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WeBSurg, the e-surgical reference of Laparoscopic surgery

Clinical Case

LATE DIAGNOSIS OF A GASTRINOMA


M Vix, MD, Hôpitaux Universitaires de Strasbourg, Strasbourg, France

1. Summary


Delay in diagnosis and treatment of a gastrinoma is not uncommon. In this particular case, the clinical history started four years before the diagnosis of gastrinoma was made. Surgical treatment consisted of a standard open pancreatic cephalic head resection (Whipple procedure).

2. Keywords


Ulcer, gastrinoma, Zollinger-Ellison, stomach

3. Patient


Patient, 59 years old

4. Past history


Chronic alcoholism

5. History of the disease


5.1. 4 years before

Four years prior to presentation the patient was hospitalized for weight loss, diarrhea and a right hernia.
Laboratory values: Hypogammaglobulinemia and macrocytosis were found and were attributed to a secondary immuno-deficiency.
Bone marrow biopsy: non contributive
Small bowel series: a thickened wall of the jejunum
Upper endoscopy: well-healed esophageal ulcers s/p medical treatment, duodenal biopsies non-contributive
CT scan: peri-pancreatic lymph nodes
Laparoscopic LN biopsy: performed for both treatment of a right inguinal hernia and to perform a biopsy of the celiac lymph node. Pathology: benign (sinusoidal histiocytosis)
Tumor markers: CA125, CA 19.9, CA15.3, ACE, alpha-fetoprotein: all normal
Standard blood tests: normal

5.2. 2 years before

2 years prior, the patient was admitted for peritonitis due to a large perforation of the first part of the jejunum. An end-block resection of small bowel with primary anastomosis was performed. On examination of the resected bowel, it appeared that the perforation was due to a non-specific ischemic lesion. The underlying cause was initially thought to be due to Zollinger-Ellison syndrome.
This diagnosis was ruled out due to the low gastrin level, 46 pg/ml (<115). The patient refused medical follow-up.
A peptic stenosis of the esophagus appears few months later. It was treated medically.

5.3. History of present illness

The patient presented with abdominal pain and recent weight loss.

6. Examinations


6.1. Physical examination

Unremarkable except for weight loss

6.2. Sonogram

An ultrasound exam of the abdomen revealed voluminous sub-hepatic and lateral duodenal lymph node.

6.3. CT scan

Poly-lobed mass that “lights-up” moderately after contrast injection. The mass appears to be independent of the pancreas.
Sagittal sections confirms the appearance and the location of the sub-hepatic mass.

6.4. MRI

Presence of a voluminous multi-lobulated mass beginning at the level of the left retro-hepatic region, extending posterior to the body of pancreas and wrapping around the head of the pancreas to envelope the duodenum. It had the appearance of either one single mass, or two masses side by side. Its horizontal and A-P diameter was about 3 centimeters and its cephalo-caudal diameter is 10 cm. It had a low T1-weighted signal and a heterogeneous high T2-weighted signal. It appeared to be well-encapsulated and “lit-up” after injection of contrast. There were no anomalies of the pancreas itself, except for apparent retraction on the head of the pancreas by the mass. There was no compression of the biliary ducts. The CBD was narrow, without any anomaly of the choledocho-duodenal junction. There were no anomalies of the liver parenchyma and no splenic anomalies. A small gallstone was noted.

6.5. Endoscopic ultrasonography

3 hypo-echoic lesions were demonstrated utilizing a linear probe which does not allow for biopsies. They appeared to be lymph nodes, with an average diameter of 3 cm. Large gastric folds were visualized. No tumor was localized in the duodenum, nor in the head of the pancreas.

6.6. Blood Tests

- elevated serum gastrin levels - varying between 1000 et 3000 pg/ml (N<115);
- VIP, catecholamines, CA19.9, ACE, Alpha FP: normal.

7. Operation


- pancreatic cephalic head resection+ antrectomy (Whipple procedure and antrectomy);
- dissection of the peri-hepatic lymph nodes.

The following pictures show the surgical specimen, with the anterior (a), posterior (b) and interior (opened) (c) views.

8. Pathology


Three polypoid, intra-duodenal lesions corresponding to the initial gastrinoma, ganglion metastases.

9. Discussion


Gastrinomas are tumor proliferations of non-B cells of the islets of Langerhans. They are the cause of elevated gastrin levels, an hormone which is normally secreted by the G-cells of the gastric antrum. They are considered paracrine tumors and the incidence is estimated at 1 case per 2 million inhabitants. The typical patient age is between 40 and 60 years old.
These tumors are often small, occult, multiple, malignant and often found as part of one of the multiple endocrine neoplasia (MEN1).
More than 90% of the tumors are localized in the “gastrinoma triangle”, located in the area delineated by the following three structures: the third part of the duodenum, the pancreatic isthmus, and the liver hilum.
Classic signs and symptoms include: ulcerous lesions of the duodenum, esophagus, stomach, and jejunum, and a history of chronic diarrhea.

In this case, the diagnosis of gastrinoma had been entertained at first presentation, but the initial laboratory data did not support it.
When the patient presented with a small bowel perforation, the diagnosis of a gastrinoma was once again entertained. A secretin simulation test had been scheduled to confirm the diagnosis, but the patient had refused it at the time.
A pancreatic cephalic head resection- was performed in order to remove all involved lymph nodes along with the primary lesion, thus resecting the entire “gastrinoma triangle”.

10. Zollinger-Ellison syndrome


Zollinger-Ellison syndrome defined by the following:
Patient profile: young, family history of ulcers or gastric endocrine pathology.
Medical history: ulcers disease highly resistance to medical treatment, frequently presenting already with complications of ulcer disease.
Signs and symptoms: frequent emesis, without evidence of pyloric stenosis and associated with diarrhea.
Ulcer topography: multiple ulcers in unusual locations.

Gastrinoma is diagnosed based on laboratory evidence of gastrin hyper-secretion and/or an elevated baseline gastrin level or elevation post-secretin stimulation test. Once the diagnosis is made, tumor-localization staging should be performed for both sporadic and MEN-related gastrinomas.

11. Imaging studies


Preoperative ultrasonography of the abdomen aids in localization of tumors in less than one third of the cases, due to the typically small size of the tumors.
IV contrast CT scan allows localization in up to 40 - 60% of cases.
MRI aids in detection of tumors which are larger than 2 cm, therefore is not useful with smaller sized tumors.
Selective angiography is operator-dependent; its effectiveness ranges from 13 to 86% of cases.
Selective arterial injection of secretin followed by hepatic or peripheral venous gastrin determinations may demonstrate the arterial supply and therefore the location of the tumor. Pancreatic venous sampling may also be performed.
Endoscopic ultrasonography may be utilized to improve localization in the gastric antrum, the duodenum and the head of the pancreas. Use of axial probes allow biopsies to be performed during the procedure.

Although imagery should provide a topographic orientation, intraoperative exploration and palpation by an experienced surgeon, aided by a preoperative imagery, remains an indispensable part of a curative resection.

12. References


  1. Nicolas D, Ortiz V et al. Zollinger-Ellison syndrome. Gastroenterol Hepatol 1999; 22:100-12.
  2. Proye C, Malvaux P, Pattou F, Filoche B, Godchaux JM, Maunoury V, Palazzo L, Huglo D, Lefebvre J, Paris JC. Non-invasive imaging of insulinomas and gastrinomas with endoscopic ultrasonography and somatostatin receptor scintigraphy. Surgery 1998;124:1134-43.
  3. Sato T, Konishi K, Kimura H, Maeda K, Yabushita K, Tsuji M, Demachi H, Miwa A. Strategy for pancreatic endocrine tumors. Hepato-Gastroenterology 2000;47:537-9.
  4. Schindl M, Kaczirek K et al. Is the new classification of neuroendocrine pancreatic tumors of clinical help? World J Surg 2000;24:1312-8.