Cases reported "Neuroendocrine Tumors"

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1/7. Middle ear adenomatous tumor with a predominant neuroendocrine component.

    A primary adenomatous tumor of the middle ear was examined by light microscopy, ultrastructural and immunohistochemical techniques. In support of its extensive neuroendocrine differentiation, was the diffuse detection of neuron-specific enolase (NSE) and positive immunoreaction with antibodies to chromogranin and synaptophysin. The great majority of tumor cells contained neurosecretory granules and intraluminal mucin production could be focally detected. These characteristics confirm the diagnosis of a middle ear adenomatous tumor (meat) of a biphasic nature and with a prominent neuroendocrine component.
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2/7. Middle ear adenoma with neuroendocrine differentiation.

    The lining of the middle ear cleft is normally a thin modified respiratory type mucosa. Normal mucosa of the middle ear is devoid of salivary type seromucous glands. Middle ear adenoma is a rare tumor that seems to be derived from the middle ear mucosa. This tumor has been previously described under a variety of names because of its different nature and biological behavior. We herein report a case of middle ear tumor that shows adenomatous and neuroendocrine features. A 64-year-old woman presented with a history of hearing loss, tinnitus and stuffy feeling of the right ear. The patient was treated two times for ear polyp. She finally underwent a tympanomastoidectomy and there was no evidence of recurrence 18 months after the procedure. Histopathological examination displayed cuboidal and columnar cells, arranged as glands, trabeculae and solid sheets. Neuroendocrine differentiation was revealed by immunohistochemical staining with polyclonal antibodies against neuron specific enolase and chromogranin antigens.
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3/7. role of liver transplantation in the management of metastatic neuroendocrine tumors.

    INTRODUCTION: In the majority of patients transplanted for unresectable liver metastases, long-term results are disappointing because of early tumor recurrence. Due to its biologically less aggressive nature, neuroendocrine metastases (NM) may represent a good indication for liver transplantation (LT). patients AND methods: Between January 1996 and May 2000, five patients with NM were transplanted. The primary tumors were located in the pancreas (n=4) and the small bowel (n=1). In three cases there were symptoms related to hormone production: two carcinoids, and one gastrinoma. The management of primary tumors was sequential in three patients with the tumor being resected before LT (one Whipple procedure and two left pancreatectomies). In two patients the resections of the primary tumors and the LT were simultaneous namely one bowel resection and one left pancreatectomy. All patients were treated with chemotherapy. RESULTS: Two patients developed recurrent disease succumbing at 15 months (nonfunctioning NE pancreatic head tumor) and 17 months (carcinoid of the pancreatic tail) post-LT. Another patient died at 3 months post-LT due to technical complications. The other two patients are alive and free of recurrence. CONCLUSION: Despite the promising results obtained with LT for NM, our experience indicates that patients must be carefully selected. Perhaps the use of more aggressive chemotherapeutic protocols combined with an individualized approach will improve the results.
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4/7. necrolytic migratory erythema associated with hyperglucagonemia and neuroendocrine hepatic tumors.

    We present a 61-year-old man with a 2-year history of persistent disseminated, psoriasiform annular pruritic lesions, acrodermatitis, weight loss, anemia and diabetes. Histopathology of the affected skin showed nonspecific subacute psoriasiform dermatitis. The computed tomographic scan of the abdomen revealed multiple hepatic tumors. Histopathological examination of ultrasound-guided needle biopsy from a hepatic lesion demonstrated a neuroendocrine tumor. somatostatin-receptor scintigraphy with radio-labelled octreotide confirmed the likelihood of the neuroendocrine nature of the hepatic tumors and excluded the presence of other such lesions throughout the rest of the body, including the pancreas. The serum glucagon level was markedly increased. The diagnosis of necrolytic migratory erythema associated with hyperglucagonemia and neuroendocrine hepatic tumors was made and therapy with the long-acting somatostatin analogue octreotide was started. The skin changes resolved after the initiation of therapy, but no improvement of other symptoms was observed. Having reached the final stage of the disease, which was further complicated by congestive heart failure, the patient died one year later. As no autopsy was performed, we were unable to establish whether the hepatic tumors represented a metastatic process of previously undetected pancreatic glucagonoma or if they were extra-pancreatic glucagon-secreting tumors. The correct diagnosis of necrolytic migratory erythema is important, since it might be the clue for early detection of glucagonoma or of extra-pancreatic glucagon-secreting tumors.
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5/7. Nonfunctioning cystic neuroendocrine neoplasms of the pancreas.

    BACKGROUND. Three types (functioning solid, nonfunctioning solid, and functioning cystic) of neuroendocrine neoplasms (benign and malignant) of the pancreas have been reported. patients. We report three patients with nonfunctioning neuroendocrine neoplasms of the pancreas with cystic components, a type of neoplasm not previously reported. One of the neoplasms we report was benign; the other two were malignant. The patients were admitted with abdominal pain. In each case an abdominal computed tomography scan defined a large multicystic pancreatic mass. RESULTS. In all instances, immunochemical staining was positive for neuroendocrine markers, confirming the neuroendocrine nature of the tumors. The nonfunctional nature of the neoplasms was confirmed by (1) the absence of clinical symptoms traditionally attributed to neuroendocrine secretagogues and (2) the lack of gastroenteropancreatic peptide serum levels. CONCLUSIONS. Two of these three cases represent another subset of pancreatic neuroendocrine pathology: a nonfunctional cystic neoplasm. Increased clinical awareness, appropriate operation, and better immunohistochemical staining techniques may yield more, heretofore unrecognized, subtypes. These cases reemphasize the need for proper surgical exploration and biopsy of all unexplained pancreatic cystic lesions, particularly if they are multicystic.
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6/7. liver transplantation for metastatic neuroendocrine tumors.

    OBJECTIVE: This article describes the experience with liver transplantation in patients with irresectable neuroendocrine hepatic metastases. SUMMARY BACKGROUND DATA: liver transplantation has become an established therapy in primary liver cancer. On contrast, there is little experience with liver transplantation in secondary hepatic tumors. So far, in the majority of patients being transplanted for irresectable liver metastases, long-term results have been disappointing because of early tumor recurrence. Because of their biologically less aggressive nature, the metastases of neuroendocrine tumors could represent a justified indication for liver grafting. methods: In a retrospective study, the data of 12 patients who underwent liver transplantation for irresectable neuroendocrine hepatic metastases were analyzed regarding survival, tumor recurrence, and symptomatic relief. RESULTS: Nine of 12 patients currently are alive with a median survival of 55 months (range, 11.0 days to 103.5 months). The operative mortality was 1 of 12, 2 patients died because of septic complications or tumor recurrences or both 6.5 months and 68.0 months after transplantation. all patients had good symptomatic relief after hepatectomy and transplantation. Four of the nine patients who are alive have no evidence of tumor with a follow-up of 2.0, 57.0, 58.0, and 103.5 months after transplantation. CONCLUSIONS: In selected patients, liver transplantation for irresectable neuroendocrine hepatic metastases may provide not only long-term palliation but even cure. Regarding the shortage of donor organs, liver grafting for neuroendocrine metastases should be considered solely in patients without evidence of extrahepatic tumor manifestation and in whom all other treatment methods are no longer effective.
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7/7. Histocytologic diagnosis of neuroendocrine tumors in the liver: a retrospective study of 23 cases.

    A series of 23 needle biopsies of neuroendocrine tumors occurring in the liver is described. Aspirate smears, core biopsies, and touch preparations were examined. Eighteen of the 23 patients had been previously diagnosed: 9 patients had been correctly identified as having a neuroendocrine tumor, and 9 patients had been originally misdiagnosed. Five of the patients in this series had no previously identified neoplasia. Immunohistochemical staining confirmed the neuroendocrine nature of the tumors in each of the cases. On the basis of cytomorphology, these cases were subtyped as either round cell type, spindle cell type, or polygonal cell type. The polygonal cell type of neuroendocrine tumor, as well as rare examples of the round cell type, demonstrated features similar to well-differentiated hepatocellular carcinoma and adenocarcinomas, and may present a diagnostic dilemma. Characteristic cytologic attributes of the polygonal cell type of neuroendocrine tumor which aid in its distinction from well-differentiated hepatocellular carcinoma include eccentrically located "plasmacytoid" nuclei and cellular discohesion. Findings on core needle biopsy which further identify the neuroendocrine tumors are thick fibrous stroma or small "nests" of tumor cells. The additional use of immunohistochemical staining provides reliable evidence of the cell of origin in confusing cases. attention to these considerations will aid in the cytologic diagnosis of neuroendocrine tumors.
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