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GastroenterologyStomach & duodenum

Malignant diseases

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Please submit a new question (maximum 100 words) or any comments to QandA@GastroHep.com. Please make sure you include the title of the Q&A you are commenting on in the subject line of your email. Please note that this service is only for medically qualified professionals, and not for the general public. The questions should be based on a clinical problem. You may nominate the expert from our Global Academic Faculty whom you would like us to contact for the first reply. Don't forget to include your full name, city, country and your email address.

I performed an EGD on a 32 year old woman with dyspepsia. Her mother died at age 40 with linitis plastica. There was mild antral erythema seen at the patient's EGD, but no other abnormalites. Antral biopsies showed signet cells. This was confirmed by immunohistochemical stains. Multiple deep biopsies taken at a subsequent were negative. EUS and CT scan were also negative.

Should this patient undergo a partial gastrectomy or be followed with surveillance endoscopy?

Dave Talabiska, Lewisburg, PA, USA   
 

Answer 1: This case sounds likely to represent one of hereditary diffuse gastric cancer associated with mutation of the E-cadherin gene (CDH1), as described in the New England Journal of Medicine 2001; 344(25): 1904-9. In this condition, it is recognized that there are superficial infiltrates of malignant signet-ring cells, which are often multi-focal. Therefore, at present, it seems the only appropriate treatment would be that of a total gastroectomy (some have even done it prophylactically).

R V Heatley, Leeds, England  

 

Answer 2: Because the distribution of signet ring cells is known to be patchy, sampling error could not be avoided by even multiple deep biopsies. Moreover, signet ring cell carcinoma is known to be of rapid progressive tumor. She should undergo a partial gastrectomy.

Nobuhiro Sato, Japan  

 

Answer 3: I would take the usual precautions of checking with the laboratory that there may not have been contamination with malignant cells from another patient during automatic processing and having the histology reviewed. (I assume this has been done already.)

Thirty years ago I was privileged to spend 6 months working in the gastroenterology unit in the Aichi Cancer Centre in Nagoya, Japan. Apart from seeing more early gastric cancer there than I have in the next 30 years in Sydney, I learned that in the situation being considered here the problem is much more likely to be a subtle mucosal or submucosal cancer rather than linitis plastica.

An early repeat endoscopy by an endoscopist with experience in diagnosing early gastric cancer would be optimal initial management. This is easier in Japan than in the West. It is a matter of experience, frequency of the lesion in a particular community and taking adequate time. Before doing the repeat endoscopy, the Western endoscopist should revise the Japanese classification of early gastric cancer and study a slide atlas of the various types of early gastric cancer. At the endoscopy, a lot of time should be devoted to looking for subtle abnormalities and taking multiple photographs and targeted biopsies in separately labeled containers. What had been written off at the first endoscopy as a "thickened fold" could be a Type 1 or Type 11a lesion; a slightly different colored patch of mucosa could be a 11b lesion or an insignificant depression a 11c. The signet cells are more likely to be found with Type 11c or Type 111 early cancer. A small lesion would be less likely to have lymph node metastases.

Dye scattering is worth considering in the very few units that do it routinely. Perhaps the patient should be referred to such a unit where they have a special interest and experience. As a one off procedure it usually doesn't help and the time spent is better devoted to detailed looking. It would be a shame to do an antrectomy if the early cancer is not in the antrum. Is it certain that the positive biopsies came from the antrum? Were the cells in the mucosa, submucosa or on the surface? Were the biopsies taken from mucosa that was clearly antral on histology? Was gastritis or metaplasia present?

One other thing I learned in Japan was that early cancers could take a long time to progress. In the patient we are considering, a follow-up gastroscopy now is recommended. Then, when additional information is available, the patient needs to be involved in further decision making but a third endoscopy in three months would be not unreasonable if doubt remains after the early second endoscopy.

The aim is to localize the lesion. Local operative resection of a documented early cancer is a possible option.

In some centers where ultrasound shows the lesion has not spread beyond the muscularis mucosae, endoscopic resection of this intestinal type adenocarcinoma might be considered and this would now be commonly done in Japan. However the cancer would need to be clearly identified at endoscopy before either of these two options would be possible.

I would be unhappy with a blind antrectomy as initial management but it might be justified in 3 months as outlined above if it were still not possible to identify the tumor.

Tim Heap, Sydney, Australia  

 

Answer 4: Obviously the mention of signet cells raises the possibility of carcinoma. However, since the histopathologist has not been more definite one way or the other makes me wonder about the degree of confidence that can be placed in the immunohistochemistry and cytokeratin-positive staining.

If the patient was my wife, I would be most reluctant that she undergoes gastric resection without clearer evidence of malignancy. One should also remember that other tumors, such as metastatic lobular breast cancer can produce this picture.

My counsel is that this patient requires close follow up and repeat endoscopic biopsy in a couple of months time. I am an upper GI surgeon and remember clearly the morbidity of gastric resection for duodenal ulcer disease. I am not suggesting that this has anything to do with DU disease, but partial gastrectomy is a fearsome operation.

I would be interested in follow up of this case.

Peter Devitt, Adelaide, Australia   

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DISCLAIMER: Clinical Q & A is for information only and is not a substitute for specific medical advice and diagnosis from a medical practitioner. Neither the content providers nor the publisher accept any legal responsibility for nor make any warranty with respect to the views expressed or procedures outlined on the site.

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 22 July 2014

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