|Year : 2020 | Volume
| Issue : 4 | Page : 277-279
A rare case of pancreatic neuroendocrine tumor
Raja Rao Nudurupati1, Ravi Kamal Kumar Akunuri1, Tarun Kumar Suvvari2, Lakshmi Venkata Simhachalam Kutikuppala3
1 Department of General Surgery, Rangaraya Medical College, Kakinada, Andhra Pradesh, India
2 Department of Medical Student, Rangaraya Medical College, Kakinada, Andhra Pradesh, India
3 Medical Student, Konaseema Institute of Medical Sciences and Research Foundation, Amalapuram, Andhra Pradesh, India
|Date of Submission||01-Aug-2020|
|Date of Decision||25-Sep-2020|
|Date of Acceptance||28-Oct-2020|
|Date of Web Publication||27-Nov-2020|
Tarun Kumar Suvvari
Rangaraya Medical College, Kakinada, Andhra Pradesh
Source of Support: None, Conflict of Interest: None
Pancreatic neuroendocrine tumors (PNETs) are a group of endocrine neoplasms that exhibit neuroendocrine phenotypes. These include the production of neuropeptides, large dense-core secretory vesicles, and a lack of neural structures. They arise in the pancreas and are among the most common neuroendocrine tumors. A case of 38 years old female presented with a painless progressive swelling in the right upper abdomen since one month which is insidious in onset, gradually increasing in size with a history of significant weight loss and patient is not a known diabetic. On Examination, a swelling of size 5x7cm noted involving epigastric and right hypochondrium, which is not moving with respiration and dull on percussion. Plane of swelling is Intra-abdominal& retroperitoneal. Computed tomography abdomen (CECT) showed well defined rounded iso dense lesion showing heterogeneous enhancement in arterial and venous phase. Magnetic resonance cholangiopancreatography (MRCP) showed well defined lobulated heterogeneous solid lesion with central areas of necrosis seen in the pancreaticoduodenal groove (PDG) involving main pancreatic duct (MPD) and loss of fat planes with surrounding structures most likely malignant etiology. The patient underwent pancreaticoduodenectomy surgery (Whipple procedure) and had an uneventful recovery. Post-operative biopsy report confirmed pancreatic neuroendocrine tumor grade II (well-differentiated type), stage II a – pT3 N0 Mx.
Keywords: Endocrine neoplasms, pancreatic neuroendocrine tumors, pancreaticoduodenal groove, Whipple procedure
|How to cite this article:|
Nudurupati RR, Kumar Akunuri RK, Suvvari TK, Simhachalam Kutikuppala LV. A rare case of pancreatic neuroendocrine tumor. Apollo Med 2020;17:277-9
| Introduction|| |
Pancreatic neuroendocrine tumors (PNETs) are rare tumors accounting for 7% of all NETs and 1%–2% of pancreatic tumors with slightly more incidence in males and females, with the majority of cases diagnosed between ages of 60 and 80 years. The patient presents with symptoms that result from the secretion of active gastrointestinal hormones or local symptoms from tumor growth such as abdominal pain, jaundice, anorexia, and weight loss. Behaviors of PNETs are highly variable and range from nearly benign to extremely aggressive, but the majority of PNETs are moderately malignant.
Appropriate imaging and histological evidence are required to diagnose PNETs accurately. A complete diagnosis should establish the nature, tumor grade, and assessment of PNETs and primary and metastatic loci identification and determine the functioning of the tumor. Treatment strategy for PNETs had a paradigm shift in the last one to two decades, with an “aggressive” approach becoming popular in tertiary care centers worldwide. The aggressive approach depends on the reasonable assumption of benefits from reducing the tumor burden, and those interventions can increasingly safe. There must be a considerable experience of the personnel in the surgical removal of PNETs. Those procedures should be performed at academic centers with a team of experts, as the surgery has intrinsic operative and anesthetic risks.
| Case Report|| |
A 38-year-old female presented with a painless progressive swelling in the right upper abdomen for 1 month, which is insidious in onset and gradually increasing in size with a history of significant weight loss, and the patient is not a known diabetic. On examination, a swelling measuring 5 cm ×7 cm was noted involving epigastric and right hypochondrium, which is not moving with respiration and dull on percussion. Plane of swelling is intra-abdominal and retroperitoneal.
Contrast-enhanced computed tomography (CT) abdomen showed a well-defined rounded isodense lesion showing heterogeneous enhancement in the arterial and venous phase predominantly. Outer two-third of the lesion with central non-enhancing area looks like cystic or necrotic component was noted in the uncinate process and head of the pancreas. It closely adjoins the first and “C” loop of the duodenum with no fat plane in between them. The lesion is abutting portal vein about half of the circumference.
Magnetic resonance cholangiopancreatography showed a well-defined lobulated heterogeneous solid lesion with central areas of necrosis seen in the pancreaticoduodenal groove involving main pancreatic duct and loss of fat planes with surrounding structures most likely malignant etiology.
The patient underwent pancreaticoduodenectomy surgery (Whipple procedure) and had an uneventful recovery [Figure 1]. The postoperative biopsy report showed pancreatic neuroendocrine tumor Grade II (well-differentiated type), Stage IIa – pT3 N0 Mx [Figure 2]. IHC markers showed positive for chromogranin, synaptophysin, CD56 Ki-67 index of 15%. Histopathology showed [Figure 3] sheets of uniform, round blue cells (left), homer wright rosettes (middle), and atypical PNETs with larger cells with prominent nucleolus (right).
|Figure 3: Histopathology of pancreatic neuroendocrine tumor (left, middle, and right)|
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| Discussion|| |
The incidence of malignancy in PNET is ~10% in insulinoma to almost 100% in glucagon or somatostatin-secreting tumors. Malignancy in PNETs definitely determined only by metastasis. NETs are graded based on appearance, mitotic rate, invasion of other organs, and Ki-67 proliferative index., Most PNETs occur sporadically, and the most common genetic syndrome associated is MEN1 syndrome, followed by VHL disease, tuberous sclerosis, and neurofibromatosis Type 1.
Localization of tumor by cross-sectional imaging by CT or magnetic resonance imaging (MRI) and capturing. Capturing vascular blush in the arterial phase is critical for the identification and differentiation from other types of pancreatic tumors. If a tumor is not localized in CT or MRI, then EUS or somatostatin scintigraphy or single-photon emission CT/CT or angiography is done.,
In the absence of metastatic disease, the primary treatment of PNETs is surgical resection. In patients with advanced PNETs, treatment options include cytotoxic chemotherapy, everolimus, sunitinib, somatostatin analogs, peptide receptor radiotherapy, and ablative approaches such as hepatic artery embolization and radiofrequency ablation.
| Conclusion|| |
PNETs are rare indolent tumors with majority belonging to the nonfunctional type in which mostly are sporadic with no significant risk factors. Treatment options include surgical resection and nonsurgical management indicated for symptomatic functional tumors, metastatic disease, high-grade disease, patient unfit for resection, and patients in whom disease progressed despite surgical resection.
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
Conflicts of interest
There are no conflicts of interest.
| References|| |
Vinik A, Perry RR, Casellini C, Hughes MS, Feliberti E. Pathophysiology and treatment of Pancreatic Neuroendocrine Tumors (PNETs): New developments. In: Feingold KR, Anawalt B, Boyce A, et al
., editors. Endotext. South Dartmouth (MA): MDText.com, Inc.; 2000.
Young K, Iyer R, Morganstein D, Chau I, Cunningham D, Starling N. Pancreatic neuroendocrine tumors: A review. Future Oncol 2015;11:853-64.
Lee DW, Kim MK, Kim HG. Diagnosis of pancreatic neuroendocrine tumors. Clin Endosc 2017;50:537-45.
Wong KP, Tsang JS, Lang BH. Role of surgery in pancreatic neuroendocrine tumor. Gland Surg 2018;7:36-41.
Paniccia A, Edil BH, Schulick RD. Pancreatic neuroendocrine tumors: An update. Indian J Surg 2015;77:395-402.
[Figure 1], [Figure 2], [Figure 3]