Cases reported "neuroendocrine tumors"

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1/142. Primary hepatic neuroendocrine tumor: successful hepatectomy in two cases and review of the literature.

    BACKGROUND/AIMS: Primary hepatic neuroendocrine tumor represents an extremely rare clinical entity with only very few cases having been reported to date. methods: The case histories of 2 patients with presumably primary hepatic neuroendocrine tumor were analyzed and a complete follow-up obtained. The literature was reviewed to provide comprehensive data collection. RESULTS: Both patients underwent partial hepatic resection. Histomorphologic diagnosis revealed a neuroendocrine tumor in both cases. Extensive preoperative as well as intra- and postoperative search for the primary tumor did not identify another site of neuroendocrine tumor tissue. Six and ten years after hepatic segmentectomy, the 2 patients are alive and show no clinical signs of malignancy. Their most recent thorough follow-up included computed tomography and somatostatin receptor scintigraphy. Neither a nonhepatic primary neuroendocrine tumor site nor recurrent disease was found in the 2 patients. The literature review resulted in a complete survey of all previously reported cases of primary hepatic neuroendocrine tumors. CONCLUSION: We conclude that the liver was the primary site of the neuroendocrine tumor in both patients. Radical surgery was successfully performed as the only treatment option with curative intention. ( info)

2/142. Gastroenteropancreatic neuroendocrine tumor metastases to the thyroid gland: differential diagnosis with medullary thyroid carcinoma.

    neuroendocrine tumors (NET) of the thyroid gland are rare. Apart from medullary thyroid carcinoma (MTC), metastases of gastroenteropancreatic (GEP) NET may also occur. Features of six patients (five men, one female: age range, 39-67 years) with thyroid metastases from a GEP-NET are described. Thyroid metastases were bilateral in all patients and were associated with enlarged neck lymph nodes in five. In four cases, the thyroid tumor was either the first sign of the disease (n = 2) or was an isolated site of recurrence (n = 2). The tumors were well (n = 3) or poorly differentiated (n = 3). Five tumors for which the primary site could be determined corresponded to foregut-derived tumors (3 lungs, 1 thymus and 1 pancreatic NET). One tumor demonstrated calcitonin (CT) production as shown by immunohistochemistry and elevated plasma CT levels. However, the disease history and the clinical features strongly favored a metastasizing GEP-NET. No tumoral RET proto-oncogene mutation was found in this patient. The differential diagnosis between metastatic GEP-NET and MTC is crucial because prognosis, work-up, and treatment differ greatly. ( info)

3/142. A case of synchronous double primary lung cancer with neuroendocrine features.

    We report a case of unique double primary lung cancers with neuroendocrine features in a 63-year-old male smoker. The mass in the left lower lobe (LLL) was a small cell/large cell carcinoma with spindle cell sarcomatous areas and organoid structure. The mass in the left upper lobe (LUL) was a tubular adenocarcinoma with neuroendocrine features including organoid nests showing occasional rosette formation, nuclear palisading in the periphery of the nests and positive immunoreaction for CD56, chromogranin a and synaptophysin. The difference in histological structures between the two masses led us to diagnose double primary lung cancer. The combination of small cell lung carcinoma and spindle cell carcinoma is very uncommon. The relationship between LLL and LUL tumors remains unclear. Multiple lung cancers with neuroendocrine features have only rarely been reported in the literature. The patient in our case died of widespread cancer 2 years and 4 months after the surgery without adjuvant chemotherapy, a longer postoperative survival time than in cases of ordinary extensive small cell lung cancer. Multiple lung cancers with neuroendocrine features are extremely rare and similar cases have not been reported in the literature. Neuroendocrine differentiation has attracted widespread attention and, therefore, examining neuroendocrine features in lung cancers is important. ( info)

4/142. Minimally invasive surgery for recurrent neuroendocrine carcinoma of the supraglottic larynx.

    calcitonin-secreting neuroendocrine carcinomas of the supraglottic larynx are infrequent tumors, making it difficult to agree on treatment plans for recurrent tumors. Furthermore, this rare malignancy is often confused with the more common medullary thyroid carcinoma, resulting in inappropriate thyroidectomies. We present a case report of a calcitonin-secreting recurrent neuroendocrine carcinoma of the supraglottic larynx, in which surgery and pentagastrin stimulation were performed repeatedly at various stages of the disease. The recurrent laryngeal tumor was ultimately identified and, after construction of a protective tracheostomy, resected transorally en bloc with the underlying arytenoid cartilage. Postoperatively, the patient did well and stimulated calcitonin levels never exceeded double baseline values. Laryngoscopic removal of smaller laryngeal carcinomas is both technically feasible and safe, even when tumors are recurrent. In calcitonin-secreting malignancies, pentagastrin stimulation may facilitate the distinction between laryngeal and medullary thyroid carcinoma and thus help avoid unnecessary thyroidectomies. ( info)

5/142. Cellular neurothekeoma with possible neuroendocrine differentiation.

    We report a case of cellular neurothekeoma showing unusual immunohistochemical findings and occurring on the left upper arm of a healthy 48-year-old woman. She presented with a 1 cm, red, asymptomatic, dermal nodule of 1 year duration. A biopsy showed dermal proliferation of plexiform fascicles of spindle-shaped or polygonal cells with plentiful eosinophilic cytoplasms. The immunohistochemical profile included negative stains for S-100, CD34, factor xiiia, CD68, HMB45, cytokeratins, and EMA, strongly positive stains for neuron specific enolase (NSE), synaptophysin, and chromogranin a, and focally positive ones for NKI/C3 and alpha-smooth muscle actin. Ultrastructural analysis showed undifferentiated mesenchymal cells with cytoplasmic projections and abundant RER. Although we couldn't find any confirmative cell type in this cellular tumor, we believe that cellular neurothekeoma is predominantly composed of undifferentiated cells that can exhibit features of neuroendocrine cells in addition to fibroblastic or myofibroblastic ones, suggesting a divergent cell origin. ( info)

6/142. A choroidal amyloid-rich neuroendocrine tumor: initial manifestation of cushing syndrome.

    A 57-year-old man with a history of hepatic adenocarcinoma was referred 3 years after his diagnosis with a choroidal tumor in the right eye. Results of a transscleral excision biopsy revealed the tumor to be an amyloid-rich neuroendocrine metastasis. The patient subsequently developed cushingoid features and investigations revealed ectopic corticotropin syndrome, an elevated urinary 5-hydroxyindoleacetic acid level, and neuroendocrine metastasis in several locations. The choroidal neuroendocrine metastasis stained negative for serotonin and corticotropin. The source of the ectopic corticotropin and the location of the primary tumor have not been found. This case demonstrates that disseminated neuroendocrine tumors may rarely cause ocular lesions before systemic endocrine sequelae arise. ( info)

7/142. Total hepatectomy and liver transplantation for metastatic neuroendocrine tumors of the pancreas - a single center experience with ten patients.

    BACKGROUND: Metastatic neuroendocrine pancreatic tumors have a poor prognosis. We have studied retrospectively the efficacy of liver transplantation as ultimate therapy of otherwise untreatable symptomatic neuroendocrine hepatic metastases originating in the pancreas. methods: We reviewed our experience of liver transplantation (LTx) for hepatic metastases of neuroendocrine pancreatic tumors in ten patients. The indication for liver grafting was seen in cases of irresectable metastases and when patients were suffering from otherwise untreatable tumor-associated symptoms due to massive hormonal release or large intra-abdominal tumor bulk. RESULTS: In four patients, the primary tumors had been removed before LTx, in five patients simultaneously with LTx and in one case 46 months after grafting. There was no operative mortality. After hepatectomy and LTx, all patients had complete relief of symptoms and all preoperatively increased hormonal levels returned to normal. In nine of ten patients, the transplant procedure had the potential for cure, whereas, in one patient, the primary tumor had remained in situ at LTx and was removed 46 months later by an R2-resection. At present, nine patients are alive with a median follow-up of 33 months (range 13.5 months to 117 months). The one patient in whom the primary tumor was removed after transplantation died due to massive intra-abdominal tumor spread 68 months after LTx. Currently, two patients are without evidence of disease, but one of them after re-operation because of lymph-node metastases 8 months after transplantation. The longest disease-free survival is now more than 7 years. In seven of nine patients, tumor recurred between 1.5 months and 48 months after transplantation. CONCLUSIONS: patients with otherwise untreatable symptomatic neuroendocrine hepatic metastases of pancreatic origin may benefit from total hepatectomy and liver transplantation with regard to symptomatic relief and long-term survival, despite frequent recurrence of disease. In some patients, liver transplantation may even offer the chance for cure. ( info)

8/142. Primary neuroendocrine tumors of the cystic duct.

    Primary neuroendocrine tumors of the cystic duct are extremely rare. Only 4 cases have been described to date. We report 2 patients in whom a primary neuroendocrine tumor of the cystic duct was incidentally detected during histological examination following cholecystectomy. With regard to the primary neuroendocrine tumor both patients were asymptomatic at the time of diagnosis. However, histologic examination did not confirm that the neuroendocrine tumors had been completely removed. Both patients underwent a second procedure. They are well after 47 and 49 months, respectively. ( info)

9/142. Partial nephrectomy for incidental primary renal neuroendocrine carcinoma: case report.

    Here we present an 83-year-old woman who was referred to our hospital and had had left flank pain and oligouria for 3 days. Plain abdominal film and ultrasonography revealed left ureteropelvic junction stone with obstructive uropathy. The serum level of creatinine fell to 3.1 mg/dl from 7.6 mg/dl after ureteral catheter drainage was given 5 days after admission. Then a left pyelolithotomy was performed and a tumor of 2 x 1 x 1 cm over the lower pole of the left kidney was found incidentally. Partial nephrectomy was performed in consideration of her age and poor renal function although the biopsy result showed it to be carcinoma. The final pathological report and immunohistochemical study results proved that it was neuroendocrine carcinoma. To our knowledge, this is the first case of primary renal neuroendocrine carcinoma to be treated using conservative surgery. The clinical course was acceptable, since she had been found to be free of disease during regular follow-up of 2.5 years with the creatinine level of about 2.5 mg/dl. ( info)

10/142. 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. ( info)
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