Gardner syndrome with giant abdominal desmoid tumor during pregnancy: a case report
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CASE REPORT
Gardner syndrome with giant abdominal desmoid tumor during pregnancy: a case report Liquan Jin1, Yunbo Tan1, Ziting Su1, Shan Huang1, Sita Pokhrel2, Hongbo Shi1 and Yiming Chen1,3*
Abstract Background: Gardner syndrome is a subtype of familial adenomatous polyposis (FAP), characterized by a combination of adenomatous intestinal polyps and extracolonic lesions such as multiple osteomas, dental abnormalities, and soft tissue tumors. Although 12% of patients with intestinal polyposis of FAP may occur intra-abdominal desmoid tumors, pregnancy complicating with giant abdominal desmoid tumors is a relatively rare case. Case presentation: A 28-year-old pregnant woman was diagnosed with Gardner syndrome in whom an intraabdominal tumor was found a year after undergoing a laparoscopic total colectomy due to family adenomatous polyposis. At 32 weeks’ gestation, she presented to our department for the third time complaining upper abdominal pain caused by the giant abdominal mass about 21 × 12 cm2 in size. After multidisciplinary consultation and discussion, the decision of fetal preservation treatment was made. After the delivery of a baby girl, abdominal mass resection was performed, and pathological examination revealed a fibrous adenoma. The patient was discharged after a week and was uneventful in the follow-up for half a year. Conclusions: Gardner syndrome is characterized by typical syndrome including family adenomatous polyposis and extra-intestinal tissue tumor. Were desmoid tumors rarely as large as fetus and local aggressively. In our case, we selected surgery to remove the intra-abdominal desmoid tumor after the natural delivery of the fetus and no abnormalities were observed during the 6 months follow-up. Women during pregnancy have an increased risk for the development of desmoid tumors, likely with the sex hormone to be one of the triggers. Therefore, we suggested that when a patient with Gardner syndrome desire to conceive again, they should go to the hospital for a regular review at least once every 3 months. Keywords: Gardner syndrome, Desmoid tumor, FAP, Pregnancy, Case report Background Gardner syndrome, mainly manifested by multiple gastrointestinal polyps and universal lesions such as soft tissue tumor, ectopic teeth, osteoma, and retinal pigment epithelium, is a rare autosomal dominant genetic disorder caused by gene mutation in adenomatous polyposis coli (APC). Occasionally, Gardner syndromes also known as "deep" or "invasive" fibromatosis, characterized by *Correspondence: [email protected] 1 1St Department of General Surgery, The First Affiliated Hospital of Dali University, 32 Carlsberg Ave, Dali 671000, Yunnan, China Full list of author information is available at the end of the article
fibroblastomas or desmoid tumors that originated from deep muscle or aponeurosis. The World Health Organization (WHO) classified desmoid tumors as one of the moderate fibroblastoma, which recurs locally and invasively rather than distant metastasis. The incidence of Gardner syndrom
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