Marked improvement in hyperammonaemic encephalopathy from multimodal treatment of metastatic neuroendocrine tumour

2021 ◽  
Vol 14 (6) ◽  
pp. e241191
Author(s):  
Alexander Davis ◽  
Stephen Clarke ◽  
Sally Ayesa ◽  
David L Chan
Keyword(s):  
Poor Prognosis ◽  
Neuroendocrine Tumours ◽  
Treatment Approach ◽  
Neuroendocrine Tumour ◽  
Multimodality Treatment ◽  
Peptide Receptor ◽  
The Poor ◽  
Heterogenous Group ◽  
Gastroenteropancreatic Neuroendocrine Tumours ◽  
New Diagnosis

Gastroenteropancreatic neuroendocrine tumours (GEPNETs) are a heterogenous group of tumours which are rising in incidence. Morbidity and mortality related to these tumours is dependent on the location of metastatic spread. Hyperammonaemia and subsequent encephalopathy has previously been described in GEPNET and is typically associated with a poor prognosis. We describe a case of a 55-year-old woman with hyperammonaemic encephalopathy and a new diagnosis of GEPNET. Given the poor prognosis and the outcomes in this patient group we feel this case highlights the benefit of a multimodality treatment approach including peptide receptor radionucleotide therapy and transarterial chemoembolisation.

scholarly journals The multidisciplinary team for gastroenteropancreatic neuroendocrine tumours: the radiologist’s challenge

2019 ◽  
Vol 53 (4) ◽  
pp. 373-387 ◽  
Author(s):  
Vincenza Granata ◽  
Roberta Fusco ◽  
Sergio Venanzio Setola ◽  
Elisabetta de Lutio di Castelguidone ◽  
Luigi Camera ◽  
...  
Keyword(s):  
Multidisciplinary Team ◽  
Neuroendocrine Tumours ◽  
Clinical Symptoms ◽  
Neuroendocrine Tumour ◽  
Imaging Techniques ◽  
Critical Role ◽  
Nuclear Imaging ◽  
Distant Metastases ◽  
Gastroenteropancreatic Neuroendocrine Tumours ◽  
Effective Diagnosis

Abstract Background Gastroenteropancreatic neuroendocrine tumours (GEP-NETs) are a heterogeneous group of tumours. An effective diagnosis requires a multimodal approach that combines evaluation of clinical symptoms, hormonelevels, radiological and nuclear imaging, and histological confirmation. Imaging plays a critical role in NETs diagnosis, prognosis and management, so the radiologists are important members of the multidisciplinary team. During diagnostic work-up two critical issues are present: firstly the need to identify tumor presence and secondly to define the primary site and assess regional and distant metastases. Conclusions The most appropriate imaging technique depends on the type of neuroendocrine tumour and the availability of specialized imaging techniques and expertise. There is no general consensus on the most efficient imaging pathway, reflecting the challenge in reliably detection of these tumours.

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.

Small cell carcinoma of the pancreas: a rare neuroendocrine tumour

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.

scholarly journals Gastroenteropancreatic neuroendocrine tumours: the current incidence and staging based on the WHO and European Neuroendocrine Tumour Society classification: an analysis based on prospectively collected parameters

2010 ◽  
Vol 17 (4) ◽  
pp. 909-918 ◽  
Author(s):  
Martin B Niederle ◽  
Monika Hackl ◽  
Klaus Kaserer ◽  
Bruno Niederle
Keyword(s):  
Digestive Tract ◽  
Neuroendocrine Tumours ◽  
Neuroendocrine Tumour ◽  
Tnm Staging ◽  
Incidence Rates ◽  
Neuroendocrine Cells ◽  
Malignant Tumours ◽  
Main Site ◽  
Well Differentiated ◽  
Gastroenteropancreatic Neuroendocrine Tumours

As incidence data on gastroenteropancreatic neuroendocrine tumours (GEP-NETs) have so far only been retrospectively obtained and based on inhomogeneous material, we conducted a prospective study in Austria collecting all newly diagnosed GEP-NETs during 1 year. Using the current WHO classification, the tumor, nodes, metastases (TNM) staging and Ki67 grading and the standard diagnostic procedure proposed by the European Neuroendocrine Tumor Society (ENETS), GEP-NETs from 285 patients (male: 148; female: 137) were recorded. The annual incidence rates were 2.51 per 100 000 inhabitants for men, 2.36 per 100 000 for women. The stomach (23%) was the main site, followed by appendix (21%), small intestine (15%) and rectum (14%). Patients with appendiceal tumours were significantly younger than patients with tumours in any other site. About 46.0% were classified as benign, 15.4% as uncertain, 31.9% as well differentiated malignant and 6.7% as poorly differentiated malignant. Patients with benign or uncertain tumours were significantly younger than patients with malignant tumours. Among the malignant tumours of the digestive tract, 1.49% arose from neuroendocrine cells. For malignant gastrointestinal NETs, the incidence was 0.80 per 100 000: 40.9% were ENETS stage I, 23.8% stage II, 11.6% stage III and 23.8% stage IV. The majority (59.7%) were grade 1, 31.2% grade 2 and 9.1% grade 3. NETs of the digestive tract are more common than previously reported; the majority show benign behaviour, are located in the stomach and are well differentiated. G3 tumours are very rare.

Can peptide receptor radionuclide therapy be safely applied in florid bone metastases?

2014 ◽  
Vol 53 (02) ◽  
pp. 54-59 ◽  
Author(s):  
A. Sabet ◽  
F. Khalaf ◽  
C. J. Yong-Hing ◽  
A. Sabet ◽  
T. Haslerud ◽  
...  
Keyword(s):  
Bone Metastases ◽  
Bone Disease ◽  
Radionuclide Therapy ◽  
Neuroendocrine Tumours ◽  
Peptide Receptor Radionuclide Therapy ◽  
Metastatic Bone Disease ◽  
Minor Response ◽  
Peptide Receptor ◽  
Gastroenteropancreatic Neuroendocrine Tumours ◽  
A Minor

Summary Aim: Highly advanced metastatic bone disease with extensive osseous infiltration of neuroendocrine tumours (NET) may preclude patients from treatment with peptide receptor radionuclide therapy (PRRT) in concern about haematotoxicity. This study aims to assess the safety and efficacy of PRRT with 177Lu-octreotate in a patient cohort with this condition. Patients, methods: 41 PRRT courses were performed in 11 patients with gastroenteropancreatic neuroendocrine tumours (GEP-NET) and florid bone metastases (severely advanced widespread metastatic bone disease). A mean activity of 6.95 GBq 177Lu-octreotate was administered per treatment cycle, aimed at four courses with standard intervals of 3 months. Haematological parameters were determined prior to each treatment course, in 2-4 weeks intervals between the courses, 8-12 weeks after the last course of PRRT and in 3 monthly intervals thereafter. Toxicity was recorded using Common Terminology Criteria for Adverse Events v3.0. Restaging was performed 3 months after termination of PRRT with CT/MRI and functional imaging (modified MDA criteria). Results: Significant (grade III-IV), reversible haematotoxicity occurred in 4 (35%) patients and after 10 (24%) administrations. It either resolved spontaneously (1 patient) or was controlled by supportive measures (3 patients), such as blood transfusions (3 patients) or deferral of the subsequent therapy cycle (1 patient). Patients returned to baseline blood values within up to 23 months after termination of PRRT. The observed treatment response of bone metastases consisted of a partial response in 2, a minor response in 1, stable disease in 7, and progressive disease in 1 patient. Of the 4 patients with metastatic bone pain, 1 experienced complete and 3 partial resolution of symptoms within 3-10 weeks after commencement of PRRT. Conclusion: These preliminary data indicate that PRRT with 177Lu-octreotate can be safely applied even in florid bone metastases with extensive, severely advanced osseous replacement. The higher myelosuppression rate was not associated with serious complications and should not preclude patients from being treated and potentially experiencing remarkable treatment efficacy despite the very advanced stage.

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