How wide do we cast a net? Neuroendocrine tumours in lymph nodes without a primary — ASN Events
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How wide do we cast a net? Neuroendocrine tumours in lymph nodes without a primary (#12)

Jinghang Luo 1 , Amas Lee 2 , Peter Fuller 3 4 , Frances Milat 3 4
  1. Endocrinology and Diabetes, Western Health, Melbourne, VIC, Australia
  2. Monash Pathology, Monash Health, Melbourne, VIC, Australia
  3. Hudson Institute of Medical Research, Melbourne, VIC, Australia
  4. Endocrinology and Diabetes, Monash Health, Melbourne, VIC, Australia

A 45-year-old Sudanese man was referred for investigation of a possible neuroendocrine tumour in the setting of recurrent abdominal pain, profuse vomiting and diarrhoea of 10-20 episodes daily with two Gallium-68 DOTATATE-avid peri-pancreatic lymph nodes. This was his fifth admission in three months, requiring intensive care for renal replacement therapy due to acute kidney injury as well as total parenteral nutrition due to nasogastric losses totalling 4–8 L/day which eventually responded to empirical octreotide.

 

His medical history included diet-controlled T2DM, alcohol excess, hypertension and thalassaemia minor. He had 22 similar but milder admissions over 5 years, each lasting 1–3 days; cross-sectional imaging had only shown eccentric gastric thickening and mildly prominent peri-pancreatic nodes, and gastroscopies had shown duodenitis and gastritis. He had no family history. His medications on admission included telmisartan, amlodipine, nizatidine, magnesium, thiamine, vitamin D and a multivitamin; octreotide and pantoprazole had been commenced as an inpatient. Examination was unremarkable apart from an elevated BMI of 30kg/m2.

Repeat abdominal CT scans showed circumferential gastric thickening, a distended stomach and jejunum; two 18mm peri-pancreatic nodes were noted. Diagnostic laparoscopy, gastroscopies and colonoscopy only demonstrated duodenitis. A DOTATATE-PET, prompted by a rapid response to octreotide, showed avidity in the two nodes; however, endoscopic fine needle aspiration was non-diagnostic due to the absence of lymphoid cells. Repeat gastroscopy showed superficial duodenal ulcers despite maximal therapy; capsule endoscopy was non-contributory.

Chromogranin A and gastrin levels during admission peaked at >15,200mcg/L [range 27–94] and 8121pmol/L [range 5–55] respectively, before reaching a nadir of 352 and 128 after withdrawal of pantoprazole. One VIP measurement and 24-hour urinary 5HIAA levels were normal. MRE enterography only showed prominent gastric folds, and an 18-FDG-PET scan showed only mild avidity in one of the nodes.

Further diagnostic tests were sent, and surgery was planned.

  1. 1. Pavel M, Öberg K, Falconi M, Krenning EP, Sundin A, Perren A, Berruti A; ESMO Guidelines Committee. Electronic address: clinicalguidelines@esmo.org. Gastroenteropancreatic neuroendocrine neoplasms: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2020 Jul;31(7):844-860. doi: 10.1016/j.annonc.2020.03.304. Epub 2020 Apr 6. PMID: 32272208.
  2. 2. Sansone A, Lauretta R, Vottari S, Chiefari A, Barnabei A, Romanelli F, Appetecchia M. Specific and Non-Specific Biomarkers in Neuroendocrine Gastroenteropancreatic Tumors. Cancers (Basel). 2019 Aug 4;11(8):1113. doi: 10.3390/cancers11081113. PMID: 31382663; PMCID: PMC6721814.
  3. 3. Ohsawa T, Hirata W, Higichi S. Effects of three H2-receptor antagonists (cimetidine, famotidine, ranitidine) on serum gastrin level. Int J Clin Pharmacol Res. 2002;22(2):29-35. PMID: 12503773.
  4. 4. Jensen RT, Cadiot G, Brandi ML, de Herder WW, Kaltsas G, Komminoth P, Scoazec JY, Salazar R, Sauvanet A, Kianmanesh R; Barcelona Consensus Conference participants. ENETS Consensus Guidelines for the management of patients with digestive neuroendocrine neoplasms: functional pancreatic endocrine tumor syndromes. Neuroendocrinology. 2012;95(2):98-119. doi: 10.1159/000335591. Epub 2012 Feb 15. PMID: 22261919; PMCID: PMC3701449.
  5. 5. Metz DC, Cadiot G, Poitras P, Ito T, Jensen RT. Diagnosis of Zollinger-Ellison syndrome in the era of PPIs, faulty gastrin assays, sensitive imaging and limited access to acid secretory testing. Int J Endocr Oncol. 2017;4(4):167-185. doi: 10.2217/ije-2017-0018. Epub 2017 Oct 11. PMID: 29326808; PMCID: PMC5757869.
  6. 6. Korse CM, Muller M, Taal BG. Discontinuation of proton pump inhibitors during assessment of chromogranin A levels in patients with neuroendocrine tumours. Br J Cancer. 2011 Oct 11;105(8):1173-5. doi: 10.1038/bjc.2011.380. PMID: 21989216; PMCID: PMC3208502.
  7. 7. Rinke A, Müller HH, Schade-Brittinger C, Klose KJ, Barth P, Wied M, Mayer C, Aminossadati B, Pape UF, Bläker M, Harder J, Arnold C, Gress T, Arnold R; PROMID Study Group. Placebo-controlled, double-blind, prospective, randomized study on the effect of octreotide LAR in the control of tumor growth in patients with metastatic neuroendocrine midgut tumors: a report from the PROMID Study Group. J Clin Oncol. 2009 Oct 1;27(28):4656-63. doi: 10.1200/JCO.2009.22.8510. Epub 2009 Aug 24. PMID: 19704057.
  8. 8. Stabile BE, Morrow DJ, Passaro E Jr. The gastrinoma triangle: operative implications. Am J Surg. 1984 Jan;147(1):25-31. doi: 10.1016/0002-9610(84)90029-1. PMID: 6691547.
  9. 9. Norton JA, Fraker DL, Alexander HR, Venzon DJ, Doppman JL, Serrano J, Goebel SU, Peghini PL, Roy PK, Gibril F, Jensen RT. Surgery to cure the Zollinger-Ellison syndrome. N Engl J Med. 1999 Aug 26;341(9):635-44. doi: 10.1056/NEJM199908263410902. PMID: 10460814.
  10. 10. Chen Y, Deshpande V, Ferrone C, Blaszkowsky LS, Parangi S, Warshaw AL, Lillemoe KD, Fernandez-Del Castillo C. Primary lymph node gastrinoma: A single institution experience. Surgery. 2017 Nov;162(5):1088-1094. doi: 10.1016/j.surg.2017.05.017. Epub 2017 Jul 10. PMID: 28705492.