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 Table of Contents  
CASE REPORT
Year : 2014  |  Volume : 34  |  Issue : 5  |  Page : 217-219

Endocrine carcinoma of the pancreatic tail exhibiting gastric variceal bleeding


1 Department of Surgery, Division of General Surgery, Tri-Service General Hospital, National Defense Medical Center, Taipei, Taiwan, Republic of China
2 Department of Medicine, Division of Gastroenterology, Tri-Service General Hospital, National Defense Medical Center, Taipei, Taiwan, Republic of China
3 Department of Biology and Anatomy, National Defense Medical Center, Taipei, Taiwan, Republic of China

Date of Submission05-Aug-2013
Date of Decision02-Jul-2014
Date of Acceptance10-Jul-2014
Date of Web Publication30-Oct-2014

Correspondence Address:
Dr. Shing-Hwa Huang
Department of Surgery, Division of General Surgery, Tri-Service General Hospital, National Defense Medical Center, No. 325, Sec 2, Cheng-Gong Road, Taipei 114, Taiwan
Republic of China
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/1011-4564.143650

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  Abstract 

Nonfunctional endocrine carcinoma of the pancreas is uncommon. Without excess hormone secretion, it is clinically silent until the enlarging or metastatic tumor causes compressive symptoms. Epigastric pain, dyspepsia, jaundice, and abdominal mass are the usual symptoms, whereas upper gastrointestinal (GI) bleeding is rare. Here, we describe the case of a 24-year-old man with the chief complaint of hematemesis. Upper GI panendoscopy revealed isolated gastric varices at the fundus and upper body. Ultrasonography and computed tomography showed a tumor mass at the pancreatic tail causing a splenic vein obstruction, engorged vessels near the fundus of the stomach, and splenomegaly. After distal pancreatectomy and splenectomy, the bleeding did not recur. The final pathologic diagnosis was endocrine carcinoma of the pancreas. Gastric variceal bleeding is a possible manifestation of nonfunctional endocrine carcinoma of the pancreas if the splenic vein is affected by a tumor. In non-cirrhotic patients with isolated gastric variceal bleeding, the differential diagnosis should include pancreatic disorders.

Keywords: Isolated gastric varices, pancreatic tumor, hematemesis, endocrine carcinoma


How to cite this article:
Wu SY, Chang YM, Yu JC, Chan DC, Li CH, Lin GJ, Huang SH. Endocrine carcinoma of the pancreatic tail exhibiting gastric variceal bleeding. J Med Sci 2014;34:217-9

How to cite this URL:
Wu SY, Chang YM, Yu JC, Chan DC, Li CH, Lin GJ, Huang SH. Endocrine carcinoma of the pancreatic tail exhibiting gastric variceal bleeding. J Med Sci [serial online] 2014 [cited 2019 Aug 23];34:217-9. Available from: http://www.jmedscindmc.com/text.asp?2014/34/5/217/143650


  Introduction Top


Gastroesophageal variceal bleeding is often secondary to liver cirrhosis. In non-cirrhotic patients, isolated gastric varices at the fundus could result from splenic vein obstruction, which causes venous hypertension and the formation of splenoportal collaterals. [1] As such, pancreatic disorders that induce splenic vein obstruction can present with upper gastrointestinal (GI) bleeding. [2] Here, we describe a young man who presented with gastric variceal bleeding, and the final diagnosis was endocrine carcinoma of the pancreas. Its cause may be segmental portal hypertension secondary to splenic vein involvement by a pancreatic neoplasm.


  Case Report Top


A 24-year-old Chinese man presented with the chief complaint of hematemesis. Physical examination in the emergency room showed no signs of acute abdomen. A blood test revealed anemia (hemoglobin, 10.6 mg/dL) and a normal platelet count. Liver function test results were normal, and hepatitis B and C serologies were negative. The serum glucose level was also normal (99 mg/dL). Upper GI endoscopy showed an ulcerative lesion with hemorrhagic stigmata at the greater curvature of the gastric fundus suggestive of bleeding gastric varices. The bleeding stopped temporally after endoscopic hemostasis by epinephrine injection and clipping [Figure 1]a. No esophageal varices were seen [Figure 1]b.
Figure 1. Upper gastrointestinal endoscopy image showing bleeding from varices in the gastric fundus. (a) Emergent hemostasis using epinephrine injection and clipping was performed. (b) EG junction: No esophageal varices or ulcers are seen

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Subsequent abdominal ultrasonography showed splenomegaly and a pancreatic tumor, while the contour, size, and echogenicity of the liver were normal. Computed tomography (CT) scans of the abdomen revealed an ill-defined lobulated mass measuring 6.8 cm × 4.4 cm × 4.2 cm at the pancreatic tail with heterogeneous contrast enhancement during a dynamic study. The neoplasm completely encased the splenic vein, within which thrombus formation was detected [Figure 2]. Multiple engorged short gastric vessels were noted at the fundus of the stomach.
Figure 2. Coronary view of a computed tomography image showing that the tumor (T) occupies the pancreatic tail and involves the splenic vein. Without cirrhosis of the liver or portal vein thrombosis, isolated engorgement of the short gastric veins and coronary vein are remarkable, showing the sequence of splenic vein obstruction by the pancreatic tumor

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One week later, the patient experienced massive hematemesis despite repeat endoscopic hematostasis for which an emergent laparotomy was performed. One 4 × 4 × 3 cm indurated mass [Figure 3] at the pancreatic tail constricted the splenic vein and was considered to be associated with splenomegaly and torturous engorgement of the short gastric and gastroepiploic veins. As such, gastric devascularization, distal pancreatectomy, and splenectomy were performed. Microscopic examination showed a low-grade, well-differentiated endocrine carcinoma. The tumor had invaded the peripancreatic fatty tissue and metastasized to the peripancreatic lymph nodes. All of the resection margins were free of tumor invasion. Immunohistochemical staining showed tumor cells positive for chromogranin-A [Figure 4], synaptophysin, and neuron-specific enolase. The final diagnosis was nonfunctional endocrine carcinoma of the pancreas (T2N1M0, stage IIB). The gastric variceal bleeding secondary to splenic vein stenosis by the tumor was confirmed and cured surgically.
Figure 3. One grey-white solid tumor arising from the pancreatic tail (black arrow) with encasement of the splenic vessels (white arrow)

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Figure 4. Histopathology image demonstrating the organoid architecture, hyalinized stroma, and coarsely clumped chromatin but few mitotic cells. This finding corresponds to well-differentiated endocrine carcinoma of the pancreas. (Brown spots: Positive immunohistochemical staining for chromogranin, ×200)

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The patient's postoperative recovery was uneventful, and he was discharged 14 days after the operation. He underwent adjuvant chemoradiotherapy with gemcitabine and cisplatin without target therapy like sunitinib. No recurrence and no GI bleeding were noted during the 3 years of follow-up at our hospital.


  Discussion Top


Endocrine tumors of the pancreas account for approximately 1-2% of pancreatic tumors. Almost half of these tumors secrete hormones (insulinoma, gastrinoma, glucagonomas, somatostatinomas, vasoactive intestinal polypeptide-secreting tumors [VIPomas]) and are associated with clinical metabolic or GI disorders. [3] Instead, nonfunctional endocrine tumors cause symptoms after the enlarging or metastatic tumor compresses the adjacent organ, which manifests as epigastric pain, anorexia, back pain, and jaundice. The increasing use of cross-sectional imaging has resulted in the identification of tumors in more asymptomatic patients. [4] However, the vague symptoms may also result in a diagnosis that is delayed until metastasis occurs, which worsens the prognosis.

Endocrine tumor of the pancreas that presents with acute upper GI bleeding is uncommon. In fact, there are only 22 cases reported in the English literature since 1970. Among them, most were nonfunctional tumor but one was a VIPoma. [5] Gastric varices were frequently found in the fundus of the stomachs of these patients. The tumors disturbed the splenic venous return in all patients as well, causing segmental portal hypertension (or left-sided [sinistral] portal hypertension). After tumor resection and splenectomy, no recurrence of variceal bleeding was reported.

Isolated gastric varices are defined as gastric varices without esophageal varices. [6] Portal hypertension secondary to cirrhosis of the liver remains the most common etiology. However, patency of the splenic vein in the presence of pancreatic disorders needs to be checked, especially when fundal varices are involved. [1] Since short gastric veins (SGVs) at the greater curvature of the gastric fundus are confluent with the splenic, gastroepiploic, and coronary veins (CORs), stenosis or occlusion of the splenic vein results in segmental portal hypertension. In this patient, the splenomegaly, engorged SGVs, and CORs noted on CT [Figure 1] could have resulted from the splenic vein obstruction by the neoplasm at the pancreatic tail.

Bleeding gastric varices should be treated with octreotide and balloon tamponade followed with either a transjugular intrahepatic portosystemic shunt or surgery. Endoscopic therapy including variceal sclerotherapy, variceal obturation with cyanoacrylate, and variceal ligation with nylon or snares or rubber bands could be attempted. [7] Our patient did not receive standard endoscopic therapy during the first acute bleeding episode due to the uncertainty of his endoscopic diagnosis and the fair initial response to the epinephrine injection and clipping. When a tumor of the pancreas is discovered on CT, a subsequent episode of gastric variceal bleeding demands emergent surgery.

Distal pancreatectomy and splenectomy are the standard treatment for neoplasms at the pancreatic tail. Splenectomy is used as curative surgery and releases the segmental portal hypertension since it cuts off venous return from the splenic hilum and a portion of the SGV. In patients with prior bleeding episodes, it is reasonable to perform a splenectomy alone with distal pancreatectomy. [8]

Nonfunctional endocrine tumor of the pancreas is difficult to diagnose owing to its vague symptoms. Upper GI bleeding is a rare but possible manifestation in such patients. In non-cirrhotic patients with isolated gastric variceal bleeding, attention should be paid to segmental portal hypertension related to the pancreatic neoplasm. Gastric varices along the greater curvature of the fundus also require cross-sectional imaging to verify splenic vein patency.


  Disclosure Top


The authors report no conflicts of interest in this work.

 
  References Top

1.
Madsen MS, Petersen TH, Sommer H. Segmental portal hypertension. Ann Surg 1986;204:72-7.  Back to cited text no. 1
    
2.
Hwang TL, Jan YY, Jeng LB, Chen MF, Hung CF, Chiu CT. The different manifestation and outcome between pancreatitis and pancreatic malignancy with left-sided portal hypertension. Int Surg 1999;84:209-12.  Back to cited text no. 2
    
3.
Zhou C, Zhang J, Zheng Y, Zhu Z. Pancreatic neuroendocrine tumors: A comprehensive review. Int J Cancer 2012;131:1013-22.  Back to cited text no. 3
    
4.
Vagefi PA, Razo O, Deshpande V, McGrath DJ, Lauwers GY, Thayer SP, et al. Evolving patterns in the detection and outcomes of pancreatic neuroendocrine neoplasms: The Massachusetts General Hospital experience from 1977 to 2005. Arch Surg 2007;142:347-54.  Back to cited text no. 4
    
5.
Joyce DL, Hong K, Fishman EK, Wisell J, Pawlik TM. Multi-visceral resection of pancreatic VIPoma in a patient with sinistral portal hypertension. World J Surg Oncol 2008;6:80.  Back to cited text no. 5
    
6.
Sarin SK, Jain AK, Lamba GS, Gupta R, Chowdhary A. Isolated gastric varices: Prevalence, clinical relevance and natural history. Dig Surg 2003;20:42-7.  Back to cited text no. 6
    
7.
Ryan BM, Stockbrugger RW, Ryan JM. A pathophysiologic, gastroenterologic, and radiologic approach to the management of gastric varices. Gastroenterology 2004;126:1175-89.  Back to cited text no. 7
    
8.
Loftus JP, Nagorney DM, Ilstrup D, Kunselman A. Sinistral portal hypertension. Splenectomy or expectant management. Ann Surg 1993;217:35.  Back to cited text no. 8
    


    Figures

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


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