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eRAM

encyclopedia of Rare Disease Annotation for Precision Medicine




Disease desmoid tumor
Comorbidity C0032580|adenomatous polyposis
Sentences 25
PubMedID- 25152189 Clinical characteristics and adequate treatment of familial adenomatous polyposis combined with desmoid tumors.
PubMedID- 23673714 [desmoid tumors associated with familial adenomatous polyposis].
PubMedID- 22701333 This paper analyzes recent evidence pertaining to the epidemiology, molecular biology, histopathology, screening, and treatment of extra-abdominal desmoid tumors associated with familial adenomatous polyposis syndrome.
PubMedID- 24760231 The historic turcot's syndrome is characterized by colonic polyposis and brain tumors.figure 5.image from a computed tomography of a desmoid tumor in a patient with familial adenomatous polyposis.
PubMedID- 22367132 An estimated 2% of all desmoid tumors are associated with familial adenomatous polyposis (fap), an autosomal dominant disease [2,3,9].
PubMedID- 21364578 Evaluation of management of desmoids tumours associated with familial adenomatous polyposis in dutch patients.
PubMedID- 22965401 Background: desmoid tumors associated with familial adenomatous polyposis show variable behavior; about 10% grow relentlessly, resulting in severe morbidity or mortality.
PubMedID- 19908178 [current diagnosis and treatment of desmoid tumours in patients with familial adenomatous polyposis - the surgical view].
PubMedID- 25656017 [scrotal desmoid tumor in a patient with familial adenomatous polyposis].
PubMedID- 21225148 desmoid tumor in patients with familial adenomatous polyposis.
PubMedID- 24212949 desmoid tumors associated with familial adenomatous polyposis (fap) have been shown to be associated with mutations in the adenomatous polyposis coli (apc) gene [11].
PubMedID- 25725653 Commentary on "clinical characteristics and adequate treatment of familial adenomatous polyposis combined with desmoid tumors".
PubMedID- 26199790 No association with metastatic disease has been reported, however, they often show a strong tendency to infiltrate local adjacent tissue causing significant morbidity and mortality.1 sporadic desmoid tumors (those not associated with the familial adenomatous polyposis coli [fap] syndrome) have an estimated incidence of two to five cases per million per year.23 pregnancy-related desmoid tumors are even more infrequent, with limited published literature available.2 the etiology of this condition is currently unknown, however it has been shown that the majority of spontaneous desmoid tumors show activating mutations of the beta catenin gene,145 as well as somatic mutations of the adenomatous polyposis coli gene.6 they have also been associated with hyperestrogenic states and trauma, but the evidence is largely based on retrospective and anecdotal case reports.2 it seems that the hormonal and immunological changes occurring in pregnancy may play a role in the severity and course of the disease, however, their true association with the pathogenesis of the disease remains unclear.
PubMedID- 23329958 desmoid tumor associated with familial adenomatous polyposis: evaluation with 64-detector ct enterography.
PubMedID- 25116575 Approximately 10% of the desmoid tumours are associated with familial adenomatous polyposis (fap).
PubMedID- 23334997 desmoid tumour biology in patients with familial adenomatous polyposis coli.
PubMedID- 22323009 Mortality of intra-abdominal desmoid tumors in patients with familial adenomatous polyposis: a single center review of 154 patients.
PubMedID- 22449158 Multiple desmoid tumors in a patient with familial adenomatous polyposis caused by the novel w421x mutation.
PubMedID- 21904137 Setting and patients: polyposis registries in the netherlands, france, denmark, finland, and italy provided information on familial adenomatous polyposis patients with desmoid tumors.
PubMedID- 26504712 desmoid fibromatosis is seen in patients with familial adenomatous polyposis (fap) with an incidence of 3.5 to 32% [3], [26], [27], [33].
PubMedID- 25725654 Reply to commentary on "clinical characteristics and adequate treatment of familial adenomatous polyposis combined with desmoid tumors".
PubMedID- 21403834 Approximately 10% of desmoids occur in patients with familial adenomatous polyposis [1] as one of the extracolonic manifestations of gardner syndrome.
PubMedID- 23819848 Enterocutaneous fistulae in familial adenomatous polyposis patients with abdominal desmoid disease.
PubMedID- 20887112 This is the first reported case of spinal desmoid-type fibromatosis in association with adenomatous polyposis syndrome.
PubMedID- 23856253 Discussion: diagnosis and treatment of desmoids tumor in patients with familial adenomatous polyposis may be difficult, especially when desmoids are located intra-abdominally and in the mesentery.

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