Goblet cell appendiceal tumors – Management dilemmas and long-term outcomes

Rossi, Roberta; Luong, Tu Vinh; Caplin, Martyn; Thirlwell, Christina; Meyer, Tim; García‐Hernández, Jorge; Baneke, Alex; Conte, Dario; Toumpanakis, Christos

doi:10.1016/j.suronc.2015.01.001

Cited by 48 publications

(39 citation statements)

References 40 publications

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“…Right hemicolectomy has been suggested by some clinicians as the standard approach for all GCC patients after resection of the primary tumour, in order to reduce the rate of relapse [45]. However, our data were unable to demonstrate a clear benefit of performing right hemicolectomy for all patients with GCC.…”

Section: Discussioncontrasting

confidence: 71%

Appendiceal Goblet Cell Carcinoids: Management Considerations from a Reference Peritoneal Tumour Service Centre and ENETS Centre of Excellence

Lamarca¹,

Nonaka²,

Escola³

et al. 2015

Neuroendocrinology

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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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Section: Discussioncontrasting

confidence: 71%

Appendiceal Goblet Cell Carcinoids: Management Considerations from a Reference Peritoneal Tumour Service Centre and ENETS Centre of Excellence

Lamarca¹,

Nonaka²,

Escola³

et al. 2015

Neuroendocrinology

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“…GCC are diagnosed in less than 1% of appendectomy specimens[7,8]. Most commonly, patients present with abdominal pain and acute appendicitis (> 50%).…”

Section: Clinical Presentationmentioning

confidence: 99%

“…About 27% of patients may present with perforated appendicitis[9]. Patients may also present sub-acutely in advanced stages with vague abdominal pain and mass[7,8]. Common in Caucasians, there is equal distribution in male and females with the average age of diagnosis in the fifth decade[1,2].…”

Section: Clinical Presentationmentioning

confidence: 99%

“…Somatostatin expression is sparse and erratic and therefore functional scans such as 111-Indium pentetreotide scintigraphy (Octreoscan) and Gallium 68-octreotide positron emission tomography (PET) scans are usually normal in patients with GCC, and thus are of limited use[11,19]. Fluorodeoxyglucose PET scan may be useful in advanced disease to detect peritoneal metastatic disease[7,27,30]. …”

Section: Immunochemistry and Mutational Findingsmentioning

confidence: 99%

“…This could be of benefit in differentiating the origin of these tumors in females with primary ovarian mucinous malignancy[58]. In postmenopausal female patients with GCC prophylactic bilateral oophorectomy, although not evidence based should be considered[7,8,12]. In female patients with mucinous ovarian and pelvic malignancies an appendectomy should always be performed in staging laparotomy as these may represent metastatic GCC[12,19,57].…”

Section: Treatmentmentioning

confidence: 99%

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Goblet cell carcinoids of the appendix: Tumor biology, mutations and management strategies

Shenoy¹

2016

WJGS

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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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