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Y Yakuwa - One of the best experts on this subject based on the ideXlab platform.
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Somatostatinoma inhibitory syndrome a statistical evaluation of 173 reported cases as compared to other pancreatic endocrinomas
Journal of Experimental & Clinical Cancer Research, 1999Co-Authors: Jun Soga, Y YakuwaAbstract:Somatostatin is known to inhibit the secretory release of other peptide hormones. Somatostatinomas associated either with or without Somatostatinoma (inhibitory) syndrome are rare neoplasms among gut-pancreatic endocrinomas. Collected from international literature, this study aimed to perform a statistical analysis on 173 patients with Somatostatinoma/inhibitory syndrome. The evaluation further attempted to provide investigators in this particular field of research with extensive and precise information on the present situation of Somatostatinoma. The 173 patients consisted of 81 with pancreatic Somatostatinomas and 92 with extrapancreatic Somatostatinomas. Most of the latter were found to have originated in the duodenum and may be termed as carcinoid Somatostatinoma. Where data were considered to be adequate, a comparative study was carried out between two groups, pancreatic and duodenal, each consisting of 81 patients. A statistically significant difference between these two groups was found in the incidence of inhibitory syndrome (18.5% versus 2.5%) and von Recklinghausen's disease (1.2% versus 43.2%), large size of tumor (>20 mm) (85.5% versus 41.4%), multisecretory activities (33.3% versus 16.3%), and presence of psammoma bodies (2.5% versus 49.4%). There was no statistically significant difference in the rate of metastases and malignancy between the two groups. The average postoperative 5-year survival rate was 75.2% in 90 patients overall, 59.9% in 44 with metastases and 100.0% in 46 without metastases. Compared with the other pancreatic endocrinomas, including PPomas, glucagonomas, vipomas, gastrinomas, and insulinomas, Somatostatinomas were characterized by the low rate of the relevant syndrome and multiple endocrine neoplasia syndrome type 1. There was a low rate of multiplicity, and a high incidence of psammoma bodies in the duodenal group particularly with von Recklinghausen's disease. A high rate of malignancy was recorded, resulting in a low postoperative survival rate of patients with metastases. In conclusion, Somatostatinomas exhibited characteristic features quite different from those of the other pancreatic endocrinomas regarding multiple points.
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Somatostatinoma/inhibitory syndrome: a statistical evaluation of 173 reported cases as compared to other pancreatic endocrinomas.
Journal of experimental & clinical cancer research : CR, 1999Co-Authors: Jun Soga, Y YakuwaAbstract:Somatostatin is known to inhibit the secretory release of other peptide hormones. Somatostatinomas associated either with or without Somatostatinoma (inhibitory) syndrome are rare neoplasms among gut-pancreatic endocrinomas. Collected from international literature, this study aimed to perform a statistical analysis on 173 patients with Somatostatinoma/inhibitory syndrome. The evaluation further attempted to provide investigators in this particular field of research with extensive and precise information on the present situation of Somatostatinoma. The 173 patients consisted of 81 with pancreatic Somatostatinomas and 92 with extrapancreatic Somatostatinomas. Most of the latter were found to have originated in the duodenum and may be termed as carcinoid Somatostatinoma. Where data were considered to be adequate, a comparative study was carried out between two groups, pancreatic and duodenal, each consisting of 81 patients. A statistically significant difference between these two groups was found in the incidence of inhibitory syndrome (18.5% versus 2.5%) and von Recklinghausen's disease (1.2% versus 43.2%), large size of tumor (>20 mm) (85.5% versus 41.4%), multisecretory activities (33.3% versus 16.3%), and presence of psammoma bodies (2.5% versus 49.4%). There was no statistically significant difference in the rate of metastases and malignancy between the two groups. The average postoperative 5-year survival rate was 75.2% in 90 patients overall, 59.9% in 44 with metastases and 100.0% in 46 without metastases. Compared with the other pancreatic endocrinomas, including PPomas, glucagonomas, vipomas, gastrinomas, and insulinomas, Somatostatinomas were characterized by the low rate of the relevant syndrome and multiple endocrine neoplasia syndrome type 1. There was a low rate of multiplicity, and a high incidence of psammoma bodies in the duodenal group particularly with von Recklinghausen's disease. A high rate of malignancy was recorded, resulting in a low postoperative survival rate of patients with metastases. In conclusion, Somatostatinomas exhibited characteristic features quite different from those of the other pancreatic endocrinomas regarding multiple points.
Jun Soga - One of the best experts on this subject based on the ideXlab platform.
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Somatostatinoma inhibitory syndrome a statistical evaluation of 173 reported cases as compared to other pancreatic endocrinomas
Journal of Experimental & Clinical Cancer Research, 1999Co-Authors: Jun Soga, Y YakuwaAbstract:Somatostatin is known to inhibit the secretory release of other peptide hormones. Somatostatinomas associated either with or without Somatostatinoma (inhibitory) syndrome are rare neoplasms among gut-pancreatic endocrinomas. Collected from international literature, this study aimed to perform a statistical analysis on 173 patients with Somatostatinoma/inhibitory syndrome. The evaluation further attempted to provide investigators in this particular field of research with extensive and precise information on the present situation of Somatostatinoma. The 173 patients consisted of 81 with pancreatic Somatostatinomas and 92 with extrapancreatic Somatostatinomas. Most of the latter were found to have originated in the duodenum and may be termed as carcinoid Somatostatinoma. Where data were considered to be adequate, a comparative study was carried out between two groups, pancreatic and duodenal, each consisting of 81 patients. A statistically significant difference between these two groups was found in the incidence of inhibitory syndrome (18.5% versus 2.5%) and von Recklinghausen's disease (1.2% versus 43.2%), large size of tumor (>20 mm) (85.5% versus 41.4%), multisecretory activities (33.3% versus 16.3%), and presence of psammoma bodies (2.5% versus 49.4%). There was no statistically significant difference in the rate of metastases and malignancy between the two groups. The average postoperative 5-year survival rate was 75.2% in 90 patients overall, 59.9% in 44 with metastases and 100.0% in 46 without metastases. Compared with the other pancreatic endocrinomas, including PPomas, glucagonomas, vipomas, gastrinomas, and insulinomas, Somatostatinomas were characterized by the low rate of the relevant syndrome and multiple endocrine neoplasia syndrome type 1. There was a low rate of multiplicity, and a high incidence of psammoma bodies in the duodenal group particularly with von Recklinghausen's disease. A high rate of malignancy was recorded, resulting in a low postoperative survival rate of patients with metastases. In conclusion, Somatostatinomas exhibited characteristic features quite different from those of the other pancreatic endocrinomas regarding multiple points.
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Somatostatinoma/inhibitory syndrome: a statistical evaluation of 173 reported cases as compared to other pancreatic endocrinomas.
Journal of experimental & clinical cancer research : CR, 1999Co-Authors: Jun Soga, Y YakuwaAbstract:Somatostatin is known to inhibit the secretory release of other peptide hormones. Somatostatinomas associated either with or without Somatostatinoma (inhibitory) syndrome are rare neoplasms among gut-pancreatic endocrinomas. Collected from international literature, this study aimed to perform a statistical analysis on 173 patients with Somatostatinoma/inhibitory syndrome. The evaluation further attempted to provide investigators in this particular field of research with extensive and precise information on the present situation of Somatostatinoma. The 173 patients consisted of 81 with pancreatic Somatostatinomas and 92 with extrapancreatic Somatostatinomas. Most of the latter were found to have originated in the duodenum and may be termed as carcinoid Somatostatinoma. Where data were considered to be adequate, a comparative study was carried out between two groups, pancreatic and duodenal, each consisting of 81 patients. A statistically significant difference between these two groups was found in the incidence of inhibitory syndrome (18.5% versus 2.5%) and von Recklinghausen's disease (1.2% versus 43.2%), large size of tumor (>20 mm) (85.5% versus 41.4%), multisecretory activities (33.3% versus 16.3%), and presence of psammoma bodies (2.5% versus 49.4%). There was no statistically significant difference in the rate of metastases and malignancy between the two groups. The average postoperative 5-year survival rate was 75.2% in 90 patients overall, 59.9% in 44 with metastases and 100.0% in 46 without metastases. Compared with the other pancreatic endocrinomas, including PPomas, glucagonomas, vipomas, gastrinomas, and insulinomas, Somatostatinomas were characterized by the low rate of the relevant syndrome and multiple endocrine neoplasia syndrome type 1. There was a low rate of multiplicity, and a high incidence of psammoma bodies in the duodenal group particularly with von Recklinghausen's disease. A high rate of malignancy was recorded, resulting in a low postoperative survival rate of patients with metastases. In conclusion, Somatostatinomas exhibited characteristic features quite different from those of the other pancreatic endocrinomas regarding multiple points.
Detlef K. Bartsch - One of the best experts on this subject based on the ideXlab platform.
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Duodenal Somatostatinoma associated with Von Recklinghausen’s disease
Journal of Hepato-Biliary-Pancreatic Surgery, 2004Co-Authors: Volker Fendrich, Annette Ramaswamy, Emily P. Slater, Detlef K. BartschAbstract:Somatostatinomas are rare, malignant, somatostatin-producing neuroendocrine tumors with a prevalence of one in 40 million. The coincidence of Von Recklinghausen’s disease and duodenal Somatostatinoma has been known since 1982. We report the case of a 57-year-old female patient with Von Recklinghausen’s disease and a tumor of the pancreatic head that was diagnosed due to painless icterus. Histopathological examination after pylorus-preserving pancreatoduodenectomy revealed the existence of a duodenal Somatostatinoma with lymph node metastases. Characteristics of the association of von Recklinghausen’s disease and Somatostatinoma, and therapy and prognosis will be discussed. In patients with Von Recklinghausen’s disease and an ampullary tumor, a Somatostatinoma should be considered. In contrast to its pancreatic counterparts, duodenal Somatostatinoma is frequently associated with Von Recklinghausen’s disease, often contains psammoma bodies, is rarely associated with a recognizable “somatostatin syndrome”, and is hardly ever associated with demonstrable metastases at the time of diagnosis. Small tumors arising in the duodenum may be treated with local excision, whereas larger tumors should be treated by total excision, which may entail a partial pancreatoduodenectomy.
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Duodenal Somatostatinoma associated with Von Recklinghausen's disease.
Journal of hepato-biliary-pancreatic surgery, 2004Co-Authors: Volker Fendrich, Annette Ramaswamy, Emily P. Slater, Detlef K. BartschAbstract:Somatostatinomas are rare, malignant, somatostatin-producing neuroendocrine tumors with a prevalence of one in 40 million. The coincidence of Von Recklinghausen’s disease and duodenal Somatostatinoma has been known since 1982. We report the case of a 57-year-old female patient with Von Recklinghausen’s disease and a tumor of the pancreatic head that was diagnosed due to painless icterus. Histopathological examination after pylorus-preserving pancreatoduodenectomy revealed the existence of a duodenal Somatostatinoma with lymph node metastases. Characteristics of the association of von Recklinghausen’s disease and Somatostatinoma, and therapy and prognosis will be discussed. In patients with Von Recklinghausen’s disease and an ampullary tumor, a Somatostatinoma should be considered. In contrast to its pancreatic counterparts, duodenal Somatostatinoma is frequently associated with Von Recklinghausen’s disease, often contains psammoma bodies, is rarely associated with a recognizable “somatostatin syndrome”, and is hardly ever associated with demonstrable metastases at the time of diagnosis. Small tumors arising in the duodenum may be treated with local excision, whereas larger tumors should be treated by total excision, which may entail a partial pancreatoduodenectomy.
Volker Fendrich - One of the best experts on this subject based on the ideXlab platform.
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Duodenal Somatostatinoma associated with Von Recklinghausen’s disease
Journal of Hepato-Biliary-Pancreatic Surgery, 2004Co-Authors: Volker Fendrich, Annette Ramaswamy, Emily P. Slater, Detlef K. BartschAbstract:Somatostatinomas are rare, malignant, somatostatin-producing neuroendocrine tumors with a prevalence of one in 40 million. The coincidence of Von Recklinghausen’s disease and duodenal Somatostatinoma has been known since 1982. We report the case of a 57-year-old female patient with Von Recklinghausen’s disease and a tumor of the pancreatic head that was diagnosed due to painless icterus. Histopathological examination after pylorus-preserving pancreatoduodenectomy revealed the existence of a duodenal Somatostatinoma with lymph node metastases. Characteristics of the association of von Recklinghausen’s disease and Somatostatinoma, and therapy and prognosis will be discussed. In patients with Von Recklinghausen’s disease and an ampullary tumor, a Somatostatinoma should be considered. In contrast to its pancreatic counterparts, duodenal Somatostatinoma is frequently associated with Von Recklinghausen’s disease, often contains psammoma bodies, is rarely associated with a recognizable “somatostatin syndrome”, and is hardly ever associated with demonstrable metastases at the time of diagnosis. Small tumors arising in the duodenum may be treated with local excision, whereas larger tumors should be treated by total excision, which may entail a partial pancreatoduodenectomy.
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Duodenal Somatostatinoma associated with Von Recklinghausen's disease.
Journal of hepato-biliary-pancreatic surgery, 2004Co-Authors: Volker Fendrich, Annette Ramaswamy, Emily P. Slater, Detlef K. BartschAbstract:Somatostatinomas are rare, malignant, somatostatin-producing neuroendocrine tumors with a prevalence of one in 40 million. The coincidence of Von Recklinghausen’s disease and duodenal Somatostatinoma has been known since 1982. We report the case of a 57-year-old female patient with Von Recklinghausen’s disease and a tumor of the pancreatic head that was diagnosed due to painless icterus. Histopathological examination after pylorus-preserving pancreatoduodenectomy revealed the existence of a duodenal Somatostatinoma with lymph node metastases. Characteristics of the association of von Recklinghausen’s disease and Somatostatinoma, and therapy and prognosis will be discussed. In patients with Von Recklinghausen’s disease and an ampullary tumor, a Somatostatinoma should be considered. In contrast to its pancreatic counterparts, duodenal Somatostatinoma is frequently associated with Von Recklinghausen’s disease, often contains psammoma bodies, is rarely associated with a recognizable “somatostatin syndrome”, and is hardly ever associated with demonstrable metastases at the time of diagnosis. Small tumors arising in the duodenum may be treated with local excision, whereas larger tumors should be treated by total excision, which may entail a partial pancreatoduodenectomy.
Francesco Tonelli - One of the best experts on this subject based on the ideXlab platform.
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Somatostatinoma clinico pathological features of three cases and literature reviewed
Journal of Gastroenterology and Hepatology, 2008Co-Authors: Gabriella Nesi, Tommaso Marcucci, Carlos A. Rubio, Maria Luisa Brandi, Francesco TonelliAbstract:Somatostatinoma is a rare endocrine tumor that comprises around 1% of all gastroenteropancreatic endocrine neoplasms. This paper gives an updated review on Somatostatinoma and describes three sporadic cases of Somatostatinoma located in the pancreas, duodenum, and jejunum. Approximately 200 case histories of Somatostatinoma have been published, with the duodenum being the most frequent site, followed by the pancreas. Somatostatinomas may be sporadic or associated with neurofibromatosis type 1, Multiple Endocrine Neoplasia type 1, and Von Hippel-Lindau syndromes. Functional Somatostatinomas release excessive amounts of somatostatin suppressing gallbladder motility and inhibiting the secretory activity of various endocrine and exocrine cell types. A triad of mild diabetes mellitus, cholelithiasis, and diarrhea/steatorrhoea characterizes the Somatostatinoma or 'inhibitory' syndrome. Non-functional Somatostatinomas tend either to be asymptomatic or to present with obstructive symptoms. These tumors are often malignant and by the time they are detected, nearly two-thirds have already metastasized to the regional lymph nodes or the liver. A comparison between our three cases and those in the literature provides useful insights into the clinical management of these patients. Interestingly, the jejunal Somatostatinoma described here is the second case ever reported.
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Somatostatinoma: Clinico‐pathological features of three cases and literature reviewed
Journal of gastroenterology and hepatology, 2007Co-Authors: Gabriella Nesi, Tommaso Marcucci, Carlos A. Rubio, Maria Luisa Brandi, Francesco TonelliAbstract:Somatostatinoma is a rare endocrine tumor that comprises around 1% of all gastroenteropancreatic endocrine neoplasms. This paper gives an updated review on Somatostatinoma and describes three sporadic cases of Somatostatinoma located in the pancreas, duodenum, and jejunum. Approximately 200 case histories of Somatostatinoma have been published, with the duodenum being the most frequent site, followed by the pancreas. Somatostatinomas may be sporadic or associated with neurofibromatosis type 1, Multiple Endocrine Neoplasia type 1, and Von Hippel-Lindau syndromes. Functional Somatostatinomas release excessive amounts of somatostatin suppressing gallbladder motility and inhibiting the secretory activity of various endocrine and exocrine cell types. A triad of mild diabetes mellitus, cholelithiasis, and diarrhea/steatorrhoea characterizes the Somatostatinoma or 'inhibitory' syndrome. Non-functional Somatostatinomas tend either to be asymptomatic or to present with obstructive symptoms. These tumors are often malignant and by the time they are detected, nearly two-thirds have already metastasized to the regional lymph nodes or the liver. A comparison between our three cases and those in the literature provides useful insights into the clinical management of these patients. Interestingly, the jejunal Somatostatinoma described here is the second case ever reported.
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Characterization of the functional and growth properties of long-term cell cultures established from a human Somatostatinoma
Endocrine-related cancer, 2006Co-Authors: Gianna Galli, Gabriella Nesi, Tommaso Marcucci, Francesco Tonelli, Roberto Zonefrati, Alessia Gozzini, Carmelo Mavilia, Valentina Martineti, Isabella Tognarini, Maria Silvia TommasiAbstract:In Somatostatinoma, a rare malignant somatostatin (SST)-secreting neoplasia, tumour regression is rarely observed, implying the need for novel antiproliferative strategies. Here, we characterized a long-term culture (SST-secreting cancer (SS-C cells)) established from a human Somatostatinoma. High concentrations of SST and chromogranin A were released by SS-C cells and SST release was stimulated by depolarizing stimuli and inhibited by the SST analogue, octreotide. SS-C cells expressed mRNA for SST receptor (SSTR) subtypes 1, 2 and 4, being also able to bind native SST. Moreover, SS-C cells were positively stained with an antibody to SSTR2. SS-C cells also expressed interferon-g (IFN-g) receptor mRNA and measurable telomerase activity. Our findings indicate that in vitro exposure of SS-C cells to native SST-28, to octreotide, to IFN-g ,o r to 3 0 -azido3 0 deoxythymidine (AZT), a telomerase inhibitor, results in inhibition of SS-C cell proliferation. Concomitant with growth inhibition, apoptosis was detected in SST-, octreotide-, IFN-g- or AZTtreated SS-C cell cultures. Taken together our results characterized native SST, SST analogues, IFN-g and a telomerase inhibitor as growth-inhibiting and proapoptotic stimuli in cultured human Somatostatinoma cells. Based on these findings, the potential of SST analogues, IFN-g and AZT, alone or in combination, should be further explored in the medical treatment of Somatostatinoma. Endocrine-Related Cancer (2006) 13 79‐93
