eRAM
encyclopedia of Rare Disease Annotation for Precision Medicine
| Disease | desmoid tumor |
| Comorbidity | C0032580|familial adenomatous polyposis |
| Sentences | 25 |
| PubMedID- 25725654 | Reply to commentary on "clinical characteristics and adequate treatment of familial adenomatous polyposis combined with desmoid tumors". |
| PubMedID- 25116575 | Approximately 10% of the desmoid tumours are associated with familial adenomatous polyposis (fap). |
| 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- 23334997 | desmoid tumour biology in patients with familial adenomatous polyposis coli. |
| PubMedID- 23329958 | desmoid tumor associated with familial adenomatous polyposis: evaluation with 64-detector ct enterography. |
| 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- 22965401 | Background: desmoid tumors associated with familial adenomatous polyposis show variable behavior; about 10% grow relentlessly, resulting in severe morbidity or mortality. |
| 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- 19908178 | [current diagnosis and treatment of desmoid tumours in patients with familial adenomatous polyposis - the surgical view]. |
| PubMedID- 22367132 | An estimated 2% of all desmoid tumors are associated with familial adenomatous polyposis (fap), an autosomal dominant disease [2,3,9]. |
| 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- 25725653 | Commentary on "clinical characteristics and adequate treatment of familial adenomatous polyposis combined with desmoid tumors". |
| PubMedID- 25656017 | [scrotal desmoid tumor in a patient with familial adenomatous polyposis]. |
| 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. |
| PubMedID- 23673714 | [desmoid tumors associated with familial adenomatous polyposis]. |
| PubMedID- 21225148 | Objectives: to evaluate the occurrence of desmoid tumors in familial adenomatous polyposis cases following prophylactic colectomy and to present patient outcome. |
| 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- 22449158 | Multiple desmoid tumors in a patient with familial adenomatous polyposis caused by the novel w421x mutation. |
| PubMedID- 22323009 | Mortality of intra-abdominal desmoid tumors in patients with familial adenomatous polyposis: a single center review of 154 patients. |
| PubMedID- 21403834 | Approximately 10% of desmoids occur in patients with familial adenomatous polyposis [1] as one of the extracolonic manifestations of gardner syndrome. |
| PubMedID- 25152189 | Clinical characteristics and adequate treatment of familial adenomatous polyposis combined 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- 23819848 | Enterocutaneous fistulae in familial adenomatous polyposis patients with abdominal desmoid disease. |
| PubMedID- 21364578 | Evaluation of management of desmoids tumours associated with familial adenomatous polyposis in dutch patients. |
| PubMedID- 25315103 | desmoid tumour in familial adenomatous polyposis patients: responses to treatments. |
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