| 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. ( info) |
2/24. Coexistence of an endocrine tumour in a serous cystadenoma (microcystic adenoma) of the pancreas, an unusual association. A pancreatic endocrine tumour arising within a serous cystadenoma is reported. A 49 year old woman was admitted with a history of epigastric pain, nausea, vomiting, and weight loss of two months duration. She had been diabetic for 12 years. An epigastric mass was palpated in the physical examination, and computed tomography revealed a multiloculated cystic lesion in the pancreas. Pathological examination of the pancreatic tumour revealed the coexistence of a serous cystadenoma and an endocrine tumour. The endocrine tumour, which was located inside the serous cystadenoma, was 1 cm in diameter. The first case of a serous cystadenoma of the pancreas containing a pancreatic endocrine tumour was reported in the literature recently. This paper reports another incidentally found pancreatic endocrine tumour arising within a serous cystadenoma. ( info) |
3/24. 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. ( info) |
4/24. Malignant endocrine tumor of the pancreas associated with von Recklinghausen's disease. We describe a case of malignant endocrine tumor of the pancreas associated with von Recklinghausen's disease. A 66-year-old woman with familial von Recklinghausen's disease was admitted to our hospital with weight loss and lower abdominal pain. endoscopy revealed a submucosal tumor in the second part of the duodenum. Contrast-enhanced computed tomography showed a heterogeneous low-density tumor, measuring 20 x 15 mm, in the head of the pancreas, and metastatic lymphadenopathy. angiography showed faint tumor staining without encasement or apparent increase in vascularity. Pancreatoduodenectomy was performed. Macroscopically, there was a solid tumor, measuring 20 x 18 mm, in the head of the pancreas. Histologically, a malignant endocrine tumor was shown, with direct invasion to the duodenum, and lymph node metastases. This is the thirteenth case of pancreatic malignant tumor and the fourth case of pancreatic endocrine tumor associated with von Recklinghausen's disease reported in the world literature. ( info) |
5/24. Cowden disease or multiple hamartoma syndrome--cutaneous clue to internal malignancy. Cowden disease (CD) #158350, also known as multiple hamartoma syndrome, is a multisystemic cancer predisposition disorder, inherited in an autosomal dominant pattern. Mucocutaneous lesions are the most constant features: facial papules, acral keratoses and oral papillomatosis. The most common associated cancers are breast, thyroid and endometrial carcinomas. The CD gene locus has been mapped to chromosome 10q22-23. Subsequently the tumor suppressor gene PTEN was located to this chromosomal region and soon after germline mutations in the PTEN gene were demonstrated in CD patients. Somatic PTEN mutations have been found in a variety of sporadic cancers. So CD is an important clinical and genetic model for carcinogenesis. We recently observed four cases of CD and reviewed the literature on CD over the last 40 years, in particular the clinical and histopathological features, genetics, and diagnostic criteria. Based on these data we propose a possible management of CD patients. With increased knowledge and awareness of the typical mucocutaneous lesions an earlier diagnosis and an appropriate cancer surveillance of these patients might be possible. ( info) |
6/24. 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. ( info) |
7/24. Pitfalls in scintigraphic detection of neuroendocrine tumours. We report 4 cases of abnormal results using iodine-123 metaiodobenzylguanidine (123I-mIBG) or technetium-99m (V) dimercaptosuccinic acid (99mTc(V)-DMSA) scintigraphy in the diagnosis and follow-up of presumed neuroendocrine tumours. The present series consisted of 2 false-positive cases (1 adenomatous polyp of the caecum with mIBG and 1 follicular adenoma of the thyroid with DMSA) and 2 cases of anomalous uptake of (V)-DMSA in a non-neuroendocrine tissue. ( info) |
8/24. Neuroendocrine carcinoma of the stomach with extensive somatostatin immunoreactivity. upper gastrointestinal tract neuroendocrine tumors producing predominantly somatostatin have thus far been described only in the duodenum; their characteristic features include the frequent presence of psammoma bodies (psammomatous somatostinomas), and the association with von Recklinghausen's neurofibromatosis. Gastric neuroendocrine tumors, on the other hand, tend to display immunoreactivity to serotonin but may include small subpopulations producing gastrin, motilin, pancreatic polypeptide, and somatostatin. In this report we describe a neuroendocrine carcinoma of the stomach with rapidly fatal outcome, displaying neurosecretory granules by electron microscopy and immunoreactivity to pan-neuroendocrine markers, ie, chromogranin and neuron-specific enolase. The only neuroendocrine regulatory peptide detected in the tumor was somatostatin, identified by immunohistochemistry in the majority of neoplastic cells. In contrast with duodenal somatostinomas, there were no psammoma bodies and no demonstrable association with von Recklinghausen's neurofibromatosis. To our knowledge this appears to be the first report of a malignant neuroendocrine tumor with diffuse somatostatin immunoreactivity. ( info) |
9/24. 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. ( info) |
10/24. hyperandrogenism in a postmenopausal woman presenting with a metastatic ileum endocrine tumor. OBJECTIVE: To elucidate the mechanism of the hyperandrogenism found in a postmenopausal woman presenting an ileum endocrine tumor with ovarian metastases. DESIGN: Case report. SETTING: University hospital. PATIENT(S): A postmenopausal woman was referred for hirsutism. Basal plasma testosterone was high, 6.6 nM/L (normal, magnetic resonance imaging revealed a 6-cm left ovarian mass. INTERVENTION(S): Bilateral salpingo-oophorectomy was performed. Pathological examination found a bilateral metastatic endocrine ovarian tumor, associated with a functional stroma. A primary ileum endocrine tumor was discovered and resected. MAIN OUTCOME MEASURE(S): Immunohistochemical study of the expression of steroidogenic enzymes and beta and alpha subunits of hCG. RESULT(S): Immunohistochemical expression of steroidogenic enzymes was found in the ovarian stromal tissue surrounding the tumor but not in the metastatic tumoral cells. A substantial percentage of the metastatic tumoral cells was immunopositive for the beta and alpha subunit of hCG but not the ileal cells. CONCLUSION(S): These data suggest an hCG paracrine effect of the ovarian metastases tumor on the adjacent interstitial cells, resulting in the virilization of the patient. ( info) |