Primary neuroendocrine tumour of the kidney in an asymptomatic patient involving a multidisciplinary approach

Primary neuroendocrine tumours of the kidney are rare, and their pathophysiology is uncertain; since their discovery in 1966, they have been described only a few times in the literature. We present a case of a well-differentiated neuroendocrine tumour of the kidney in an asymptomatic patient, which required a multidisciplinary approach by the hospital’s team, including precise surgical treatment and an effective radiopathological diagnosis. The patient underwent right radical nephrectomy. During follow-up, he remained asymptomatic, and no metastases or complications were identified.

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Classification and pathology of gastroenteropancreatic neuroendocrine neoplasms

Endocrine Related Cancer ◽

10.1530/erc-11-0013 ◽

2011 ◽

Vol 18 (S1) ◽

pp. S1-S16 ◽

Cited By ~ 180

Author(s):

Günter Klöppel

Keyword(s):

Gastrointestinal Tract ◽

Neuroendocrine Tumours ◽

Neuroendocrine Tumour ◽

Tnm Stage ◽

Neuroendocrine Neoplasms ◽

Gastroenteropancreatic Neuroendocrine Neoplasms ◽

Poorly Differentiated ◽

Well Differentiated ◽

Neuroendocrine Carcinomas

Gastroenteropancreatic neuroendocrine neoplasms (GEP-NENs) are composed of cells with a neuroendocrine phenotype. The old and the new WHO classifications distinguish between well-differentiated and poorly differentiated neoplasms. All well-differentiated neoplasms, regardless of whether they behave benignly or develop metastases, will be called neuroendocrine tumours (NETs), and graded G1 (Ki67 <2%) or G2 (Ki67 2–20%). All poorly differentiated neoplasms will be termed neuroendocrine carcinomas (NECs) and graded G3 (Ki67 >20%). To stratify the GEP-NETs and GEP-NECs regarding their prognosis, they are now further classified according to TNM-stage systems that were recently proposed by the European Neuroendocrine Tumour Society (ENETS) and the AJCC/UICC. In the light of these criteria the pathology and biology of the various NETs and NECs of the gastrointestinal tract (including the oesophagus) and the pancreas are reviewed.

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Results of surgical treatment of children with PFAPA syndrome

Medical Council ◽

10.21518/2079-701x-2019-17-244-251 ◽

2019 ◽

pp. 244-251

Author(s):

A. S. Yunusov ◽

T. I. Garashchenko ◽

P. I. Belavina ◽

A. G. Ryazanskaya ◽

E. V. Molodtsova ◽

...

Keyword(s):

Quality Of Life ◽

Surgical Treatment ◽

Multidisciplinary Approach ◽

Pediatric Population ◽

Quality Of Life Indicators ◽

Before And After ◽

Pfapa Syndrome ◽

Primary School Age

Rationale. Currently, the literature describes about 374 cases of Marshall syndrome. According to some authors, the prevalence of Marshall syndrome is 2.3 per 10,000 children, in general, the epidemiology of PFAPA syndrome in the pediatric population is unknown.Purpose. The aim of our study is to justify surgical treatment in children with Marshall syndrome. Objectives. The objectives of this study are to give a comparative assessment of the effectiveness of tonsillectomy and adenotonzyllectomy for this syndrome and to evaluate the quality of life indicators before and after surgical treatment.Materials and methods. The study included 26 patients with Marshall syndrome, of which 18 patients underwent adenotonzyllectomy and 8 patients underwent bilateral tonsillectomy. A clinical case of a typical manifestation of Marshall syndrome is also described, and follow-up results of the effectiveness of surgical treatment for Marshall syndrome are presented. In the course of the work, an adapted questionnaire was developed to assess the quality of life of parents of children with Marshall syndrome.Results. The result of our work is the fact that in relation to the relief of PFAPA-syndrome, children of preschool and primary school age have more effective adenotonsillectomy (88.2%), while tonsillectomy was effective only in 55.6% of patients.Conclusion. A multidisciplinary approach is required to address the diagnosis and treatment of Marshall syndrome.

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AUA and NCCN surveillance guidelines for RCC: Do they effectively capture recurrences following nephrectomy?

Journal of Clinical Oncology ◽

10.1200/jco.2014.32.4_suppl.402 ◽

2014 ◽

Vol 32 (4_suppl) ◽

pp. 402-402

Author(s):

Suzanne B. Stewart ◽

Christine M. Lohse ◽

Sarah P. Psutka ◽

John C. Cheville ◽

Stephen A. Boorjian ◽

...

Keyword(s):

Renal Cell Carcinoma ◽

Surgical Treatment ◽

Partial Nephrectomy ◽

Radical Nephrectomy ◽

Clear Cell ◽

Risk Groups ◽

Site Specific ◽

American Urological Association ◽

Cancer Network

402 Background: The American Urological Association (AUA) and the National Comprehensive Cancer Network (NCCN) are highly utilized sources for surveillance strategies following surgical treatment for renal cell carcinoma (RCC). However, the duration of follow-up may be inadequate to capture the majority of recurrences. Herein, we assess the ability of these guidelines to effectively capture recurrences of RCC following primary surgical resection. Methods: We reviewed our institutional database of 3,725 patients treated with radical or partial nephrectomy for M0 sporadic RCC between 1970-2008. For comparison to the AUA guidelines, patients were stratified into low risk following partial nephrectomy (LRp) or radical nephrectomy (LRr) = pT1N0, and moderate/high risk (M/HR) = pT2-4 N0-1. Guideline effectiveness was assessed by calculating the percentage of recurrences detected within the prescribed follow-up periods given for site-specific recurrence: AUA—LRp: 3yrs for all sites; LRr: 1yr for abdominal and 3yrs for chest/bone/other sites; M/HR: 5yrs for all sites; NCCN—6 months for abdominal/chest sites and 5yrs for bone/other sites. Results: Of the 3,725 patients, 2721 (73.1%) underwent radical nephrectomy, 2,210 (59.3%) were classified as pT1 NX-0 and 2,910 (78.1%) as clear cell RCC. Median postoperative follow-up was 8.9yrs (IQR 5.5-14.2) during which 1,114 (29.9%) patients developed recurrence. Of these recurrences, 760 (68.2%) would have been detected using the AUA guidelines and 432 (38.8%) by NCCN recommendations. Within AUA risk groups, 37.2% recurrences were captured in LRp, 31.4% in LRr and 80.2% in M/HR. Capture of 90% of recurrences in the abdomen and chest would require surveillance for 9yrs and 8 yrs, respectively in LRp, 15yrs and 12yrs in LRr and 11yrs and 10yrs in M/HR. Conclusions: Duration of follow-up recommended by current surveillance algorithms by the AUA and NCCN do not adequately capture many recurrences in RCC following radical or partial nephrectomy. Guidelines using risk stratification and site-specific recurrence parameters to assign length of surveillance may allow providers to better individualize surveillance regimens.

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Management and Follow-up of Patients with a Bronchial Neuroendocrine Tumor in the Last Twenty Years in Ireland: Expected Inconsistencies and Unexpected Discoveries

International Journal of Endocrinology ◽

10.1155/2018/1043287 ◽

2018 ◽

Vol 2018 ◽

pp. 1-8 ◽

Cited By ~ 3

Author(s):

Asta Agasarova ◽

Clare Harnett ◽

Niall Mulligan ◽

Muhammad Shakeel Majeed ◽

Alberto Caimo ◽

...

Keyword(s):

Multidisciplinary Approach ◽

Clinical Laboratory ◽

Large Cell ◽

High Rate ◽

Diagnostic Process ◽

Good Survival ◽

Small Cell Lung ◽

Well Differentiated ◽

Neuroendocrine Carcinomas

Bronchial neuroendocrine tumors (NET) are classified into well-differentiated typical carcinoids (TC), atypical carcinoids (AC), large cell neuroendocrine carcinomas (LCNEC), and small cell lung carcinomas (SCLC). We retrospectively reviewed and analyzed the diagnostic and therapeutic aspects, follow-up data, and outcomes of all patients diagnosed with a bronchial NET from 1995 to 2015 at our institution. Patients with LCNEC or SCLC were excluded due to the biological and clinical differences from the other bronchial NET. The clinical, laboratory, imaging, treatment, and follow-up data were collected and analyzed keeping in mind the recently published international recommendations. Forty-six patients were included in the study. Of these, 37 had a TC and 5 an AC. In 4 patients, the histological characterization was inadequate. Forty-four patients underwent surgery. Four patients developed metastatic disease. Interestingly, 14 patients had one or more other tumors diagnosed at some stage and 3 of them had three different tumors. A total of 7 patients died. The analysis of the laboratory and pathology assessment identified some inconsistencies when compared to the international recommendations. Although the treatment of bronchial NET at our institution was consistent with the successively published recommendations, it appears that the diagnostic process and the follow-up surveillance were not. We think that a systematic multidisciplinary approach might improve bronchial NET patient care. A relatively high rate of occurrence of a second, or also a third, non-NET tumor was observed, though the statistical value of such observation could not be exhaustively elucidated in this numerically limited patient population. In our opinion, the observed high rate of second malignancies in this patient cohort highlights the necessity of optimizing the follow-up of the bronchial NET patients, also considering the very good survival rate achieved with regard to the bronchial NET.

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A Multimodal Approach to the Management of Neuroendocrine Tumour Liver Metastases

International Journal of Hepatology ◽

10.1155/2012/819193 ◽

2012 ◽

Vol 2012 ◽

pp. 1-13 ◽

Cited By ~ 17

Author(s):

Ron Basuroy ◽

Rajaventhan Srirajaskanthan ◽

John K. Ramage

Keyword(s):

Liver Metastases ◽

Patient Care ◽

Metastatic Disease ◽

Multidisciplinary Approach ◽

Neuroendocrine Tumours ◽

Systemic Treatment ◽

Neuroendocrine Tumour ◽

Therapeutic Options ◽

Treatment Modalities ◽

Multimodal Approach

Neuroendocrine tumours (NETs) are often indolent malignancies that commonly present with metastatic disease in the liver. Surgical, locoregional, and systemic treatment modalities are reviewed. A multidisciplinary approach to patient care is suggested to ensure all therapeutic options explored.

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Requiem for the Term ‘Carcinoid Tumour’ in the Gastrointestinal Tract?

Canadian Journal of Gastroenterology ◽

10.1155/2008/270905 ◽

2008 ◽

Vol 22 (4) ◽

pp. 357-358 ◽

Cited By ~ 19

Author(s):

Runjan Chetty

Keyword(s):

Gastrointestinal Tract ◽

Neuroendocrine Carcinoma ◽

Neuroendocrine Tumours ◽

Neuroendocrine Tumour ◽

Carcinoid Tumour ◽

World Health ◽

Low Grade ◽

Biological Behaviour ◽

Unique Type ◽

Well Differentiated

Use of the term ‘carcinoid tumour’ to describe a unique type of tumour in the gastroenteropancreatic system is endemic in the medical literature and in daily clinical and pathological parlance. However, it is a somewhat misleading moniker because a spectrum of histopathological changes and hence, biological outcomes may occur in these tumours. The World Health Organization classification scheme recommends the use of the terms neuroendocrine tumours or carcinomas, which may be stratified as well-differentiated neuroendocrine tumours with benign or uncertain behaviour, well-differentiated tumours with low-grade neuroendocrine carcinoma behaviour and high-grade neuroendocrine carcinomas. These categories may be applied within different sites in the gastrointestinal tract and pancreas, and convey a sense of biological behaviour. In addition, a recently suggested tumour-node-metastasis scheme has been proposed and awaits clinical validation and acceptance. Thus, the term ‘carcinoid’ has served its purpose well, but its use should be phased out in favour of ‘neuroendocrine tumour’ or ‘neuroendocrine carcinoma’.

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Bronchial Carcinoid Tumours in Children — A Review

European Oncology & Haematology ◽

10.17925/eoh.2011.07.03.196 ◽

2011 ◽

Vol 07 (03) ◽

pp. 196 ◽

Cited By ~ 5

Author(s):

Giovanna Rizzardi ◽

Luca Bertolaccini ◽

Alberto Terzi ◽

◽

...

Keyword(s):

Neuroendocrine Tumours ◽

Primary Lung Cancer ◽

Radical Resection ◽

Carcinoid Tumours ◽

Long Term Follow Up ◽

Bronchoplastic Procedures ◽

Well Differentiated ◽

Treatment Surgery

Bronchial carcinoids (BCs) are rare, well-differentiated malignant neuroendocrine tumours that account for 25 % of all lung neoplasms in adults. In paediatric patients, carcinoids represent the most frequent primary lung cancer. Although BCs in childhood often have an endobronchial location causing airway obstruction, they are frequently misdiagnosed as benign conditions, resulting in a delay in definitive diagnosis and treatment. Surgery represents the treatment of choice for BCs, and lung-sparing resections (sleeve or bronchoplastic procedures) are recommended in central carcinoid tumours; pneumonectomy should be avoided, particularly in childhood. If promptly diagnosed and radically treated, BCs in children have an excellent prognosis. Relapses can occur many years after a radical resection, highlighting the necessity for long-term follow-up.

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Exploring the current status of neuroendocrine tumours: a population-based analysis of epidemiology, management and use of resources

BMC Cancer ◽

10.1186/s12885-019-6412-8 ◽

2019 ◽

Vol 19 (1) ◽

Cited By ~ 3

Author(s):

Josep Darbà ◽

Alicia Marsà

Keyword(s):

Disease Management ◽

Neuroendocrine Tumours ◽

Neuroendocrine Tumour ◽

Fold Increase ◽

Current Status ◽

Metastatic Tumour ◽

Neuroendocrine Neoplasms ◽

Use Of Resources ◽

Poorly Differentiated Neuroendocrine Carcinoma ◽

Well Differentiated

Abstract Background Neuroendocrine tumours (NETs) are rare malignancies characterised by its capacity to synthesise and secrete monoamines, due to its neuroendocrine origin. Its varied locations and symptoms have traditionally been responsible for extended delays in their diagnosis. The interest of this study was to characterise the patient population diagnosed with NETs in Spain and to revise how the disease is managed, together with the hospitalisation costs of these patients. Methods The database included records of all patients diagnosed with a NET between 2010 and 2015. Admission records were used to evaluate hospitalisation, disease management data and costs, and single-patient files were used to characterise the population. Results Nine Thousand One Hundred Twenty patients were diagnosed with a neuroendocrine tumour between 2010 and 2015, with a 2 fold increase in the diagnosis rate over the study period. 42.25% of the patients were females, while 57.75% were males, and mean diagnosis age was 62.58 years (SD = 14.65). Considering all the registered neuroendocrine neoplasms, 46.86% of the patients had malignant well-differentiated NETs, 32.02% had a malignant poorly differentiated neuroendocrine carcinoma and 42.93% of patients developed metastatic NETs. In addition, 18.59% of patients were diagnosed with benign well-differentiated NETs. The most common tumour sites were the bronchus, lung and other sites, including pancreatic tumours; metastasis was found in the liver and distant lymph nodes. Pancreatic resection was the most common surgical procedure utilised in these patients, summing 19% of total expenses, the injection of an unspecified therapeutic substance (including targeted therapies) was registered in 11.40% of admissions, while chemotherapy was registered in only 6.85% of admissions. The annual healthcare cost of NETs was €15,373,961, corresponding to €9092 per patient. Conclusions The implementation of standard diagnosis procedures should be prioritised, with a focus on the pancreas and lung, and taking into account that 42.93% of the patients develop a metastatic tumour. The presence of comorbidities and multimorbidities should be considered in order to develop more efficient disease management protocols.

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Surgical treatment of renal cell carcinoma recurrence at the renal fossa following radical nephrectomy

Sao Paulo Medical Journal ◽

10.1590/s1516-31802008000300011 ◽

2008 ◽

Vol 126 (3) ◽

pp. 194-196 ◽

Cited By ~ 1

Author(s):

Carlos Márcio Nóbrega de Jesus ◽

Filemón Anastásio Silva Casafus ◽

Aparecido Donizetti Agostinho

Keyword(s):

Renal Cell Carcinoma ◽

Surgical Treatment ◽

Cell Carcinoma ◽

Renal Cell ◽

Radical Nephrectomy ◽

Left Lung ◽

Rare Event ◽

Good Method ◽

Rare Situation

CONTEXT: Isolated renal cell carcinoma recurrence at the renal fossa is a rare event. This condition occurs in 1 to 2% of radical nephrectomy cases. It is usually seen in postoperative follow-up imaging examinations such as abdominal computed tomography or abdominal ultrasound. There is controversy among urologists and oncologists regarding the best way to treat this rare situation, because of the few cases in the literature. CASE REPORT: We report on a case of isolated recurrence at the renal fossa due to renal cell carcinoma (RCC), four and a half years after radical nephrectomy, without evidence of metastases in other organs. The diagnosis was made from abdominal tomography performed during outpatient follow-up, in which a retroperitoneal mass was observed in the renal fossa. Excision was carried out by means of a subcostal transversal incision, without complications. One and a half years after the procedure, there was evidence of metastasis in the left lung and, six months later, another recurrence at the ninth anterior right rib, while the patient remained asymptomatic. Aggressive surgical treatment is a good method for controlling this rare situation of single retroperitoneal RCC recurrence. Abdominal tomography must continue to be performed over long periods of follow-up, to monitor for RCC following radical nephrectomy, in order to diagnose any late retroperitoneal recurrences. These must be treated as single RCC metastases.

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Small cell carcinoma of the pancreas: a rare neuroendocrine tumour

BMJ Case Reports ◽

10.1136/bcr-2020-235397 ◽

2020 ◽

Vol 13 (7) ◽

pp. e235397

Author(s):

Theakarajan Rajendran ◽

Bhavana Katta ◽

Oseen Hajilal Shaikh ◽

Uday Shamrao Kumbhar

Keyword(s):

Cell Carcinoma ◽

Small Cell Carcinoma ◽

Poor Prognosis ◽

Neuroendocrine Tumours ◽

Palliative Chemotherapy ◽

Neuroendocrine Tumour ◽

Small Cell ◽

Immunohistochemical Markers ◽

Poorly Differentiated

Primary small cell carcinoma (SCC) of the pancreas is a rare disease with poor prognosis. Very few cases have been reported in the literature. It is a type of poorly differentiated variety of neuroendocrine tumours of the pancreas with specific immunohistochemical markers. Imaging is not diagnostic for disease, and diagnosis is mainly by biopsy. We report a rare case of SCC of the pancreas who presented with features of obstructive jaundice without any paraneoplastic features. The patient is planned for palliative chemotherapy because of metastasis and is under regular follow-up.

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