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 Table of Contents  
CASE REPORT
Year : 2013  |  Volume : 3  |  Issue : 1  |  Page : 76-78

A case of nonfunctioning pancreatic islet cell tumor in a young female


1 Department of Pathology, Sri Venkateswara Institute of Medical Sciences, Tirupati, Andhra Pradesh, India
2 Department of General Surgery, Sri Venkateswara Institute of Medical Sciences, Tirupati, Andhra Pradesh, India
3 Department of Surgical Gastroenterology, Sri Venkateswara Institute of Medical Sciences, Tirupati, Andhra Pradesh, India
4 Department of Surgical Oncology, Sri Venkateswara Institute of Medical Sciences, Tirupati, Andhra Pradesh, India

Date of Web Publication28-Aug-2013

Correspondence Address:
Amitabh Jena
Department of Surgical Oncology, Sri Venkateswara Institute of Medical Sciences, Tirupati, Andhra Pradesh - 517 507
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2278-9596.117144

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  Abstract 

Pancreatic endocrine tumors are uncommon and represent 1-2% of all pancreatic neoplasms. Nonfunctioning pancreatic endocrine tumors constitute 30-40% of pancreatic endocrine tumors. They can occur at any age, but are rare in childhood. About 65-80% of nonfunctioning pancreatic endocrine tumors are associated with clear-cut evidence of malignant behavior. A 14-year-old girl presented with abdominal pain of 2 weeks duration with occasional vomiting. Abdominal examination did not reveal any abnormality. Ultrasound and computed tomography (CT) scan of the abdomen showed solid mass lesion located in the head of pancreas. The patient underwent Whipple's pancreaticodudenectomy. Microscopically, the mass was circumscribed, containing oval to polygonal cells. There was no pleomorphism, significant mitotic activity or evidence of capsular or vascular invasion, and a diagnosis of benign pancreatic islet cell tumor was made. Although benign nonfunctioning pancreatic islet cell tumor is rare in children, such diagnosis should be considered when there is an isolated mass in the pancreas. Appropriate resection of such mass is attended with good outcome.

Keywords: Nonfunctioning islet cell tumor, pancreas, pancreatic endocrine tumor, pancreatic islet cell tumor


How to cite this article:
Patnayak R, Yootla M, Parthasarathy S, Reddy V, Jena A, Reddy MK. A case of nonfunctioning pancreatic islet cell tumor in a young female. Arch Int Surg 2013;3:76-8

How to cite this URL:
Patnayak R, Yootla M, Parthasarathy S, Reddy V, Jena A, Reddy MK. A case of nonfunctioning pancreatic islet cell tumor in a young female. Arch Int Surg [serial online] 2013 [cited 2024 Mar 28];3:76-8. Available from: https://www.archintsurg.org/text.asp?2013/3/1/76/117144


  Introduction Top


Pancreatic islet cell tumors are rare pancreatic or peripancreatic tumors which constitute a broad spectrum of endocrine neoplasms. [1],[2] They are classified clinically as either functioning or nonfunctioning based on the symptoms produced from hormone production. Functioning islet cell tumors (FIT) produce symptoms because of excessive production of hormones. [2],[3] The important tumors that produce clinical syndromes due to excessive production of hormones include insulinomas, gastrinomas, glucagonomas, somatostitonomas, VIPomas (vasoactive intestinal polypeptide), and ACTH (adrenocorticotropic hormone) producing tumors. The classical manifestations of insulinomas include symptoms of hypoglycemia and inappropriately elevated levels of insulin. The Whipple's triad comprising of hypoglycemia, low serum glucose level, and resolution of symptoms with administration of glucose is a classical sign of insulinomas. [2] The hallmarks of somatostatinoma syndrome include elevated serum levels of somatostatin, diabetes mellitus, gallbladder disease, and steatorrhea. VIPoma also called as the Verner-Morrison syndrome or the watery diarrhea, hypokalemia, achlorhydria syndrome results due to excessive quantities of vasoactive intestinal polypeptide. Excessive levels of ACTH results in increased cortisol levels and produce a number of signs and symptoms; including impaired glucose tolerance, central obesity, hypertension, oligomenorrhea, osteoporosis, purpura and striae, and muscle atrophy. [1] Therefore, these tumors are diagnosed early, even when the lesions are smaller than 2 cm. [2] Whereas nonfunctioning islet cell tumors (NIT) are usually large in size and commonly present as an abdominal mass or symptom complex related to invasion of adjacent structures. [2],[3]

Nonfunctioning pancreatic endocrine tumors constitute 30-40% of pancreatic endocrine tumors. [1] They can occur at any age, but are rare in childhood. [2] We present the management of a rare case of NIT in a child aged 14 years.


  Case Report Top


A 14-year-old girl presented with abdominal pain of 2 weeks duration and vomiting for 4 days. At that time, she had intermittent attacks of right upper quadrant abdominal pain. The pain was dull aching in nature, not radiating, associated with no aggravating factors, and was relieved with analgesics. The pain increased in severity since 1 week and was not relieved with medication. The pain was not associated with hemetemesis or malena, alteration of bowel habits, abdominal distention, weight loss, or jaundice. There was no history of intermittent attacks of headache or blurred vision or seizures.

She had no previous history of diabetes or hypertension, but had complaints of oligomenorrhoea. There was no significant family history. Abdominal examination did not reveal any abnormality. The abdomen was soft and nontender. There was no organomegally or ascites. Serum urea, electrolytes, and creatinine levels were within normal limits. Abdominal ultrasound revealed space occupying lesion in the head of pancreas. Computed tomography (CT) scan of abdomen showed a solid heterogeneously enhancing mass lesion arising from the head and uncinate process of the pancreas [Figure 1]. A diagnosis of pancreatic tumor was made and the patient was prepared for operation. Intraoperatively, the soft mass was in the head of the pancreas. There was no evidence of secondaries in the liver or other adjacent organs. The patient underwent Whipple's pancreaticodudenectomy. Retrocolic hepatojejunostomy, gastrojejunostomy, and pancreaticojejunostomy were done. Feeding jejunostomy was also done. Macroscopically the lesion was 5.0 × 4.0 × 3.0 cm. Cut section showed a well-demarcated lesion with fleshy areas [Figure 2]. Histopathological examination of the mass showed a well-circumscribed lesion comprising oval to polygonal cells arranged in nests and sheets without any significant pleomorphism, mitotic activity, necrosis, or any evidence of capsular or vascular invasion [Figure 3]. Immunohistochemically, the tumor cells were strongly positive for endocrine markers like synaptophysin; neuron specific enolase; chromogranin; and were negative for hormonal markers like somatostatin, insulin, glucagon, and gastrin [Figure 4]. The serum prolactin levels assessed afterwards were within normal limits. The patient is doing well after 5 years of follow-up.
Figure 1: Computed tomography (CT) scan of abdomen showed a solid mass lesion arising from the head and uncinate process of the pancreas

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Figure 2: Gross picture of well-demarcated mass lesion with fleshy areas

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Figure 3: Microscopic picture showing islet tumor with polygonal cells arranged in nests and sheets (hematoxylin and eosin (H and E), ×20)

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Figure 4: Immunoreactivity for synaptophysin (immunohistochemistry, ×20)

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  Discussion Top


Nonfunctioning islet cell tumors are rare. [4] NITs represent one-half of all islet cell tumors and are usually greater than 5 cm at the time of diagnosis. [3] Clinically, NITs affect patients from 24-74 years with a mean age of 57 years. Though NITs do not produce symptoms related to excessive hormone production, these tumors can still be hormonally active. [2],[3] NITs occur more often in female than in male. Buetow et al., in their study have opined that nonhyperfunctioning islet tumors are large heterogeneous masses and commonly show cystic change, necrosis, calcification, local invasion, vascular invasion, and distant metastases. [5]

The present case is a 14-year-old young female. Very few cases have been described in this age group. The size of the present tumor was 5 cm and it showed homogenous fleshy areas without any cystic change. Mostly NITs are located in the pancreatic body and tail. [2] The location of tumor in our case was the head of the pancreas. Usually, NIT patients are asymptomatic. Symptoms like abdominal pain, jaundice, variceal bleeding, palpable mass, and gastric outlet obstruction are attributed to mass effect from their large size resulting in compression of the adjacent structures. [2] In our patient, such symptoms were not present probably because the tumor was small at the time of presentation. Regarding their histogenesis, it has been proposed that islet tumors originate either from amine precursor uptake and decarboxylation (APUD) cells or from multipotential cells in the pancreatic ductal epithelium. [1]

The preoperative diagnostic rate of NIT is rather low, owing to difficulty in its differentiation from pancreatic tumor or retroperitoneal mass. [4] As shown in our patient, with advancement of imaging technology, even smaller nonfunctioning islet cell tumors are being diagnosed in asymptomatic patients. [5] However, the imaging features of pancreatic NIT can overlap with other pancreatic neoplasms. Therefore, according to Sukaiti et al., combining the imaging features of pancreatic NIT and its hepatic metastases helps in narrowing the differential diagnosis. [2]

Histologically, functioning and nonfunctioning islet cell tumors are identical with three observed patterns: (1) Solid, diffuse pattern; (2) ribbon like, trabecular pattern; and (3) an acinar or duct-like pattern. Immunohistochemical staining also may not be reliable in differentiating between functioning and nonfunctioning islet cell tumors. [3] Therefore, emphasis should be placed on good history and biochemical tests in order to identify NIT preoperatively. Since the prognosis of even malignant NIT after surgical removal is favorable with 5-year survival rate being 53.1%, active treatment is strongly recommended. [4],[6]

In conclusion, the present case is an additional rare benign nonfunctioning pancreatic islet cell tumor in a 14-year-old girl. Following diagnosis and appropriate evaluation, prompt surgical intervention is attended with good outcome.


  Acknowledgment Top


We thank the Department of Radiology and senior laboratory technicians Mrs. Ushanandini and Mr. Ramana for their help.

 
  References Top

1.Buetow PC, Miller DL, Parrino TV, Buck JL. Islet cell tumors of the pancreas: Clinical, radiologic, and pathologic correlation in diagnosis and localization. Radiographics 1997;17:453-72.   Back to cited text no. 1
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2.Sukaiti RA, Robinson K, Menias C. Retrospective Review of Cross Sectional Imaging Findings of Pancreatic Non-functional Islet Cell Tumor (NFICT) and its Hepatic Metastases. Oman Med J 2011;26:39-42.  Back to cited text no. 2
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3.White TJ, Edney JA, Thompson JS, Karrer FW, Moor BJ. Is there a prognostic difference between functional and nonfunctional islet cell tumors? Am J Surg 1994;168:627-9.  Back to cited text no. 3
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4.Yang Z, Zhao P, Liu Z, Tang W, Zhong S. Non-functional islet-cell tumor: Analysis of 237 cases. Zhonghua Yi Xue Za Zhi 2002;82:376-8.  Back to cited text no. 4
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5.Buetow PC, Parrino TV, Buck JL, Pantongrag-Brown L, Ros PR, Dachman AH, et al. Islet cell tumors of the pancreas: Pathologic-imaging correlation among size, necrosis and cysts, calcification, malignant behavior, and functional status. AJR Am J Roentgenol 1995;165:1175-9.  Back to cited text no. 5
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6.Yang CS, Shyr YM, Chiu CT, Su CH, Lin CP, Lin JT. Non-functioning islet cell tumors of the pancreas--a multicentric clinical study in Taiwan. Hepatogastroenterology 2000;47:1747-9.  Back to cited text no. 6
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    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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