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The American Joint Committee on Cancer (AJCC) staging system for all cancer sites, including gastroenteropancreatic neuroendocrine tumors (GEP‐NETs), is meant to be dynamic, requiring periodic updates to optimize AJCC staging definitions. This entails the collaboration of experts charged with evaluating new evidence that supports changes to each staging system. GEP‐NETs are the second most prevalent neoplasm of gastrointestinal origin after colorectal cancer. Since publication of the AJCC eighth edition, the World Health Organization has updated the classification and separates grade 3 GEP‐NETs from poorly differentiated neuroendocrine carcinoma. In addition, because of major advancements in diagnostic and therapeutic technologies for GEP‐NETs, AJCC version 9 advocates against the use of serum chromogranin A for the diagnosis and monitoring of GEP‐NETs. Furthermore, AJCC version 9 recognizes the increasing role of endoscopy and endoscopic resection in the diagnosis and management of NETs, particularly in the stomach, duodenum, and colorectum. Finally, T1NXM0 has been added to stage I in these disease sites as well as in the appendix. [ABSTRACT FROM AUTHOR]

INTRODUCTION: The American Joint Committee on Cancer (AJCC) staging system undergoes periodic revisions to maintain contemporary survival outcomes related to stage. Recently, the AJCC has developed a novel, systematic approach incorporating survival data to refine stage groupings. The objective of this study was to demonstrate data‐driven optimization of the version 9 AJCC staging system for anal cancer assessed through a defined validation approach. METHODS: The National Cancer Database was queried for patients diagnosed with anal cancer in 2012 through 2017. Kaplan–Meier methods analyzed 5‐year survival by individual clinical T category, N category, M category, and overall stage. Cox proportional hazards models validated overall survival of the revised TNM stage groupings. RESULTS: Overall, 24,328 cases of anal cancer were included. Evaluation of the 8th edition AJCC stage groups demonstrated a lack of hierarchical prognostic order. Survival at 5 years for stage I was 84.4%, 77.4% for stage IIA, and 63.7% for stage IIB; however, stage IIIA disease demonstrated a 73.0% survival, followed by 58.4% for stage IIIB, 59.9% for stage IIIC, and 22.5% for stage IV (p <.001). Thus, stage IIB was redefined as T1‐2N1M0, whereas Stage IIIA was redefined as T3N0‐1M0. Reevaluation of 5‐year survival based on data‐informed stage groupings now demonstrates hierarchical prognostic order and validated via Cox proportional hazards models. CONCLUSION: The 8th edition AJCC survival data demonstrated a lack of hierarchical prognostic order and informed revised stage groupings in the version 9 AJCC staging system for anal cancer. Thus, a validated data‐driven optimization approach can be implemented for staging revisions across all disease sites moving forward. Evaluation of the 8th edition American Joint Committee on Cancer staging system demonstrated a lack of hierarchical prognostic order for anal cancer. Thus, the version 9 American Joint Committee on Cancer staging system for anal cancer was revised through a validated and data‐driven approach and should similarly be applied across all disease sites moving forward. [ABSTRACT FROM AUTHOR]

The standard for cancer staging in the United States for all cancer sites, including primary carcinomas of the appendix, is the American Joint Committee on Cancer (AJCC) staging system. AJCC staging criteria undergo periodic revisions, led by a panel of site‐specific experts, to maintain contemporary staging definitions through the evaluation of new evidence. Since its last revision, the AJCC has restructured its processes to include prospectively collected data because large data sets have become increasingly robust and available over time. Thus survival analyses using AJCC eighth edition staging criteria were used to inform stage group revisions in the version 9 AJCC staging system, including appendiceal cancer. Although the current AJCC staging definitions were maintained for appendiceal cancer, incorporating survival analysis into the version 9 staging system provided unique insight into the clinical challenges in staging rare malignancies. This article highlights the critical clinical components of the now published version 9 AJCC staging system for appendix cancer, which (1) justified the separation of three different histologies (non‐mucinous, mucinous, signet‐ring cell) in terms of prognostic variance, (2) demonstrated the clinical implications and challenges in staging heterogeneous and rare tumors, and (3) emphasized the influence of data limitations on survival analysis for low‐grade appendiceal mucinous neoplasms. [ABSTRACT FROM AUTHOR]

The American Joint Committee on Cancer (AJCC) staging system for all cancer sites, including anal cancer, is the standard for cancer staging in the United States. The AJCC staging criteria are dynamic, and periodic updates are conducted to optimize AJCC staging definitions through a panel of experts charged with evaluating new evidence to implement changes. With greater availability of large data sets, the AJCC has since restructured and updated its processes, incorporating prospectively collected data to validate stage group revisions in the version 9 AJCC staging system, including anal cancer. Survival analysis using AJCC eighth edition staging guidelines revealed a lack of hierarchical order in which stage IIIA anal cancer was associated with a better prognosis than stage IIB disease, suggesting that, for anal cancer, tumor (T) category has a greater effect on survival than lymph node (N) category. Accordingly, version 9 stage groups have been appropriately adjusted to reflect contemporary long‐term outcomes. This article highlights the changes to the now published AJCC staging system for anal cancer, which: (1) redefined stage IIB as T1–T2N1M0 disease, (2) redefined stage IIIA as T3N0–N1M0 disease, and (3) eliminated stage 0 disease from its guidelines altogether. [ABSTRACT FROM AUTHOR]

Background: Although the lifetime risk of melanoma is disproportionately higher in whites, blacks have a poorer overall survival with an absolute survival difference of 25%. Significant progress has been made in melanoma treatment in the past decade; however, these successes may not be available or accessible to all segments of the population. Methods: In this review, we highlight important studies in melanoma as well as informative retrospective studies from databases and nonmelanoma cancers where appropriate. Results: There are no level I evidence-based studies on disparities in melanoma, and most likely there will never be, but the studies presented herein and clinical experience demonstrate that disparities in clinical outcomes from melanoma exists. Conclusions: By becoming aware of the disparities, we can help mitigate them by engagement, education, and corrective and empowering actions through awareness campaigns, appropriate clinical trial design, encouraging participation in clinical trials, increasing the diversity of providers, and advocacy. [ABSTRACT FROM AUTHOR]

Background: Development of distant metastases (DM) is associated with markedly decreased survival in parathyroid carcinoma (PC). We sought to identify factors associated with development of DM and to quantify the effect that development of DM had on overall survival (OS). Methods: Patients with surgically resected local/regional PC treated or surveilled at a tertiary-referral cancer hospital from 1980 to 2017 were included. We assessed the association between biochemical and clinicopathologic factors (preoperative parathyroid hormone (PTH) levels, sex, race, age, preoperative serum calcium levels, serum calcium levels at 6 months postop, tumor size, and extent of resection) with the development of DM. We also assessed the effect of development of DM on OS. Results: Seventy-five patients with PC were assessed; 17 (22.7%) developed DM at a median follow-up of 77 months. The cumulative incidence of DM in the cohort was 20, 30, and 38% at 5, 10, and 20 years respectively. Tumor size > 3.2 cm based on recursive partitioning analysis was the only significant predictor for development of DM (hazard ratio (HR) = 3.51; 95% confidence interval [CI] 1.04–11.91; p = 0.04). Median OS for the entire cohort was 17 years compared with 40 months for the cohort who developed DM. The HR for death after distant metastasis was 9.6 (95% CI 4.2–22.3; p < 0.0001). Conclusions: Development of distant metastasis during surveillance is associated with decreased OS, including late recurrences. Primary tumor size should be considered in future interval surveillance and development of treatment algorithms. [ABSTRACT FROM AUTHOR]

Background: Adenocarcinomas of the appendix represent a heterogeneous disease depending on the presence of mucinous histology, histologic grade, and stage. In the current study, the authors sought to explore the interplay of these factors with systemic chemotherapy in a large population data set.Methods: Patients in the National Cancer Data Base (NCDB) who were diagnosed with mucinous, nonmucinous, and signet ring cell-type appendiceal neoplasms from 1985 through 2006 were selected. Multivariable Cox proportional hazards regression models were developed.Results: A total of 11,871 patients met the inclusion criteria for the current study: 50.3% had mucinous neoplasms, 40.5% had nonmucinous neoplasms, and 9.2% had signet ring cell-type neoplasms. The 5-year overall survival (OS) stratified by grade was similar among patients with American Joint Committee on Cancer stage I to stage III disease but not for those with stage IV disease. The median OS for patients with stage IV mucinous and nonmucinous tumors was 6.4 years and 2.3 years, respectively, for those with well differentiated histology (P<.0001) and was 1.5 years and 0.8 years, respectively, for those with poorly differentiated histology (P<.0001). In multivariable modeling for stage I to III disease, adjuvant chemotherapy improved OS for both mucinous and nonmucinous histologies, with hazard ratios (HRs) of 0.78 (95% confidence interval [95% CI], 0.68-0.89 [P = .0002]) and 0.83 (95% CI, 0.74-0.94 [P = .002]), respectively. For patients with stage IV disease, systemic chemotherapy significantly improved OS for those with nonmucinous (HR, 0.72; 95% CI, 0.64-0.82 [P<.0001]) but not mucinous (HR, 0.95; 95% CI, 0.86-1.04 [P = .2) histologies, although this was grade-dependent. The median OS for chemotherapy versus no chemotherapy was 6.4 years versus 6.5 years (P value not significant) for patients with mucinous, well-differentiated tumors and 1.6 years versus 1.0 years (P = .0007) for patients with mucinous, poorly differentiated tumors.Conclusions: Adjuvant chemotherapy demonstrated a significant OS benefit regardless of histology. However, for patients with stage IV disease, the benefit of systemic chemotherapy varied by tumor histology and grade, with patients with well-differentiated, mucinous, appendiceal adenocarcinomas deriving no survival benefit from systemic chemotherapy. Cancer 2016;122:213-221. © 2015 American Cancer Society. [ABSTRACT FROM AUTHOR]

Background: Parathyroid carcinoma is a rare disease. Conflicting results on prognostic factors and extent of surgical resection for patients with parathyroid carcinoma have been made based on small sample sizes. A large, robust dataset is needed to help address some of the controversies. Methods: A retrospective review of patients with parathyroid carcinoma in the National Cancer Data Base from 1985 to 2006 was performed. Characteristics of the cohort and type of treatment were evaluated. Prognostic factors were assessed with Cox proportional hazards regression models and 5- and 10-year OS rates were determined. Results: There were 733 evaluable patients with a mean age of 56.1 ± 15.3 years (median 57, range 15-89) and mean tumor size of 29.6 ± 18.4 mm (median 25.0 mm, range 10.0-150.0). Tumor size, age at diagnosis, male sex, positive nodal status, and complete tumor resection had hazard ratios for death of 1.02 (1.01-1.02, p < 0.0001), 1.06 (1.05-1.07, p < 0.0001), 1.67 (1.24-2.25, p = 0.0008), 1.25 (0.57-2.76, p = 0.6), and 0.42 (0.22-0.81, p = 0.01), respectively, on multivariable analysis. Patients who had removal of the parathyroid tumor with concomitant resection of adjacent organs had HR for death of 0.70 (0.35-1.41, p = 0.3). The 5- and 10-year OS rates were 82.3 and 66 % respectively. Conclusions: Patient age, tumor size, and sex have modest effects on survival in patients with parathyroid carcinoma. A staging system with prognostic value for parathyroid carcinoma should include at least these pertinent prognostic factors. [ABSTRACT FROM AUTHOR]

The author discusses efforts of American Joint Committee on Cancer (AJCC) to improve the quality of cancer staging. Topics discussed include its collaboration with Union for International Cancer Control to provide unified anatomic staging system for cancer worldwide, the vital role of registrars in the collection of cancer data and a study commissioned by the AJCC which examined the quality of existing cancer prognostic calculators and highlighted the poor quality of existing prognostic tools.

Introduction: The management of perirectal tumors often requires rectal wall resection, and sometimes a complete proctectomy is required. Access to posterior perirectal masses via a posterior, transcoccygeal approach (Kraske procedure) avoids dissection of the intraperitoneal rectum.Patient: The patient was a 63-year-old male who presented to his primary care physician with debilitating perirectal pain of several months’ duration. He did not respond to therapy with pain medications and topical agents, and underwent a lateral internal sphincterotomy for what was thought to be an anal fissure, without relief prior to referral. Diagnostic workup showed a low signal intensity mass on magnetic resonance imaging (MRI), and biopsy revealed high-grade leiomyosarcoma with myxoid features. Staging workup included a contrast-enhanced computed tomography chest, abdomen and pelvis, flexible sigmoidoscopy and endoscopic ultrasound. A lytic lesion in his left ilium on MRI was found to be avid on fluorodeoxyglucose-positron emission tomography scan and was therefore consistent with oligometastatic disease. He received six cycles of adriamycin, cyclophosphamide and dacarbazine, with good response. The metastatic lesion was treated with 24 Gy of radiotherapy, while the primary tumor was treated with 50 Gy of radiotherapy. The patient underwent the Kraske approach with radical resection of the perirectal mass. The rectal wall was closed with interrupted silk sutures, and layered closure of incision over a drain was performed. An R0 resection was achieved. A laparoscopic diverting loop ileostomy to protect the rectal repair was performed.Conclusion: The Kraske approach allows for adequate resection, while avoiding the morbidity of the transabdominal approach, and allowing the patient to maintain a continent rectum. [ABSTRACT FROM AUTHOR]

Cancer registry data items need to be updated to reflect current practice trends. Close collaboration between clinicians and registrars is needed to improve the quality of the data collected. [ABSTRACT FROM AUTHOR]