Corticotropin secreting pancreatic neuroendocrine tumour, a therapeutic management challenge. A presentation of 2 cases

Simó-Servat, Andreu; Peiró, Inmaculada; Villabona, Carles

doi:10.1016/j.endien.2018.09.001

Cited by 2 publications

(6 citation statements)

References 5 publications

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“…In non-operable cases, the aim of the treatment is to inhibit hormone secretion and to prevent tumour progression. Changes in tumour functionality due to cell pluripotency can complicate management ( 1 ). As in this case, tumours expressing somatostatin receptor 2 and 5 can be treated with somatostatin analogues, which inhibit both tumour growth and hormone secretion ( 1 ).…”

Section: Discussionmentioning

confidence: 99%

“…Changes in tumour functionality due to cell pluripotency can complicate management ( 1 ). As in this case, tumours expressing somatostatin receptor 2 and 5 can be treated with somatostatin analogues, which inhibit both tumour growth and hormone secretion ( 1 ). Systemic anti-cancer therapy involves conventional chemotherapy, mechanistic target of rapamycin (mTOR) inhibitors, small molecule tyrosine kinase inhibitors or radionucleotide therapy when somatostatin receptor scintigraphy or 68Ga-DOTA-peptide-PET/CT is positive.…”

Section: Discussionmentioning

confidence: 99%

“…If this is proven ineffective, bilateral adrenalectomy may be required, and this has been shown to improve survival in the first 2 years ( 14 ). However overall, there is a lack of evidence-based treatment strategies for ACTH-secreting p-NETs and further studies are needed to establish the best treatment approaches and to improve patient outcomes ( 1 ).…”

Section: Discussionmentioning

confidence: 99%

“…Approximately 1% of all pancreatic tumours are functioning neuroendocrine tumours (p-NETs) ( 1 ). P-NETs are known to secrete a variety of hormones including insulin, gastrin, vasoactive intestinal peptide (VIP), somatostatin, glucagon, pancreatic polypeptide (PP) and more rarely adrenocorticotropic hormone (ACTH).…”

Section: Introductionmentioning

confidence: 99%

“…Patients usually develop profound metabolic disturbance with alkalosis, hypokalaemia, hypertensive crisis and hyperglycaemia. When surgical resection of p-NETs is not achievable, there are limited effective systemic therapies and their management is often challenging ( 1 ).…”

Section: Introductionmentioning

confidence: 99%

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Posterior reversible encephalopathy syndrome in a patient with a metastatic pancreatic neuroendocrine tumour and ectopic Cushing’s syndrome

Palmer

Weerasuriya

Whitelaw

et al. 2021

Endocrinology, Diabetes &Amp; Metabolism Case Reports

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Summary We report a rare case of posterior reversible encephalopathy syndrome (PRES), precipitated by ectopic Cushing’s syndrome, in a patient with a metastatic pancreatic neuroendocrine tumour. A 55-year-old female presented as a hypertensive emergency with seizures and severe biochemical disturbance, including alkalosis, hypokalaemia and hyperglycaemia. MRI showed vasogenic oedema in the parieto-occipital region, consistent with a diagnosis of PRES. She had a significantly raised serum cortisol (>6000 nmol/L) which did not suppress with dexamethasone. Plasma adrenocorticotropic hormone (ACTH) concentrations were neither suppressed nor raised but were consistently within the normal reference range. The unexpected finding of a normal ACTH may be explained by either tumour secretion of unmeasured ACTH-related peptides, immunoassay antibody interference or episodic ACTH secretion. PRES is usually reversible with prompt and appropriate treatment. Hypercortisolism associated PRES is rare and may be associated with a worse outcome. Learning points PRES secondary to ectopic Cushing’s syndrome is very rare. PRES in this context may indicate a worse prognosis. In ectopic Cushing’s syndrome, if the serum ACTH level is normal, consider testing for ACTH-related peptides or interfering antibodies. Further research is required to establish the best treatment approach and to improve patients’ outcomes.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 3 more Smart Citations

Posterior reversible encephalopathy syndrome in a patient with a metastatic pancreatic neuroendocrine tumour and ectopic Cushing’s syndrome

Palmer

Weerasuriya

Whitelaw

et al. 2021

Endocrinology, Diabetes &Amp; Metabolism Case Reports

View full text Add to dashboard Cite

show abstract

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

Weerasuriya

Palmer

Gregory

et al. 2021

Case Rep Gastroenterol

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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.

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Corticotropin secreting pancreatic neuroendocrine tumour, a therapeutic management challenge. A presentation of 2 cases

Cited by 2 publications

References 5 publications

Posterior reversible encephalopathy syndrome in a patient with a metastatic pancreatic neuroendocrine tumour and ectopic Cushing’s syndrome

Posterior reversible encephalopathy syndrome in a patient with a metastatic pancreatic neuroendocrine tumour and ectopic Cushing’s syndrome

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

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