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Boström L, Jovic V, Dahlberg M, Holtenius F, Sandblom G, Järnbert-Pettersson H. Survival among 148 patients with an incidentally detected appendiceal tumours at surgery for acute appendicitis: a population-based cohort follow-up study. Eur J Trauma Emerg Surg 2024; 50:2113-2122. [PMID: 39020129 PMCID: PMC11599532 DOI: 10.1007/s00068-024-02580-1] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/11/2024] [Accepted: 06/11/2024] [Indexed: 07/19/2024]
Abstract
PURPOSE To investigate the long-term prognosis of appendiceal tumours incidentally detected at appendicectomy for suspicion of benign appendicitis. METHODS A retrospective register-based single centre cohort study was carried out, using data from the local acute appendicectomy quality register of cases operated on at the Department of Surgery, South General Hospital, Stockholm, Sweden. The local colorectal cancer register was also used to identify appendix tumours. The study period was between January 2004 and January 2023. Survival was calculated according to the Kaplan-Meier method. RESULTS A total of 11,888 patients were registered in the acute acute appendicectomy register, 54% males and 46% females, median age 32 (Q1 = 21, Q3 = 47) (with 33.7% were 41 years or older). From the appendicectomy and colorectal registers 148 (1.2% of the total cohort) appendiceal tumours were found; 60% in females and 40% in males, median age 56 (Q1 = 43, Q3 = 70) (with 78.4% being 41 years or older). Tumours found were: Low grade Appendiceal Mucinous Neoplasms (LAMN, N = 64); Neuroendocrine Tumours (NET N = 24); adenocarcinomas or other form of carcinomas (N = 57); and adenomas (N = 3). The overall 5-year survival in patients operated for LAMN was 96.8%, for NET 93.3% and for adenocarcinoma 69.7%. The overall 5-year survival for all tumour patients was 85.7%. For the younger patients (< 51 years) with LAMN and NET, almost all survived to the end of follow-up. Survival of patients in the carcinoma group was statistically significantly lower than for the LAMN and NET groups, especially in females 51 years or older. In the group of tumour patients undergoing surgery (n = 146), primary surgery was laparoscopic in 47% and open in 52%. Two patients did not undergo surgery due to widespread disease. In 64% of cases operation was acute, whereas it was delayed and/or planned in 34%. Most procedures were laparoscopic appendicectomy 36%, followed by open appendicectomy 30%, right-sided hemicolectomy 14.6% (open 11.6% and laparoscopic 3%, acute operation 5.5%), ileocaecal resection 5% (acute operation 3.4%), and staging laparoscopy 7%. In 38% of the operated patients the tumour was discovered incidentally at histopathology examination. Two patients had CRS and HIPEC as the initial operation. Forthy-three per cent of the 146 tumour patients operated underwent a second procedure: CRS and HIPEC in 23.3% and right-sided hemicolectomy in 13.6% (laparoscopic 8.2% open 5.4%). CONCLUSION Survival was high for patients with incidentally detected appendiceal LAMN or NET, but not so for carcinoma. Survival was lower in the carcinoma group older than 50 years, especially those sick and females.
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Affiliation(s)
- Lennart Boström
- Department of Surgery, South General Hospital (Södersjukhuset), Sjukhusbacken 10, Stockholm, SE-11883, Sweden.
- Department of Clinical Science and Education, Karolinska Institutet, South General Hospital, Stockholm, Sweden.
| | - Viktor Jovic
- Department of Pathology, Karolinska University Hospital, Karolinska Institutet, Stockholm, Sweden
| | - Martin Dahlberg
- Department of Surgery, South General Hospital (Södersjukhuset), Sjukhusbacken 10, Stockholm, SE-11883, Sweden
- Department of Clinical Science and Education, Karolinska Institutet, South General Hospital, Stockholm, Sweden
| | - Fredrik Holtenius
- Department of Surgery, South General Hospital (Södersjukhuset), Sjukhusbacken 10, Stockholm, SE-11883, Sweden
| | - Gabriel Sandblom
- Department of Surgery, South General Hospital (Södersjukhuset), Sjukhusbacken 10, Stockholm, SE-11883, Sweden
- Department of Clinical Science and Education, Karolinska Institutet, South General Hospital, Stockholm, Sweden
| | - Hans Järnbert-Pettersson
- Department of Clinical Science and Education, Karolinska Institutet, South General Hospital, Stockholm, Sweden
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Salama MM, O’Neill MA, Ryan ÉJ, O’Sullivan NJ, Nugent TS, Temperley HC, Mehigan BJ, Larkin JO, Gallagher D, O’Kane G, McCormick PH, Kelly ME. The Optimal Approach to Surgical Management of Goblet Cell Carcinoid of the Appendix: A Systematic Review. Diagnostics (Basel) 2024; 14:1773. [PMID: 39202258 PMCID: PMC11353492 DOI: 10.3390/diagnostics14161773] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/17/2024] [Revised: 08/05/2024] [Accepted: 08/06/2024] [Indexed: 09/03/2024] Open
Abstract
INTRODUCTION Goblet cell carcinoid (GCC) is a rare and poorly understood appendiceal neoplasm, exhibiting mixed histological and aggressive clinical features. Current guidelines recommend right hemicolectomy in all cases, although there is conflicting evidence that appendicectomy alone may be sufficient. This review aims to identify the optimal surgical management for appendiceal GCC. METHODS A systematic review was performed by searching MEDLINE, Embase, Scopus and the Cochrane Register of Controlled Trials. Randomised controlled trials, cohort studies or large case series (>5 patients) reporting clinical outcomes for patients undergoing surgical management of GCC of the appendix were included. Outcomes extracted included participant and tumour characteristics, type of surgery and survival data. RESULTS A total of 1341 studies were retrieved. After duplicate removal, 796 titles were screened for relevance prior to abstract and full text review. A total of six studies were included for analysis, comprising 3177 patients-1629 females and 1548 males. The median age ranged from 51 to 72 years. A total of 2329 patients underwent right hemicolectomy, while 824 were treated with appendicectomy only. Overall, the included studies report increased survival in patients undergoing right hemicolectomy compared to appendicectomy alone. A meta-analysis was not possible due to insufficient data reported in the published literature to date. CONCLUSIONS There is no consensus regarding the optimal surgical management of appendiceal GCC, as outcomes-based data comparing surgical interventions are lacking. It is possible that some patients with favourable features are overtreated. The absence of robust evidence to support a more conservative approach means that right hemicolectomy remains the standard of care for all patients, in keeping with current international guidelines. The rarity of this condition and limited data in the published studies remain barriers to evidence-based best clinical practice.
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Affiliation(s)
- Mahmoud M. Salama
- Department of Surgery, St. James’s Hospital, D08 NHY1 Dublin, Ireland; (M.M.S.)
- School of Medicine, University of Galway, H91T K33 Galway, Ireland
| | - Maeve A. O’Neill
- Department of Surgery, St. James’s Hospital, D08 NHY1 Dublin, Ireland; (M.M.S.)
| | - Éanna J. Ryan
- Department of Surgery, St. James’s Hospital, D08 NHY1 Dublin, Ireland; (M.M.S.)
| | - Niall J. O’Sullivan
- Department of Surgery, St. James’s Hospital, D08 NHY1 Dublin, Ireland; (M.M.S.)
- School of Medicine, Trinity College Dublin, D02 PN40 Dublin, Ireland
| | - Timothy S. Nugent
- Department of Surgery, St. James’s Hospital, D08 NHY1 Dublin, Ireland; (M.M.S.)
| | - Hugo C. Temperley
- Department of Surgery, St. James’s Hospital, D08 NHY1 Dublin, Ireland; (M.M.S.)
- School of Medicine, Trinity College Dublin, D02 PN40 Dublin, Ireland
| | - Brian J. Mehigan
- Department of Surgery, St. James’s Hospital, D08 NHY1 Dublin, Ireland; (M.M.S.)
- School of Medicine, University of Galway, H91T K33 Galway, Ireland
| | - John O. Larkin
- Department of Surgery, St. James’s Hospital, D08 NHY1 Dublin, Ireland; (M.M.S.)
| | - David Gallagher
- Trinity St. James’s Cancer Institute, D08 NHY1 Dublin, Ireland
| | - Grainne O’Kane
- Trinity St. James’s Cancer Institute, D08 NHY1 Dublin, Ireland
| | - Paul H. McCormick
- Department of Surgery, St. James’s Hospital, D08 NHY1 Dublin, Ireland; (M.M.S.)
- School of Medicine, Trinity College Dublin, D02 PN40 Dublin, Ireland
| | - Michael E. Kelly
- Department of Surgery, St. James’s Hospital, D08 NHY1 Dublin, Ireland; (M.M.S.)
- School of Medicine, Trinity College Dublin, D02 PN40 Dublin, Ireland
- Trinity St. James’s Cancer Institute, D08 NHY1 Dublin, Ireland
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Toshima T, Inada R, Sakamoto S, Takeda E, Yoshioka T, Kumon K, Mimura N, Takata N, Tabuchi M, Oishi K, Sato T, Sui K, Okabayashi T, Ozaki K, Nakamura T, Shibuya Y, Matsumoto M, Iwata J. Goblet cell carcinoid of the appendix: Six case reports. World J Clin Cases 2024; 12:5217-5224. [DOI: 10.12998/wjcc.v12.i22.5217] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/11/2024] [Revised: 05/21/2024] [Accepted: 05/30/2024] [Indexed: 06/30/2024] Open
Abstract
BACKGROUND Goblet cell carcinoid (GCC) of the appendix is a rare tumor characterized by neuroendocrine and adenocarcinoma features. Accurate preoperative diagnosis is very difficult, with most patients complaining mainly of abdominal pain. Computed tomography shows swelling of the appendix, so diagnosis is usually made incidentally after appendectomy based on a preoperative diagnosis of appendicitis. Even if a patient undergoes preoperative colonoscopy, accurate endoscopic diagnosis is very difficult because GCC shows a submucosal growth pattern with invasion of the appendiceal wall.
CASE SUMMARY Between 2017 and 2022, 6 patients with GCC were treated in our hospital. The presenting complaint for 5 of these 6 patients was abdominal pain. All 5 patients underwent appendectomy, including 4 for a preoperative diagnosis of appendicitis and the other for diagnosis and treatment of an appendiceal tumor. The sixth patient presented with vomiting and underwent ileocecal resection for GCC diagnosed from preoperative biopsy. Although 2 patients with GCC underwent colonoscopy, no neoplastic changes were identified. Two of the six patients showed lymph node metastasis on pathological examination. As of the last follow-up (median: 15 mo), all cases remained alive without recurrence.
CONCLUSION As preoperative diagnosis of GCC is difficult, this possibility must be considered during surgical treatments for presumptive appendicitis.
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Affiliation(s)
- Toshiaki Toshima
- Department of Gastroenterological Surgery, Kochi Health Sciences Center, Kochi 781-8555, Japan
| | - Ryo Inada
- Department of Gastroenterological Surgery, Kochi Health Sciences Center, Kochi 781-8555, Japan
| | - Shinya Sakamoto
- Department of Gastroenterological Surgery, Kochi Health Sciences Center, Kochi 781-8555, Japan
| | - Eri Takeda
- Department of Gastroenterological Surgery, Kochi Health Sciences Center, Kochi 781-8555, Japan
| | - Takahiro Yoshioka
- Department of Gastroenterological Surgery, Kochi Health Sciences Center, Kochi 781-8555, Japan
| | - Kento Kumon
- Department of Gastroenterological Surgery, Kochi Health Sciences Center, Kochi 781-8555, Japan
| | - Naoki Mimura
- Department of Gastroenterological Surgery, Kochi Health Sciences Center, Kochi 781-8555, Japan
| | - Nobuo Takata
- Department of Gastroenterological Surgery, Kochi Health Sciences Center, Kochi 781-8555, Japan
| | - Motoyasu Tabuchi
- Department of Gastroenterological Surgery, Kochi Health Sciences Center, Kochi 781-8555, Japan
| | - Kazuyuki Oishi
- Department of Gastroenterological Surgery, Kochi Health Sciences Center, Kochi 781-8555, Japan
| | - Takuji Sato
- Department of Gastroenterological Surgery, Kochi Health Sciences Center, Kochi 781-8555, Japan
| | - Kenta Sui
- Department of Gastroenterological Surgery, Kochi Health Sciences Center, Kochi 781-8555, Japan
| | - Takehiro Okabayashi
- Department of Gastroenterological Surgery, Kochi Health Sciences Center, Kochi 781-8555, Japan
| | - Kazuhide Ozaki
- Department of Gastroenterological Surgery, Kochi Health Sciences Center, Kochi 781-8555, Japan
| | - Toshio Nakamura
- Department of Gastroenterological Surgery, Kochi Health Sciences Center, Kochi 781-8555, Japan
| | - Yuichi Shibuya
- Department of Gastroenterological Surgery, Kochi Health Sciences Center, Kochi 781-8555, Japan
| | - Manabu Matsumoto
- Department of Diagnostic Pathology, Kochi Health Sciences Center, Kochi 781-8555, Japan
| | - Jun Iwata
- Department of Diagnostic Pathology, Kochi Health Sciences Center, Kochi 781-8555, Japan
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Ji JN, Yin ZB. Visualizing the landscape of appendiceal tumor research after 2010: A bibliometric study. World J Gastrointest Surg 2024; 16:1894-1909. [PMID: 38983319 PMCID: PMC11230007 DOI: 10.4240/wjgs.v16.i6.1894] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/26/2024] [Revised: 05/03/2024] [Accepted: 05/17/2024] [Indexed: 06/27/2024] Open
Abstract
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 co-citations 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.
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Affiliation(s)
- Jia-Nan Ji
- Department of General Surgery, The Affiliated Huishan Hospital of Xinglin College, Nantong University, Wuxi Huishan District People’s Hospital, Wuxi 214187, Jiangsu Province, China
| | - Zhi-Bin Yin
- Department of General Surgery, The Affiliated Huishan Hospital of Xinglin College, Nantong University, Wuxi Huishan District People’s Hospital, Wuxi 214187, Jiangsu Province, China
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Dong Y, Huang S, Wu H, Cao M, Huang Y, Tang G, Zhou W. Superiority of 18F-FAPI-42 PET/CT in the detection of primary tumor and management of appendiceal neoplasm to 18F-FDG PET/CT and CE-CT. Cancer Imaging 2024; 24:58. [PMID: 38715096 PMCID: PMC11077780 DOI: 10.1186/s40644-024-00706-7] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/18/2024] [Accepted: 04/27/2024] [Indexed: 05/12/2024] Open
Abstract
BACKGROUND In the present study, we investigated the value of 18F-fibroblast-activation protein inhibitor (FAPI) positron emission tomography/computed tomography (18F-FAPI-42 PET/CT) to preoperative evaluations of appendiceal neoplasms and management for patients. METHODS This single-center retrospective clinical study, including 16 untreated and 6 treated patients, was performed from January 2022 to May 2023 at Southern Medical University Nanfang Hospital. Histopathologic examination and imaging follow-up served as the reference standard. 18F-FAPI-42 PET/CT was compared to 18F-fluorodeoxyglucose (18F-FDG) PET/CT and contrast-enhanced CT (CE-CT) in terms of maximal standardized uptake value (SUVmax), diagnostic efficacy and impact on treatment decisions. RESULTS The accurate detection of primary tumors and peritoneal metastases were improved from 28.6% (4/14) and 50% (8/16) for CE-CT, and 43.8% (7/16) and 85.0% (17/20) for 18F-FDG PET/CT, to 87.5% (14/16) and 100% (20/20) for 18F-FAPI-42 PET/CT. Compared to 18F-FDG PET/CT, 18F-FAPI-42 PET/CT detected more regions infiltrated by peritoneal metastases (108 vs. 43), thus produced a higher peritoneal cancer index (PCI) score (median PCI: 12 vs. 5, P < 0.01). 18F-FAPI-42 PET/CT changed the intended treatment plans in 35.7% (5/14) of patients compared to CE-CT and 25% (4/16) of patients compared to 18F-FDG PET/CT but did not improve the management of patients with recurrent tumors. CONCLUSIONS The present study revealed that 18F-FAPI-42 PET/CT can supplement CE-CT and 18F-FDG PET/CT to provide a more accurate detection of appendiceal neoplasms and improved treatment decision making for patients.
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Affiliation(s)
- Ye Dong
- NanFang PET Center, NanFang Hospital, Southern Medical University, Guangzhou, Guangdong Province, 510515, China
- GDMPA Key Laboratory for Quality Control and Evaluation of Radiopharmaceuticals, Department of Nuclear Medicine, Nanfang Hospital, Southern Medical University, Guangzhou, Guangdong Province, 510515, China
| | - Shun Huang
- Department of Nuclear Medicine, PET Center, The Tenth Affiliated Hospital of Southern Medical University (Dongguan People's Hospital), Dongguan, 523059, P. R. China
| | - Hubing Wu
- NanFang PET Center, NanFang Hospital, Southern Medical University, Guangzhou, Guangdong Province, 510515, China
- GDMPA Key Laboratory for Quality Control and Evaluation of Radiopharmaceuticals, Department of Nuclear Medicine, Nanfang Hospital, Southern Medical University, Guangzhou, Guangdong Province, 510515, China
| | - Min Cao
- NanFang PET Center, NanFang Hospital, Southern Medical University, Guangzhou, Guangdong Province, 510515, China
- GDMPA Key Laboratory for Quality Control and Evaluation of Radiopharmaceuticals, Department of Nuclear Medicine, Nanfang Hospital, Southern Medical University, Guangzhou, Guangdong Province, 510515, China
| | - Yanchao Huang
- NanFang PET Center, NanFang Hospital, Southern Medical University, Guangzhou, Guangdong Province, 510515, China
- GDMPA Key Laboratory for Quality Control and Evaluation of Radiopharmaceuticals, Department of Nuclear Medicine, Nanfang Hospital, Southern Medical University, Guangzhou, Guangdong Province, 510515, China
| | - Ganghua Tang
- NanFang PET Center, NanFang Hospital, Southern Medical University, Guangzhou, Guangdong Province, 510515, China.
- GDMPA Key Laboratory for Quality Control and Evaluation of Radiopharmaceuticals, Department of Nuclear Medicine, Nanfang Hospital, Southern Medical University, Guangzhou, Guangdong Province, 510515, China.
| | - Wenlan Zhou
- NanFang PET Center, NanFang Hospital, Southern Medical University, Guangzhou, Guangdong Province, 510515, China.
- GDMPA Key Laboratory for Quality Control and Evaluation of Radiopharmaceuticals, Department of Nuclear Medicine, Nanfang Hospital, Southern Medical University, Guangzhou, Guangdong Province, 510515, China.
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Marks VA, Kerekes D, Butensky S, Ahuja N, Johnson C, Turaga K, Khan SA. Role of colectomy in the management of appendiceal tumors: a retrospective cohort study. BMC Gastroenterol 2023; 23:398. [PMID: 37978348 PMCID: PMC10655451 DOI: 10.1186/s12876-023-03019-4] [Citation(s) in RCA: 4] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/11/2023] [Accepted: 10/29/2023] [Indexed: 11/19/2023] Open
Abstract
BACKGROUND Appendiceal tumors represent a range of histologies that vary in behavior. Recommendations for treatment with appendectomy versus right hemicolectomy (RHC) for different tumor types are evolving and sometimes conflicting. This study sought to characterize variation in the United States around surgical treatment of major appendiceal tumor types over time and describe differences in outcomes based on procedure. METHODS Patients diagnosed with appendiceal goblet cell adenocarcinoma (GCA), mucinous adenocarcinoma, neuroendocrine neoplasm (NEN), or non-mucinous adenocarcinoma from 2004-2017 were identified in the National Cancer Database. Trends in RHC over time and predictors of RHC were identified. Surgical outcomes for each histologic type and stage were compared. RESULTS Of 18,216 patients, 11% had GCAs, 34% mucinous adenocarcinoma, 31% NENs, and 24% non-mucinous adenocarcinoma. Rate of RHC for NEN decreased from 68% in 2004 to 40% in 2017 (p = 0.008) but remained constant around 60-75% for other tumor types. Higher stage was associated with increased odds of RHC for all tumor types. RHC was associated with higher rate of unplanned readmission (5% vs. 3%, p < 0.001) and longer postoperative hospital stay (median 5 days vs. 3 days, p < 0.001). On risk-adjusted analysis, RHC was significantly associated with increased survival versus appendectomy for stage 2 disease of all tumor types (HRs 0.43 to 0.63) and for stage 1 non-mucinous adenocarcinoma (HR = 0.56). CONCLUSIONS Most patients with appendiceal tumors undergo RHC, which is associated with increased readmission, longer length of stay, and improved survival for stage 2 disease of all types. RHC should be offered selectively for appendiceal tumors.
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Affiliation(s)
- Victoria A Marks
- Department of Surgical Oncology, Yale School of Medicine, Yale University School of Medicine, 330 Cedar Street, FMB130, New Haven, CT, 06520, USA
| | - Daniel Kerekes
- Department of Surgical Oncology, Yale School of Medicine, Yale University School of Medicine, 330 Cedar Street, FMB130, New Haven, CT, 06520, USA
| | - Samuel Butensky
- Department of Surgical Oncology, Yale School of Medicine, Yale University School of Medicine, 330 Cedar Street, FMB130, New Haven, CT, 06520, USA
| | - Nita Ahuja
- Department of Surgical Oncology, Yale School of Medicine, Yale University School of Medicine, 330 Cedar Street, FMB130, New Haven, CT, 06520, USA
| | - Caroline Johnson
- Yale School of Public Health, 60 College St, New Haven, CT, 06510, USA
| | - Kiran Turaga
- Department of Surgical Oncology, Yale School of Medicine, Yale University School of Medicine, 330 Cedar Street, FMB130, New Haven, CT, 06520, USA
| | - Sajid A Khan
- Department of Surgical Oncology, Yale School of Medicine, Yale University School of Medicine, 330 Cedar Street, FMB130, New Haven, CT, 06520, USA.
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Gaillard M, Van Eyken P, Verswijvel G, Van der Speeten K. Appendiceal Goblet Cell Carcinoma: Role of Cytoreductive Surgery (CRS) and Hyperthermic Intraperitoneal Chemotherapy (HIPEC). Indian J Surg Oncol 2023; 14:240-249. [PMID: 37359931 PMCID: PMC10284751 DOI: 10.1007/s13193-023-01748-2] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/06/2023] [Accepted: 04/06/2023] [Indexed: 06/28/2023] Open
Abstract
Goblet cell carcinoma (GCC) encompasses a separate entity in appendiceal neoplasms with mixed glandular and neuro-endocrine pathological features. GCC mostly presents as an acute appendicitis duo to luminal obstruction or as an incidental finding on the surgical appendectomy specimen. In case of tumour perforation or presence of other risk factors, guidelines suggest additional treatment with a completing right hemicolectomy or cytoreductive surgery (CRS) with hyperthermic intraperitoneal chemotherapy (HIPEC). We report the case of a 77-year-old male with symptoms of appendicitis for which an appendectomy was performed. The appendix was ruptured during the procedure. There was an incidental finding of GCC on the pathological specimen. Because of possible tumour soiling, the patient received a prophylactic CRS-HIPEC. A literature review was performed to investigate the potential role for CRS-HIPEC as a curative treatment in patients with GCC. GCC of the appendix is an aggressive type of tumour with a high risk of peritoneal and systemic dissemination. CRS and HIPEC is a treatment option: both in a prophylactic setting and in patients with established peritoneal metastases.
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Affiliation(s)
- M. Gaillard
- Department of Surgical Oncology, Ziekenhuis Oost-Limburg, Schiepse Bos 6, 3600 Genk, Belgium
| | - P. Van Eyken
- Department of Pathology, Ziekenhuis Oost-Limburg, Schiepse Bos 6, 3600 Genk, Belgium
| | - G. Verswijvel
- Department of Radiology, Ziekenhuis Oost-Limburg, Schiepse Bos 6, 3600 Genk, Belgium
| | - K. Van der Speeten
- Department of Surgical Oncology, Ziekenhuis Oost-Limburg, Schiepse Bos 6, 3600 Genk, Belgium
- Department of Medicine and Life Sciences, Hasselt University, Hasselt, Belgium
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Li M, Yao X. Goblet cell adenocarcinoma of the anal canal with perianal Paget disease: A rare case report with literature review. Medicine (Baltimore) 2023; 102:e33598. [PMID: 37083794 PMCID: PMC10118358 DOI: 10.1097/md.0000000000033598] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/14/2023] [Accepted: 04/03/2023] [Indexed: 04/22/2023] Open
Abstract
INTRODUCTION AS an uncommon neoplasm, goblet cell adenocarcinoma (GCA) is characterized by mixed endocrine-exocrine features. It is almost exclusively found in the appendix. Primary GCA of the anal canal is extremely rare. CASE PRESENTATION Herein we describe a novel rare case of 74-year-old Chinese female who is diagnosed with GCA in the anal canal with perianal Paget disease, including a brief review of the literature. In the lesion of anal canal, the tumor was composed of signet-ring-like cells on confluent growth model and copious mucin was produced as well. Simultaneously, the results of immunohistochemistry showed signet-ring-like cells were positive for CK20, CDX2, synaptophysin (Syn), CD56, carcinoembryonic antigen (CEA) and Villin. Meanwhile, the Ki67-labeling index reached 40%. In the lesion of perianal Paget disease, the small groups of atypical neoplastic cells were present in the epidermis. Immunohistochemically, the neoplastic cells were positive for CK20, CDX2 and epithelial membrane antigen, but negative for CK7, GCDFP15, S100, HMB45, and P63. The Ki67-labeling index reached 60% in the most concentrated spot. CONCLUSIONS Extra-appendiceal GCA was rare and easily under-recognizable. The diagnosis of GCA was seldom made preoperatively. Occasionally, GCA could occur in the anal canal accompanied by perianal Paget disease. So careful rectal examination was important in the patient with perianal Paget disease for avoid missing diagnosis of GCA on anal canal. GCA may show aggressive clinical behavior compared with typical well-differentiated neuroendocrine tumors. Therefore, we should pay more attention on the recognization of this rare disease.
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Affiliation(s)
- Minhua Li
- Department of Pathology, Shaoxing People’s Hospital, Shaoxing, Zhejiang, China
| | - Xiaofei Yao
- Department of Pathology, Shaoxing People’s Hospital, Shaoxing, Zhejiang, China
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Kiyosawa N, Koyama M, Miyagawa Y, Kitazawa M, Tokumaru S, Soejima Y. Goblet cell adenocarcinoma of the appendix: A case report of three cases. Int J Surg Case Rep 2023; 106:108229. [PMID: 37084554 PMCID: PMC10140787 DOI: 10.1016/j.ijscr.2023.108229] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/09/2022] [Revised: 04/13/2023] [Accepted: 04/14/2023] [Indexed: 04/23/2023] Open
Abstract
INTRODUCTION AND IMPORTANCE Appendiceal goblet cell adenocarcinoma is in 0.3-0.9 % of appendectomy specimens. There is still controversy regarding whether surgery with dissection or additional resection is necessary for goblet cell adenocarcinoma and whether adjuvant chemotherapy is practical. We present three cases of goblet cell adenocarcinomas. CASE PRESENTATION Case 1: A 30-year-old woman was diagnosed with appendicitis and underwent appendicectomy. Histopathological evaluation revealed a malignant neoplasm with goblet-like cells and tumour infiltration into the subserosa. The patient underwent laparoscopic ileocecal resection, and the main lymph nodes at the root of the feeding vessels were removed. Case 2: A 50-year-old man was diagnosed with appendicitis and underwent appendicectomy. Histopathological evaluation revealed a malignant neoplasm with goblet-like cells; malignant cells were found at the surgical resection margins. The patient underwent laparoscopic ileocolic resection. Case 3: A 60-year-old man undergoing treatment for malignant melanoma. He was diagnosed with appendicitis associated with an appendiceal tumour, and emergency laparoscopic caecal resection was performed and diagnosed as goblet cell adenocarcinoma. We decided to prioritize treatment for malignant melanoma, and the patient is under follow-up for goblet cell adenocarcinoma and no metastasis was detected. CLINICAL DISCUSSION We performed additional resection in two case of goblet cell adenocarcinoma. Diagnosing appendiceal goblet cell adenocarcinoma is difficult, and the prognosis of patients with positive lymph nodes is poor. Surgical treatment should be considered for the advanced stages of this disease. CONCLUSION Goblet cell adenocarcinoma, diagnosed after appendectomy, additional resection including lymph node dissection may provide a long-term prognosis.
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Affiliation(s)
- Nami Kiyosawa
- Division of Gastroenterological, Hepato-Biliary-Pancreatic, Transplantation and Pediatric Surgery, Department of Surgery, Shinshu University School of Medicine, 3-1-1 Asahi, Matsumoto, Nagano 390-8621, Japan
| | - Makoto Koyama
- Division of Gastroenterological, Hepato-Biliary-Pancreatic, Transplantation and Pediatric Surgery, Department of Surgery, Shinshu University School of Medicine, 3-1-1 Asahi, Matsumoto, Nagano 390-8621, Japan.
| | - Yusuke Miyagawa
- Division of Gastroenterological, Hepato-Biliary-Pancreatic, Transplantation and Pediatric Surgery, Department of Surgery, Shinshu University School of Medicine, 3-1-1 Asahi, Matsumoto, Nagano 390-8621, Japan
| | - Masato Kitazawa
- Division of Gastroenterological, Hepato-Biliary-Pancreatic, Transplantation and Pediatric Surgery, Department of Surgery, Shinshu University School of Medicine, 3-1-1 Asahi, Matsumoto, Nagano 390-8621, Japan
| | - Shigeo Tokumaru
- Division of Gastroenterological, Hepato-Biliary-Pancreatic, Transplantation and Pediatric Surgery, Department of Surgery, Shinshu University School of Medicine, 3-1-1 Asahi, Matsumoto, Nagano 390-8621, Japan
| | - Yuji Soejima
- Division of Gastroenterological, Hepato-Biliary-Pancreatic, Transplantation and Pediatric Surgery, Department of Surgery, Shinshu University School of Medicine, 3-1-1 Asahi, Matsumoto, Nagano 390-8621, Japan
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10
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Ricci C, Campana D, Ingaldi C, Lamberti G, Alberici L, Tateo V, Castagna G, Ricco G, Calderaro F, Malvi D, Rosini F, Casadei R. Appendiceal goblet cell carcinoma has marginal advantages from perioperative chemotherapy: a population-based study with an entropy balancing analysis. Langenbecks Arch Surg 2023; 408:65. [PMID: 36695921 PMCID: PMC9876863 DOI: 10.1007/s00423-023-02791-x] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/22/2022] [Accepted: 12/02/2022] [Indexed: 01/26/2023]
Abstract
PURPOSE The aim is to clarify the use of perioperative chemotherapy in resectable goblet cell carcinoma (GCC). METHODS A retrospective study was carried out based on the Surveillance, Epidemiology, and End Results study. The population was divided: into patients who received only radical surgery (group A) and those who received radical surgery plus chemotherapy (group B). An entropy balancing was carried out to correct the imbalance between the two groups. Two models were generated. Model 1 contained only high-risk patients: group B and a "virtual" group A with similar characteristics. Model 2 included only low-risk patients: group A and "virtual" group B with identical attributes. The efficacy of entropy balancing was evaluated with the d value. The overall survival was compared and reported with Hazard Ratio (HR) within a confidence interval of 95% (95 CI). RESULTS The groups A and B were imbalanced for tumor size (d = 0.392), T (d = 1.128), N (d = 1.340), M (d = 1.456), mean number of positive lymph nodes (d = 0.907), and LNR (d = 0.889). Before the balancing, the risk of death was higher in group B than in A (4.3; 2.5 to 7.4). After reweighting, all large differences were eliminated (d < 0.200). In high-risk patients, the risk of death was higher in patients who underwent surgery alone than those who received perioperative chemotherapy (HR 0.5; 0.2 to 1.3) without statistical significance (p = 0.187). In low-risk patients, the risk of death was similar (HR 1.1; 0.3 to 3.3). CONCLUSION Perioperative chemotherapy could provide some marginal advantages to high-risk patients.
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Affiliation(s)
- Claudio Ricci
- Division of Pancreatic Surgery, IRCCS Azienda Ospedaliero-Universitaria Di Bologna, Via Albertoni 15, Bologna, Italia.
- Department of Internal Medicine and Surgery (DIMEC), Alma Mater Studiorum, University of Bologna, Bologna, Italy.
| | - Davide Campana
- Division of Oncology, IRCCS Azienda Ospedaliero-Universitaria Di Bologna, Via Albertoni 15, Bologna, Italia
- Department of Specialized,Diagnostic and Experimental Medicine (DIMES), Alma Mater Studiorum, University of Bologna, Bologna, Italy
| | - Carlo Ingaldi
- Division of Pancreatic Surgery, IRCCS Azienda Ospedaliero-Universitaria Di Bologna, Via Albertoni 15, Bologna, Italia
- Department of Internal Medicine and Surgery (DIMEC), Alma Mater Studiorum, University of Bologna, Bologna, Italy
| | - Giuseppe Lamberti
- Division of Oncology, IRCCS Azienda Ospedaliero-Universitaria Di Bologna, Via Albertoni 15, Bologna, Italia
| | - Laura Alberici
- Division of Pancreatic Surgery, IRCCS Azienda Ospedaliero-Universitaria Di Bologna, Via Albertoni 15, Bologna, Italia
- Department of Internal Medicine and Surgery (DIMEC), Alma Mater Studiorum, University of Bologna, Bologna, Italy
| | - Valentina Tateo
- Division of Oncology, IRCCS Azienda Ospedaliero-Universitaria Di Bologna, Via Albertoni 15, Bologna, Italia
| | - Giovanni Castagna
- Division of Pancreatic Surgery, IRCCS Azienda Ospedaliero-Universitaria Di Bologna, Via Albertoni 15, Bologna, Italia
- Department of Internal Medicine and Surgery (DIMEC), Alma Mater Studiorum, University of Bologna, Bologna, Italy
| | - Gianluca Ricco
- Division of Oncology, IRCCS Azienda Ospedaliero-Universitaria Di Bologna, Via Albertoni 15, Bologna, Italia
| | - Fulvio Calderaro
- Division of Pancreatic Surgery, IRCCS Azienda Ospedaliero-Universitaria Di Bologna, Via Albertoni 15, Bologna, Italia
- Department of Internal Medicine and Surgery (DIMEC), Alma Mater Studiorum, University of Bologna, Bologna, Italy
| | - Deborah Malvi
- Division of Pathology, IRCCS Azienda Ospedaliero-Universitaria Di Bologna, Via Albertoni 15, Bologna, Italia
| | - Francesca Rosini
- Division of Pathology, IRCCS Azienda Ospedaliero-Universitaria Di Bologna, Via Albertoni 15, Bologna, Italia
| | - Riccardo Casadei
- Division of Pancreatic Surgery, IRCCS Azienda Ospedaliero-Universitaria Di Bologna, Via Albertoni 15, Bologna, Italia
- Department of Internal Medicine and Surgery (DIMEC), Alma Mater Studiorum, University of Bologna, Bologna, Italy
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11
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Palmer K, Weerasuriya S, Chandrakumaran K, Rous B, White BE, Paisey S, Srirajaskanthan R, Ramage JK. Goblet Cell Adenocarcinoma of the Appendix: A Systematic Review and Incidence and Survival of 1,225 Cases From an English Cancer Registry. Front Oncol 2022; 12:915028. [PMID: 35903705 PMCID: PMC9314749 DOI: 10.3389/fonc.2022.915028] [Citation(s) in RCA: 16] [Impact Index Per Article: 5.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/07/2022] [Accepted: 06/08/2022] [Indexed: 12/19/2022] Open
Abstract
BackgroundGoblet cell adenocarcinoma (GCA) of the appendix is a rare and aggressive tumour with varying nomenclature and classification systems. This has led to heterogeneity in published data, and there is a lack of consensus on incidence, survival, and management.MethodsWe provide an overview of GCA with a comprehensive systematic review using Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) methodology and a retrospective analysis of all cases recorded in the English National Cancer Registration and Analysis Service database between 1995 and 2018. The Kaplan–Meier estimator was used to calculate overall survival, and Cox proportional hazards regression was used to identify prognostic factors.ResultsThe systematic review demonstrated an incidence of 0.05–0.3 per 100,000 per year among North American registry studies. The 1-, 3-, and 5-year survival rate was 95.5%, 85.9%–87.6%, and 76.0%–80.6%, respectively. Age, stage, and grade were identified as prognostic factors for survival. Our analysis included 1,225 cases. Age-standardised incidence was 0.0335 per year in 1995 and gradually rose to 0.158 per year in 2018. The 1-, 3-, and 5-year survival rate was 90.0% [95% confidence interval (95% CI): 85.4–94.0], 76.0% (95% CI: 73.8–80.9), and 68.6% (95% CI: 65.9–72.2), respectively. On univariate Cox regression analyses, female sex, stage, and grade were associated with worse overall survival. On multivariate analysis, only stage remained a statistically significant prognostic factor.ConclusionsGCA of the appendix is rare, but incidence is increasing. We report a lower incidence and survival than North American registry studies. Higher stage was associated with decreased survival. Further prospective studies are required to establish optimal management.
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Affiliation(s)
- Kieran Palmer
- Barts Cancer Centre, St Bartholomew’s Hospital, London, United Kingdom
- *Correspondence: Kieran Palmer,
| | - Scott Weerasuriya
- Department of Critical Care Medicine, King’s College Hospital National Health Service (NHS) Foundation Trust, London, United Kingdom
| | - Kandiah Chandrakumaran
- Surgical Division, Hampshire Hospitals National Health Service (NHS) Foundation Trust, Basingstoke, United Kingdom
| | - Brian Rous
- National Health Service (NHS) Digital, Leeds, United Kingdom
| | - Benjamin E. White
- Surgical Division, Hampshire Hospitals National Health Service (NHS) Foundation Trust, Basingstoke, United Kingdom
| | - Sangeeta Paisey
- Surgical Division, Hampshire Hospitals National Health Service (NHS) Foundation Trust, Basingstoke, United Kingdom
| | - Rajaventhan Srirajaskanthan
- Department of Critical Care Medicine, King’s College Hospital National Health Service (NHS) Foundation Trust, London, United Kingdom
- Faculty of Life Sciences and Medicine, King’s College London, London, United Kingdom
| | - John K. Ramage
- Surgical Division, Hampshire Hospitals National Health Service (NHS) Foundation Trust, Basingstoke, United Kingdom
- Faculty of Life Sciences and Medicine, King’s College London, London, United Kingdom
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12
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Tamiya M, Matsuda K, Iwamoto H, Mitani Y, Mizumoto Y, Nakamura Y, Sakanaka T, Takemoto N, Hyo T, Iwamoto R, Yamaue H. Appendiceal goblet cell adenocarcinoma with peritoneal recurrence 9 years after surgery. Int Cancer Conf J 2021; 11:57-61. [DOI: 10.1007/s13691-021-00519-6] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/26/2021] [Accepted: 10/19/2021] [Indexed: 11/28/2022] Open
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13
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Alabraba E, Pritchard DM, Griffin R, Diaz-Nieto R, Banks M, Cuthbertson DJ, Fenwick S. Appendiceal goblet cell carcinomas have poor survival despite completion surgery. Endocrine 2021; 73:734-744. [PMID: 33891259 DOI: 10.1007/s12020-021-02727-9] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/07/2021] [Accepted: 04/05/2021] [Indexed: 12/11/2022]
Abstract
PURPOSE Appendiceal goblet cell carcinomas (aGCCs) are rare but aggressive tumours associated with significant mortality. We retrospectively reviewed the outcomes of aGCC patients treated at our tertiary referral centre. METHODS We analysed aGCC patients, diagnosed between 1990-2016, assessing the impact of completion surgery and tumour factors on survival. Survival was assessed using Kaplan-Meier analysis. RESULTS We identified 41 patients (23 F, 18 M); median age 61 (range 27-79) years. Mean tumour size was 10.5 (range 0.5-50) mm; most tumours were located in the appendiceal tip (n = 18, 45%). Appendicectomy was the index surgery in 32 patients, 24 of whom subsequently underwent completion surgery at median 3 (range 1.3-13.3) months later. Histology from completion surgery showed residual disease in 8 patients: nodal disease (n = 2) or residual tumour (n = 6). Index surgery for the rest was either colectomy (n = 7) or cytoreductive surgery plus intraperitoneal chemotherapy (CRS-HIPEC) (n = 1). Index and completion surgery had 0% mortality and 2.5% morbidity. Overall and recurrence-free survival were not significantly affected by tumour grade or completion surgery. Disease recurred in 9 patients after a median follow-up of 57.0 (4.6-114.9) months; 7 of these patients died during follow-up. Recurrences were treated with CRS-HIPEC (n = 1), palliative chemotherapy (n = 3) or supportive care (n = 5). Five- and ten- year overall survival were 85.3% and 62.3% respectively; 5-year and 10-year recurrence-free survival were 73.6% and 50.6%. CONCLUSION The prognosis of aGCCs remains relatively poor. Completion surgery did not prevent recurrence or improve survival, but this needs to be verified with a larger patient cohort. The high mortality associated with tumour recurrence questions current treatment recommendations.
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Affiliation(s)
- Edward Alabraba
- Department of Hepatobiliary and Pancreatic Surgery, Queen's Medical Centre, Nottingham University Hospitals NHS Trust, Nottingham, United Kingdom.
| | - David Mark Pritchard
- ENETS Centre of Excellence, Liverpool University Hospitals NHS Foundation Trust, Liverpool, United Kingdom
- Department of Molecular and Clinical Cancer Medicine; Institute of Systems, Molecular and Integrative Biology, University of Liverpool, Liverpool, United Kingdom
| | - Rebecca Griffin
- Liverpool Clinical and Cancer Research UK Trials Unit, University of Liverpool, Liverpool, United Kingdom
| | - Rafael Diaz-Nieto
- ENETS Centre of Excellence, Liverpool University Hospitals NHS Foundation Trust, Liverpool, United Kingdom
| | - Melissa Banks
- ENETS Centre of Excellence, Liverpool University Hospitals NHS Foundation Trust, Liverpool, United Kingdom
| | - Daniel James Cuthbertson
- ENETS Centre of Excellence, Liverpool University Hospitals NHS Foundation Trust, Liverpool, United Kingdom
- Department of Cardiovascular and Metabolic Medicine, Institute of Life Course and Medical Sciences, University of Liverpool, Liverpool, United Kingdom
| | - Stephen Fenwick
- ENETS Centre of Excellence, Liverpool University Hospitals NHS Foundation Trust, Liverpool, United Kingdom
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Ciarrocchi A, Rindi G, Pietroletti R. Diagnosis and Treatment of Primary Tumors of the Appendix: a Critical Review. J Gastrointest Cancer 2021; 52:471-475. [PMID: 33740229 DOI: 10.1007/s12029-021-00628-8] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 03/14/2021] [Indexed: 12/24/2022]
Abstract
INTRODUCTION The aim of the current paper was to critically collect, select and summarize the evidence regarding diagnosis, treatment and follow up of primary tumors of the appendix. METHODS A literature review was performed by search and review of the scientific studies pertaining to the subject of our inquiry. RESULTS Recommendations regarding pimary tumors of the appendix were formulated on the basis of the collected evidence. CONCLUSION : Primary tumors of the appendix are rare and a high index of suspicion is required not to miss a potentially life threatening medical condition.
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Affiliation(s)
| | - Guido Rindi
- Fondazione Policlinico Universitario A. Gemelli IRCCS-Università Cattolica del Sacro Cuore, Rome, Italy
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15
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A Case of Appendiceal Goblet Cell Carcinoid Tumor: Getting it right under the Microscope. J ASEAN Fed Endocr Soc 2021; 35:102-104. [PMID: 33442175 PMCID: PMC7784227 DOI: 10.15605/jafes.035.01.16] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/19/2020] [Accepted: 04/01/2020] [Indexed: 11/17/2022] Open
Abstract
Goblet cell carcinoid (GCC) is a rare neoplasm of the vermiform appendix and can be mistaken as a typical neuroendocrine tumour (TNET). The natural history of this disease is more aggressive compared to TNETs and requires a more aggressive approach. We report a case of a 37-year-old male who was initially diagnosed with TNET, but subsequently revised as Tang's A GCC. He underwent appendectomy and right hemicolectomy. Aside from a persistently elevated carcinoembyrogenic antigen (CEA) result, his 18F-fluorodeoxyglucose (FDG) PET/CT and a 68-Gallium DOTATATE PET/CT scan showed no FDG or DOTATATE avid lesions.
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16
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Zakka K, Williamson S, Jiang R, Reid MD, Alese OB, Shaib WL, Wu C, Behera M, El-Rayes BF, Akce M. Is adjuvant chemotherapy beneficial for stage II-III goblet cell carcinoid/goblet cell adenocarcinoma of the appendix? Surg Oncol 2020; 36:120-129. [PMID: 33360118 DOI: 10.1016/j.suronc.2020.12.003] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/28/2020] [Revised: 11/06/2020] [Accepted: 12/06/2020] [Indexed: 01/21/2023]
Abstract
BACKGROUND Goblet cell carcinoma (GCC), formerly known as goblet cell carcinoid, of the appendix constitutes less than 14% of all primary appendiceal neoplasms. Surgical resection is the main treatment and the role of adjuvant chemotherapy (AC) is not established. This study aims to evaluate the impact of AC in stage II-III appendiceal GCC. METHODS Patients with pathological stage II and III GCC who underwent surgical resection between 2006 and 2015 were identified from the National Cancer Database (NCDB) using ICD-O-3 morphology and topography codes: 8243/3 (goblet cell carcinoid) and C18.1. Patients treated with neoadjuvant systemic and/or radiation therapy and adjuvant radiation were excluded. Univariate and multivariable analyses were conducted, and Kaplan-Meier Curves were used to compare overall survival (OS) based on treatment received with Log-rank test. RESULTS A total of 619 patients were identified. 54.4% males and 89.0% Caucasian; median age 56 (range, 23-90) years. Distribution across pathological stages II-III was 82.7% (N = 512) and 17.3% (N = 107) respectively. AC was administered in 9.4% (N = 48) of stage II and 47.7% (N = 51) of stage III patients. For stage II patients, AC was not associated with better OS in univariate (HR 0.32; 95% CI 0.04-2.34; p = 0.261) or multivariable analyses (HR 0.29; 95% CI 0.04-2.12; p = 0.221). By contrast, in stage III patients, AC was associated with better OS in univariate (HR 0.35; 95% CI 0.17-0.71; p = 0.004) and multivariable analyses (HR 0.25; 95% CI 0.07-0.88; p = 0.031). In the entire cohort 5-year OS for patients that received AC was 85.5% (74.0%, 92.1%) versus 82.7% (77.5%, 86.8%) (p = 0.801) with no AC. For stage II patients, 5-year OS was 96.9% with AC vs. 89.1% with no AC (p = 0.236). For stage III patients, 5-year OS was 77.1% with AC vs. 42.8% with no AC (p = 0.003). CONCLUSION AC was associated with improved OS in patients with pathological stage III GCC of the appendix, but not with pathological stage II.
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Affiliation(s)
- Katerina Zakka
- Department of Hematology and Medical Oncology, Winship Cancer Institute, Emory University, Atlanta, GA, USA
| | - Shayla Williamson
- Winship Research Informatics, Winship Cancer Institute, Emory University, Atlanta, GA, USA
| | - Renjian Jiang
- Winship Research Informatics, Winship Cancer Institute, Emory University, Atlanta, GA, USA
| | - Michelle D Reid
- Department of Pathology and Laboratory Medicine, Emory University School of Medicine, USA
| | - Olatunji B Alese
- Department of Hematology and Medical Oncology, Winship Cancer Institute, Emory University, Atlanta, GA, USA
| | - Walid L Shaib
- Department of Hematology and Medical Oncology, Winship Cancer Institute, Emory University, Atlanta, GA, USA
| | - Christina Wu
- Department of Hematology and Medical Oncology, Winship Cancer Institute, Emory University, Atlanta, GA, USA
| | - Madhusmita Behera
- Department of Hematology and Medical Oncology, Winship Cancer Institute, Emory University, Atlanta, GA, USA; Winship Research Informatics, Winship Cancer Institute, Emory University, Atlanta, GA, USA
| | - Bassel F El-Rayes
- Department of Hematology and Medical Oncology, Winship Cancer Institute, Emory University, Atlanta, GA, USA
| | - Mehmet Akce
- Department of Hematology and Medical Oncology, Winship Cancer Institute, Emory University, Atlanta, GA, USA.
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Saito M, Asanuma K, Hatta W, Koike T, Hata T, Fujishima F, Furukawa T, Unno M, Masamune A. Duodenal Obstruction Caused by the Long-term Recurrence of Appendiceal Goblet Cell Carcinoid. Intern Med 2020; 59:3001-3007. [PMID: 32759580 PMCID: PMC7759692 DOI: 10.2169/internalmedicine.4548-20] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/21/2023] Open
Abstract
A 38-year-old Japanese man who had been diagnosed with appendiceal carcinoid and undergone ileocecal resection 8 years before presented with duodenal obstruction caused by a submucosal tumor-like appearance. He was diagnosed with long-term recurrence of appendiceal goblet cell carcinoid (GCC) with a multi-morphological pattern based on the histological assessment of a duodenal biopsy and his previously resected appendix. He underwent subtotal stomach-preserving pancreaticoduodenectomy combined with resection of an ileo-colic anastomotic lesion. The GCC recurred at the nearby ileo-colic anastomosis and invaded the duodenum. This late recurrence might have resulted from the unique features of his GCC, which contained cells with different degrees of malignancy.
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Affiliation(s)
- Masashi Saito
- Division of Gastroenterology, Tohoku University Graduate School of Medicine, Japan
| | - Kiyotaka Asanuma
- Division of Gastroenterology, Tohoku University Graduate School of Medicine, Japan
| | - Waku Hatta
- Division of Gastroenterology, Tohoku University Graduate School of Medicine, Japan
| | - Tomoyuki Koike
- Division of Gastroenterology, Tohoku University Graduate School of Medicine, Japan
| | - Tatsuo Hata
- Division of Gastroenterological Surgery, Tohoku University Graduate School of Medicine, Japan
| | - Fumiyoshi Fujishima
- Department of Pathology, Tohoku University Graduate School of Medicine, Japan
| | - Toru Furukawa
- Department of Investigative Pathology, Tohoku University Graduate School of Medicine, Japan
| | - Michiaki Unno
- Division of Gastroenterological Surgery, Tohoku University Graduate School of Medicine, Japan
| | - Atsushi Masamune
- Division of Gastroenterology, Tohoku University Graduate School of Medicine, Japan
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18
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Kato J, Maruta A, Shirakami Y, Mizutani T, Ozawa N, Ideta T, Takada J, Kubota M, Sakai H, Ibuka T, Araki H, Shimizu M. Advanced appendiceal goblet cell carcinoids with intestinal obstruction: two case reports. Clin J Gastroenterol 2020; 13:1205-1212. [PMID: 32989687 DOI: 10.1007/s12328-020-01232-2] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/18/2020] [Accepted: 08/31/2020] [Indexed: 01/20/2023]
Abstract
A goblet cell carcinoid is quite rare and has features, wherein, a carcinoid-like image and an adenocarcinoma-like image coexist. We encountered two cases of rare goblet cell carcinoid originating in the appendix. Case 1 is that of a 48-year-old man with a chief complaint of abdominal distension and case 2 is that of a 64-year-old woman with a chief complaint of constipation. At the time of diagnosis, both cases had already metastasized to the peritoneum and other organs, and no radical surgical treatment could be administered in either case. Chemotherapies were performed according to the regimen for colon cancer, and they were effective to a certain extent. During the course of treatment, however, both cases developed intestinal obstruction, presumably due to peritoneal dissemination, which led to worse condition and death several months afterwards. Chemotherapy for goblet cell carcinoids has not yet reached a consensus, and further studies and establishment of therapeutic strategy are desired in the future.
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Affiliation(s)
- Junichi Kato
- Department of Gastroenterology, Gifu University Graduate School of Medicine, 1-1 Yanagido, Gifu, 501-1194, Japan
| | - Akinori Maruta
- Department of Gastroenterology, Gifu University Graduate School of Medicine, 1-1 Yanagido, Gifu, 501-1194, Japan
| | - Yohei Shirakami
- Department of Gastroenterology, Gifu University Graduate School of Medicine, 1-1 Yanagido, Gifu, 501-1194, Japan.
| | - Taku Mizutani
- Department of Gastroenterology, Gifu University Graduate School of Medicine, 1-1 Yanagido, Gifu, 501-1194, Japan
| | - Noritaka Ozawa
- Department of Gastroenterology, Gifu University Graduate School of Medicine, 1-1 Yanagido, Gifu, 501-1194, Japan
| | - Takayasu Ideta
- Department of Gastroenterology, Gifu University Graduate School of Medicine, 1-1 Yanagido, Gifu, 501-1194, Japan
| | - Jun Takada
- Department of Gastroenterology, Gifu University Graduate School of Medicine, 1-1 Yanagido, Gifu, 501-1194, Japan
| | - Masaya Kubota
- Department of Gastroenterology, Gifu University Graduate School of Medicine, 1-1 Yanagido, Gifu, 501-1194, Japan
| | - Hiroyasu Sakai
- Department of Gastroenterology, Gifu University Graduate School of Medicine, 1-1 Yanagido, Gifu, 501-1194, Japan
| | - Takashi Ibuka
- Department of Gastroenterology, Gifu University Graduate School of Medicine, 1-1 Yanagido, Gifu, 501-1194, Japan
| | - Hiroshi Araki
- Department of Gastroenterology, Gifu University Graduate School of Medicine, 1-1 Yanagido, Gifu, 501-1194, Japan
| | - Masahito Shimizu
- Department of Gastroenterology, Gifu University Graduate School of Medicine, 1-1 Yanagido, Gifu, 501-1194, Japan
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Sluiter NR, van der Bilt JD, Croll DMR, Vriens MR, de Hingh IHJT, Hemmer P, Aalbers AGJ, Bremers AJA, Ceelen W, D'Hoore A, Schoonmade LJ, Coupé V, Verheul H, Kazemier G, Tuynman JB. Cytoreduction and Hyperthermic Intraperitoneal Chemotherapy (HIPEC) Versus Surgery Without HIPEC for Goblet-Cell Carcinoids and Mixed Adenoneuroendocrine Carcinomas: Propensity Score-Matched Analysis of Centers in the Netherlands and Belgium. Clin Colorectal Cancer 2020; 19:e87-e99. [PMID: 32651131 DOI: 10.1016/j.clcc.2020.01.002] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/26/2019] [Revised: 07/22/2019] [Accepted: 01/06/2020] [Indexed: 12/16/2022]
Abstract
BACKGROUND The value of cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) for patients with peritoneally metastasized goblet-cell carcinoids (GCCs) and mixed adenoneuroendocrine carcinomas (MANECs) is currently unclear. We compared outcomes of CRS-HIPEC to surgery alone for peritoneally metastasized GCCs and MANECs. PATIENTS AND METHODS Two cohorts were obtained from the Netherlands Cancer Registry (n = 569): patients with peritoneally metastasized GCCs and MANECs treated with CRS-HIPEC in Dutch and Belgian centers (n = 45), and patients treated with surgery alone. Primary outcome was overall survival (OS). Secondary outcomes were morbidity and hospital mortality. After propensity score matching, OS was compared in univariate and multivariate analyses. A systematic literature review was conducted following the PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-analyses) guidelines from database inception to June 25, 2018. RESULTS After matching for sex, tumor stage, lymph node stage, and liver metastases, CRS-HIPEC was associated with improved median OS in the combined GCC and MANEC group and the separate GCC subgroup in univariate (GCC + MANEC: 39 vs. 12 months, P < .001; GCC: 39 vs. 12 months, P = .017) and multivariate analysis (GCC + MANEC: hazard ratio 4.27, 95% confidence interval 1.88-9.66, P = .001; GCC: hazard ratio 2.77, 95% confidence interval 1.06-7.26, P = .038). Acceptable grade III-IV morbidity (17.5%) and mortality (0) were seen after CRS-HIPEC. The literature review supported these findings. CONCLUSION CRS-HIPEC is associated with substantial survival benefit in patients with peritoneally metastasized GCCs and MANECs compared to surgery alone and is a safe treatment option. These data support centralized care of GCC and MANEC patients with peritoneal spread in expert centers offering CRS-HIPEC.
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Affiliation(s)
- Nina R Sluiter
- Department of Surgery, Cancer Center Amsterdam, Amsterdam UMC, Vrije Universiteit Amsterdam, Amsterdam, The Netherlands.
| | | | - Dorothée M R Croll
- Department of Surgery, University Medical Center Utrecht, Utrecht, The Netherlands
| | - Menno R Vriens
- Department of Surgery, University Medical Center Utrecht, Utrecht, The Netherlands
| | | | - Patrick Hemmer
- Department of Surgery, University Medical Center Groningen, Groningen, The Netherlands
| | - Arend G J Aalbers
- Department of Surgery, Netherlands Cancer Institute, Amsterdam, The Netherlands
| | - Andreas J A Bremers
- Department of Surgery, Radboud University Medical Center, Nijmegen, The Netherlands
| | - Wim Ceelen
- Department of Surgery, University Hospital Ghent, Gent, Belgium
| | - Andre D'Hoore
- Department of Surgery, University Hospital Leuven, Leuven, Belgium
| | - Linda J Schoonmade
- Medical Library, Amsterdam UMC, Vrije Universiteit Amsterdam, Amsterdam, The Netherlands
| | - Veerle Coupé
- Department of Epidemiology and Biostatistics, Amsterdam UMC, Vrije Universiteit Amsterdam, Amsterdam, The Netherlands
| | - Henk Verheul
- Department of Medical Oncology, Cancer Center Amsterdam, Amsterdam UMC, Vrije Universiteit Amsterdam, Amsterdam, The Netherlands
| | - Geert Kazemier
- Department of Surgery, Cancer Center Amsterdam, Amsterdam UMC, Vrije Universiteit Amsterdam, Amsterdam, The Netherlands
| | - Jurriaan B Tuynman
- Department of Surgery, Cancer Center Amsterdam, Amsterdam UMC, Vrije Universiteit Amsterdam, Amsterdam, The Netherlands
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20
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Appendiceal neoplasms and histological involvement of the mesoappendix: A case series. Ann Med Surg (Lond) 2020; 56:64-67. [PMID: 32577233 PMCID: PMC7305355 DOI: 10.1016/j.amsu.2020.05.037] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/12/2020] [Revised: 05/19/2020] [Accepted: 05/19/2020] [Indexed: 11/22/2022] Open
Abstract
Introduction Acute appendicitis is a common presentation to surgical departments, typically resulting in appendicectomy. Appendiceal tumours may not be visible intraoperatively, and are present in roughly 0.5–1% of specimens. Routine resection of the mesoappendix is not universally practiced, despite the mesoappendix being commonly involved in appendiceal tumours. This is a case series of the histological findings of 21 patients with appendiceal tumours, with consideration to tumour within the resected mesoappendix. Methods We reviewed the histology of 1344 patients undergoing laparoscopic appendicectomy over a 6-year period assessing for the presence of appendiceal tumours and resected mesoappendix. Results Twenty-one patients were found to have appendiceal tumours, with a mean maximum tumour dimension of 7.2 mm. Sixteen of these patients had simultaneous mesoappendix resection, of whom six (38%) were found to have direct or indirect tumour tissue within the mesoappendix. Conclusion Further evidence for routine removal of the mesoappendix, and the need for larger prospective studies to investigate for any survival benefit. We note the worrying trend of conservative management of acute appendicitis.
The mesoappendix is a key site of appendiceal tumour spread, directly & indirectly. Routine mesoappendix resection may reduce the need for reoperation. Conservative management of appendicitis may delay diagnosis of appendiceal tumours.
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21
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The American Society of Colon and Rectal Surgeons, Clinical Practice Guidelines for the Management of Appendiceal Neoplasms. Dis Colon Rectum 2019; 62:1425-1438. [PMID: 31725580 DOI: 10.1097/dcr.0000000000001530] [Citation(s) in RCA: 84] [Impact Index Per Article: 14.0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/18/2022]
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22
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Zambrano-Vera K, Sardi A, Munoz-Zuluaga C, Studeman K, Nieroda C, Sittig M, King MC, Sipok A, Gushchin V. Outcomes in Peritoneal Carcinomatosis from Appendiceal Goblet Cell Carcinoma Treated with Cytoreductive Surgery and Hyperthermic Intraperitoneal Chemotherapy (CRS/HIPEC). Ann Surg Oncol 2019; 27:179-187. [PMID: 31646450 DOI: 10.1245/s10434-019-07932-5] [Citation(s) in RCA: 10] [Impact Index Per Article: 1.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/26/2019] [Indexed: 12/15/2022]
Abstract
BACKGROUND Appendiceal goblet cell adenocarcinoma (GCA) is often misclassified and mistreated due to mixed histologic features. In general, cytoreductive surgery plus hyperthermic intraperitoneal chemotherapy (CRS/HIPEC) is standard of care for peritoneal carcinomatosis (PC) from mucinous appendiceal tumors; however, in PC from GCA, data are limited and the role of CRS/HIPEC is controversial. We report outcomes in PC from appendiceal GCA treated with CRS/HIPEC. PATIENTS AND METHODS A prospective institutional database of 391 CRS/HIPEC patients with appendiceal carcinomatosis from 1998 to 2018 was reviewed. Twenty-seven patients with GCA were identified. Perioperative variables were described. Survival was estimated using the Kaplan-Meier method. RESULTS GCA occurred in 7% (27/391) of appendiceal CRS/HIPEC patients. Seven (26%) cases were aborted. Two patients underwent a second CRS/HIPEC for peritoneal recurrence. Median age at diagnosis was 53 years (range 39-72 years), and 12 (60%) were female. All underwent previous surgery. Seven (35%) had prior chemotherapy and received a median of 5 cycles (range 3-8). Median PCI was 6 (range 1-39). Complete cytoreduction was achieved in 95% (19/20). Grade III complications occurred in three (15%) patients, and no perioperative deaths occurred. Median follow-up was 97 months. Overall survival at 1, 3 and 5 years was 100%, 74% and 67%, respectively. Progression-free survival at 1, 3, and 5 years was 94%, 67% and 59%, respectively. CONCLUSION CRS/HIPEC should be considered as the main treatment option for patients with PC from appendiceal GCA. When performed at a CRS/HIPEC specialty center, 5-year OS of 67% can be achieved.
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Affiliation(s)
| | - Armando Sardi
- The Institute for Cancer Care, Mercy Medical Center, Baltimore, MD, USA.
| | | | | | - Carol Nieroda
- The Institute for Cancer Care, Mercy Medical Center, Baltimore, MD, USA
| | - Michelle Sittig
- The Institute for Cancer Care, Mercy Medical Center, Baltimore, MD, USA
| | - Mary Caitlin King
- The Institute for Cancer Care, Mercy Medical Center, Baltimore, MD, USA
| | - Arkadii Sipok
- The Institute for Cancer Care, Mercy Medical Center, Baltimore, MD, USA
| | - Vadim Gushchin
- The Institute for Cancer Care, Mercy Medical Center, Baltimore, MD, USA
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23
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Fields AC, Lu P, Enzinger A, Goldberg J, Irani J, Bleday R, Nash G, Melnitchouk N. Treatment patterns and outcomes in goblet cell carcinoid tumors of the appendix. J Surg Oncol 2019; 120:1096-1101. [PMID: 31592538 DOI: 10.1002/jso.25723] [Citation(s) in RCA: 9] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/19/2019] [Accepted: 09/21/2019] [Indexed: 01/16/2023]
Abstract
BACKGROUND Goblet cell carcinoid (GCC) tumors of the appendix are a rare malignancy. We aim to examine the overall survival per stage and the relationship between different treatment modalities and outcomes for patients with GCC tumors of the appendix. METHODS We identified patients with GCC tumors of the appendix from the National Cancer Database. The main outcome was overall patient survival and cox proportional hazard models were used to ascertain predictors of survival. RESULTS There were 2552 patients identified. The median age of diagnosis was 57 (interquartile range: 49-65) and 52.3% of patients were female. The 5-year survival for Stage I disease was 91.1% (95% confidence interval [CI]: 82.2%-95.7%), for Stage II disease was 90.5% (95% CI: 85.8%-93.7%), for Stage III disease was 57.0% (95% CI: 45.0%-67.3%), and for Stage IV disease was 18.9% (95% CI: 9.3%-31.0%). In a Cox proportional hazard model, older age (hazard ratio [HR]: 1.1; 95% CI: 1.03-1.12; P < .001), lymph node metastasis (HR: 6.9; 95% CI: 2.76-17.01; P < .001), and positive surgical margins (HR: 2.9; 95% CI:1.13-7.26; P = .003) were associated with worse overall survival for Stages I to III disease while only older age (HR: 1.03; 95% CI: 1.002-1.06; P = .04) was associated with worse overall survival for Stage IV disease. CONCLUSIONS Patients with GCC tumors of the appendix who have the nonmetastatic disease have a high 5-year survival. We have identified several prognostic factors for GCC.
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Affiliation(s)
- Adam C Fields
- Division of Colorectal Surgery, Department of Surgery, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts
| | - Pamela Lu
- Division of Colorectal Surgery, Department of Surgery, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts.,Department of Surgery, Center for Surgery and Public Health, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts
| | - Andrea Enzinger
- Center for Gastrointestinal Oncology, Dana Farber Cancer Center, Boston, Massachusetts
| | - Joel Goldberg
- Division of Colorectal Surgery, Department of Surgery, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts
| | - Jennifer Irani
- Division of Colorectal Surgery, Department of Surgery, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts
| | - Ronald Bleday
- Division of Colorectal Surgery, Department of Surgery, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts
| | - Garrett Nash
- Department of Surgery, Memorial Sloan Kettering Cancer Center, New York, New York
| | - Nelya Melnitchouk
- Division of Colorectal Surgery, Department of Surgery, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts.,Department of Surgery, Center for Surgery and Public Health, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts
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24
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Prieto-Nieto MI, Pastor D, Rodríguez-Cobos J, Pérez JP, Méndez C, Palacios E, Arranz-Alvarez M, Santos-López J, Cano-Vega M, Viñal D, Rodríguez N, Domínguez G. ΔNp73 status in peritoneal and ovarian dissemination of appendicular adenocarcinoids (goblet cells). Clin Transl Oncol 2019; 21:1432-1439. [PMID: 31025168 DOI: 10.1007/s12094-019-02091-1] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/29/2022]
Abstract
INTRODUCTION Goblet cell carcinoma (GCC) is an appendicular neoplasia representing less than 5% of all appendicular tumors, found in 0.3-0.9% of the appendectomies, 35-58% of all appendicular neoplasms, and less than 14% of malign appendix tumors. The most frequent clinical presentation is abdominal pain associated with a picture of acute appendicitis. MATERIALS AND METHODS We present 3 clinical cases of appendix GCC, 2 subjected to cytoreductory surgery plus intraperitoneal hyperthermic chemotherapy and a third, who is currently receiving neoadjuvant treatment with a good response to chemotherapy and who will be offered the same treatment as the first two patients. Given the unpredictable behavior of these tumors, the use of molecular markers could help us to predict their behavior and prognosis. In this context, the TP73 gene would make an interesting putative marker. ∆Np73 has been described as overexpressed in a great variety of tumor types including colon cancer and this up-regulation is associated with a poor prognosis. To evidence its role in this malignancy, we evaluate here the status of ∆Np73 in the primary tumor and normal counterpart tissues, in the metastatic implants and in healthy areas of the peritoneum from the appendicular GCC patients. In addition, we checked the expression levels of this p73 variant in the tumor and normal tissue of 26 patients with colon cancer. RESULTS Remarkably, 2 patients showed significant ∆Np73 down-regulation in both the primary tumor and the implants. Case 1 presented a fourfold decrease of levels in the primary tumor and 20-fold decrease in the implants. Case 2 showed a seven- and fourfold down-regulation in the primary tumor and implants, respectively. However, Case 3 showed an up-regulation of 53- and threefold in the primary tumor and implants, respectively. CONCLUSION Goblet cell carcinoma of the appendix is very rate. It tends to seed throughout the peritoneum, making aggressive surgical cytoreduction and chemotherapy viable treatment options. Investigation into the molecular basis of these tumors may improve the diagnosis, prognosis and therapeutic decisions regarding these patients. ∆Np73 seems a good candidate for further analysis in longer series.
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Affiliation(s)
- M I Prieto-Nieto
- Peritoneal Oncological Surgery Unit, Hospital La Paz, Madrid, Spain.
| | - D Pastor
- Peritoneal Oncological Surgery Unit, Hospital La Paz, Madrid, Spain
| | - J Rodríguez-Cobos
- Department of Medicine, Faculty of Medicine, Institute of Biomedical Research Alberto Sols, CSIC-UAM, Madrid, Spain
| | - J P Pérez
- Peritoneal Oncological Surgery Unit, Hospital La Paz, Madrid, Spain
| | - C Méndez
- Pathological Department, Hospital La Paz, Madrid, Spain
| | - E Palacios
- Pathological Department, Hospital La Paz, Madrid, Spain
| | - M Arranz-Alvarez
- Biobanco Idipaz, Hospital Universitario La Paz, Idipaz, Madrid, Spain
| | - J Santos-López
- Department of Medicine, Faculty of Medicine, Institute of Biomedical Research Alberto Sols, CSIC-UAM, Madrid, Spain
| | - M Cano-Vega
- Department of Medicine, Faculty of Medicine, Institute of Biomedical Research Alberto Sols, CSIC-UAM, Madrid, Spain
| | - D Viñal
- Medical Oncology Department, Hospital La Paz, Madrid, Spain
| | - N Rodríguez
- Medical Oncology Department, Hospital La Paz, Madrid, Spain
| | - G Domínguez
- Department of Medicine, Faculty of Medicine, Institute of Biomedical Research Alberto Sols, CSIC-UAM, Madrid, Spain.
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25
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Abstract
Goblet cell carcinoid (GCC) or goblet cell carcinoma is a unique mixed endocrine-exocrine neoplasm that is almost exclusively seen in the appendix. The hallmark of GCC is the concentric infiltration of the appendiceal wall by small tight clusters, nests or cords of tumor cells that exhibit a goblet cell morphology with a small compressed nucleus and conspicuous intracytoplasmic mucin. The coexistence of high-grade adenocarcinoma with GCC has been increasingly recognized as a common finding, which has been called adenocarcinoma ex GCC or mixed GCC-adenocarcinoma. A number of studies have shown that it is the high-grade adenocarcinomatous component that dictates the prognosis. Several histologic classification/grading systems have been proposed, which correlate with overall patient survival. Treatment options are primarily based on tumor stage and the presence or absence of a high-grade adenocarcinomatous component.
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26
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Shibuya H, Hijioka S, Mizuno N, Kuwahara T, Okuno N, Tanaka T, Ishihara M, Hirayama Y, Oonishi S, Murakami Y, Yatabe Y, Tajika M, Niwa Y, Hara K. A Rare Case of Ampullary Goblet Cell Carcinoid. Intern Med 2018; 57:2489-2496. [PMID: 29607953 PMCID: PMC6172535 DOI: 10.2169/internalmedicine.0516-17] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/06/2022] Open
Abstract
An asymptomatic 70-year-old woman was referred to our hospital because of liver enzyme elevation. Enhanced abdominal computed tomography demonstrated a small, round-shaped tumor with dilation of the common bile duct and main pancreatic duct. A biopsy specimen from the papilla showed mucin-containing cells that were positive for endocrine markers on immunohistochemical staining. Endoscopic snare resection was done, and there was a positive vertical margin on pathology. Pancreaticoduodenectomy was then performed later. The final diagnosis was goblet cell carcinoid, pT2N0M0, pStage IIA [Union for International Cancer Control (UICC) 7th edition]. Ampullary goblet cell carcinoid is an extremely rare disease of which there have been no recent reports.
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Affiliation(s)
- Hitoshi Shibuya
- Departments of Gastroenterology, Aichi Cancer Center Hospital, Japan
- Division of Gastroenterology and Hepatology, Department of Medicine, Nihon University School of Medicine, Japan
| | - Susumu Hijioka
- Departments of Gastroenterology, Aichi Cancer Center Hospital, Japan
- Department of Hepatobiliary and Pancreatic Oncology, National Cancer Center Hospital, Japan
| | - Nobumasa Mizuno
- Departments of Gastroenterology, Aichi Cancer Center Hospital, Japan
| | | | - Nozomi Okuno
- Departments of Gastroenterology, Aichi Cancer Center Hospital, Japan
| | - Tsutomu Tanaka
- Departments of Endoscopy, Aichi Cancer Center Hospital, Japan
| | - Makoto Ishihara
- Departments of Endoscopy, Aichi Cancer Center Hospital, Japan
| | - Yutaka Hirayama
- Departments of Endoscopy, Aichi Cancer Center Hospital, Japan
| | - Sachiyo Oonishi
- Departments of Endoscopy, Aichi Cancer Center Hospital, Japan
| | - Yoshiko Murakami
- Departments of Pathology and Molecular Diagnosis, Aichi Cancer Center Hospital, Japan
| | - Yasushi Yatabe
- Departments of Pathology and Molecular Diagnosis, Aichi Cancer Center Hospital, Japan
| | - Masahiro Tajika
- Departments of Endoscopy, Aichi Cancer Center Hospital, Japan
| | - Yasumasa Niwa
- Departments of Endoscopy, Aichi Cancer Center Hospital, Japan
| | - Kazuo Hara
- Departments of Gastroenterology, Aichi Cancer Center Hospital, Japan
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27
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Goretzki PE, Mogl MT, Akca A, Pratschke J. Curative and palliative surgery in patients with neuroendocrine tumors of the gastro-entero-pancreatic (GEP) tract. Rev Endocr Metab Disord 2018; 19:169-178. [PMID: 30280290 DOI: 10.1007/s11154-018-9469-9] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/17/2022]
Abstract
The incidence of neuroendocrine tumors (NET) increases with age. Lately, the diagnosis of NET was mainly caused by early detection of small NET (<1 cm) in the rectum and stomach, which are depicted by chance during routine and prophylactic endoscopy. Also in patients with large and metastatic pancreatic and intestinal tumors thorough pathologic investigation with use of different immunohistologic markers discovers more neuroendocrine tumors with low differentiation grade (G2-G3) and more neuroendocrine carcinomas (NEC), nowadays, than in former times. While gastric and rectal NET are discovered as small (<1 cm in diameter) and mainly highly differentiated tumors, demonstrating lymph node metastases in less than 10% of the patients, the majority of pancreatic and small bowel NET have already metastasized at the time of diagnosis. This is of clinical importance, since tumor stage and differentiation grade not only influence prognosis but also surgical procedure and may define whether a combination of surgery with systemic biologic therapy, chemotherapy or local cytoreductive procedures may be used. The indication for surgery and the preferred surgical procedure will have to consider personal risk factors of each patient (i.e. general health, additional illnesses, etc.) and tumor specific factors (i.e. tumor stage, grade of differentiation, functional activity, mass and variety of loco regional as well as distant metastases etc.). Together they define, whether radical curative or only palliative surgery can be applied. Altogether surgery is the only cure for locally advanced NET and helps to increase quality of life and overall survival in many patients with metastatic neuroendocrine tumors. The question of cure versus palliative therapy sometimes only can be answered with time, however. Many different aspects and various questions concerning the indication and extent of surgery and the best therapeutic procedure are still unanswered. Therefore, a close multidisciplinary cooperation of colleagues involved in biochemical and localization diagnostics and those active in various treatment areas is warranted to search for the optimal strategy in each individual patient. How far genetic screening impacts survival remains to be seen. Since surgeons do have a central role in the treatment of NET patients, they have to understand the need for integration into such an interdisciplinary team.
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Affiliation(s)
- Peter E Goretzki
- Department of Surgery, Campus Charité Mitte | Campus Virchow-Klinikum, Augustenburger Platz 1, 13353, Berlin, Germany.
- Leiter Arbeitsbereich endokrine Chirurgie, Chirurgische Klinik, Campus Charité Mitte | Campus Virchow-Klinikum, Charité - Universitätsmedizin Berlin, Augustenburger Platz 1, 13353, Berlin, Germany.
| | - Martina T Mogl
- Department of Surgery, Campus Charité Mitte | Campus Virchow-Klinikum, Augustenburger Platz 1, 13353, Berlin, Germany
| | - Aycan Akca
- Surgical Clinic 1, Lukaskrankenhaus Neuss, Preußenstrasse 84, 41456, Neuss, Germany
| | - Johann Pratschke
- Department of Surgery, Campus Charité Mitte | Campus Virchow-Klinikum, Augustenburger Platz 1, 13353, Berlin, Germany
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28
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Jesinghaus M, Konukiewitz B, Foersch S, Stenzinger A, Steiger K, Muckenhuber A, Groß C, Mollenhauer M, Roth W, Detlefsen S, Weichert W, Klöppel G, Pfarr N, Schlitter AM. Appendiceal goblet cell carcinoids and adenocarcinomas ex-goblet cell carcinoid are genetically distinct from primary colorectal-type adenocarcinoma of the appendix. Mod Pathol 2018; 31:829-839. [PMID: 29327707 DOI: 10.1038/modpathol.2017.184] [Citation(s) in RCA: 34] [Impact Index Per Article: 4.9] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/25/2017] [Revised: 10/24/2017] [Accepted: 11/08/2017] [Indexed: 12/23/2022]
Abstract
The appendix gives rise to goblet cell carcinoids, which represent special carcinomas with distinct biological and histological features. Their genetic background and molecular relationship to colorectal adenocarcinoma is largely unknown. We therefore performed a next-generation sequencing analysis of 25 appendiceal carcinomas including 11 goblet cell carcinoids, 7 adenocarcinomas ex-goblet cell carcinoid, and 7 primary colorectal-type adenocarcinomas, using a modified Colorectal Cancer specific Panel comprising 32 genes linked to colorectal and neuroendocrine tumorigenesis. The mutational profiles of these neoplasms were compared with those of conventional adenocarcinomas, mixed adenoneuroendocrine carcinomas, and neuroendocrine carcinomas of the colorectum. In addition, a large-scale pan-cancer sequencing panel covering 409 genes was applied to selected cases of goblet cell carcinoid/adenocarcinoma ex-goblet cell carcinoid (n=2, respectively). Mutations in colorectal cancer-related genes (eg, TP53, KRAS, APC) were rare to absent in both, goblet cell carcinoids and adenocarcinomas ex-goblet cell carcinoid, but frequent in primary colorectal-type adenocarcinomas of the appendix. Additional large-scale sequencing of selected goblet cell carcinoids and adenocarcinomas ex-goblet cell carcinoid revealed mutations in Wnt-signaling-associated genes (USP9X, NOTCH1, CTNNA1, CTNNB1, TRRAP). These data suggest that appendiceal goblet cell carcinoids and adenocarcinomas ex-goblet cell carcinoid constitute a morphomolecular entity, histologically and genetically distinct from appendiceal colorectal-type adenocarcinomas and its colorectal counterparts. Altered Wnt-signaling associated genes, apart from APC, may act as potential drivers of these neoplasms. The absence of KRAS/NRAS mutations might render some of these tumors eligible for anti-EGFR directed therapy regimens.
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Affiliation(s)
- Moritz Jesinghaus
- Institute of Pathology, Technical University of Munich, Munich, Germany.,German Cancer Consortium (DKTK), Partner Site Munich, Munich, Germany
| | - Björn Konukiewitz
- Institute of Pathology, Technical University of Munich, Munich, Germany
| | | | - Albrecht Stenzinger
- Institute of Pathology, University Hospital Heidelberg, Heidelberg, Germany.,National Center for Tumor Diseases (NCT), Heidelberg, Germany
| | - Katja Steiger
- Institute of Pathology, Technical University of Munich, Munich, Germany
| | - Alexander Muckenhuber
- Institute of Pathology, Technical University of Munich, Munich, Germany.,German Cancer Consortium (DKTK), Partner Site Munich, Munich, Germany
| | - Claudia Groß
- Institute of Pathology, Technical University of Munich, Munich, Germany
| | | | - Wilfried Roth
- Institute of Pathology, University Hospital Mainz, Mainz, Germany
| | - Sönke Detlefsen
- Department of Clinical Pathology, University Hospital Odense, Odense, Denmark
| | - Wilko Weichert
- Institute of Pathology, Technical University of Munich, Munich, Germany.,German Cancer Consortium (DKTK), Partner Site Munich, Munich, Germany
| | - Günter Klöppel
- Institute of Pathology, Technical University of Munich, Munich, Germany
| | - Nicole Pfarr
- Institute of Pathology, Technical University of Munich, Munich, Germany
| | - Anna Melissa Schlitter
- Institute of Pathology, Technical University of Munich, Munich, Germany.,German Cancer Consortium (DKTK), Partner Site Munich, Munich, Germany
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29
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Clift AK, Kornasiewicz O, Drymousis P, Faiz O, Wasan HS, Kinross JM, Cecil T, Frilling A. Goblet cell carcinomas of the appendix: rare but aggressive neoplasms with challenging management. Endocr Connect 2018; 7:268-277. [PMID: 29335251 PMCID: PMC5801558 DOI: 10.1530/ec-17-0311] [Citation(s) in RCA: 22] [Impact Index Per Article: 3.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/10/2018] [Accepted: 01/15/2018] [Indexed: 12/17/2022]
Abstract
Goblet cell carcinomas (GCC) are a rare, aggressive sub-type of appendiceal tumours with neuroendocrine features, and controversy exists with regards to therapeutic strategy. We undertook a retrospective review of GCC patients surgically treated at two tertiary referral centres. Clinical and histopathological data were extracted from a prospectively maintained database. Survival analyses utilised Kaplan-Meier methodology. Twenty-one patients were identified (9 females). Median age at diagnosis was 55 years (range 32-77). There were 3, 6 and 9 grade 1, 2 and 3 tumours, respectively. One, 10, 5 and 5 patients had stage I, II, III and IV disease at diagnosis, respectively. There were 8, 10 and 3 Tang class A, B and C tumours, respectively. Index operation was appendectomy (n = 12), right hemicolectomy (n = 6) or resections including appendix/right colon, omentum and the gynaecological system (n = 3). Eight patients underwent completion right hemicolectomy. Surgery for recurrence included small bowel resection (n = 2), debulking with peritonectomy and heated intraperitoneal chemotherapy, and hysterectomy and bilateral salpingo-oophorectomy (all n = 1). Median follow-up was 30 months (range 2.5-123). One-, 3- and 5-year OS was 79.4, 60 and 60%, respectively. Mean OS (1-, 3-, and 5-year OS) for Tang class A, B and C tumours were 73.1 months (85.7, 85.7, 51.4%), 83.7 months (all 66.7%) and 28.5 months (66.7, 66.7%, not reached), respectively. Chromogranin A/B and 68Ga-DOTATATE PET/CT were not useful in follow-up, but CEA, CA 19-9, CA 125 and 18F-FDG PET/CT identified tumour recurrence. GCC must be clearly discriminated from relatively indolent appendiceal neuroendocrine neoplasms. 18F-FDG PET/CT and CEA/CA19-9/CA 125 are useful in detecting recurrence of GCC.
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Affiliation(s)
- Ashley K Clift
- Department of Surgery and CancerImperial College London, London, UK
| | - Oskar Kornasiewicz
- Department of Surgery and CancerImperial College London, London, UK
- Department of SurgeryWarsaw Medical University, Warsaw, Poland
| | | | - Omar Faiz
- Department of SurgerySt Mark's Hospital, London, UK
| | - Harpreet S Wasan
- Department of Surgery and CancerImperial College London, London, UK
| | - James M Kinross
- Department of Surgery and CancerImperial College London, London, UK
| | - Thomas Cecil
- Peritoneal Malignancy UnitBasingstoke and North Hampshire Hospital, Basingstoke, UK
| | - Andrea Frilling
- Department of Surgery and CancerImperial College London, London, UK
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Madsen AH, Ladekarl M, Villadsen GE, Grønbæk H, Sørensen MM, Stribolt K, Verwaal VJ, Iversen LH. Effects of Cytoreductive Surgery and Hyperthermic Intraperitoneal Chemotherapy (HIPEC) in the Treatment of Goblet Cell Carcinoma: A Prospective Cohort Study. Ann Surg Oncol 2017; 25:422-430. [DOI: 10.1245/s10434-017-6272-x] [Citation(s) in RCA: 20] [Impact Index Per Article: 2.5] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/06/2017] [Indexed: 12/12/2022]
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Maedler C, Arnason T, Dorreen A, Sapp H, Castonguay M, Murphy J, Selegean S, Huang WY. Goblet cell carcinoid of the appendix - An interobserver variability study using two proposed classification systems. Ann Diagn Pathol 2017; 32:51-55. [PMID: 29414399 DOI: 10.1016/j.anndiagpath.2017.11.001] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/20/2017] [Accepted: 11/04/2017] [Indexed: 10/18/2022]
Abstract
Goblet cell carcinoid (GCC) is an uncommon tumor of the vermiform appendix. Due to a broad spectrum of morphological differentiation, subclassification and grading of GCCs remains an area of controversy. Two separate systems have proposed classifying GCC tumors into three (classical GCC; adenocarcinoma ex-GCC, signet ring cell type; adenocarcinoma ex-GCC, poorly differentiated carcinoma type) OR two subgroups (low and high grade GCC) based on morphological criteria. We independently compared the inter-observer variability associated with each classification system. Overall, both systems had moderate interobserver agreement, with the two-tiered system (κ=0.54) performing slightly better than the three-tiered system (κ=0.42). GI-specialist pathologists had substantial agreement for both two and three-tiered systems (κ=0.65 vs. 0.65). Non-GI trained pathologists had lower overall agreement than GI trained pathologists, but their agreement was better using the two-tiered system (κ=0.44) than the three-tiered system (κ=0.22). A sub-analysis of 6 cases with a high rate of discordant classification revealed several challenges that exist in applying current criteria, including differentiating "goblet" vs. "signet ring" cell morphology, applying a 1 mm2 criteria to multifocal non-contiguous glandular and single infiltrating cell architecture, differentiating fibro-inflammatory stroma from desmoplastic stroma, and solid architecture in cases with abundant extracellular mucin, and distinguishing "reactive" nuclear atypia from true "cytologic atypia". Despite these challenges, the study identified better agreement among GI pathologists than non-GI trained pathologists. While GI pathologist review may be helpful, further research on objective classification criteria remains an area of interest.
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Affiliation(s)
- Chelsea Maedler
- Department of Pathology, Dalhousie University, Halifax, Nova Scotia, Canada
| | - Thomas Arnason
- Department of Pathology, Dalhousie University, Halifax, Nova Scotia, Canada
| | - Alastair Dorreen
- Department of Medicine, Dalhousie University, Halifax, Nova Scotia, Canada
| | - Heidi Sapp
- Department of Pathology, Dalhousie University, Halifax, Nova Scotia, Canada
| | - Mathieu Castonguay
- Department of Pathology, Dalhousie University, Halifax, Nova Scotia, Canada
| | - Joanne Murphy
- Department of Pathology, Dalhousie University, Halifax, Nova Scotia, Canada
| | - Sorin Selegean
- Department of Pathology, Dalhousie University, Halifax, Nova Scotia, Canada
| | - Weei-Yuarn Huang
- Department of Pathology, Dalhousie University, Halifax, Nova Scotia, Canada.
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Abstract
AIMS Neuroendocrine gastroenteropancreatic tumors are infrequently found neoplasms. Our objective was to analyze the survival rates for all sites that they occur in by studying different variables. MATERIALS AND METHODS A retrospective study was carried out using records for a 7-year period from January 1, 2008 to December 31, 2014 on neuroendocrine gastroenteropancreatic tumors patients diagnosed at the Pontevedra-Salnés Hospital Complex. The variables used were as follows: age at diagnosis, tumor size, presence or absence of metastases at diagnosis, cell proliferation index, Ki-67 of each tumor, treatments received, postdiagnosis survival time, existence or not of tumor progression, and time from diagnosis to progression and from diagnosis to mortality. In relation to treatments, the information recorded was whether the treatment was endoscopic, surgical, or pharmacological. RESULTS Ninety-three neuroendocrine tumors made up a ratio of 4.42 cases per 100,000 inhabitants per annum. The median patient follow-up time was 44 months. The overall 5-year survival rate for patients who were followed up for a minimum of 60 months (49 patients) was 65.3%. The progression-free survival was 75.6% for 41 patients who were followed up for a minimum of 60 months. The survival rate for patients receiving endoscopic treatment was 100%, as there was no patient mortality recorded for those treated by endoscopic resection during the follow-up period. CONCLUSION Pancreatic neuroendocrine tumors may be managed conservatively in elderly patients by either monitoring them with imaging studies or treating them with somatostatin analogs. In the case of digestive tract tumors (stomach, duodenum, and rectum) that meet the criteria for endoscopic resection, this is a reliable and safe technique in the long term.
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Clift AK, Frilling A. Neuroendocrine, goblet cell and mixed adeno-neuroendocrine tumours of the appendix: updates, clinical applications and the future. Expert Rev Gastroenterol Hepatol 2017; 11:237-247. [PMID: 28081662 DOI: 10.1080/17474124.2017.1282314] [Citation(s) in RCA: 9] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/04/2023]
Abstract
Appendiceal neuroendocrine neoplasms are rare, clinically challenging tumours that are typically incidentally diagnosed, have a poorly understood biology and have controversy surrounding their management. Most are adequately treated with appendectomy, and although distant metastases are rare, the threat of disease dissemination remains and current guidelines possess poor accuracy in terms of selecting patients requiring more extensive surgery, i.e. oncological right-hemicolectomy. Areas covered: In this article, we discuss the presentation and diagnostic work-up of patients with appendiceal neuroendocrine neoplasms, and also examine the evidence base for existing management strategies. We highlight controversies within the management of these tumours, and anticipate avenues for further progress. Although no longer classified as neuroendocrine neoplasms, we also discuss two related forms of tumours with neuroendocrine features - goblet cell cancers and mixed adeno-neuroendocrine carcinomas. Expert commentary: Existing guidelines for the treatment of appendiceal neuroendocrine neoplasms are derived from a limited evidence base and are unable to accurately predict which patients require extensive attempts at surgical disease control. Future advances in the field of improved patient selection for more extensive surgery may be possible with multi-factorial tumour assessment integrating morphological and molecular analyses.
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Affiliation(s)
- Ashley K Clift
- a Department of Surgery and Cancer , Imperial College London , London , UK
| | - Andrea Frilling
- a Department of Surgery and Cancer , Imperial College London , London , UK
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Koganti SB, Gilchrist BF, Bell TC. Perforated Goblet Cell Carcinoid Tumors of the Appendix: Navigating the Management Conundrum. Int Surg 2017; 102:131-136. [DOI: 10.9738/intsurg-d-17-00071.1] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/17/2022] Open
Abstract
Appendix is the most common site of occurrence for a goblet cell carcinoid tumor. A diagnosis of an appendiceal goblet cell carcinoid is made in retrospect the majority of the time. These tumors are best treated with a right hemicolectomy and adjuvant therapies tailored according to the presence or absence of residual disease. Presentation as a perforated appendix is seen in 16% of these tumors. The natural history and the ideal management strategy in such a scenario are not well described. In those with peritoneal spread cytoreductive surgery with HIPEC (hyperthermic intraperitoneal chemotherapy) offers the best disease-free and progression-free survival. Close follow-up with cross-sectional imaging helps in identifying recurrences at the earliest. Multimodality management involving patient participation in every aspect of care accomplishes high-value care in the treatment of these tumors.
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Affiliation(s)
- Suman B. Koganti
- Department of General Surgery, Bronx-Lebanon Hospital Center, Icahn School of Medicine at Mount Sinai Hospital, New York, New York
| | - Brian F. Gilchrist
- Department of General Surgery, Bronx-Lebanon Hospital Center, Icahn School of Medicine at Mount Sinai Hospital, New York, New York
| | - Tresara C. Bell
- Department of General Surgery, Bronx-Lebanon Hospital Center, Icahn School of Medicine at Mount Sinai Hospital, New York, New York
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Shenoy S. Goblet cell carcinoids of the appendix: Tumor biology, mutations and management strategies. World J Gastrointest Surg 2016; 8:660-669. [PMID: 27830037 PMCID: PMC5081547 DOI: 10.4240/wjgs.v8.i10.660] [Citation(s) in RCA: 25] [Impact Index Per Article: 2.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/17/2016] [Revised: 08/14/2016] [Accepted: 08/27/2016] [Indexed: 02/06/2023] Open
Abstract
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.
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Lamarca A, Nonaka D, Lopez Escola C, Hubner RA, O'Dwyer S, Chakrabarty B, Fulford P, Valle JW. Appendiceal Goblet Cell Carcinoids: Management Considerations from a Reference Peritoneal Tumour Service Centre and ENETS Centre of Excellence. Neuroendocrinology 2016; 103:500-17. [PMID: 26356507 DOI: 10.1159/000440725] [Citation(s) in RCA: 35] [Impact Index Per Article: 3.9] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/02/2015] [Accepted: 08/28/2015] [Indexed: 12/24/2022]
Abstract
BACKGROUND Appendix goblet cell carcinoids are known to share histological features of adenocarcinoma and neuroendocrine tumours. Due to their low incidence, quality evidence is lacking for the management of these patients. METHODS We performed a single-centre retrospective study of patients with a confirmed diagnosis of appendiceal goblet cell carcinoid (GCC; 1996-2014). Patients were divided into curative intent (CI) and palliative intent (PI) cohorts. Our primary end point was overall survival (OS). RESULTS Seventy-four patients were eligible; 76% were treated with CI [surgery only (36%), cytoreductive surgery (CRS) and hyperthermic intra-peritoneal chemotherapy (HIPEC; 36%), adjuvant chemotherapy (20%) and a combination of CRS and HIPEC followed by adjuvant chemotherapy (9%)], and 23% had advanced-stage disease amenable to palliative treatment (chemotherapy or supportive care) only. Completion right hemicolectomy, performed in 64% of the CI cohort, did not impact on the relapse rate or disease-free survival. FOLFOX chemotherapy was used in both the adjuvant and palliative settings; safety was as expected, and we observed a high rate (60%) of disease control in the palliative cohort. The estimated median OS (all patients), disease-free survival (CI patients) and progression-free survival (PI patients) were 52.1 (95% CI 29.4-90.3), 75.9 (26.6-not reached) and 5.3 (0.6-5.7) months, respectively. Age and stage were independent factors associated with OS in the multivariable analysis. Tang classification showed a trend for impact on OS. No benefit from specific adjuvant approach was identified; however, selection bias for treatment approach was observed. CONCLUSION Prospective trials are needed to define optimal approaches in GCC. All GCC patients should be managed by specialized centres due to their esoteric behaviour; we provide management considerations based on our experience and conclusions.
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