Since its identification in 1969, understanding of this disease has evolved greatly, but issues regarding its histogenesis, nomenclature and management are still conjectural. The published English language literature from 1966 to 2009 was retrieved via PubMed and reviewed. Various other names have been used for this entity such as adenocarcinoid, mucinous carcinoid, crypt cell carcinoma, and mucin-producing neuroendocrine tumor, although none have been found to be completely satisfactory or universally accepted. The tumor is thought to arise from pluripotent intestinal epithelial crypt-base stem cells by dual neuroendocrine and mucinous differentiation. GCCs present in the fifth to sixth decade and show no definite sex predominance. The most common clinical presentation is acute appendicitis, followed by abdominal pain and a mass. Fifty percent of the female patients present with ovarian metastases. The histologic hallmark of this entity is the presence of clusters of goblet cells in the lamina propria or submucosa stain for various neuroendocrine markers, though the intensity is often patchy. Atypia is usually minimal, but carcinomatous growth patterns may be seen. These may be of signet ring cell type or poorly differentiated adenocarcinoma. Recently molecular studies have shown these tumors to lack the signatures of adenocarcinoma but they have some changes similar to that of ileal carcinoids (allelic loss of chromosome 11q, 16q and 18q). The natural history of GCC is intermediate between carcinoids and adenocarcinomas of the appendix. The 5-year overall survival is 76%. The most important prognostic factor is the stage of disease. Appendectomy and right hemicolectomy are the main modalities of treatment, followed by adjuvant chemotherapy in select cases. There is some debate about the surgical approach for these tumors, and a summary of published series and recommendations are provided.
