Incidence and Survival of Appendiceal Mucinous Neoplasms

Shaib, Walid Labib; Goodman, Michael; Chen, Zhengjia; Kim, Sungjin; Brutcher, Edith; Bekaii‐Saab, Tanios; El‐Rayes, Bassel F.

doi:10.1097/coc.0000000000000210

Cited by 53 publications

(46 citation statements)

References 26 publications

(30 reference statements)

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“…The incidence of AMN in the U.S. has increased over time, from 0.6 cases per million persons in 1973 [1] to 2.8 cases per million persons in 2011, with an annual percentage increase of 3.1%. In addition, age at diagnosis has decreased over the same time period . A similar pattern of increase in incidence and decrease in age at diagnosis was observed in a large registry study from the Netherlands spanning 1980–2010 .…”

Section: Epidemiology and Characteristicssupporting

confidence: 70%

Appendiceal Mucinous Neoplasms: Diagnosis and Management

et al. 2017

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Objective Appendiceal mucinous neoplasms (AMNs) are a rare and heterogeneous disease for which clinical management is challenging. We aim to review the literature regarding modalities of treatment to guide the management of AMNs. Methods and Review Criteria We conducted a PubMed search in February 2016 for English‐language publications, using the terms “appendiceal,” “appendix,” “carcinoma,” “cancer,” “mucinous,” “treatment,” “genes,” “target,” “genomic,” and terms listed in the articles' subheadings. Published reports and abstracts from the American Society of Clinical Oncology meetings were also searched. Results In this review, we summarize current data and controversies in AMN classification, clinical presentation, molecular alterations, treatment outcomes with regard to cytoreductive surgery, hyperthermic intraperitoneal chemotherapy (HIPEC), and the role of systemic chemotherapy. Conclusion Appendiceal mucinous neoplasms are a heterogeneous group of tumors with a rising incidence. Treatment is based on stage and histology. Low‐grade tumors are treated surgically with resection of the primary site in early stage disease, or peritoneal debulking and HIPEC in patients with advanced stage disease. Treatment of high‐grade tumors requires further prospective trials, and options include debulking surgery and HIPEC with or without preoperative chemotherapy. Trials evaluating novel therapies based on the molecular profiling of AMN tumors are needed to evaluate therapeutic options in patients who are not surgical candidates. Implications for Practice This review provides a reference to guide gastroenterologists, pathologists, surgeons, and oncologists in the management of appendiceal mucinous neoplasms (AMNs), a rare and heterogeneous disease with no consensus on histologic classification or guidelines for treatment algorithms. This review summarizes all AMN classifications and proposes a treatment algorithm based on stage and histology of disease.

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Section: Epidemiology and Characteristicssupporting

confidence: 70%

Appendiceal Mucinous Neoplasms: Diagnosis and Management

et al. 2017

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“…4,5 Appendiceal cancer is most often diagnosed at late stages, with the majority of patients presenting with distant disease. 3 Another issue raised is the increased risk of morbidity and mortality in cases of elderly patients who undergo operative treatment for acute appendicitis. This is certainly important to consider, as the elderly population is also the population at increased risk of malignancy.…”

Section: Shared Decision-making and Nonoperative Management Of Appendmentioning

confidence: 99%

“…While the age range of acute appendicitis in adults tends to peak between 20 and 40 years of age, the median age of diagnosis for appendiceal cancer is reported to be late 50s to early 60s . However, recent studies have demonstrated that the rates of appendiceal cancer have not only increased over time but that the age of diagnosis has significantly decreased as well . Therefore, while cancer risk may be lower in those under age 50 relative to an older population, there is still risk of appendiceal cancer and we must be vigilant and fully discuss risks and benefits of nonoperative management with these patients.…”

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confidence: 99%

“…To this point, is important to note that while the incidence of malignancy found in appendectomy specimens is approximately 1% overall, up to 39% of appendiceal cancer cases have been diagnosed as a result of surgery performed for a preoperative diagnosis of acute appendicitis; further, there may be low detection with colonoscopy after nonoperative management as fewer than 2% of cases have been reported to be identified on screening colonoscopies . Appendiceal cancer is most often diagnosed at late stages, with the majority of patients presenting with distant disease …”

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Shared decision‐making and nonoperative management of appendicitis

McCarty

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2019

Journal of Surgical Oncology

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“…The main clinical and pathological prognostic factors for PMP developing are stage at diagnosis and pathologic features of primary tumor [101]. Data from the National Cancer Institute's Surveillance Epidemiology and End Results (SEER) showed that the overall incidence of PMP and disease diagnosis in younger patients increased and survival improved from 1973 to 2006 mainly because patients with appendiceal mucinous tumors with advanced stage disease underwent CRS + HIPEC [102]. The finding that three-quarters of the patients with appendiceal mucinous tumors presented with symptoms of acute appendicitis or right iliac fossa pain suggests that many attend general surgical services.…”

Section: Proactive Management Of Peritoneal Metastases From Appendicementioning

confidence: 99%

Proactive Management for Gastric, Colorectal and Appendiceal Malignancies: Preventing Peritoneal Metastases with Hyperthermic Intraperitoneal Chemotherapy (HIPEC)

Sammartino

Biacchi

Cornali

et al. 2016

Indian J Surg Oncol

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An integrated treatment strategy using peritonectomy procedures plus hyperthermic intraperitoneal chemotherapy (HIPEC) is now a clinical standard of care in selected patients with peritoneal metastases and primary peritoneal tumors. This comprehensive approach can offer many patients, who hitherto had no hope of cure, a good quality of life and survival despite limited morbidity. The increasingly successful results and chance of interfering in the natural history of disease has prompted research to develop for some clinical conditions a therapeutic strategy designed to prevent malignant peritoneal dissemination before it becomes clinically evident and treat it microscopically (tertiary prevention). The main factor governing successful cytoreductive surgery and predicting outcome is the extent of peritoneal spread assessed with the peritoneal cancer index (PCI). In peritoneal metastases from colorectal and gastric cancer the PCI score acquires a specific role acting as the cut-off between patients who can undergo curative surgery or palliation. Long-term results show that the only group enjoying favorable results are patients with limited disease (a statistical minority). By applying to appropriately selected patients with primary malignancies a proactive management strategy including HIPEC we can treat patients with microscopic peritoneal dissemination and therefore at PCI 0. Among treated conditions pseudomyxoma peritonei enjoys the best results. But a major future advance comes from identifying among lesions at major risk of pseudomyxoma.

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Incidence and Survival of Appendiceal Mucinous Neoplasms

Abstract: There has been an increase in the overall incidence of AMN with an observed increase in the proportion of younger age and distant stage at diagnosis. The OS has improved over time.

Cited by 53 publications

References 26 publications

Appendiceal Mucinous Neoplasms: Diagnosis and Management

Appendiceal Mucinous Neoplasms: Diagnosis and Management

Shared decision‐making and nonoperative management of appendicitis

Proactive Management for Gastric, Colorectal and Appendiceal Malignancies: Preventing Peritoneal Metastases with Hyperthermic Intraperitoneal Chemotherapy (HIPEC)

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