Goblet Cell Carcinoid Tumor, Mixed Goblet Cell Carcinoid-Adenocarcinoma, and Adenocarcinoma of the Appendix: Comparison of Clinicopathologic Features and Prognosis

Taggart, Melissa W.; Abraham, Susan C.; Overman, Michael J.; Mansfield, Paul F.; Rashid, Asif

doi:10.5858/arpa.2013-0047-oa

Cited by 84 publications

(98 citation statements)

References 37 publications

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“…Unlike classic midgut carcinoids, serum chromogranin A levels are normal and have no value in detecting and monitoring GCC. 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%

“…In patients who fulfill all the following criteria: Tumor less than 2 cm localized to appendix with negative margins, pT1 or pT2 tumors, and typical GCC histology group A (Tang et al[8] classification) tumors, an appendectomy alone may be sufficient as the definitive treatment[13]. Right hemicolectomy is recommended in tumors greater than two centimeters, locally advanced, positive margins, T3, T4 tumors and histology suggestive of group B, group C (Tangs classification) in the appendectomy specimens[8,11,12,55]. …”

Section: Treatmentmentioning

confidence: 99%

“…Since metastatic GCC shows clinical and histological resemblance to colorectal adenocarcinoma and not metastatic carcinoids the choice of adjuvant therapy in GCC is similar to colorectal adenocarcinoma. 5-fluorouracil (5-FU) and leucovorin based FOLFOX (5-FU, leucovorin, oxaliplatin) and FOLFIRI (5-FU, folic acid, irinotecan) chemotherapy are standard regimens recommended[11,30]. …”

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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Section: Immunochemistry and Mutational Findingsmentioning

confidence: 99%

Section: Treatmentmentioning

confidence: 99%

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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“…They usually involve both adenoma and well-differentiated neuroendocrine components, explaining the usually intermediate or low grade of malignancy. Most appendicular MiNEN are discovered at the systematic pathological examination of resected surgical specimens in patients operated on for an acute appendicitis, although 20-30% are identified incidentally [57][58][59] . Their prognosis is much worse than that of "pure" appendicular well-differentiated NEN and rather close to that of adenocarcinoma, depending on the rate of adenocarcinoma component and the presence of signet-ring adenocarcinoma [56][57][58][59][60][61] .…”

Section: Small Intestinementioning

confidence: 99%

Digestive System Mixed Neuroendocrine-Non-Neuroendocrine Neoplasms

Cros²,

et al. 2017

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Mixed neuroendocrine-non-neuroendocrine neoplasms (MiNEN) are a heterogeneous subgroup of rare neoplasms that represent about a third of all poorly differentiated neuroendocrine carcinomas (PDNEC). MiNEN combine a neuroendocrine component, usually a PDNEC, and a non-neuroendocrine component, generally an adenocarcinoma, both accounting for at least 30% of the neoplasm. MiNEN are classified as high-, intermediate-, or low-grade malignancies depending on the metastatic potential of the tumour components. High-grade malignant component should be considered even if it represents <30% of the tumour. The prognosis of MiNEN is generally intermediate between those of the two “pure” components composing it. The aim of this comprehensive review of the literature is to suggest a standardized management of MiNEN. An increasing body of evidence suggests that PDNEC components share molecular abnormalities with their adenocarcinoma counterparts, but also display additional alterations. This advocates for a common origin, and that the presence of a PDNEC component in an adenocarcinoma could indicate a turning point in carcinogenesis.

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“…Adjuvant chemotherapy -qualification according to the same principles as in cancer of the large intestine: FOLFOX is recommended in stage III (pT3, pT4, N+), although there are no reports from randomised studies, only scientific reports from individual centres [5,106].…”

Section: Goblet Cell Carcinoma (Gcc)mentioning

confidence: 99%

Nowotwory neuroendokrynne jelita cienkiego i wyrostka robaczkowego — zasady postępowania (rekomendowane przez Polską Sieć Guzów Neuroendokrynnych)

Bednarczuk¹,

Bolanowski²,

Zemczak³

et al. 2017

Endokrynologia Polska

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 224Neuroendocrine neoplasms of the small intestine and appendix -management guidelines Bednarczuk Tomasz et al. SZKOLENIE PODYPLOMOWE AbstractThis study presents the revised Polish guidelines regarding the management of patients suffering from neuroendocrine neoplasms (NENs) of the small intestine and appendix. The small intestine, especially the ileum, is the most common location for these neoplasms. Most are well differentiated and slow growing. Their symptoms may be atypical, which can result in delayed or accidental diagnosis. Appendicitis is usually the first manifestation of NEN in this location. Typical symptoms of carcinoid syndrome occur in approximately 20-30% of patients suffering from small intestinal NENs with distant metastases. The main cause of death in patients with carcinoid syndrome is carcinoid heart disease.The most useful laboratory test is the determination of chromogranin A, while concentration of 5-hydroxyindoleacetic acid is helpful in the diagnostics of carcinoid syndrome. For visualisation, ultrasound, computed tomography, magnetic resonance imaging, colonoscopy, video capsule endoscopy, double-balloon enteroscopy, and somatostatin receptor scintigraphy may be used. A detailed histological report is crucial for the proper diagnostics and therapy of NENs of the small intestine and appendix. The treatment of choice is surgical management, either radical or palliative. The pharmacological treatment of the hormonally active and non-active small intestinal NENs as well as NENs of the appendix is based on long-acting somatostatin analogues. In patients with generalised NENs of the small intestine in progress during the SSA treatment, with good expression of somatostatin receptors, the first-line treatment should be radioisotope therapy, while targeted therapies, such as everolimus, should be considered afterwards. When the above therapies are exhausted, in certain cases chemotherapy may be considered.(Endokrynol Pol 2017; 68 (2): 223-236)

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Goblet Cell Carcinoid Tumor, Mixed Goblet Cell Carcinoid-Adenocarcinoma, and Adenocarcinoma of the Appendix: Comparison of Clinicopathologic Features and Prognosis

Cited by 84 publications

References 37 publications

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

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

Digestive System Mixed Neuroendocrine-Non-Neuroendocrine Neoplasms

Nowotwory neuroendokrynne jelita cienkiego i wyrostka robaczkowego — zasady postępowania (rekomendowane przez Polską Sieć Guzów Neuroendokrynnych)

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