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Primary retroperitoneal tumours
Published in Anju Sahdev, Sarah J. Vinnicombe, Husband & Reznek's Imaging in Oncology, 2020
Isaac R Francis, Richard H Cohan, William R Burns
Solitary fibrous tumours (SFTs) are rare spindle cell neoplasms. These were previously classified as hemangiopericytomas and/or giant cell angiofibromas. These tumours are CD34 positive and overexpress the STAT6 protein. The majority of these tumours are benign, but up to 20% may demonstrate aggressive malignant behaviour. While SFTs most commonly arise from the pleura, the most common extrapleural site is the retroperitoneum (2,6,7). Fewer than 5% of patients may present with hypoglycaemia—Doege–Potter syndrome (41).
The urinary tract
Published in Spencer W. Beasley, John Hutson, Mark Stringer, Sebastian K. King, Warwick J. Teague, Paediatric Surgical Diagnosis, 2018
Spencer W. Beasley, John Hutson, Mark Stringer, Sebastian K. King, Warwick J. Teague
Persistence of mucosal folds attached to the verumonta- num block the posterior urethra (type I valve). Rarely, there may be persistence of the urogenital part of the cloacal membrane, producing a more distal obstruction (type III valve). Boys with a posterior urethral valve present with a poor urinary stream, sepsis from urinary tract infection or renal failure. The urinary obstruction has been present throughout the formation of the urinary tract: if the obstruction is severe, renal dysplasia may ensue. On occasion, this is so severe the child has intrauterine renal failure and dies shortly after birth with Potter syndrome. A micturating cystourethrogram confirms the diagnosis postnatally and a renal nuclear scan allows assessment of renal function and upper tract obstruction. Severe vesicoureteric reflux is often coexistent.
Doege-Potter syndrome in a patient with a giant abdominal solitary fibrous tumor: a case report and review of the literature
Published in Acta Clinica Belgica, 2023
Joris Rötgens, Bruno Lapauw, Guy T’Sjoen
In about half of the patients with Doege-Potter syndrome, development of hypoglycemia leads to the diagnosis of the tumor. In other cases, hypoglycemia occurs after the tumor has already been diagnosed, mainly because of symptoms due to the tumor mass itself. Because small tumors are generally asymptomatic, SFTs may attain large sizes before presentation, with intra-abdominal SFTs being typical more than 20 cm at the time of diagnosis. When hypoglycemia develops, both neuroglycopenic and autonomic symptoms may occur. Neuroglycopenic symptoms can range from mild to severe (confusion, amnesia, psychosis, seizures and coma). Due to repeated hypoglycemic events and insidious progression, autonomic symptoms (sweating, anxiety and tremor) are frequently blunted or absent [10]. Interestingly, there are a few cases in which there was a spectacular improvement in metabolic control in pre-existing diabetes. Acromegalic features (due to increased activation of the IGF-I receptor by big IGF-II) are a rare manifestation and disappear after surgery. Our patient presented with multiple skin tags, which could be a manifestation of increased activation of IGF-R, as reported in earlier cases. Hypokalemia has also been described (due to insulin-like activity of big IGF-II) [6,11]. In addition to Doege-Potter syndrome, SFTs can also give rise to another paraneoplastic manifestation, namely Pierre-Marie-Bamberger syndrome (characterized by clubbing of the fingers, long bone periostitis and arthritis due to hypertrophic osteoarthropathy). This occurs in up to 35% of cases with SFT and is thus more prevalent than Doege-Potter syndrome. Cases have been described were patients exhibit both paraneoplastic features [12].