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CASE REPORT
Year : 2022  |  Volume : 25  |  Issue : 6  |  Page : 971-973

Diagnostic challenge and surgical management of multiple pancreatic neuroendocrine tumors – A case report


1 Department for Radiology, General Hospital “Bel Medic”, Bulevar Oslobodjenja; Department for HBP Surgery, Clinic for Digestive Surgery, University Clinical Centre of Serbia, Dr Koste Todorovica, Belgrade, Serbia
2 Department for HBP Surgery, Clinic for Digestive Surgery, University Clinical Centre of Serbia, Dr Koste Todorovica; Department for Surgery with Anesthesiology, Faculty of Medicine, University of Belgrade, Dr Subotica, Belgrade, Serbia

Date of Submission28-Nov-2021
Date of Acceptance31-Jan-2022
Date of Web Publication16-Jun-2022

Correspondence Address:
Dr. S Jovanovic
General Hospital Bel Medic, Koste Jovanovic 87, 11000 Belgrade, Serbia (Primarily Carried Out from 2009-2019 at Clinical Centre of Serbia, Clinic for Digestive Surgery, Koste Todorovica 6, Belgrade
Serbia
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/njcp.njcp_1971_21

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   Abstract 


Insulinoma is the most common functional solitary, benign pancreatic neuroendocrine tumor. In this paper, we share our experience of triple pancreatic insulinomas, with an idea to highlight the fact that despite the high frequency of solitary insulinoma, there is the possibility of multiple lesions and point out the need for a systematic and detailed radiographic and clinical examination. A 33-year-old woman was admitted to our clinic due to chronic fatigue, sweating, sporadic episodes of palpitations, unsteady hands, and blurred images, which resolve spontaneously after a few minutes. A comprehensive radiological examination revealed two lesions in the pancreatic tail, highly indicative of neuroendocrine tumor – insulinoma, so we elected for surgical treatment. Intraoperative exploration by manual palpation and ultrasonography revealed two more lesions. In the case of sporadically distributed lesions in the pancreas and if it is not possible to accurately differentiate those that represent the insulinoma for sure, the surgical procedure should be delayed. Additionally, tests such as selective arterial calcium stimulation testing should be carried out, and then, with more information in hands, make a re-plan of the potential surgical treatment which should be made.

Keywords: Distal pancreatectomy, enucleation, insulinoma, multiple insulinomas, neuroendocrine tumor


How to cite this article:
Jovanovic S, Tadic B, Knezevic D J, Ostojic S. Diagnostic challenge and surgical management of multiple pancreatic neuroendocrine tumors – A case report. Niger J Clin Pract 2022;25:971-3

How to cite this URL:
Jovanovic S, Tadic B, Knezevic D J, Ostojic S. Diagnostic challenge and surgical management of multiple pancreatic neuroendocrine tumors – A case report. Niger J Clin Pract [serial online] 2022 [cited 2022 Jul 5];25:971-3. Available from: https://www.njcponline.com/text.asp?2022/25/6/971/347623




   Introduction Top


At the moment of diagnosis, insulinomas are not bigger than 2 cm in size.[1] Surgical management is the method of choice but if the diagnosis is inconclusive or uncertain, can be a challenge. We are sharing our experience of triple localization in the pancreas, pointing out the need for multimodality radiographic and detailed clinical examination in specific situations.


   Case Report Top


A 33-year-old woman was admitted to our hospital due to chronic fatigue, sweating, palpitations, trembling hands, blurred images that resolve spontaneously. In previous medical history, she reported a weight gain of about 15 kg during the last 2 years, loss of consciousness in the last 6 months, and a decrease in glucose values with complete recovery after its ingestion.

After an overnight fastening, laboratory findings were within normal range except serum blood glucose level of 3,1 mmol/L. To confirm the diagnosis of endogenous hyperinsulinemia, the patient underwent a supervised 72-h fast test. The test was terminated after 17 h when she developed neuroglycopenic symptoms, confusion, and blurred vision, with a serum glucose level of 1,8 mmol/L. The C-peptide suppression test was completed after 60 min (1,9 mmol glucose/L, C-peptide 2,25 ng/mL). A prolonged 5-h oral glucose tolerance test showed reactive hypoglycemia with hyperinsulinemia. Endogenous hyperinsulinemia was suspected.

A magnetic resonance (MR) scanner (Avanto Siemens 1.5T, Germany) was completed with the administration of a paramagnetic contrast agent (Gadovist, Schering, Germany). We imagined a demarcated larger lesion in the pancreatic tail and a smaller prominent nodule at the anterior margin of the pancreatic body. Both lesions showed low signal intensity (SI) in the T1w sequence [Figure 1]a, while on T2w the larger nodule was presented with intermediate SI, but the smaller was almost clearly highly hyperintense [Figure 1]b. After the contrast administration, lesions showed an inhomogeneous increase in SI, predominantly in the arterial contrast phase [Figure 1]c. It was characterized as a possible neuroendocrine tumor. Immunoreactive insulin and chromogranin A (CgA) levels were 2.1 μIU/mL and 180 ng/mL, respectively.
Figure 1: MRI: hypo SI in T1w (a), hyper in T2w (smaller nodule), intermediate SI (bigger nodule) (b), an inhomogeneous increase in SI in the bigger lesion after contrast enhancement (CE) (c); intraoperative (d) triple lesions (yellow arrow: undetected lesion preoperatively)

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Due to a suspected diagnosis of insulinoma with multiple localization, the patient was examined for the existence of multiple endocrine neoplasia (MEN1) syndrome. In her family history, there was no existence of a similar disease, but because of the high cost of genetic testing, she refused the testing for MEN1 gene mutation. Magnetic resonance imaging (MRI) of the pituitary gland as well as of its serum hormone analysis showed normal results.

Intraoperative exploration noted a large lesion in the tail of the pancreas. Manual palpation and intraoperative ultrasonography (IOUS) revealed two nodules: on the right side to the main one bulging the pancreatic parenchyma [Figure 1]d. Tumors were excised by performing spleen-preserving distal pancreatectomy with conservation of the splenic vessels.

Histological findings showed strong positivity for synaptophysin which was observed in all three lesions; however, the largest lesion showed stronger positivity for CgA, while the positivity for pro-insulin and insulin was registered only in the largest tumor [Figure 2]. Resection was classified in the category as R0.
Figure 2: Abundant hyalinized stroma, unevenly distributed nests, and trabeculas of cells with “carcinoid-like” cytomorphology (a) with cytoplasmic hormonal immune expression (b). Trabecular and focally microcystically organized cells (c), with a proliferation index of 3% (d), Ki-67 protein immunolabeling

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At regular check-ups in the first 6 months, she lost body weight by about 10 kg. Five years after the operation, the patient is feeling well and there is no evidence of disease recurrence.


   Discussion Top


Insulinoma is a rare condition, with a prevalence of 1–4/1 million/year.[2] Poorly differentiated insulinoma cells are lacking the normal physiological functions of autoregulation, which results in uncontrolled excessive secretion of insulin. They may occur at any age with a specific peak in the fifth decade more often in women (57%). Even if the incidence of insulinoma is low, the possibility of successful treatment and long-time survival are the reasons that the prevalence of this cancer is much higher compared to the incidence.[3] They are presented as a benign solitary lesion (90%) with a size between 1 and 2 cm at the time of the detection.[4] In 10% of cases, two or more insulinomas could be in the pancreas, usually associated with MEN1.[5] Multiple insulinomas have a higher percentage of malignant alteration (25–50%).[6]

Inconclusive findings on imaging present diagnostic and management challenges. Computerized tomography (CT), MRI, and IOUS are the diagnostic methods of choice with a sensitivity of 74–79%, 56–85%, and 84–90, respectively.[7] The invasive radiological procedures (pancreatic arteriography, percutaneous transhepatic portal venous sampling) and calcium stimulation test have the main role with a sensitivity of 87%.[8] Manual palpation and IOUS are reported with sensitivities up to 80–99% and 75–95%, respectively.[7]

Surgical management results in a high cure rate, and today laparoscopic enucleation is considered a method of choice, to spare most of the tissue of the pancreas.[9] As the sporadic occurrence of insulinoma with multiple localization and small size, some dilemmas are still present. Is the observed lesion the only one, and can it be detected using radiologic imaging? In our experience, careful intraoperative palpation and IOUS should be proposed as standard and additional protocols. Depending on the size and the number of lesions, intraglandular localization, and their relationship with the main pancreatic duct, the surgeon should decide to perform either the enucleation or the resection procedure. The distance between the main pancreatic duct and tumor should be at least 3 mm, to consider the enucleation safe.[10] Intraoperative monitoring of blood glucose allows us to assess the effect of surgery. In hypoglycemic patients, normalization of blood glucose levels occurs when the tumor is removed.

Having all in mind, additional methods either invasive or not, should be carried out for making a re-plan for choosing the adequate surgical treatment.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
He M, Xu J, Sun Z, Wang X, Wang J, Feng F, et al. Prospective comparison of reduced field-of-view (rFOV) and Full FOV (fFOV) diffusion-weighted imaging (DWI) in the assessment of insulinoma: Image quality and lesion detection. Acad Radiol 2020;27:1572-9.  Back to cited text no. 1
    
2.
Fisher WE, Andersen DK, Winsdor JA, Saluja AK, Brunicardi CF. Pancreas. In: Brunicardi FC, Andersen D, Billiar TR, Dunn DL, Kao LS, Hunter JG, et al., editors. Schwartz's Principles of Surgery. 11th ed. New York, N.Y.: McGraw-Hill Education LLC; 2019. p. 1391-2.  Back to cited text no. 2
    
3.
Scoazec JY. Insulinoma. In: La Rosa S, Sessa F, editors. Pancreatic Neuroendocrine Neoplasms Practical Approach to Diagnosis, Springer, Switzerland. Classification, and Therapy. 2019. p. 75-80.  Back to cited text no. 3
    
4.
Shao S, Zeng Z, Hu S. An observational analysis of insulinoma a from a single institution. QJM 2018;111:237-41.  Back to cited text no. 4
    
5.
Kwon EB, Jeong HR, Shim YS, Lee HS, Hwang JS. Multiple endocrine neoplasia type 1 presenting as hypoglycemia due to insulinoma. J Korean Med Sci 2016;31:1003-6.  Back to cited text no. 5
    
6.
Anlauf M, Bauersfeld J, Raffel A, Koch CA, Henopp T, Alkatout I, et al. Insulinomatosis: A multicentric insulinoma disease that frequently causes early recurrent hyperinsulinemic hypoglycemia. Am J Surg Pathol 2009;33:339-46.  Back to cited text no. 6
    
7.
Jyotsna VP, Pal S, Kandasamy D, Gamanagatti S, Garg PK, Raizada N, et al. Evolving management of insulinoma: Experience at a tertiary care center. Indian J Med Res 2016;144:771.  Back to cited text no. 7
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8.
Pillai AK, Silvers W, Christensen P, Riegel M, Adams-Huet B, Lingvay I, et al. Comparative evaluation of two venous sampling techniques for the assessment of pancreatic insulin and zinc release upon glucose challenge. J Diabetes Res 2015;2015:789359. doi: 10.1155/2015/789359.  Back to cited text no. 8
    
9.
Tsang Y-P, Lang BH-H, Shek TW-H. Assessing the short- and long-term outcomes after resection of benign insulinoma. ANZ J Surg 2016;86:706-10.  Back to cited text no. 9
    
10.
Falconi M, Zerbi A, Crippa S, Balzano G, Boninsegna L, Capitanio V, et al. Parenchyma-preserving resections for small nonfunctioning pancreatic endocrine tumors. Ann Surg Oncol 2010;17:1621-7.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2]



 

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