Clinical signs of fibrosis in small intestinal neuroendocrine tumours

Daskalakis, Kosmas; Karakatsanis, Andreas; Stålberg, Peter; Norlén, Olov; Hellman, Per

doi:10.1002/bjs.10333

Cited by 70 publications

(70 citation statements)

References 24 publications

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“…In our cohort, the median OS was 8.7 years and 5-year OS was 71%. While this is comparable to OS rates for SI NETs [1, 12], a large ( n = 824) retrospective study of SI NETs from Uppsala showed that extensive fibrosis associated with intestinal ischaemia is a poor prognostic factor for survival [5]. Similarly, a recent survival analysis of all SI NETs from our centre [13] showed that the median OS was longer (10.7 years), suggesting that the subset of patients with mesenteric fibrosis may have distinct prognostic features that merit separate consideration.…”

Section: Discussionmentioning

confidence: 99%

“…It may be due to a variety of processes such as the mass effect, bowel ischaemia, or obstruction. Encasement of the superior mesenteric artery or its branches may lead to bowel ischaemia, which is associated with poor outcome [5]. Involvement of the superior mesenteric vein may lead to ascites, ectopic small bowel varices, and venous ischaemia, resulting in bowel wall oedema and, ultimately, malabsorption, malnutrition, and cachexia.…”

Section: Introductionmentioning

confidence: 99%

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Prognostic Factors for Survival among Patients with Small Bowel Neuroendocrine Tumours Associated with Mesenteric Desmoplasia

et al. 2018

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Background: Small intestinal neuroendocrine tumours (SI NETs) represent 30–50% of small bowel neoplasms and are often associated with diverse fibrotic complications. Mesenteric fibrosis is a hallmark of SI NETs which may cause substantial morbidity and is considered an adverse feature. However, survival analyses in this group of patients are lacking. Methods: The aim of this retrospective study was to determine the overall survival (OS) and factors affecting prognosis in a large cohort of 147 patients with SI NETs and radiological evidence of mesenteric desmoplasia from our centre. The severity of desmoplasia was graded radiologically and its effect on OS and long-term complications was assessed. The median follow-up period was 82 months. Results: The median OS was 8.7 years (95% CI 6.8–9.9) with an overall 5-year survival of 71%. The univariate analysis demonstrated that an age >65 years, a liver tumour burden >50% of the hepatic parenchyma, carcinoid heart disease, chromogranin A levels >10 times the upper limit of normal, and urinary 5-hydroxyindoleacetic acid (5-HIAA) levels >5 times the upper limit of normal were poor prognosticators, while primary resection was associated with a longer OS. However, only an age >65 years and urinary 5-HIAA levels >10 times the upper limit of normal remained statistically significant after multivariate analysis. The severity of mesenteric desmoplasia did not seem to demonstrate a statistically significant relationship to OS or long-term outcomes. Conclusion: This study is the first comprehensive survival analysis of patients with SI NETs associated with mesenteric desmoplasia and has provided important and clinically relevant epidemiological data for this group of patients.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Prognostic Factors for Survival among Patients with Small Bowel Neuroendocrine Tumours Associated with Mesenteric Desmoplasia

et al. 2018

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“…Hidrosel, varikosel, testiküler ağrı, poliüri ve artmış idrar sıklığı, bacaklarda klaudikasyon ve ödem ve kabızlık saptanabilir (11). Hastalığın yaklaşık % 10'u malignensiye sekonder RPF olduğu için bu malignensiye spesifik belirtilerle karşılaşmak olasıdır (12)(13)(14). Serimizde öz geçmişinde bir malignite hikayesi olan hasta oranı % 15 olarak saptandı.…”

Section: Discussionunclassified

Retroperitoneal Fibrozis Tedavisi Deneyimi: Marmara Üniversitesi Üroloji ve Nefroloji Bölümlerinin 26 Yıllık Deneyimi

Sekerci¹,

Tanıdır²,

Özgen³

et al. 2017

Turk Neph Dial Transpl

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BUlgUlAr: Hastaların beşi kadın, 15'i erkek; ortalama takip süresi 11,3 yıldı (1-18 yıl). Yirmi hastanın altısına unilateral, beşine bilateral, toplam 11 (%55) hastaya üreterolizis uygulandı. Tanı anındaki ortanca kreatinin değerleri kadınlarda 2,75 mg/dl (0,7 -6,9 mg/dl); erkeklerde 3,2 mg/dl (0,84 -7,2 mg/dl), tüm grupta ortanca 3,11 mg/dl (0,7 -7,2 mg/dl) olarak saptandı. Cerrahi (n=11) sonrası ortanca bazal kreatinin değeri 3,63 mg/dl'den (1,4-7,2 mg/dl) 1,46 mg/dl'ye (0,8 -2,6 mg/dl) geriledi. Cerrahi uygulanan ve uygulanmayan hastaların bazal kreatinin değerleri ve düşüşleri kıyaslandığında istatistiksel fark ortaya konulamamıştır. SOnUÇ:Retroperitoneal fibrosis nedeniyle uygulanan tedavi seçenekleri cerrahi ve farmakolojik tedaviler olmak üzere ikiye ayrılır. Çalışmamız, hastaların her iki tedavi yönteminden de benzer şekilde fayda gördüklerini göstermektedir. Tedavi seçimi her hastaya özel olmalıdır ve multidisipliner düzenlenmelidir.AnAhTAr SÖzcükler: Retroperitoneal fibrozis, Kreatinin, Üreterolizis, Tamoksifen ABSTrAcT OBJecTIVe: Retroperitoneal fibrosis (RPF) is a rare condition characterized by the presence of inflammation and fibrosis in the retroperitoneum. This study describes our multidisciplinary approach and our results. MATerIAl and MeThOdS:Patients diagnosed with RPF at Marmara University between January 1990 and May 2007 were evaluated. Etiologies, demographic characteristics, renal functions, treatments and prognoses of each patient were recorded and data were analyzed. reSUlTS: Of all patients, five were female, 15 were male and the mean follow-up was 11.3 years (1 -18 years). Six patients were operated unilaterally and five were operated bilaterally with ureterolysis.Median creatinine values at diagnosis were 2.75 mg/dl (0.7-6.9 mg/dl) in females, 3.2 mg/dl (0.84-7.2 mg/dl) in males and 3.11 mg/dl (0.7 -7.2 mg/dl) in the whole group. Median baseline creatinine level after surgery (n=11) was found to decrease from 3.63 mg/dl (1.4-7.2 mg/dl) to 1.46 mg/dl (0.8-2.6 mg/dl). Baseline and drop in creatinine were compared among patients treated with either surgical or pharmacological methods, and no statistical significance was found.cOnclUSIOn: Retroperitoneal fibrosis can be treated surgically and pharmacologically. Our study has shown similar success for both methods. Treatment should be specific for each patient in a multidisciplinary fashion.

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“…Occasionally, distant para-aortic lymph node metastases are present and may accompany extensive retroperitoneal fibrosis, particularly in the subset of patients with NENs of small intestine origin [15]. Nevertheless, distant para-aortic lymph node metastases have previously been confirmed as an independent negative prognostic factor for survival in NENs of the small intestine [2].…”

Section: Discussionmentioning

confidence: 99%

Lung Metastases in Patients with Well-Differentiated Gastroenteropancreatic Neuroendocrine Neoplasms: An Appraisal of the Validity of Thoracic Imaging Surveillance

et al. 2019

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Background/Aims: To evaluate the impact of lung metastases (LM) on overall survival (OS) in well-differentiated (WD) stage IV gastroenteropancreatic neuroendocrine neoplasms (GEP-NEN) patients along with developing surveillance strategies for thoracic imaging. Methods: Thirty-four patients with LM, from 3 centres, were identified (22 small intestine/12 pancreatic; 17 grade 1/15 grade 2/2 of unknown grade). For comparison, we used 106 stage IV WD, grade 1 and 2 GEP-NEN patients with metastatic disease confined in the abdomen. Results: LM prevalence was 4.9% (34/692). Eleven patients (32%) presented with synchronous LM whereas 23 (68%) developed metachronous LM at a median of 25 months (range 1–150 months). Patients with metachronous LM had already established liver and/or para-aortic lymph node metastases. Eighteen of 23 patients (78%) with metachronous LM exhibited concomitant progression in the abdomen. Median OS of WD GEP-NEN patients with LM was shorter than for those with stage IV disease without extra-abdominal metastases (56 [95% CI 40.6–71.6] vs. 122.7 [95% CI 70.7–174.8] months; log-rank p = 0.001). Among patients with progressive stage IV disease, the subset of patients with LM exhibited shorter OS (log-rank p = 0.005). LM were also confirmed as an independent prognostic factor for survival in multivariable analysis (HR 0.18; 95% CI 0.07–0.45; p < 0.0001). Conclusion: LM, although relatively rare in patients with WD stage IV GEP-NENs, may impact patients’ outcome. The development of metachronous LM is associated with concomitant disease progression in established abdominal metastases in most patients. These patient-related parameters could be utilized for a stratified surveillance approach, mainly reserving thoracic imaging for GEP-NEN patients with progressive disease in the abdomen.

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Clinical signs of fibrosis in small intestinal neuroendocrine tumours

Abstract: Extensive abdominal fibrosis associated with clinically significant symptoms of intestinal ischaemia and/or obstructive uropathy was linked to advanced disease in patients with SI-NETs. Prompt recognition and minimally invasive intervention was effective in disease palliation.

Cited by 70 publications

References 24 publications

Prognostic Factors for Survival among Patients with Small Bowel Neuroendocrine Tumours Associated with Mesenteric Desmoplasia

Prognostic Factors for Survival among Patients with Small Bowel Neuroendocrine Tumours Associated with Mesenteric Desmoplasia

Retroperitoneal Fibrozis Tedavisi Deneyimi: Marmara Üniversitesi Üroloji ve Nefroloji Bölümlerinin 26 Yıllık Deneyimi

Lung Metastases in Patients with Well-Differentiated Gastroenteropancreatic Neuroendocrine Neoplasms: An Appraisal of the Validity of Thoracic Imaging Surveillance

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