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

2021 ◽  
Vol 2021 ◽  
Author(s):  
Kieran Palmer ◽  
Scott Weerasuriya ◽  
Benjamin Whitelaw ◽  
Rajaventhan Srirajaskanthan
Keyword(s):  
Cushing’S Syndrome ◽  
Posterior Reversible Encephalopathy Syndrome ◽  
Neuroendocrine Tumour ◽  
Cushing's Syndrome ◽  
Unexpected Finding ◽  
List Type ◽  
Posterior Reversible Encephalopathy ◽  
Ectopic Cushing’S Syndrome ◽  
Pancreatic Neuroendocrine Tumour ◽  
Reversible Encephalopathy

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.

scholarly journals Functional pancreatic neuroendocrine tumour causing Cushing’s syndrome: the effect of chemotherapy on clinical symptoms

2017 ◽  
Vol 11 ◽  
Author(s):  
Paulo Henrique do Amor Divino ◽  
Katia Regina Marchetti ◽  
Madson Q Almeida ◽  
Rachel P Riechelmann
Keyword(s):  
Cushing’S Syndrome ◽  
Clinical Symptoms ◽  
Neuroendocrine Tumour ◽  
Cushing's Syndrome ◽  
Pancreatic Neuroendocrine Tumour

scholarly journals A critical reappraisal of bilateral adrenalectomy for ACTH-dependent Cushing's syndrome

2015 ◽  
Vol 173 (4) ◽  
pp. M23-M32 ◽  
Author(s):  
Martin Reincke ◽  
Katrin Ritzel ◽  
Andrea Oßwald ◽  
Christina Berr ◽  
Günter Stalla ◽  
...  
Keyword(s):  
Cushing’S Syndrome ◽  
Neuroendocrine Tumour ◽  
Cushing's Syndrome ◽  
Bilateral Adrenalectomy ◽  
Treatment Modalities ◽  
Adrenal Crisis ◽  
Surgical Morbidity ◽  
Ectopic Cushing’S Syndrome ◽  
Long Term Mortality

ObjectiveOur aim was to review short- and long-term outcomes of patients treated with bilateral adrenalectomy (BADx) in ACTH-dependent Cushing's syndrome.MethodsWe reviewed the literature and analysed our experience with 53 patients treated with BADx since 1990 in our institution.ResultsBADx is considered if ACTH-dependent Cushing's syndrome is refractory to other treatment modalities. In Cushing's disease (CD), BADx is mainly used as an ultima ratio after transsphenoidal surgery and medical therapies have failed. In these cases, the time span between the first diagnosis of CD and treatment with BADx is relatively long (median 44 months). In ectopic Cushing's syndrome, the time from diagnosis to BADx is shorter (median 2 months), and BADx is often performed as an emergency procedure because of life-threatening complications of severe hypercortisolism. In both situations, BADx is relatively safe (median surgical morbidity 15%; median surgical mortality 3%) and provides excellent control of hypercortisolism; Cushing's-associated signs and symptoms are rapidly corrected, and co-morbidities are stabilised. In CD, the quality of life following BADx is rapidly improving, and long-term mortality is low. Specific long-term complications include the development of adrenal crisis and Nelson's syndrome. In ectopic Cushing's syndrome, long-term mortality is high but is mostly dependent on the prognosis of the underlying malignant neuroendocrine tumour.ConclusionBADx is a relatively safe and highly effective treatment, and it provides adequate control of long-term co-morbidities associated with hypercortisolism.

Ectopic Cushing’s syndrome due to thymic neuroendocrine tumours: a systematic review

2021 ◽  
Author(s):  
Fernando Guerrero-Pérez ◽  
Inmaculada Peiró ◽  
Agustina Pia Marengo ◽  
Alex Teulé ◽  
José Carlos Ruffinelli ◽  
...  
Keyword(s):  
Systematic Review ◽  
Cushing’S Syndrome ◽  
Neuroendocrine Tumours ◽  
Cushing's Syndrome ◽  
Ectopic Cushing’S Syndrome

Rare case of pancreatic neuroendocrine tumour presenting as paraneoplastic hypercalcaemia

2021 ◽  
Vol 14 (4) ◽  
pp. e240786
Author(s):  
Benjamin Ferrel ◽  
Jan Franko ◽  
May C Tee
Keyword(s):  
Parathyroid Hormone ◽  
Surgical Management ◽  
Rare Case ◽  
Neuroendocrine Tumours ◽  
Neuroendocrine Tumour ◽  
Related Peptide ◽  
Pancreatic Neuroendocrine Tumours ◽  
Successful Removal ◽  
Pancreatic Neuroendocrine Tumour ◽  
Parathyroid Hormone Related Peptide

An asymptomatic 68-year-old woman who presented with an isolated hypercalcaemia was diagnosed with a rare, previously unsuspected parathyroid hormone-related peptide (PTHrP)-producing pancreatic neuroendocrine tumour. She underwent an extensive operation including vascular resection and reconstruction, resulting in successful removal of the tumour with negative margins. Medical and surgical management of pancreatic neuroendocrine tumours and PTHrP-mediated paraneoplastic hypercalcaemia is discussed.

scholarly journals Evolution in functionality of a metastatic pancreatic neuroendocrine tumour (pNET) causing Cushing’s syndrome: treatment response with chemotherapy

2014 ◽  
Vol 14 (1) ◽  
Author(s):  
Surya Panicker Rajeev ◽  
Steffan McDougall ◽  
Monica Terlizzo ◽  
Daniel Palmer ◽  
Christina Daousi ◽  
...  
Keyword(s):  
Treatment Response ◽  
Cushing’S Syndrome ◽  
Neuroendocrine Tumour ◽  
Cushing's Syndrome ◽  
Pancreatic Neuroendocrine Tumour

scholarly journals A thymic neuroendocrine tumour in a young female: a rare cause of relapsing and remitting Cushing’s syndrome

2016 ◽  
Vol 2016 ◽  
Author(s):  
M J Trott ◽  
G Farah ◽  
V J Stokes ◽  
L M Wang ◽  
A B Grossman
Keyword(s):  
Cushing’S Syndrome ◽  
Neuroendocrine Tumours ◽  
Neuroendocrine Tumour ◽  
Cushing's Syndrome ◽  
Young Female ◽  
Diagnostic Workup ◽  
Acth Secretion ◽  
Ectopic Acth ◽  
Ectopic Acth Secretion ◽  
Men 1

Summary We present a case of a young female patient with a rare cause of relapsing and remitting Cushing’s syndrome due to ectopic ACTH secretion from a thymic neuroendocrine tumour. A 34-year-old female presented with a constellation of symptoms of Cushing’s syndrome, including facial swelling, muscle weakness and cognitive impairment. We use the terms ‘relapsing and remitting’ in this case report, given the unpredictable time course of symptoms, which led to a delay of 2 years before the correct diagnosis of hypercortisolaemia. Diagnostic workup confirmed ectopic ACTH secretion, and a thymic mass was seen on mediastinal imaging. The patient subsequently underwent thymectomy with complete resolution of her symptoms. Several case series have documented the association of Cushing’s syndrome with thymic neuroendocrine tumours (NETs), although to our knowledge there are a few published cases of patients with relapsing and remitting symptoms. This case is also notable for the absence of features of the MEN-1 syndrome, along with the female gender of our patient and her history of non-smoking. Learning points Ectopic corticotrophin (ACTH) secretion should always be considered in the diagnostic workup of young patients with Cushing’s syndrome There is a small but growing body of literature describing the correlation between ectopic ACTH secretion and thymic neuroendocrine tumours (NETs) The possibility of a MEN-1 syndrome should be considered in all patients with thymic NETs, and we note the observational association with male gender and cigarette smoking in this cohort An exception to these associations is the finding of relatively high incidence of thymic NETs among female non-smoking MEN-1 patients in the Japanese compared with Western populations The relapsing and remitting course of our patient’s symptoms is noteworthy, given the paucity of this finding among other published cases

scholarly journals Ectopic Cushing’s syndrome due to a mesenteric neuroendocrine tumour

2012 ◽  
Vol 94 (8) ◽  
pp. e20-e22 ◽  
Author(s):  
N Mashoori ◽  
AH Rabani ◽  
AR Kazemeini
Keyword(s):  
Small Intestine ◽  
Cushing’S Syndrome ◽  
Neuroendocrine Tumour ◽  
Treatment Options ◽  
Hormone Secretion ◽  
Cushing's Syndrome ◽  
Gastrointestinal System ◽  
Tumour Resection ◽  
Similar Treatment ◽  
Ectopic Cushing’S Syndrome

INTRODUCTION Neuroendocrine tumours (NETs) are tumours that commonly involve the gastrointestinal system. Common primary sites in the gastrointestinal system include the small intestine, appendix, rectum and pancreas. Mesenteric NETs are extremely rare entities and are sparsely reported in the literature. CASE HISTORY We report the case of a 62-year-old woman with ectopic Cushing’s syndrome due to excessive adrenocorticotropic hormone secretion by a primary mesenteric tumour in the small intestine and its liver metastases. CONCLUSIONS Although rare, the mesentery can be a primary site for NETs. It can cause similar symptoms and require similar treatment options. Tumour resection and debulking are acceptable ways to improve both the survival and symptoms.

Sign in / Sign up

Export Citation Format

Share Document