Ectopic insulin secreting neuroendocrine tumor of kidney with recurrent hypoglycemia: a diagnostic dilemma

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Abstract

Background

Hypoglycemia secondary to ectopic insulin secretion of non-pancreatic tumors is rare.

Case presentation

We describe a middle aged woman with recurrent hypoglycemia. On evaluation, she was detected to have hyperinsulinemic hypoglycemia and right sided renal mass lesion. 68Ga-Dotanoc and 99mTc-HYNICTOC scans confirmed the intrarenal mass to be of neuroendocrine origin. Right nephrectomy was done and it turned out to be an insulin secreting neuroendocrine tumour. Neuroendocrine nature of this tumour was further confirmed by ultra-structural examination. Her hypoglycemia did not recur after resection of this tumour.

Conclusion

Few cases of ectopic insulin secretion have been reported though some are not proven convincingly. This case addresses all the issues raised in previous case reports and proves by clinical, laboratory, functional imaging and immunohistochemical analysis that ectopic origin of insulin by non-pancreatic tumors does occur. To our knowledge, this is the first reported case of ectopic insulinoma arising from the kidney.

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Most cited references 16

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Clinical features of insulin-like growth factor-II producing non-islet-cell tumor hypoglycemia.

N Hizuka, Kazue Takano, Y Ishikawa … (2006)

In some patients with non-islet-cell tumor hypoglycemia (NICTH), a high molecular weight form of IGF-II (big IGF-II) derived from tumors is present in the circulation and might be associated with recurrent hypoglycemia. In this study, in order to survey the clinical characteristics of patients with IGF-II producing NICTH, we analyzed the medical records of 78 patients with NICTH (M/F 44/34, age 62+/-1.8, range; 9-86 years.) whose serum contained a large amount of big IGF-II. Hepatocellular carcinoma and gastric carcinoma were the most common causes of NICTH. The diameters of the tumors were more than 10 cm in 70% of the patients. Basal immunoreactive insulin (IRI) levels were less than 3 microU/dl in 79% of the patients. Hypoglycemic attack was the onset of disease in 31 of 65 cases (48%), but the tumor was revealed prior to the occurrence of hypoglycemia in 34 cases (52%). Twenty-five of 47 (53%) patients had decreased serum potassium levels. These data suggested that hypoinsulinemic hypoglycemia associated with the presence of a large tumor supports the diagnosis of IGF-II producing NICTH. Hypokalemia was associated with hypoglycemia in some patients. The BMI (21.4+/-0.6 kg/m2) and serum total protein levels (6.6+/-0.1g/dl) were preserved at the occurrence of first hypoglycemic attack suggesting that malnutrition might not be the main cause of hypoglycemia in most patients.

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Detection of somatostatin receptors in surgical and percutaneous needle biopsy samples of carcinoids and islet cell carcinomas.

Jean Claude Reubi, D. M. Nagorney, L. Kvols … (1990)

Somatostatin (SS) receptor status was investigated in the tumor tissues from 62 patients with carcinoid tumors and 15 patients with islet cell carcinomas using receptor autoradiography techniques with two different iodinated somatostatin analogues as radioligands, a [Leu8, DTrp22, Tyr25]somatostatin-28 and a somatostatin octapeptide, Tyr3-octreotide. The carcinoid tumors were either primaries (n = 32) or metastases (n = 43), sampled as surgical specimens or as small needle liver biopsies. Fifty-four of 62 carcinoid patients had SS receptor-positive tumors (87%). All 15 islet cell carcinoma patients had positive tumors (4 primaries, 11 metastases), i.e., 3 vipomas, 3 insulinomas, 2 glucagonomas, 1 gastrinoma, 2 polyfunctional tumors, and 4 nonfunctioning tumors. Saturation and competition experiments on tissue sections revealed saturable, high affinity binding sites pharmacologically specific for bioactive SS analogues. In a majority of the tumors, the receptors were densely distributed and were always homogeneously found in the whole tumor. All except two tumors were labeled with both radioligands. Multiple liver metastases (n = 16) from three different patients were all shown to contain a comparable amount of receptors. SS receptors could be demonstrated even in very small tissue samples of liver metastases obtained by percutaneous liver biopsies (mean weight, 6.8 mg). The majority of the eight SS receptor-negative carcinoids were mainly bronchial carcinoids (n = 5), usually poorly differentiated. On the contrary, SS receptor-positive cases were never found to be anaplastic. All tumors except one from patients pretreated with octreotide (3 days to 3.8 years) were SS receptor positive. In the majority of carcinoids or islet cell carcinomas, the SS receptor status correlated with the in vivo biochemical response (hormone inhibition) to octreotide. These data demonstrate (a) the high prevalence of SS receptors in the primary tumors of both carcinoids and islet cell carcinomas, (b) their presence in metastases as well, (c) their continuous expression even during long term octreotide therapy, (d) the possibility of measuring SS receptors in percutaneous needle liver biopsies, and (e) the evidence of their functionality. This study therefore suggests that tumoral SS receptors may be the likely molecular basis for octreotide action and may be an important parameter for predicting the therapeutic efficacy of SS analogues in carcinoids and islet cell carcinomas.

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Nuclear imaging of neuroendocrine tumours.

Ulrike Garske-Román, Anders Sundin, H Orlefors (2007)

The diagnosis of neuroendocrine tumours (NETs) and monitoring of therapy in many patients relies mainly on morphological imaging techniques such as computed tomography (CT), ultrasound (US) and magnetic resonance imaging (MRI). However, functional imaging modalities--such as somatostatin receptor scintigraphy (SRS)--have great impact on patient management by providing tools for better staging of the disease, visualization of occult tumour, and evaluation of eligibility for somatostatin analogue treatment. Positron emission tomography (PET) using (18)F-fluoro-deoxy-glucose (FDG) is a powerful functional modality for oncological imaging. Unfortunately, FDG is not accumulated in NETs except in the case of dedifferentiated tumours and tumours with high proliferative activity. Based on the concept of amine precursor uptake and decarboxylation (APUD), the (18)F- and (11)C-labelled amine precursors L-dihydroxyphenylalanine and 5-hydroxy-L-tryptophan (5-HTP) have been utilized for PET imaging of NETs. In comparative studies of patients with a variety of NETs, (11)C5-HTP-PET proved better than CT and SRS by visualizing additional small lesions. With carbidopa premedication orally before (11)C5-HTP-PET examination the tumour uptake could be increased and the urinary radioactivity concentration considerably reduced. This concept may also be applied to (18)F-L-DOPA-PET, a method which in a limited number of studies has gained additional diagnostic information in NET patients compared to SRS and morphological imaging. (68)Ga is available from an in-house generator and has been utilized for labelling of somatostatin analogues for PET imaging of NETs with promising results in a small number of patients. However, SRS is an established functional imaging method for patients with NETs, whereas the role for PET in the clinical routine needs further evaluation in comparative studies in larger groups of patients.

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Author and article information

Contributors

S Ramkumar

Atul Dhingra

VP Jyotsna

Mohd Ashraf Ganie

Chandan J Das

Amlesh Seth

Mehar C Sharma

Chandra Sekhar Bal

Journal

Journal ID (nlm-ta): BMC Endocr Disord

Journal ID (iso-abbrev): BMC Endocr Disord

Title: BMC Endocrine Disorders

Publisher: BioMed Central

ISSN (Electronic): 1472-6823

Publication date Collection: 2014

Publication date (Electronic): 17 April 2014

Volume: 14

Page: 36

Affiliations

[1 ]Department of Endocrinology and Metabolism, All India Institute of Medical Sciences, Ansari Nagar, New Delhi 110029, India

[2 ]Departments of Radiology, All India Institute of Medical Sciences, New Delhi, India

[3 ]Departments of Urology, All India Institute of Medical Sciences, New Delhi, India

[4 ]Departments of Pathology, All India Institute of Medical Sciences, New Delhi, India

[5 ]Departments of Nuclear Medicine, All India Institute of Medical Sciences, New Delhi, India

Article

Publisher ID: 1472-6823-14-36

DOI: 10.1186/1472-6823-14-36

PMC ID: 4046058

PubMed ID: 24741994

SO-VID: 992803c3-95f6-471d-90cb-0b926dae0324

License:

This is an Open Access article distributed under the terms of the Creative Commons Attribution License ( http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly credited. The Creative Commons Public Domain Dedication waiver ( http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.

Ectopic insulin secreting neuroendocrine tumor of kidney with recurrent hypoglycemia: a diagnostic dilemma

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Abstract

Background

Case presentation

Conclusion

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GLUT4 biology

Most cited references 16

Clinical features of insulin-like growth factor-II producing non-islet-cell tumor hypoglycemia.

Detection of somatostatin receptors in surgical and percutaneous needle biopsy samples of carcinoids and islet cell carcinomas.

Nuclear imaging of neuroendocrine tumours.

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