These guidelines update previous guidance published in 2005. They have been revised by a group who are members of the UK and Ireland Neuroendocrine Tumour Society with endorsement from the clinical committees of the British Society of Gastroenterology, the Society for Endocrinology, the Association of Surgeons of Great Britain and Ireland (and its Surgical Specialty Associations), the British Society of Gastrointestinal and Abdominal Radiology and others. The authorship represents leaders of the various groups in the UK and Ireland Neuroendocrine Tumour Society, but a large amount of work has been carried out by other specialists, many of whom attended a guidelines conference in May 2009. We have attempted to represent this work in the acknowledgements section. Over the past few years, there have been advances in the management of neuroendocrine tumours, which have included clearer characterisation, more specific and therapeutically relevant diagnosis, and improved treatments. However, there remain few randomised trials in the field and the disease is uncommon, hence all evidence must be considered weak in comparison with other more common cancers.

https://gut.bmj.com/content/gutjnl/61/1/6.full.pdf

Guidelines for the management of gastroenteropancreatic neuroendocrine (including carcinoid) tumours (NETs)

https://www.endo.theclinics.com/article/S0889-8529(10)00099-X/pdf

The Epidemiology of Gastroenteropancreatic Neuroendocrine Tumors

http://www.klinikum.uni-muenchen.de/GEPNET/download/inhalt/Fachgesellschaften/ENETS/ENETS2016/07-Pulmonary-carcinoids.pdf

Pulmonary neuroendocrine (carcinoid) tumors: European Neuroendocrine Tumor Society expert consensus and recommendations for best practice for typical and atypical pulmonary carcinoids

Seventy-eight examples of intraductal carcinoma (DCIS) were identified after pathologic review of 2072 specimens obtained from National Surgical Adjuvant Breast Project protocol 6. This randomized clinical trial compares the therapeutic merit of total mastectomy (TM) with lumpectomy (L), with (LX) and without (LO) postoperative irradiation. All patients were subjected to axillary lymph node dissection. Seven (14%) of the 51 patients with DCIS treated by L exhibited breast recurrence within or close to the site of the initial lesion 4 to 53 months (average, 16 months) after L. Only 2 (7%) of these events occurred in the 29 women treated by LX, as opposed to 23% in the LO group. No pathologic features were noticed that might have been considered predictive of local breast recurrence. The three DCIS recurrences and the four invasive forms noted are considered to represent overlooked or incompletely excised foci of cancer because of the multifocality (not multicentricity) of some breast cancers. The possibility that DCIS may represent a marker of risk for the development of cancer rather than a precursor lesion per se is suggested. Despite apparent difficulties in the pathologic diagnosis of DCIS as well as uncertainty concerning its natural history, no evidence was found to indicate that it represents a more ominous disease than invasive cancer. Indeed, treatment failure occurred in only one patient treated by LX and a similar number subjected to TM (4% versus 2%). Although these observations are short term (average follow-up, 39 months), estimates of the probability of local recurrence or survival suggest that they will not be significantly altered after longer periods of surveillance. Thus, there are no compelling reasons why DCIS may not be treated in a cosmetically acceptable manner by LX. A randomized clinical trial addressing this issue is now in progress.

Pathologic findings from the National Surgical Adjuvant Breast Project (protocol 6). I. Intraductal carcinoma (DCIS)

BACKGROUND.
The National Cancer Institute's Surveillance, Epidemiology, and End Results (SEER) program has proven to be a significant resource in US neuroendocrine tumor (NET) epidemiology. Norway also holds a robust and detailed cancer registry: the Norwegian Registry of Cancer (NRC).

METHODS.
SEER NET data were compared with corresponding NRC data in the time period 1993 to 2004 to determine whether there are differences in NET epidemiology between Norway and the United States.

RESULTS.
The SEER and NRC reported 17,312 and 2030 NETs, respectively. The overall Caucasian SEER NET incidence was 4.44, compared with 3.24 in the NRC. In the SEER white subset, bronchopulmonary NETs were the most common (incidence = 1.42; 32% of all NETs), compared with small intestinal NETs in the NRC (0.81; 26%). A marked increase in SEER NET incidence (37%-40%) was observed in the period 2000 to 2004, compared with 1993 to 1997; an even more pronounced increase (72%) was seen in the NRC. African Americans exhibited a remarkably high overall NET incidence of 6.50; furthermore, among African Americans, rectal NETs were most common (1.65; 27%). Small intestinal NET incidence was ∼30% higher in men compared with women in all populations. The highest 5-year survival rates were for rectal NETs (74%-88%) in both databases, whereas prostatic NETs had the worst outcome (0%-23%). At diagnosis, NETs were localized in 27% to 46% of patients.

CONCLUSIONS.
NET incidence in the US Caucasian population and in Norway is similar, but considerably higher (∼50%) among African Americans. NETs have been regarded as indolent tumors; however, the 5-year survival is only ∼55%. Cancer 2008. © 2008 American Cancer Society.

https://acsjournals.onlinelibrary.wiley.com/doi/pdf/10.1002/cncr.23883

Neuroendocrine tumor epidemiology: contrasting Norway and North America.

Lumpectomy specimens are commonly divided into six sides: superficial, deep, superior, inferior, medial, and lateral. Orienting stitches are placed on the specimen during surgery to allow reorientation by pathology. Despite those efforts, specimen disorientation may occur. The aim of this study was to assess the correlation in orientation between surgeons and pathologists. Lumpectomy specimens were routinely oriented. An additional Prolene suture was randomly placed by the surgeon on one side to be localized by pathology. The results were recorded and the disorientation rate calculated. Specimen size and presence of skin and/or muscle were also recorded. There were 122 lumpectomy specimens prospectively entered. Average specimen volume was 95.5 cm(3). Twenty-four specimens had segments of skin or muscle. The additional sutures were evenly divided between the six sides. The overall disorientation rate was 31.1% (95% confidence interval, 23.1-40.2).The side-specific disorientation rates were 43%, 40%, 35%, 29%, 28%, and 14% for the deep, superficial, lateral, medial, superior, and inferior surfaces, respectively (no statistical difference). Presence of skin or muscle on the specimen did not contribute to better orientation. Specimen volumes, however, were highly associated with orientation. Specimens of <20 cm(3) had a disorientation rate of 78%, while larger specimen had a disorientation rate of 20% (p < .001). Specimen orientation with stitches placed on two surfaces is associated with a high disorientation rate. Better orientation techniques are necessary to minimize the specimen disorientation.

Breast Specimen Orientation

Introduction
The aim of the study was to determine outcomes for respiratory and gastrointestinal carcinoid tumors utilizing a large cancer registry.

7201 carcinoids: increasing incidence overall and disproportionate mortality in the elderly.

Screening mammography has increased the number of patients diagnosed with ductal carcinoma-in-situ (DCIS) in the past 20 years. The Florida Cancer Data System is the largest single source incident cancer registry in the United States. We analyzed this registry to determine the changing incidence and treatment patterns for DCIS. Patients with DCIS from 1981 to 2001 were identified. Age-adjusted rate, descriptive statistics, and incidence of future DCIS and invasive breast cancer were calculated. A total of 23,810 DCIS patients were identified. The age-adjusted rate of DCIS has risen from 2.4 to 27.7 per 100,000 women between 1981 and 2001. Median age was 64 years; 85% of patients were white, 6.6% African American, and 7.5% Hispanic. Median tumor size was .9 cm. Forty-seven percent of patients had breast-conserving therapy (BCT). Half of the 53% of patients undergoing mastectomy underwent a modified radical mastectomy. Eight percent received no surgical treatment. Sentinel lymph node biopsy was used in 2.7% of patients who underwent a mastectomy. After BCT, 37.5% received adjuvant radiotherapy, and only 13% were treated with hormonal therapy. The incidence of DCIS has risen dramatically with the advent of screening mammography. Increasing numbers of these patients are treated with BCT, although a large proportion are still treated with mastectomy, in some cases combined with axillary dissection. Sentinel lymph node biopsy and tamoxifen are important components of therapy, the use of which is slowly increasing in the treatment of DCIS.

Results of 23,810 Cases of Ductal Carcinoma-in-situ

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Breast Specimen Orientation

7201 carcinoids: increasing incidence overall and disproportionate mortality in the elderly.

Results of 23,810 Cases of Ductal Carcinoma-in-situ