The Experts below are selected from a list of 219 Experts worldwide ranked by ideXlab platform
Leonardo Resta - One of the best experts on this subject based on the ideXlab platform.
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doege Potter Syndrome by malignant solitary fibrous tumor of the liver a case report and review of literature
World Journal of Gastrointestinal Surgery, 2019Co-Authors: Antonella Delvecchio, Loren Duda, Maria Conticchio, Felicia Fiore, Stefano Lafranceschina, Umberto Riccelli, Antonella Cristofano, Bianca Pascazio, Anna Colagrande, Leonardo RestaAbstract:Doege-Potter Syndrome by malignant solitary fibrous tumor of the liver: A case report and review of literature
Charleen M Moore - One of the best experts on this subject based on the ideXlab platform.
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trisomy 7 and Potter Syndrome
Clinical Genetics, 2008Co-Authors: Solveig M V Pflueger, Charles I Scott, Charleen M MooreAbstract:: A patient with mosaic trisomy 7 and features of Potter Syndrome is described. The patient was the product of a 35-week gestation and survived fourteen hours, demise being attributed to respiratory insufficiency. Autopsy confirmed pulmonary hypoplasia and renal agenesis. Additional findings included malformed, low-set ears, a flattened nasal bridge, redundant nuchal skin, positional deformation of the extremities, rocker-bottom feet, and clitorimegaly. Cytogenetic study of peripheral blood and skin fibroblast culture revealed mosaicism for full trisomy 7, the skin showing 12% of the cells to have an extra 7. Comparison with one previously confirmed case of trisomy 7 and two cases of trisomy C suggests a correlation between trisomy 7 and Potter Syndrome.
Paul Van Schil - One of the best experts on this subject based on the ideXlab platform.
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solitary fibrous tumor of the pleura with associated doege Potter Syndrome
Acta Chirurgica Belgica, 2016Co-Authors: Reinder J D Reuvers, Martijn Van Dorp, Paul Van SchilAbstract:AbstractDoege–Potter Syndrome is a paraneoplastic Syndrome characterized by tumor-associated hypoglycemia secondary to a solitary fibrous tumor of the pleura. We present a case of an 84-year-old man, who presented with acute mental confusion and therapy-resistant hypoglycemia. Diagnostic imaging revealed a large sharply defined pleural tumor based on the left diaphragm, after surgical resection the diagnosis was made of a malignant solitary fibrous tumor of the pleura and restoration of the glucose homeostasis was observed.
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solitary fibrous tumor of the pleura with associated hypoglycemia doege Potter Syndrome a case report
Journal of Thoracic Oncology, 2006Co-Authors: Bram Balduyck, Patrick Lauwers, Karl Govaert, Jeroen Hendriks, Marianne G De Maeseneer, Paul Van SchilAbstract:Tumor-associated hypoglycemia as a paraneoplastic phenomenon is a well-known entity and is referred to as Doege-Potter Syndrome. A man was admitted because of acute confusion and drowsiness. Laboratory results showed profound hypoglycemia. All investigations proved to be normal, except for a chest x-ray, which showed a large pleural mass. On transthoracic puncture, a tumor of pleural origin was diagnosed. This tumor, presenting as a large, well-circumscribed encapsulated mass, was removed by thoracotomy. On pathologic examination, the diagnosis of a solitary fibrous tumor with benign characteristics was made. After surgical removal, the hypoglycemia resolved. Solitary fibrous tumors are localized tumors of the pleura with an unpredictable behavior. The therapy consists of resection.
Giorgio Annoni - One of the best experts on this subject based on the ideXlab platform.
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a rare diagnosis after the fall of a 96 year old woman doege Potter Syndrome
Current Aging Science, 2019Co-Authors: Angela Ida Pincelli, Mario Perotti, Francesca Massariello, Antonella Gatti, Damiano Calella, Vincenzo Cimino, Justin Haas, Giuseppe Bellelli, Paolo Mazzola, Giorgio AnnoniAbstract:INTRODUCTION: Doege-Potter Syndrome (DPS) is a rare but life-threatening paraneoplastic Syndrome, characterized by Non-Islet Cell Tumor-Induced Hypoglycemia (NICTH) secondary to a Solitary Fibrous Tumor (SFT), which secretes an incompletely processed form of Insulin-like Growth Factor 2 (IGF-2). RESULTS: A 96-year-old woman was admitted with head trauma due to an accidental fall. During her hospital stay she experienced frequent hypoglycemic episodes. Multiple injections of 33% dextrose and continuous infusion with 10% dextrose were required to maintain normal blood glucose levels. Biochemical analyses revealed hypoinsulinemic hypoglycemia, low C-peptide levels, suppressed insulin-like growth factor-1, normal insulin-like growth factor-2, and an elevated IGF-2:IGF-1 ratio, all consistent with IGF-2 secretion by a non-islet cell tumor. A contrast-enhanced chest and abdominal CT scans showed a single large pleural mass in the left lower hemithorax measuring 15x14 cm without secondary lesions. Histological analysis of biopsied specimens suggested a solitary fibrous pleural tumor; accordingly, a diagnosis of Doege-Potter Syndrome was considered. Due to extensive tumor burden and the advanced age of the patient, supportive and non-invasive management was chosen. Dexamethasone therapy was started, and while receiving this therapy she was able to discontinue glucose infusion and successfully maintain euglycemia. DISCUSSION: In the elderly, a sudden and unexplained fall can be the expression of severe hypoglycemia, usually as a complication of insulin therapy or of oral hypoglycemic agents administered to patients with diabetes. However, in patients without diabetes, other causes should be investigated, and the hypothesis of neoplastic diseases should be considered. CONCLUSION: In this case report we describe an uncommon cause of paraneoplastic hypoglycemia occurring in the oldest patient with a non-islet cell tumor reported thus far.
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A Rare Diagnosis After the Fall of a 96-Year-Old Woman: Doege-Potter Syndrome.
Current Aging Science, 2019Co-Authors: Angela Ida Pincelli, Mario Perotti, Francesca Massariello, Antonella Gatti, Damiano Calella, Vincenzo Cimino, Justin Haas, Giuseppe Bellelli, Paolo Mazzola, Giorgio AnnoniAbstract:Doege-Potter Syndrome (DPS) is a rare but life-threatening paraneoplastic Syndrome, characterized by Non-Islet Cell Tumor-Induced Hypoglycemia (NICTH) secondary to a Solitary Fibrous Tumor (SFT), which secretes an incompletely processed form of Insulin-like Growth Factor 2 (IGF-2). A 96-year-old woman was admitted with head trauma due to an accidental fall. During her hospital stay she experienced frequent hypoglycemic episodes. Multiple injections of 33% dextrose and continuous infusion with 10% dextrose were required to maintain normal blood glucose levels. Biochemical analyses revealed hypoinsulinemic hypoglycemia, low C-peptide levels, suppressed insulin-like growth factor-1, normal insulin-like growth factor-2, and an elevated IGF-2:IGF-1 ratio, all consistent with IGF-2 secretion by a non-islet cell tumor. A contrast-enhanced chest and abdominal CT scans showed a single large pleural mass in the left lower hemithorax measuring 15x14 cm without secondary lesions. Histological analysis of biopsied specimens suggested a solitary fibrous pleural tumor; accordingly, a diagnosis of Doege-Potter Syndrome was considered. Due to extensive tumor burden and the advanced age of the patient, supportive and non-invasive management was chosen. Dexamethasone therapy was started, and while receiving this therapy she was able to discontinue glucose infusion and successfully maintain euglycemia. In the elderly, a sudden and unexplained fall can be the expression of severe hypoglycemia, usually as a complication of insulin therapy or of oral hypoglycemic agents administered to patients with diabetes. However, in patients without diabetes, other causes should be investigated, and the hypothesis of neoplastic diseases should be considered. In this case report we describe an uncommon cause of paraneoplastic hypoglycemia occurring in the oldest patient with a non-islet cell tumor reported thus far. Copyright© Bentham Science Publishers; For any queries, please email at epub@benthamscience.net.
Joao Roberto Sa - One of the best experts on this subject based on the ideXlab platform.
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Seborrheic keratoses and severe hypoinsulinemic hypoglycemia associated with insulin grow factor 2 secretion by a malignant solitary fibrous tumor
Diabetology & Metabolic Syndrome, 2016Co-Authors: Andreia Latanza Gomes Mathez, Debora Moroto, Joao Roberto SaAbstract:A rare sign of some malignant tumors is a sudden eruption of multiple seborrheic keratoses called Leser-Trélat sign. Overproduction of insulin-like growth factor-2 (IGF2) or its precursor is the main mechanism related to non-islet cell tumor hypoglycemia. Doege-Potter Syndrome is the name given to paraneoplastic hypoinsulinemic hypoglycemia in presence of a solitary fibrous tumor. This report describes a case of a patient with hypoinsulinemic hypoglycemia and Leser-Trélat sign associated with a malignant solitary fibrous tumor with IGF2 secretion. Both conditions have improved after tumor excision.