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|Year : 1992 | Volume
| Issue : 4 | Page : 210-1,207
Primary extrapancreatic gastrinoma.
PR Pai, RB Shahani, HK Shah, AN Dalvi, AB Samsi, IM Vora
Dept of Surgery, Seth GS Medical College, Parel, Bombay, Maharashtra.
P R Pai
Dept of Surgery, Seth GS Medical College, Parel, Bombay, Maharashtra.
Source of Support: None, Conflict of Interest: None
Extrapancreatic gastrinoma is a rare clinical entity encountered in surgical practice. A patient was referred to us who had a history of recurring symptoms of peptic ulcer disease and ulcer perforation located at an unusual site. Serum gastrin levels were abnormally high. Scopy revealed multiple ulcers in the antrum and duodenum. A mass superior to the head of the pancreas was detected on USG, which later on found to be a separate mass on CT scan. The tumour was excised and confirmed on histopathology. Results of conservative surgery were found to be satisfactory.
Keywords: Adolescent, Case Report, Endoscopy, Gastrointestinal, Gastrinoma, blood,diagnosis,surgery,Gastrins, blood,Human, Male, Pancreatic Neoplasms, blood,diagnosis,surgery,Tomography, X-Ray Computed, Vagotomy, Truncal,
|How to cite this article:|
Pai P R, Shahani R B, Shah H K, Dalvi A N, Samsi A B, Vora I M. Primary extrapancreatic gastrinoma. J Postgrad Med 1992;38:210-1,207
Extrapancreatic gastrinoma is a rare clinical entity encountered in surgical practice. Diagnosis and management of gastrinomas had undergone a profound change over the last thirty years. Better pre-operative localisation and newer drugs have changed the management from radical to conservative therapy. We had one such case under our care. Rarity of this condition and changing trends in management, have prompted us to publish this data. The literature is also briefly reviewed.
SS, a 16-year-old male, was referred to us with suspicion of Zollinger-Ellison syndrome from a peripheral Hospital. Two months prior to his presentation to us, he had undergone an emergency laparotomy for peritionitis where a perforation was found to be located in an unusual oosition viz. in the first part of jejunum, 5 cm from duodeno-jejunal flexture raising the possibility of Zoilinger-Effison syndrome. He also gave a 2 year history for H2 receptor antagonist controlled recurrent duodenal ulcer symptoms.
On examination, he was found to be averagely built and nourished with a well healed, right upper paramedian scar on the abdomen.
Basic biochemical profile was within normal limits. Oesophago-gastro-duodenoscopy revealed multiple ulcers in the antrum as well as first and second part of duodenum. Biopsies from the antrum, body and fundus of the stomach were normal. Uitransonography revealed presence of a well-circumscribed mass superior to the head of the pancreas. No other abnormality was detected. Serum gastrin levels were 919 ng/1 (Normal upto 90 ng/l). CT scan of abdomen confirmed the presence of a well-encapsulated mass lying separate form the pancreas (See [Figure - 1] on page 207). Serum calcium, phosphorus, parathormone levels along with radiographs of skull, hands and spine were normal ruling out hyperparathyroidism (MEN 1).
The patient was explored through the previous incision. A well-encapsulated yellowish orange mass 4cm x 4 cm was seen lying superior to the head of pancreas in the gastrinoma triangle. The mass was not adherent to any vital structures and could be easily excised. Rest of the pancreas was normal to palpation. Duodenum was normal. No other abnormality was detected. Truncal vagotomy with pyloroplasty was done. Patient had an uneventful post- operative course and was well nine months after surgery. Post- operative serum gastrin levels were normal.
Tumour was composed of monotonous and uniform cells, which were rounded or polyhedral with scanty cytoplasm and uniform hyperchromatic nuclei. Tumour cells were arranged in a typical endocrine pattern having ribbon like cords, solid alveoli, rosette like acinar formation or a sheet like pattern with in between connective tissue stroma. (See [Figure - 2]).
The gastrinoma or Zollinger-Ellison syndrome is one of the fulminant clinical presentations of peptic ulcer disease caused by gastric hyperacidity and gastrin producing islet cell tumour.
A gastrinoma is typically suspected in a young with symptoms of fulminant complications of peptic ulcer disease or those with poor response to therapy. Incidence of gastrinoma is reported to by anywhere between 1 in 200 to 1 in 1000 cases of duodenal ulcer disease . Perforations occur in about 15% of cases.
Most of these tumours are localised in the gastrinoma triangle as described by Stabile et al. Localisation of these tumours especially when small is possible only in 30-40% cases in spite of newer investigations like ultrasonography, CT scan, angiography, isotope scanning and selective venous sampling.
In our case, the tumour was found to be located superiorly free from the head of pancreas, posterior to the lesser omentum. Extrapancreatic, extraintestinal gastrinomas usually occur in lymph nodes,,,. Absence of lymph nodal architecture in our tumour and presence of normal pancreas without involvement of peripancreatic nodes led us to the conclusion of extra-pancreatic, extraintestinal, extranodal gastrinoma.
Management of Zollinger-Ellison syndrome has undergone a radical change following advances in diagnostic localizing modalities and drug treatment. Drugs like omeprazole and SMS-201-905 (somatostatin analogue) effectively control hyperacidity and symptomatology. Age-old therapy of i:otal gastrectomy with attendant morbidity/mortality has now been replaced by enucleation or local excision of tumour with or without parietal cell vagotomy,. Patients so treated have done well even at 5 year follow-up.
In our case, local excision with truncal vagotomy and drainage succeeded in achieving normal post-operative gastrin levels and abolishing symptomatology of the patient at 9 months follow-up.
We thank Dr PM Pai, the Dean, Seth GS Medical College and King Edward Memorial Hospital, Mumbai for allowing us to publish the hospital data.
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[Figure - 1], [Figure - 2]