scholarly journals Presence of Cytokeratin 19-Expressing Cholangiocarcinoma-Like Tumour in a Liver Metastatic Lesion of Rectal Neuroendocrine Tumour

2016 ◽  
Vol 10 (2) ◽  
pp. 431-439 ◽  
Author(s):  
Norihiro Ishii ◽  
Kenichiro Araki ◽  
Takehiko Yokobori ◽  
Mariko Tsukagoshi ◽  
Takamichi Igarashi ◽  
...  
Keyword(s):  
Neuroendocrine Tumours ◽  
Neuroendocrine Tumour ◽  
Primary Tumour ◽  
Metastatic Lesion ◽  
Pathological Examination ◽  
Cytokeratin 19 ◽  
Tumour Resection ◽  
Case Presentation ◽  
Metastatic Lesions ◽  
Mixed Tumours

Introduction: Tumours with adenocarcinoma and neuroendocrine components have often been reported, although the reason underlying the dual components remains unclear. Case Presentation: A 43-year-old woman with multiple liver metastatic lesions of rectal neuroendocrine tumour underwent primary tumour resection and subsequent liver transplantation. Pathological examination indicated a cholangiocarcinoma-like tumour with gland formation, adjacent to a liver metastatic lesion of the neuroendocrine tumour. This tumour comprised atypical columnar epithelium, and stained positively for neuroendocrine markers and the ductal marker cytokeratin 19, indicating amphicrine properties and a partial cholangiocarcinoma phenotype – features not observed in the primary and metastatic neuroendocrine tumours. Conclusion: The presence of adenocarcinoma only at the metastatic site indicated that neuroendocrine tumour cells acquired stemness and differentiated into adenocarcinoma through metastasis, or that the adenocarcinoma newly arose from the adjacent epithelium influenced by the neuroendocrine tumour. We propose a novel mechanism for the pathogenesis of mixed tumours in neuroendocrine tumours.

Evaluation of Clinical Prognostic Factors and Further Delineation of the Effect of Mesenteric Fibrosis on Survival in Advanced Midgut Neuroendocrine Tumours

2018 ◽  
Vol 107 (3) ◽  
pp. 292-304 ◽  
Author(s):  
Faidon-Marios Laskaratos ◽  
Martin Walker ◽  
Dominic Wilkins ◽  
Alexander Tuck ◽  
Shashank Ramakrishnan ◽  
...  
Keyword(s):  
Prognostic Factors ◽  
Large Scale ◽  
Neuroendocrine Tumours ◽  
Primary Tumour ◽  
Stage Iv ◽  
Epidemiological Data ◽  
Tumour Resection ◽  
Carcinoid Heart Disease ◽  
Factors Affecting ◽  
Stage Iv Disease

Background: Small intestinal neuroendocrine tumours (SI NETs) represent 30–50% of small bowel neoplasms and often present at an advanced stage. To date, there is relatively limited literature regarding prognostic factors affecting overall survival (OS) in stage IV disease. In addition, the prevalence of mesenteric fibrosis (MF) in SI NETs and its effect on OS have not been sufficiently explored in the literature. Aim: The primary aim of this study was to perform a large-scale survival analysis in an institutional cohort of 387 patients with metastatic (stage IV) SI NETs. The secondary aim was to provide epidemiological information regarding the prevalence of MF and to evaluate its effect on OS. Results: The median OS was 101 months (95% CI 84, 118). Age > 65 years, mesenteric metastases with and without desmoplasia, liver metastases, carcinoid heart disease (CHD) and bone metastases were associated with a significantly shorter OS, while primary tumour resection was predictive of a longer OS. The benefit of surgical resection was limited to symptomatic patients. MF was present in approximately 50% of patients with mesenteric lymphadenopathy. Elevated urinary 5-HIAA levels correlated strongly with the presence of CHD (p < 0.001) and to a lesser extent (p = 0.02) with MF. MF and CHD did not usually co-exist, suggesting that different mechanisms are likely to be involved in the development of these fibrotic complications. Conclusions: This study has identified specific prognostic factors in a large cohort of 387 patients with advanced SI NETs and has provided useful epidemiological data regarding carcinoid-related fibrotic complications.

scholarly journals Neuroendocrine Tumour of the Appendix in Pregnancy

2013 ◽  
Vol 13 (2) ◽  
pp. 81-83
Author(s):  
Andrejs Vanags ◽  
Ilze Strumfa ◽  
Zane Simtniece ◽  
Jānis Gardovskis
Keyword(s):  
Acute Appendicitis ◽  
Digestive System ◽  
Neuroendocrine Tumours ◽  
Neuroendocrine Tumour ◽  
Pathological Examination ◽  
Solitary Lesion ◽  
Documented Case ◽  
Pubmed Database ◽  
Anatomic Localization ◽  
In Pregnancy

Abstract Despite the fact that the appendix is one of the rarest anatomic localization of neuroendocrine tumours (NETs) in the digestive system, the appendicular neuroendocrine tumour (A-NET) remains among the most common tumours of the appendix. The A-NET usually presents as a small solitary lesion revealed only after pathological examination of appendix that has been surgically removed for acute appendicitis. Only 18 papers in the Pubmed database (1965-2012) are dedicated to A-NET in pregnancy. Here we present a well-documented case of A-NET in a pregnant lady.

Treatment of metastatic midgut neuroendocrine tumour associated with carcinoid syndrome

2014 ◽  
Vol 155 (5) ◽  
pp. 194-198
Author(s):  
Andrea Uhlyarik ◽  
Erika Lahm ◽  
József Vachaja ◽  
Zsuzsanna Pápai
Keyword(s):  
Metastatic Disease ◽  
Case History ◽  
Carcinoid Syndrome ◽  
Neuroendocrine Tumours ◽  
Neuroendocrine Tumour ◽  
Somatostatin Receptor ◽  
Tumour Response ◽  
Tumour Resection ◽  
Peptide Receptor ◽  
Long Acting

Although the incidence of neuroendocrine tumours is low, their prevalence is high due to the usually slow course of the disease. Between July 1, 2008 and July 1, 2013 the authors evaluated 56 patients with well-differentiated or moderately differentiated neuroendocrine tumours; 36 patients with metastatic disease underwent treatment while 17 patients who had tumour resection were followed without additional treatment. All patients with metastatic disease received long acting octreotide, and additional therapy was based on the site of origin, grade of differentiation, Ki67 index, and focal labelling of the tumours during somatostatin-receptor or metaiodo-benzyl-guanidine scintigraphy. The authors present a detailed case history of a patient with carcinoid syndrome due to a metastatic midgut neuroendocrine tumour, who received long acting octreotide and peptide receptor radionuclide treatment. In this patient an objective tumour response was reached in addition to the resolution of symptoms of carcinoid syndrome. The authors conclude that the case history confirms previous observations showing that long acting octreotide combined with peptide receptor radionuclide treatment may provide long survival with good quality of life in a patient with metastatic midgut neuroendocrine tumour accompanied with carcinoid syndrome. Orv. Hetil., 2014, 155(5), 194–198.

scholarly journals ENETS TNM Staging Predicts Prognosis in Small Bowel Neuroendocrine Tumours

ISRN Oncology ◽  
2013 ◽  
Vol 2013 ◽  
pp. 1-7 ◽  
Author(s):  
Rajaventhan Srirajaskanthan ◽  
A. Ahmed ◽  
A. Prachialias ◽  
P. Srinivasan ◽  
N. Heaton ◽  
...  
Keyword(s):  
Prognostic Factors ◽  
Small Bowel ◽  
Neuroendocrine Tumours ◽  
Primary Tumour ◽  
Staging System ◽  
Tnm Staging ◽  
Tumour Resection ◽  
Carcinoid Heart Disease ◽  
Tnm Staging System ◽  
Primary Tumour Resection

Introduction. Small bowel neuroendocrine tumours (NETs) are the most common type of gastrointestinal neuroendocrine tumours. The incidence and prevalence of these tumours are on the rise. The aims of this study were to determine prognostic clinicopathological features and whether the ENETS TNM staging system predicts prognosis and also. Method. Clinical data was collected retrospectively from 138 patients with histologically proven small bowel NETs managed at King’s College Hospital. Histology was reviewed and small bowels tumours, were staged according to the ENETS TNM staging system. Results. Median age was 65 years (range 29–87). The 5-year survival was 79.5% and the 10-year survival was 48.5%. Resection of the primary tumour was associated with improved survival (120 versus 56 months, P<0.05). On multivariate analysis prognostic factors were primary tumour resection and not having a carcinoid heart disease. TNM staging significantly separated survival of stage 2 and stage 3 from stage 4 NETs. Conclusion. Small bowel primary tumour resection and not having carcinoid heart disease are prognostic factors. The ENETS TNM staging and grading system appears to be of prognostic relevance to small bowel NETs.

A rare case of syncronous solitary para-pharyngeal metastasis of clear cell carcinoma in a patient with small renal mass

2021 ◽  
pp. 039156032110204
Author(s):  
Fabrizio Gallo ◽  
Emilio Gastaldi ◽  
Gaetano Ninotta ◽  
Federico Baricalla ◽  
Alessandra Pastorino ◽  
...  
Keyword(s):  
Cell Carcinoma ◽  
Rare Case ◽  
Primary Tumour ◽  
Performance Status ◽  
Poor Performance ◽  
Metastatic Lesion ◽  
Pathological Examination ◽  
Poor Performance Status ◽  
First Case ◽  
Vertebral Bodies

Introduction: Unusual metastatic sites of renal cell carcinoma (RCC) are not infrequent. We report a rare case of solitary pharyngeal metastasis as first presentation of RCC. Case description: A 74 years-old man was referred to our hospital due to rapidly progressive dyspnoea and dysphagia. Physical examination showed a large right para-pharyngeal mass. Imaging findings showed a 5.5 cm mass, suspicious for malignancy, which extended to right para and retro-pharyngeal spaces with compression of the major right cervical vessels, C2-C3 vertebral bodies osteolysis, dural sac compression and dislocation. Futhermore, a small (2.6 × 2 cm) mass located at the upper pole of the right kidney was shown. Patient underwent partial trans-oral removal of the mass. Pathological examination and immunochemistry resulting strongly suggestive for metastatic RCC. Considering the metastatic stage of the tumour and the rapidly progressive clinical worsening with poor performance status, we offered the patient a palliative treatment with tyrosine kinase and cytoreductive radiotherapy on vertebral bodies. The patient developed a rapidly progressive multifocal metastatic disease and died 4 months after the presentation. Conclusion: We think that our case is noteworthy for some aspects. Firstly, pharyngeal localizations of RCC are very rare and this is the first case of solitary pharyngeal metastasis. Secondly, this metastatic lesion was really particular because it was synchronous and twice as big than the primary tumour. Thirdly, this case is consistent with previous evidence that synchronous compared with metachronous metastasis RCC is associated with adverse effect on outcome and response to targeted treatment.

scholarly journals Mesenteric Variceal Haemorrhage and Ectopic Cushing’s Syndrome as Presenting Features of a Pancreatic Neuroendocrine Tumour Recurrence

2021 ◽  
pp. 919-926
Author(s):  
Scott Weerasuriya ◽  
Kieran Palmer ◽  
Stephen Gregory ◽  
Benjamin C. Whitelaw ◽  
Elisa Gonzalez ◽  
...  
Keyword(s):  
Cushing’S Syndrome ◽  
Neuroendocrine Tumours ◽  
Neuroendocrine Tumour ◽  
Primary Tumour ◽  
Cushing's Syndrome ◽  
Tumour Recurrence ◽  
Metabolic Disturbances ◽  
Ectopic Cushing’S Syndrome ◽  
Pancreatic Neuroendocrine Tumours ◽  
Pancreatic Neuroendocrine Tumour

Pancreatic neuroendocrine tumours can have varied and complex presentations. Whilst hormone hypersecretion often induces characteristic clinical syndromes, non-specific symptoms may arise due to localized tumour effects. Malignant invasion of local vasculature is an increasingly recognized complication of these neoplasms and can be associated with significant morbidity. Herein, we present the case of a 47-year-old male with a recurrence of a pancreatic neuroendocrine tumour who presented with unusual upper gastrointestinal bleeding. The tumour had recurred within the superior mesenteric vein, replacing the vessel and invading its branches. This resulted in porto-mesenteric hypertension and the formation of bleeding mesenteric varices. The patient subsequently developed progressive metabolic disturbances and was diagnosed with ectopic Cushing’s syndrome, despite his primary tumour having been non-functional. This case demonstrates not only a rare pattern of tumour recurrence but also the potential for pancreatic neuroendocrine tumours to de-differentiate and change from non-functional to hormone secreting, a phenomenon which may complicate diagnosis and management.

scholarly journals Carcinoid Syndrome and Carcinoid Heart Disease as Manifestations of Non-Metastatic Ovarian Neuroendocrine Tumour

2017 ◽  
Vol 30 (5) ◽  
pp. 421 ◽  
Author(s):  
Joana Simões-Pereira ◽  
Lai Mun Wang ◽  
Attila Kardos ◽  
Ashley Grossman
Keyword(s):  
Heart Disease ◽  
Carcinoid Syndrome ◽  
Neuroendocrine Tumours ◽  
Neuroendocrine Tumour ◽  
Early Recognition ◽  
Tumour Resection ◽  
Carcinoid Heart Disease ◽  
Additional Burden ◽  
Gastrointestinal Neuroendocrine Tumours ◽  
Surgical Valve Replacement

The carcinoid syndrome is rare but it is associated with carcinoid heart disease in more than a half of the cases. Carcinoid heart disease is typically characterised by morphological and functional modifications of right-sided valves. Its aetiology is probable multifactorial but serotonin appears to play a key role in the development of this valvular disease. Unlike gastrointestinal neuroendocrine tumours, ovarian neuroendocrine tumours can present with carcinoid syndrome and carcinoid heart disease in the absence of liver metastases; such ovarian neuroendocrine tumours are a unique clinical entity. The additional burden of cardiac impairment in these patients represents a significant reduction in survival. Early recognition and surgical valve replacement before advanced heart failure is established may improve the clinical outcome. We report the case of a woman with an ovarian neuroendocrine tumour and highly symptomatic carcinoid heart disease who was submitted to tumour resection followed by valvuloplasty. She demonstrated an outstanding clinical improvement and has remained free of tumour and symptomatology.

scholarly journals P-L13 Proposed sampling methodology for improved accuracy of hepatic burden assessment in neuroendocrine tumours

2021 ◽  
Vol 108 (Supplement_9) ◽  
Author(s):  
Thomas Thorne ◽  
Simon Hughes ◽  
Rupaly Pande ◽  
Samuel Ford
Keyword(s):  
Neuroendocrine Tumours ◽  
Primary Tumour ◽  
Liver Volume ◽  
Small Sample ◽  
Tumour Resection ◽  
Kendall’S Tau ◽  
Sampling Methodology ◽  
Kendall's Tau ◽  
Total Liver Volume ◽  
Primary Tumour Resection

Abstract Background Hepatic burden is a significant confounder in the assessment of impact of primary tumour resection in metastatic small bowel neuroendocrine tumours (SI-NET). For SI-NET metastatic hepatic burden &gt;10% disease replacement or &gt; 5 hepatic metastases are known prognostic markers, though nomograms and scores do not adequately account for this. Most trials do not adequately account for hepatic burden when assessing the survival difference between SI-NET primary tumour resection and no resection. We propose a sampling methodology to more accurately assess metastatic liver burden in SI-NET and correlate with delayed resection vs. upfront primary tumour resection at a specialist NET surgical unit. Methods Patients referred for metastatic SI-NET between January 2003 and February 2020 were identified from a prospective dataset. The earliest CT scan after diagnosis was used. The axial, coronal and sagittal slice position limits of the whole liver were recorded. These limits allowed equitable slice position of the liver, with 8 equally distributed axial, 4 equally distributed coronal and 4 equally distributed sagittal slices. Each slice was used to define the liver and metastatic area as assessed using liver CT windows. Liver burden was estimated as percentage total metastatic area summed from all 8 axial, 4 coronal and 4 sagittal slices. Results 157 total patients were on the collated data base and 46 patients were identified with an appropriate CT. Liver burden was positively skewed. Liver burden was significantly higher for delayed resection vs. upfront resection in all planes of assessment (axial: 11.61% vs. 0.14%, p = 0.003; coronal: 13.46% vs. 0.33%, p = 0.006; sagittal: 10.46% vs. 0.16%, p = 0.008). All planar assessments correlated well with one another (all Kendall’s tau ≥0.851, all p &lt; 0.001). Liver metastatic burden correlated with total liver volume (Kendall’s tau 0.549-0.573, all p &lt; 0.001). Conclusions Hepatic burden differs between resection groups in a small sample at our centre, highlighting the unmeasured confounders favouring primary tumour resection via positive bias. Therefore, hepatic burden needs quantifying in prospective studies that assess primary tumour resection in SI-NET. This is to ensure comparable groups after randomisation. Our method provides an assessment of this metastatic SI-NET liver burden.

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