NEUROENDOCRINE TUMOUR OF UNKNOWN PRIMARY ORIGIN: UNUSUAL CASE OF METASTATIC HEPATIC FOCI IN A FEMALE PATIENT

Authors

  • Anosh Aslam Khan Dow University of Health Sciences
  • Syed Hamza Bin Waqar Dow University of Health Sciences
  • Osama Mohiuddin Dow University of Health Sciences
  • Moiz Ehtesham Dow University of Health Sciences
  • Sumeen Jalees Dow University of Health Sciences

Abstract

Under the banner of cancers of unknown primary origin (CUPs), neuroendocrine tumours account for less than five percent of the neoplasms. The clinical manifestations and management depend upon the tumour's grade and differentiation and its site of growth. At times, despite of aggressive search for primary origin, cancer remains hidden. Herein, we present a case of a middle-aged woman who presented to our tertiary set-up with complaints of abdominal pain and distension. After a series of radiologic and interventional investigations including positron emission tomography with liver biopsy and immunohistochemical analysis, a diagnosis of the well-differentiated neuroendocrine tumour was made, located in the right lobe of the liver. However, the primary origin could not be identified. The patient was managed with trans-arterial chemoembolization (TACE) followed by hepatic resection and was followed biennially afterwards. In our case, hepatic metastasis was treated with chemoembolization and staged-resection and provided a good prognosis to the patient. Our case is unique as only a few case reports have been published with following presentation and documentation of efficacious treatment is needed to contribute to the literature. Proper trials with exteriorization of bowel and radiological imaging is necessary to stage the primary tumour, even if end result is in vain. This will help to further improve the prognosis.

Keywords: Neuroendocrine tumour; cancer of unknown aetiology; metastasis; TACE

References

Spigel DR, Hainsworth JD, Greco FA. Neuroendocrine carcinoma of unknown primary site. Semin Oncol 2009;36(1):52-9).

Gurung A, Yoshida EM, Scudamore CH, Hashim A, Erb SR, Webber DL. Primary hepatic neuroendocrine tumour requiring live donor liver transplantation: case report and concise review. Ann Hepatol 2015;11(5):715-20.

Bellizzi AM. Assigning site of origin in metastatic neuroendocrine neoplasms: a clinically significant application of diagnostic immunohistochemistry. Adv Anat Pathol 2013;20(5):285-314.

Liu T, Liao J, Dang J, Li G. Treatments for patients with advanced neuroendocrine tumours: a network meta-analysis. Ther Adv Med Oncol 2019;11:1758835919853673.

Hainsworth JD, Johnson DH, Greco FA. Poorly differentiated neuroendocrine carcinoma of unknown primary site: a newly recognized clinicopathologic entity. Ann Intern Med 1988;109(5):364-71.

Riihimäki M, Hemminki A, Sundquist K, Sundquist J, Hemminki K. The epidemiology of metastases in neuroendocrine tumours. Int J Cancer 2016;139(12):2679-86.

Kerr SE, Schnabel CA, Sullivan PS, Zhang Y, Huang VJ, Erlander MG, et al. A 92-gene cancer classifier predicts the site of origin for neuroendocrine tumours. Mod Pathol 2014;27(1):44-54.

Sadowski SM, Neychev V, Millo C, Shih J, Nilubol N, Herscovitch P, et al. Prospective study of 68Ga-DOTATATE positron emission tomography/computed tomography for detecting gastro-entero-pancreatic neuroendocrine tumours and unknown primary sites. J Clin Oncol 2016;34(6):588-96.

Bergsland EK, Nakakura EK. Neuroendocrine tumours of unknown primary: is the primary site really not known? JAMA Surg 2014;149(9):889-90.

Hope TA, Bergsland EK, Bozkurt MF, Graham M, Heaney AP, Herrmann K, et al. Appropriate use criteria for somatostatin receptor PET imaging in neuroendocrine tumours. J Nucl Med 2018;59(1):66-74.

Hicks RJ. Use of molecular targeted agents for the diagnosis, staging and therapy of neuroendocrine malignancy. Cancer Imaging 2010;10(1A):S83-91.

Yao JC, Buzzoni R, Carnaghi C, Fazio N, Singh S, Wolin EM, et al. Baseline demographics of the randomized, placebo-controlled, double-blind, phase III RADIANT-4 study of everolimus in nonfunctional gastrointestinal (GI) or lung neuroendocrine tumours (NET). J Clin Oncol 2015;33(3 Suppl):276.

Strosberg JR, Wolin EM, Chasen B, Kulke MH, Bushnell DL, Caplin ME, et al. NETTER-1 phase III: Progression-free survival, radiographic response, and preliminary overall survival results in patients with midgut neuroendocrine tumours treated with 177-Lu-dotatate. J Clin Oncol 2016;34(4 Suppl):194.

Kulke MH, Siu LL, Tepper JE, Fisher G, Jaffe D, Haller DG, et al. Future directions in the treatment of neuroendocrine tumours: consensus report of the National Cancer Institute Neuroendocrine Tumour clinical trials planning meeting. J Clin Oncol 2011;29(7):934-43.

Kennedy A, Bester L, Salem R, Sharma RA, Parks RW, Ruszniewski P. Role of hepatic intra-arterial therapies in metastatic neuroendocrine tumours (NET): guidelines from the NET-Liver-Metastases Consensus Conference. HPB (Oxford) 2015;17(1):29-37.

Downloads

Published

2020-01-27

How to Cite

Khan, A. A., Bin Waqar, S. H., Mohiuddin, O., Ehtesham, M., & Jalees, S. (2020). NEUROENDOCRINE TUMOUR OF UNKNOWN PRIMARY ORIGIN: UNUSUAL CASE OF METASTATIC HEPATIC FOCI IN A FEMALE PATIENT. Journal of Ayub Medical College Abbottabad, 32(1), 127–131. Retrieved from https://jamc.ayubmed.edu.pk/index.php/jamc/article/view/6775

Issue

Vol. 32 No. 1 (2020): JOURNAL OF AYUB MEDICAL COLLEGE, ABBOTTABAD

Section

CASE REPORT

License

Journal of Ayub Medical College, Abbottabad is an OPEN ACCESS JOURNAL which means that all content is FREELY available without charge to all users whether registered with the journal or not. The work published by J Ayub Med Coll Abbottabad is licensed and distributed under the creative commons License  CC BY ND Attribution-NoDerivs. Material printed in this journal is OPEN to access, and are FREE for use in academic and research work with proper citation. J Ayub Med Coll Abbottabad accepts only original material for publication with the understanding that except for abstracts, no part of the data has been published or will be submitted for publication elsewhere before appearing in J Ayub Med Coll Abbottabad. The Editorial Board of J Ayub Med Coll Abbottabad makes every effort to ensure the accuracy and authenticity of material printed in J Ayub Med Coll Abbottabad. However, conclusions and statements expressed are views of the authors and do not reflect the opinion/policy of J Ayub Med Coll Abbottabad or the Editorial Board.

USERS are allowed to read, download, copy, distribute, print, search, or link to the full texts of the articles, or use them for any other lawful purpose, without asking prior permission from the publisher or the author. This is in accordance with the BOAI definition of open access.

AUTHORS retain the rights of free downloading/unlimited e-print of full text and sharing/disseminating the article without any restriction, by any means including twitter, scholarly collaboration networks such as ResearchGate, Academia.eu, and social media sites such as Twitter, LinkedIn, Google Scholar and any other professional or academic networking site.