Cases reported "Endocrine Gland Neoplasms"

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1/5. Mixed ductal-endocrine carcinoma of the pancreas presenting as gastrinoma with zollinger-ellison syndrome: an autopsy case with a 24-year survival period.

    We report an autopsy case of mixed ductal-endocrine carcinoma of the pancreas presenting as gastrinoma with zollinger-ellison syndrome. A 38-year-old Japanese male was found to have zollinger-ellison syndrome and pancreatic gastrinoma, and gastrectomy and resection of the pancreatic tumor were performed. However, hypergastrinemia persisted, and the patient died of disseminated carcinomatosis at 62 years of age, 24 years after the onset of zollinger-ellison syndrome. At autopsy, the main tumor was present in the residual pancreas, and metastases were noted in many organs. In the pancreas and other organs, ductal and endocrine carcinoma areas were mixed and there was a gradual transition between the two. No acinar differentiation was noted. The ductal elements were positive for mucins and carcinoembryonic antigen but negative for neuroendocrine markers, while endocrine elements were positive for chromogranin a and synaptophysin and to a lesser extent for gastrin, but negative for mucins and carcinoembryonic antigen. The ductal elements comprised about 30% of the tumor cells, and endocrine elements 70%. According to the revised world health organization classification, our case was diagnosed as mixed ductal-endocrine carcinoma. Our case is rare because the tumor manifested as gastrinoma with zollinger-ellison syndrome and the patient survived for 24 years. To the best of our knowledge, no such case has been reported. Our case suggests that pancreatic endocrine tumors may evolve into mixed ductal-endocrine carcinomas.
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ranking = 1
keywords = duct
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2/5. Mammary endocrine ductal carcinoma in situ: a case report.

    Endocrine differentiation represents a pathway of neoplastic development available to a range of breast cancers. This pattern occurs in tumors with different morphological appearances as ductal carcinoma in situ (DCIS), mucinous carcinoma, a variant of lobular carcinoma, and low-grade invasive ductal carcinoma. Endocrine ductal carcinoma in situ is an uncommon entity. It occurs in older women with a mean age of 70 years. Histologically it shows expansile intraductal growth forming solid sheets and festoons transversed by delicate fibrovascular septa. Conventional microscopy permits the diagnosis in most cases. Specialized techniques such as immunohistochemistry and electron microscopy can serve as the basis of diagnosis in the absence of the appropriate morphological features. We present a 68-year-old female with a 1.5-cm firm mobile nodule of the left breast. mammography and ultrasounds showed a 15 x 15-mm circumscribed solid lobulated nodule. The mass was excised and pathology was positive for endocrine DCIS. Although endocrine DCIS has a biologic marker profile similar to that of well-differentiated or noncomedo DCIS it may constitute a different histogenetic pathway of carcinogenesis in the breast. The tumor may exhibit the invasive characteristics of a neuroendocrine neoplasm. Larger studies and longer follow-up are needed for the determination of the clinical behavior.
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ranking = 0.81744631889044
keywords = duct, bile
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3/5. Familial adenomatous polyposis: case report and review of extracolonic manifestations.

    FAP, if left untreated, results in colorectal cancer. Appropriate early surgical intervention is of utmost importance. With the reduction in mortality from colorectal carcinoma, an increasing number of patients with FAP need lifelong follow-up to screen for extracolonic manifestations. Today, the major causes of death in patients with FAP who are cured of colorectal cancer, or have had a colectomy before its development, are desmoid tumors and periampullary carcinomas. In family members at risk, screening with flexible sigmoidoscopy should be initiated in adolescence; symptoms of diarrhea and rectal bleeding would warrant an even earlier examination. The upper gastrointestinal tract should be assessed endoscopically at the time the diagnosis of FAP is made. If any polyps are detected, a biopsy is essential. If no gastric or duodenal polyps are found, repeated examinations at 3-5 year intervals probably suffice in asymptomatic patients. Surgically, colectomy will be necessary. It is usually deferred until late adolescence, when it is thought that the patient will be mature enough to handle the emotional aspects of the operation as well as the possible future morbidity due to the procedure. The presence or absence of rectal polyps as well as the site and depth of any invasive rectal carcinoma will determine the appropriate surgical procedure. Alternatives must be well understood by the physician and discussed carefully with the patient preoperatively.
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ranking = 0.1
keywords = duct
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4/5. Somatostatin-producing neuroendocrine tumor of the ampulla (ampullary somatostatinoma). Evidence of prosomatostatin production.

    Two cases of somatostatin-producing ampullary neuroendocrine tumors (somatostatinoma) are reported. The authors have characterized their immunoreactivity using antibodies specific for the amino- and carboxyl-terminal portions of prosomatostatin, the precursor of somatostatin in the normal synthetic pathway. Cytoplasmic staining was found using each of these two antibodies in the tumor cells of both ampullary somatostatinomas as well as in the cytoplasm of cells in the hypothalamus, crypt cells of the duodenal mucosa, mucosal cells of the biliary tract, D cells of the pancreatic islets, and parafollicular cells of fetal thyroid. These studies suggest that the synthesis of somatostatin in ampullary somatostatinomas occurs through the normal pathway from the precursor prosomatostatin.
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keywords = duct
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5/5. Pancreatic islet cell tumor producing vasoactive intestinal polypeptide and calcitonin.

    A patient with a pancreatic islet cell tumor concurrently producing vasoactive intestinal polypeptide (VIP) and calcitonin with related symptoms is presented. The patient was given palliative treatment consisting of octreotide, streptozotocin and 5-fluorouracil, and survival now exceeds 72 months. The concurrent production of VIP and calcitonin by a pancreatic islet cell tumor has not been described before.
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ranking = 0.1
keywords = duct
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