Year : 2007 | Volume
: 13 | Issue : 4 | Page : 197--199
Concurrence of duodenal carcinoid and diffuse gastric adenocarcinoma: A rare phenomenon
Parampalli S Srilatha
Department of Pathology, Melaka Manipal Medical College (Manipal Campus), Manipal, Karnataka, India
Parampalli S Srilatha
Department of Pathology, Melaka Manipal Medical College (Manipal campus), Manipal - 576 104, Karnataka
Synchronous tumors are well documented in the gastrointestinal tract. Various combinations have been studied. There are a few studies, which highlight the incidental finding of the yellow beauty-the «DQ»carcinoid«DQ» of the duodenum simultaneously occurring with the venomous «DQ»diffuse gastric adenocarcinoma«DQ». This is a report one such case in a 58 year-old man, who unfortunately died on the 4 th postoperative day due to cardiopulmonary arrest.
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Srilatha PS. Concurrence of duodenal carcinoid and diffuse gastric adenocarcinoma: A rare phenomenon.Saudi J Gastroenterol 2007;13:197-199
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Srilatha PS. Concurrence of duodenal carcinoid and diffuse gastric adenocarcinoma: A rare phenomenon. Saudi J Gastroenterol [serial online] 2007 [cited 2021 Dec 6 ];13:197-199
Available from: https://www.saudijgastro.com/text.asp?2007/13/4/197/36754
Carcinoids of the gastrointestinal tract are often associated with another primary cancer. However, both the occurrence of duodenal carcinoid (1.5-5%) by itself and its association with synchronous primary tumors are rare. Our case showed two completely independent tumors (double primary) that occurred simultaneously-duodenal carcinoid and diffuse gastric adenocarcinoma.
A 58 year-old chronic alcoholic, smoker and known case of rheumatic heart disease presented with complaints of abdominal pain associated with nonbilious vomiting immediately after food intake, for the past one month. There were no complaints of melena or loose stools. He gave a past history of abdominal pain, which started 18 years back and was aggravated in the last one month. Endoscopy was done then and the patient was diagnosed to have a gastric ulcer. No records were available for whether any endoscopic biopsy was done.
When endoscopy was repeated for the present problem, an ulceroproliferative growth with slough was found in the gastric body and the antrum. The gastroesophageal junction was free. An ultrasonogram of the abdomen showed irregular thickening of the gastric body and the antral wall. The coeliac lymph nodes were also enlarged. An echocardiogram showed severe calcific aortic stenosis, grade-II aortic and mitral regurgitation, mitral valve and annulus calcification, mild pulmonary hypertension and concentric left ventricular hypertrophy.
An endoscopic biopsy of the gastric growth was done. The biopsy report revealed a diffuse gastric adenocarcinoma. The patient was discharged with advice to come for surgery. Within a week, he got readmitted with severe abdominal pain of two days' duration. He underwent surgery which revealed a perforated gastric carcinoma in the antrum. Enlarged coeliac plexus lymph nodes and multiple lymph nodes were present all along the blood vessels.
Since the patient was in shock with intraoperative hypotension, a radical total gastrectomy with D2/D3 lymphadenectomy was avoided. A distal subtotal gastrectomy and gastrojejunostomy were performed. Intraoperatively, hypotension was managed with ionotropic drugs and postoperatively, the patient was put on a ventilator. He was given antibiotics and diuretics. His condition deteriorated and on the 4 th postoperative day, he died of severe cardiopulmonary arrest.
Grossly, the gastrectomy specimen with attached omentum showed a thickened wall and flattened mucosa. The cut surface of the wall showed gray-white areas. A perforated ulcer was seen 2 cm from the pyloric end. It also showed gray-white areas. The resected margins showed normal mucosal folds and appeared free of tumor. Four lymph nodes were identified.
Microscopically, the ulcer and the thickened gray-white wall showed sheets of adenocarcinoma cells of the signet ring-type up to the serosa. The malignant cells [Figure 1] exhibited abundant vacuolated to eosinophilic cytoplasm and eccentric to centrally placed hyperchromatic, irregular nuclei. Some showed prominent nucleoli. Focal areas of necrosis and bacterial colonies were also seen. The proximal resected margin was free of tumor. The distal resected margin showed normal pyloric mucosa and the beginning of duodenal mucosa. They were free of the gastric adenocarcinoma.
Incidentally, a small carcinoid [Figure 2] was found in the duodenal submucosa, invested in a fibrous stroma. The small round cells were arranged in a trabecular and glandular pattern and had moderate cytoplasm with nuclei showing classical "salt and pepper" chromatin. Two out of four lymph nodes studied showed metastatic deposits from diffuse gastric adenocarcinoma.
Concurrence of adenocarcinoma and carcinoid tumors in the gastrointestinal tract has been observed infrequently.
In Japan where there is a high incidence of gastric carcinoma, a study done by Kawano et al.  discussed 46 cases of duodenal carcinoid associated with gastric carcinoma.
Gerstle et al. reviewed 69 patients with carcinoids in the gastrointestinal tract and found that 29 (42%) had synchronous and three (4%) had metachronous tumors. Almost 43% of the second primary cancer was in the gastrointestinal tract of which colorectal carcinoma was the most common.  Similar to our patient, none of the patients with synchronous tumor had symptoms relating to the carcinoid tumor.
Another series of study by Berge and Linell showed that out of 199 patients with carcinoid tumor, 40.7% had one or more coexisting synchronous or metachronous second malignancy. Most of them (35.8%) occurred in the gastrointestinal tract. 
In one more study by Saha et al. in a series of 112 patients with gastrointestinal carcinoids, a second cancer was noted in 25% of the cases. Of the second malignancy, 53% were adenocarcinoma of the gastrointestinal tract (large bowel: 39%, stomach: 7%, small bowel: 7%) and the remaining occurred in the lung (7%), uterine cervix (7%), prostate (7%) and other sites. 
Other reported unique combinations of synchronous tumors coexisting with gastrointestinal carcinoid include gastric leiomyoblastoma in association with duodenal carcinoid,  carcinoid of the appendix occurring with ileal T-cell lymphoma  and esophageal carcinoid occurring with esophageal adenocarcinoma in Barrett's esophagitis. 
The explanation for concurrent occurrence of carcinoid with gastric adenocarcinoma is unclear. Some studies show that long-term acid inhibitory therapy can lead to enterochromaffin-like cell hyperplasia, which can lead to carcinoid.
Low acid-related state is also a predisposing factor to gastric adenocarcinoma. It is postulated (but not proven) that carcinoid formed due to low acid state can release various mitogenic growth factors and cause alterations of adjacent mucosal cells leading to the occurrence of adenocarcinoma. 
Most carcinoids have an asymptomatic, indolent course with late metastases. Only with metastases does a carcinoid become symptomatic due to increased 5-hydroxytryptamine (5-HT) production giving rise to symptoms like flushing, palpitation, wheezing, anxiety and diarrhoea. 5-HT levels can be estimated in urine. On the other hand, gastric adenocarcinoma is more aggressive and symptomatic. Hence, the second primary, carcinoid is missed initially.
In conclusion, the possibility of synchronous/concurrent tumors in the gastrointestinal tract should be kept in mind. A detailed histological evaluation of the resected specimen should be done for any patient presenting with gastrointestinal neoplasm as most of these tumors are small and submucosal in location.
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