scholarly journals Giant Intra-Abdominal Desmoid Tumor in a Young Male without History of Surgery, Trauma, or Familial Adenomatous Polyposis

2018 ◽  
Vol 2018 ◽  
pp. 1-5
Author(s):  
Noritoshi Mizuta ◽  
Kozo Tsunemi
Keyword(s):  
Familial Adenomatous Polyposis ◽  
Desmoid Tumor ◽  
Treatment Plan ◽  
Abdominal Cavity ◽  
Beta Catenin ◽  
Desmoid Tumors ◽  
Adenomatous Polyposis ◽  
History Of Surgery ◽  
History Of ◽  
Abdominal Desmoid Tumor

Desmoid tumors are rare, monoclonal myofibroblastic neoplasms that occur in the extremities, the trunk, and the abdominal cavity. We present a case that is significant for its rarity and for consideration of its treatment plan. A 17-year-old male reported swelling of his abdomen and abdominal pain. He was referred to our hospital with no history of surgery, trauma, or familial adenomatous polyposis. A large tumor in the abdominal cavity was detected by computed tomography, and surgical resection was performed. The tumor was thought to have developed from the anterior lobe of the transverse colon mesentery. It weighed 5.9 kg. Tumor cells with collagen fibers were observed in histopathological examination, but heteromorphism and the nuclear fission image were not apparent. Immunostaining revealed beta-catenin expression in the tumor cell nucleus. Diagnosis was an intra-abdominal desmoid tumor. Currently, there are no signs of recurrence. In this case, preoperative diagnosis was difficult, but surgery was the optimal treatment according to the symptoms. Desmoid tumors have invasive development and common local recurrence, so sufficient range of resection including normal tissue and strict follow-up are necessary.

Giant Desmoid Tumor of the Abdominal Wall Associated with Familial Adenomatous Polyposis

2003 ◽  
Vol 89 (3) ◽  
pp. 331-332 ◽  
Author(s):  
Ugo Marone ◽  
Alfonso Amore ◽  
Luciano Pezzullo ◽  
Nicola Mozzillo
Keyword(s):  
Familial Adenomatous Polyposis ◽  
Abdominal Wall ◽  
Desmoid Tumor ◽  
Complementary Therapies ◽  
Surgical Excision ◽  
Metastatic Potential ◽  
Tumor Type ◽  
Desmoid Tumors ◽  
Adenomatous Polyposis ◽  
Benign Histology

In this paper a case of vast desmoid tumor of the abdominal wall associated with familial adenomatous polyposis is reported. Desmoid tumors represent a particular type of fibrous neoplasms with a higher prevalence in females. They are extremely rare in the sporadic form, while they are found in about 10% of patients affected by familial adenomatous polyposis. Despite their benign histology and the absence of metastatic potential, they can be considered as fibrosarcomas with a low level of malignancy because of their locally invasive nature. The treatment of choice is surgical excision, as wide as possible, aimed at preventing recurrences, which are frequent in this tumor type. The usefulness of complementary therapies such as radiotherapy, hormone or chemotherapy, is not entirely clear.

Noncytotoxic drug therapy for intra-abdominal desmoid tumor in patients with familial adenomatous polyposis

1992 ◽  
Vol 35 (1) ◽  
pp. 29-33 ◽  
Author(s):  
Kunio Tsukada ◽  
James M. Church ◽  
David G. Jagelman ◽  
Victor W. Fazio ◽  
Ellen McGannon ◽  
...  
Keyword(s):  
Drug Therapy ◽  
Familial Adenomatous Polyposis ◽  
Desmoid Tumor ◽  
Adenomatous Polyposis ◽  
Abdominal Desmoid Tumor

scholarly journals Gardner syndrome with giant abdominal desmoid tumor during pregnancy: a case report

BMC Surgery ◽  
2020 ◽  
Vol 20 (1) ◽  
Author(s):  
Liquan Jin ◽  
Yunbo Tan ◽  
Ziting Su ◽  
Shan Huang ◽  
Sita Pokhrel ◽  
...  
Keyword(s):  
Desmoid Tumor ◽  
Abdominal Mass ◽  
Pathological Examination ◽  
Desmoid Tumors ◽  
Adenomatous Polyposis ◽  
Abdominal Tumor ◽  
Gardner Syndrome ◽  
Increased Risk ◽  
Abdominal Desmoid Tumor

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 intra-abdominal 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.

scholarly journals Recurrent giant cranial desmoid tumor in a 3-year-old boy with familial adenomatous polyposis requiring bifrontoparietal cranioplasty: case report

2016 ◽  
Vol 18 (6) ◽  
pp. 703-707
Author(s):  
Luyuan Li ◽  
John N. Jensen ◽  
Sara Szabo ◽  
Peter VanTuinen ◽  
Sean M. Lew
Keyword(s):  
Familial Adenomatous Polyposis ◽  
Desmoid Tumor ◽  
Genetic Disorder ◽  
Aggressive Fibromatosis ◽  
Colorectal Polyps ◽  
Desmoid Tumors ◽  
Adenomatous Polyposis ◽  
Connective Tissues ◽  
Total Resection ◽  
Cranial Fasciitis

Desmoid tumors, also known as aggressive fibromatosis, are locally infiltrating musculoaponeurotic neoplasms arising in connective tissues. Desmoid tumors may be associated with familial adenomatous polyposis (FAP), a genetic disorder that presents with hundreds to thousands of precancerous colorectal polyps. The authors report the case of an 18-month-old boy who underwent resection of a right temporal desmoid tumor (initially diagnosed as cranial fasciitis) and developed a bilateral frontoparietal calvarial desmoid tumor 2 years later. The patient underwent gross-total resection of the tumor that required a large cranioplasty. He was subsequently diagnosed with FAP. The patient has been without tumor recurrence for 9 years afterwards and has not required revision of his cranioplasty. This is the first report describing a recurrent cranial desmoid tumor in a pediatric patient with FAP. The authors believe, however, that some of the cases previously reported as cranial fasciitis are likely desmoid tumors pathobiologically and genetically.

scholarly journals Desmoid tumor in patients with familial adenomatous polyposis

2010 ◽  
Vol 47 (4) ◽  
pp. 373-378 ◽  
Author(s):  
Raquel Franco Leal ◽  
Patricia V. V. Tapia Silva ◽  
Maria de Lourdes Setsuko Ayrizono ◽  
João José Fagundes ◽  
Eliane M. Ingrid Amstalden ◽  
...  
Keyword(s):  
Familial Adenomatous Polyposis ◽  
Median Time ◽  
Desmoid Tumor ◽  
Treatment Options ◽  
Tumor Resection ◽  
Signs And Symptoms ◽  
Desmoid Tumors ◽  
Adenomatous Polyposis ◽  
Antiinflammatory Drugs ◽  
Surgical Data

CONTEXT: Desmoid tumors constitute one of the most important extraintestinal manifestations of familial adenomatous polyposis. The development of desmoids is responsible for increasing morbidity and mortality rates in cases of familial adenomatous polyposis. OBJECTIVES: To evaluate the occurrence of desmoid tumors in familial adenomatous polyposis cases following prophylactic colectomy and to present patient outcome. METHODS: Between 1984 and 2008, 68 patients underwent colectomy for familial adenomatous polyposis at the School of Medical Sciences Teaching Hospital, University of Campinas, SP, Brazil. Desmoid tumors were found in nine (13.2%) of these patients, who were studied retrospectively by consulting their medical charts with respect to clinical and surgical data. RESULTS: Of nine patients, seven (77.8%) were submitted to laparotomy for tumor resection. Median age at the time of surgery was 33.9 years (range 22-51 years). Desmoid tumors were found in the abdominal wall in 3/9 cases (33.3%) and in an intra-abdominal site in the remaining six cases (66.7%). Median time elapsed between ileal pouch-anal anastomosis and diagnosis of desmoid tumor was 37.5 months (range 14-60 months), while the median time between colectomy with ileorectal anastomosis and diagnosis was 63.7 months (range 25-116 months). In 6/9 (66.7%) patients with desmoid tumors, the disease was either under control or there was no evidence of tumor recurrence at a follow-up visit made a mean of 63.1 months later (range 12-240 months). CONCLUSIONS: Desmoid tumors were found in 13.2% of cases of familial adenomatous polyposis following colectomy; therefore, familial adenomatous polyposis patients should be followed-up and surveillance should include abdominal examination to detect signs and symptoms. Treatment options include surgery and clinical management with antiestrogens, antiinflammatory drugs or chemotherapy.

scholarly journals Extra-Abdominal Desmoid Tumors Associated with Familial Adenomatous Polyposis

Sarcoma ◽  
2012 ◽  
Vol 2012 ◽  
pp. 1-11 ◽  
Author(s):  
George T. Calvert ◽  
Michael J. Monument ◽  
Randall W. Burt ◽  
Kevin B. Jones ◽  
R. Lor Randall
Keyword(s):  
Familial Adenomatous Polyposis ◽  
Natural History ◽  
Molecular Biology ◽  
Desmoid Tumors ◽  
Adenomatous Polyposis ◽  
Polyposis Syndrome ◽  
The Past ◽  
History Of ◽  
Basic Biology ◽  
Extra Abdominal Desmoid

Extra-abdominal desmoid tumors are a significant cause of morbidity in patients with familial adenomatous polyposis syndrome. Understanding of the basic biology and natural history of these tumors has increased substantially over the past decade. Accordingly, medical and surgical management of desmoid tumors has also evolved. 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.

Desmoid Tumor in Familial Adenomatous Polyposis (FAP)

2008 ◽  
Vol 24 (1) ◽  
pp. 20 ◽  
Author(s):  
Dae Dong Kim ◽  
Chang Sik Yu ◽  
Dong Hyun Hong ◽  
Sang Hun Jung ◽  
Pyong Wha Choi ◽  
...  
Keyword(s):  
Familial Adenomatous Polyposis ◽  
Desmoid Tumor ◽  
Adenomatous Polyposis
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