Authors: | Schmidt, M.; Hinterleitner, C.; Singer, S.; Lauer, U. M.; Zender, L.; Hinterleitner, M. |
Article Title: | Diagnostic approaches for neuroendocrine neoplasms of unknown primary (NEN-UPs) and their prognostic relevance—A retrospective, long-term single-center experience |
Abstract: | Neuroendocrine neoplasms (NENs) represent a rare and heterogenous group of tumors with predominantly gastroenteropancreatic or pulmonary origin. Despite numerous diagnostic efforts, the primary tumor site remains unknown in up to 20% of the patients diagnosed with NEN. In this subgroup of NEN patients, a standard diagnostic algorithm has not yet been integrated into clinical routine. Of note, an undetermined primary tumor site in NENs is associated with an impaired clinical outcome by at least “formally” limiting treatment options exclusively approved for NENs of a certain histological origin. In this retrospective study, a patient cohort of 113 patients initially diagnosed with NEN of unknown primary (NEN-UP) was analyzed. In 13 patients (11.5%) a primary tumor site could be identified subsequently, amongst others, by performing somatostatin receptor (SSTR)-PET-based imaging, which was irrespective of the initial clinical or demographic features. Diagnostic work-up and therapeutic regimens did not differ significantly between patients with an identified or unidentified primary tumor site; only a detailed immunohistochemical assessment providing additional information on the tumor origin proved to be significantly associated with the detection of a primary tumor site. Our study revealed that a profound diagnostic work-up, particularly including SSTR-PET-based imaging, leads to additional treatment options, finally resulting in significantly improved clinical outcomes for patients with NEN-UPs. © 2023 by the authors. |
Keywords: | immunohistochemistry; adult; cancer chemotherapy; cancer survival; human tissue; aged; primary tumor; human cell; major clinical study; overall survival; histopathology; sunitinib; bone metastasis; cancer patient; cancer radiotherapy; cancer staging; outcome assessment; positron emission tomography; lymph node metastasis; ki 67 antigen; computer assisted tomography; ca 19-9 antigen; carcinoembryonic antigen; cohort analysis; hemoglobin blood level; retrospective study; tumor marker; distant metastasis; neuroendocrine tumor; liver metastasis; body mass; algorithm; colonoscopy; diagnosis; fluorodeoxyglucose f 18; long term care; imaging; chromosomal instability; merkel cell carcinoma; cytokeratin 20; lactate dehydrogenase; cancer of unknown primary site; blood cell count; leukocyte count; urinalysis; observational study; radiofrequency ablation; endoscopy; gastroscopy; lymphocyte count; predictive value; receiver operating characteristic; platelet count; neuroendocrine carcinoma; transcription factor cdx2; serotonin; somatostatin receptor; synaptophysin; molecularly targeted therapy; 5 hydroxyindoleacetic acid; chromogranin a; gastrin; diagnostic test accuracy study; clinical outcome; cd56 antigen; neuroendocrine neoplasm; hematological parameters; cancer prognosis; high throughput sequencing; proliferation index; absolute lymphocyte count; prognosis; human; male; female; article; absolute neutrophil count; cancer of unknown primary; supportive care need |
Journal Title: | Cancers |
Volume: | 15 |
Issue: | 17 |
ISSN: | 2072-6694 |
Publisher: | MDPI |
Date Published: | 2023-01-01 |
Start Page: | 4316 |
Language: | English |
DOI: | 10.3390/cancers15174316 |
PROVIDER: | scopus |
PMCID: | PMC10486951 |
PUBMED: | 37686593 |
DOI/URL: | |
Notes: | Article -- Source: Scopus |