open access
Oligosymptomatic neuroendocrine neoplasm of small intestine with metastases spread to heart, bones, muscles and intraperitoneally after few years in remission – diagnostic and therapeutic difficulties
open access
Abstract
Fifty-one years old male patient with the history of recurring abdominal pains and signs of subileus, without carcinoid syndrome signs, underwent laparatomy with the resection of small intestine segment. Histopathology revealed 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. Computed tomography performed once a year did not indicate the recurrence of the neoplasm. Patient did not report any “red flag” symptoms. After few years in remission [68Ga]-DOTATE PET/CT revealed dynamic development of the illness. Patient was qualified for palliative treatment with long-acting somatostatin analogue. Due to the treatment’s ineffectiveness and further progression of the disease patient received Peptide Receptor Radionuclide Therapy (PRRT). In spite of the therapy his condition did not improve and progression was observed. Patient died because of malfunction of cardiac conduction system caused by the metastases in the heart.
Abstract
Fifty-one years old male patient with the history of recurring abdominal pains and signs of subileus, without carcinoid syndrome signs, underwent laparatomy with the resection of small intestine segment. Histopathology revealed 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. Computed tomography performed once a year did not indicate the recurrence of the neoplasm. Patient did not report any “red flag” symptoms. After few years in remission [68Ga]-DOTATE PET/CT revealed dynamic development of the illness. Patient was qualified for palliative treatment with long-acting somatostatin analogue. Due to the treatment’s ineffectiveness and further progression of the disease patient received Peptide Receptor Radionuclide Therapy (PRRT). In spite of the therapy his condition did not improve and progression was observed. Patient died because of malfunction of cardiac conduction system caused by the metastases in the heart.
Keywords
neuroendocrine neoplasm; carcinoid syndrome; somatostatin analogue; radioisotope therapy


Title
Oligosymptomatic neuroendocrine neoplasm of small intestine with metastases spread to heart, bones, muscles and intraperitoneally after few years in remission – diagnostic and therapeutic difficulties
Journal
Nowotwory. Journal of Oncology
Issue
Article type
Case report
Published online
2021-01-25
DOI
10.5603/NJO.a2021.0002
Keywords
neuroendocrine neoplasm
carcinoid syndrome
somatostatin analogue
radioisotope therapy
Authors
Natalia Tyrybon
Agnieszka Żyłka
Joanna Długosińska
Małgorzata Benke
Marek Dedecjus


- 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.
- Maggard MA, O'Connell JB, Ko CY. Updated population-based review of carcinoid tumors. Ann Surg. 2004; 240(1): 117–122.
- 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.
- 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.
- 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.
- 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.
- Neumann H, Fry LC, Neurath MF. Review article on current applications and future concepts of capsule endoscopy. Digestion. 2013; 87(2): 91–99.
- 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.
- 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.
- 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.
- 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.
- 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.
- 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.
- Luis SA, Pellikka PA. Carcinoid heart disease: Diagnosis and management. Best Pract Res Clin Endocrinol Metab. 2016; 30(1): 149–158.
- 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.