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

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.

Duodenal Gastrointestinal Stromal Tumour Imitating as Pancreatic Head Tumour: A Typical Case of Whodunit

Duodenal gastrointestinal stromal tumours (D-GISTs) are rare disease. It may arise commonly from second or third part of the duodenum and can be erroneously diagnosed as a pancreatic head tumour due to proximity and morphology on imaging studies. We present a case of a sixty-year-old woman who presented with abdominal pain and was diagnosed as a case of pancreatic neuroendocrine tumour on radiologic imaging and granulomatous lesion on aspiration cytology. On laparotomy, a ~5x3 cm mass was noted in the pancreatic head and pancreatoduodenectomy was performed. Histopathology reported an exophytic GIST arising from the second part of the duodenum.Hence, D-GIST can invade into the pancreas and mimic pancreatic head tumour; therefore, these tumours should be kept in the differential diagnosis of an atypical pancreatic head mass.

Multiple primary malignancies: synchronous lymphoma, pancreatic neuroendocrine tumour and colorectal cancer

A 67-year-old woman underwent a medical check-up by her general practitioner after complaining of atypical pain in the shoulder girdle. Due to the important inflammatory syndrome noticed on blood testing, a polymyalgia rheumatica was suspected and she was started on corticosteroid treatment with good clinical response, but no impact on inflammation. She underwent extensive imaging with a thoraco-abdominal CT scanner that demonstrated a pancreatic mass, then later a PET-CT showed 3 different hyperactive lesions. Biopsies then revealed simultaneous diffuse large B-cell lymphoma (DLBCL), colorectal adenocarcinoma and pancreatic neuroendocrine tumour. She benefited from low rectal endoscopic excision of the colorectal tumour, R-CHOP chemotherapy for DLBCL and laparoscopic left pancreatectomy. Successful treatment required a good multidisciplinary collaboration between the different specialists. The patient made a good recovery and achieved complete remission at 1 year. This an unusual presentation of multiple primary malignancies.

Castleman’s disease masquerading as pancreatic neuroendocrine tumour

Castleman’s disease (CD) is a rare lymphoproliferative disorder. This case report, to the best of our knowledge, is the first report of CD simulating a pancreatic neuroendocrine tumour . The patient was a 58-year-old woman who initially presented with bilateral iritis and underwent investigation for possible systemic rheumatological disease. CT of the chest demonstrated an incidental finding of a well-demarcated retropancreatic mass. As the mass was found to enhance on DOTATATE (tetraazacyclododecanetetraacetic acid-DPhe1-Tyr3-octreotate) positron emission tomography, a diagnosis of pancreatic neuroendocrine tumour was made. The patient underwent an open distal pancreatectomy and splenectomy. Histopathological examination revealed the unexpected diagnosis of hyaline vascular CD of a lymph node posterior to the pancreas. After 2 years of follow-up, there is no evidence of disease recurrence.

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

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.

Rare case of pancreatic neuroendocrine tumour presenting as paraneoplastic hypercalcaemia

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.