open access

Vol 71, No 2 (2021)
Case report
Published online: 2021-01-25
Get Citation

Oligosymptomatic neuroendocrine neoplasm of the small intestine with metastases spread to the heart, bones, muscles and intraperitoneally after a few years in remission – diagnostic and therapeutic challenges

Natalia Tyrybon1, Agnieszka Żyłka1, Joanna Długosińska1, Małgorzata Benke1, Marek Dedecjus1
·
Nowotwory. Journal of Oncology 2021;71(2):111-114.
Affiliations
  1. Department of Endocrine Oncology and Nuclear Medicine, M. Sklodowska-Curie National Research Institute of Oncology, Warsaw, Poland

open access

Vol 71, No 2 (2021)
Case report
Published online: 2021-01-25

Abstract

A fifty-one-year-old male patient with a history of recurring abdominal pains and signs of subileus, without carcinoid syndrome signs, underwent a laparatomy with a resection of the small intestine segment. Histopathology revealed a well­-differentiated neuroendocrine neoplasm of the small intestine. Due to the lack of hormonal activity and low malignancy potential the patient was not qualified for adjuvant therapy. The yearly computed tomography did not indicate a recur­rence of the neoplasm. The patient did not report any “red flag” symptoms. After a few years in remission [68Ga]-DOTATATE PET/CT revealed a dynamic development of the illness. The patient was qualified for palliative treatment with long-acting somatostatin analogue. Due to the treatment’s ineffectiveness and further progression of the disease, the patient received Peptide Receptor Radionuclide Therapy (PRRT). In spite of the therapy his condition did not improve and progression was observed. The patient died because of a malfunction of the cardiac conduction system caused by metastases in the heart.

Abstract

A fifty-one-year-old male patient with a history of recurring abdominal pains and signs of subileus, without carcinoid syndrome signs, underwent a laparatomy with a resection of the small intestine segment. Histopathology revealed a well­-differentiated neuroendocrine neoplasm of the small intestine. Due to the lack of hormonal activity and low malignancy potential the patient was not qualified for adjuvant therapy. The yearly computed tomography did not indicate a recur­rence of the neoplasm. The patient did not report any “red flag” symptoms. After a few years in remission [68Ga]-DOTATATE PET/CT revealed a dynamic development of the illness. The patient was qualified for palliative treatment with long-acting somatostatin analogue. Due to the treatment’s ineffectiveness and further progression of the disease, the patient received Peptide Receptor Radionuclide Therapy (PRRT). In spite of the therapy his condition did not improve and progression was observed. The patient died because of a malfunction of the cardiac conduction system caused by metastases in the heart.

Get Citation

Keywords

neuroendocrine neoplasm; carcinoid syndrome; somatostatin analogue; radioisotope therapy

About this article
Title

Oligosymptomatic neuroendocrine neoplasm of the small intestine with metastases spread to the heart, bones, muscles and intraperitoneally after a few years in remission – diagnostic and therapeutic challenges

Journal

Nowotwory. Journal of Oncology

Issue

Vol 71, No 2 (2021)

Article type

Case report

Pages

111-114

Published online

2021-01-25

Page views

535

Article views/downloads

550

DOI

10.5603/NJO.a2021.0002

Bibliographic record

Nowotwory. Journal of Oncology 2021;71(2):111-114.

Keywords

neuroendocrine neoplasm
carcinoid syndrome
somatostatin analogue
radioisotope therapy

Authors

Natalia Tyrybon
Agnieszka Żyłka
Joanna Długosińska
Małgorzata Benke
Marek Dedecjus

References (15)
  1. Kos-Kudła B, Blicharz-Dorniak J, Strzelczyk J, et al. Consensus Conference, Polish Network of Neuroendocrine Tumours, oraz Pozostali Uczestnicy Konferencji Okragłego Stołu. [Diagnostic and therapeutic guidelines for gastrointestinal neuroendocrine tumors (recommended by the Polish Network of Neuroendocrine Tumors)]. Endokrynol Pol. 2008; 59(1): 41–56.
  2. Maggard MA, O'Connell JB, Ko CY. Updated population-based review of carcinoid tumors. Ann Surg. 2004; 240(1): 117–122.
  3. Rubin de Celis Ferrari AC, Glasberg J, Riechelmann RP. Carcinoid syndrome: update on the pathophysiology and treatment. Clinics (Sao Paulo). 2018; 73(suppl 1): e490s.
  4. Davar J, Connolly HM, Caplin ME, et al. Diagnosing and Managing Carcinoid Heart Disease in Patients With Neuroendocrine Tumors: An Expert Statement. J Am Coll Cardiol. 2017; 69(10): 1288–1304.
  5. Bednarczuk T, Bolanowski M, Zemczak A, et al. Neuroendocrine neoplasms of the small intestine and appendix - management guidelines (recommended by the Polish Network of Neuroendocrine Tumours). Endokrynol Pol. 2017; 68(2): 223–236.
  6. Niederle B, Pape UF, Costa F, et al. Vienna Consensus Conference participants. ENETS Consensus Guidelines Update for Neuroendocrine Neoplasms of the Jejunum and Ileum. Neuroendocrinology. 2016; 103(2): 125–138.
  7. Neumann H, Fry LC, Neurath MF. Review article on current applications and future concepts of capsule endoscopy. Digestion. 2013; 87(2): 91–99.
  8. Kos-Kudła B, Blicharz-Dorniak J, Handkiewicz-Junak D, et al. Consensus Conference, Polish Network of Neuroendocrine Tumours. Diagnostic and therapeutic guidelines for gastro-entero-pancreatic neuroendocrine neoplasms (recommended by the Polish Network of Neuroendocrine Tumours). Endokrynol Pol. 2013; 64(6): 418–443.
  9. Yao JC, Pavel M, Phan AT, et al. Chromogranin A and neuron-specific enolase as prognostic markers in patients with advanced pNET treated with everolimus. J Clin Endocrinol Metab. 2011; 96(12): 3741–3749.
  10. Krenning EP, Kwekkeboom DJ, Oei HY, et al. Somatostatin-receptor scintigraphy in gastroenteropancreatic tumors. An overview of European results. Ann N Y Acad Sci. 1994; 733: 416–424.
  11. Schreiter NF, Bartels AM, Froeling V, et al. Searching for primaries in patients with neuroendocrine tumors (NET) of unknown primary and clinically suspected NET: Evaluation of Ga-68 DOTATOC PET/CT and In-111 DTPA octreotide SPECT/CT. Radiol Oncol. 2014; 48(4): 339–347.
  12. Rinke A, Wittenberg M, Schade-Brittinger C, et al. 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 (PROMID): Results of Long-Term Survival. Neuroendocrinology. 2017; 104(1): 26–32.
  13. Maroun J, Kocha W, Kvols L, et al. Guidelines for the diagnosis and management of carcinoid tumours. Part 1: the gastrointestinal tract. A statement from a Canadian National Carcinoid Expert Group. Curr Oncol. 2006; 13(2): 67–76.
  14. Luis SA, Pellikka PA. Carcinoid heart disease: Diagnosis and management. Best Pract Res Clin Endocrinol Metab. 2016; 30(1): 149–158.
  15. Knigge U, Capdevila J, Bartsch DK, et al. Antibes Consensus Conference Participants, Antibes Consensus Conference participants. ENETS Consensus Recommendations for the Standards of Care in Neuroendocrine Neoplasms: Follow-Up and Documentation. Neuroendocrinology. 2017; 105(3): 310–319.

Regulations

Important: This website uses cookies. More >>

The cookies allow us to identify your computer and find out details about your last visit. They remembering whether you've visited the site before, so that you remain logged in - or to help us work out how many new website visitors we get each month. Most internet browsers accept cookies automatically, but you can change the settings of your browser to erase cookies or prevent automatic acceptance if you prefer.

Wydawcą serwisu jest VM Media Group sp. z o.o., ul. Świętokrzyska 73, 80–180 Gdańsk

tel.:+48 58 320 94 94, faks:+48 58 320 94 60, e-mail: viamedica@viamedica.pl