engleski

Multiorgan Resection Due to Rectal Cancer and Multiple Adenoma Coli Associated with Multiple Endocrine Neoplasia Type 1 - Related Tumors: a Case Report

Multiple primary cancers occurring in the same patients are uncommon and have been reported to represent 1.8-3.9% of all cancers. We describe one extremely rare case with rectal cancer and multiple adenoma coli syndrome and an serous oligocystic (microcystic) cystadenoma of the pancreas and pheocromocytoma. It is generally accepted that patients with adenomatous polyposis syndrome, such as familial adenomatous polyposis (FAP), or those with hereditary nonpolyposis colorectal cancer (HNPCC) syndrome type II (Lynch II) often have extracolonic malignant tumors or precancerous lesions in various organs. Such cancers include those of thyroid, stomach, duodenum, duodenal papilla, gallbladder, jejunum, ileum, breast, thymus and skin (melanoma). With improved long-term survival rates, secondary or tertiary cancers are increasingly found in these patients. Variant of familial adenomatous polyposis coli is so-called multiple adenoma patients, clinically characterized by many, but usually fewer than 100, colonic lesions, but most have no demonstrable germ-line adenomatous polyposis coli mutation, as do patients with FAP. Serous oligocystic, ill-demarcated adenoma of the pancreas is very rare tumor classified among the serous cystic neoplasms of the pancreas. To our knowledge there are only 19 cases of this tumour in the English-language medical literature. We describe the first case of patient with serous oligocystic pancreatic adenoma in whom additionally existed rectal cancer, multiple colonic adenomas and pheochromocytoma. In our patient laparotomy was performed due to endoscopically proven rectal cancer. During the abdominal exploration, additional tumors in the left adrenal gland and in the tail of the pancreas were found. Therefore, abdominoperineal rectal resection, left adrenalectomy and resection of the tail of pancreas were performed. Pathohistological examination revealed polypous adenocarcinoma in the rectum with multiple adenomatous polyps in the rest resected bowel related to some type of adenomatous polyposis syndromes (FAP or so-called multiple adenoma coli), pheocromocytoma in the adrenal gland and microcystic cystadenoma in the pancreas.

multiple adenoma coli; hereditary nonpolyposis colorectal cancer

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