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.

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.

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 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.

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

scholarly journals 516P Utility of colonoscopy in diagnosing familial adenomatous polyposis (FAP) among patients (pts) with desmoid tumors (DT)

2016 ◽  
Vol 27 ◽  
pp. ix167
Author(s):  
Z. Zhang ◽  
M. Teo ◽  
K.C. Soo ◽  
M.H. Tan ◽  
A.Y.-F. Chung ◽  
...  
Keyword(s):  
Familial Adenomatous Polyposis ◽  
Desmoid Tumors ◽  
Adenomatous Polyposis

scholarly journals Desmoid Tumor Associated With Familial Adenomatous Polyposis: Evaluation With 64-Detector CT Enterography

2012 ◽  
Vol 9 (1) ◽  
pp. 32-6 ◽  
Author(s):  
Oktay Algin ◽  
Sehnaz Evrimler ◽  
Evrim Ozmen ◽  
Melike Metin ◽  
Osman Ersoy ◽  
...  
Keyword(s):  
Familial Adenomatous Polyposis ◽  
Desmoid Tumor ◽  
Adenomatous Polyposis ◽  
Ct Enterography

Patterns of Adenoma Recurrence in Familial Adenomatous Polyposis Patients after Ileal Pouch-Anal Anastomosis

2015 ◽  
Vol 32 (6) ◽  
pp. 421-425 ◽  
Author(s):  
Adam L. Goldstein ◽  
Revital Kariv ◽  
Joseph M. Klausner ◽  
Hagit Tulchinsky
Keyword(s):  
Familial Adenomatous Polyposis ◽  
Desmoid Tumor ◽  
Ileal Pouch ◽  
Medical Center ◽  
Colonic Polyps ◽  
Ileal Pouch Anal Anastomosis ◽  
Adenomatous Polyposis ◽  
Adenoma Recurrence ◽  
Duodenal Adenomas ◽  
Anal Anastomosis

Aim: This study aims at identifying the risk factors for the development of pre-cancerous pouch and/or cuff adenomas post ileal pouch anal anastomosis (IPAA) in familial adenomatous polyposis patients. Method: We retrospectively studied 59 patients operated at a single medical center during a 26-year period. Data on the timing and location of adenoma recurrence were recorded and possible correlations with patients' gender, age, presence of desmoid tumor, duodenal adenomas, type of anastomosis and number of operation stages were analyzed. Results: Thirty-five (59%) patients had at least one adenoma in either the cuff or the pouch, including 20 with isolated cuff adenomas, 4 with isolated pouch adenomas and 11 patients with adenomas in both the pouch and cuff. There was no significant correlation between gender, age at surgery, type of anastomosis, number of operative stages and the development of pouch or cuff adenomas. Desmoid tumor and pouch adenomas were significantly correlated with cuff adenomas formation. Duodenal adenomas were associated with pouch adenomas. There was some relationship between the development of cuff adenomas and the burden of colonic polyps, as well as the presence of duodenal adenomas. Conclusion: Adenomas in both the pouch and cuff commonly occur following IPAA, mandating lifelong annual endoscopic surveillance.

Desmoid tumor of mesentery in familial adenomatous polyposis: a case report

2010 ◽  
Vol 14 (S1) ◽  
pp. 61-62 ◽  
Author(s):  
G. Basdanis ◽  
V. N. Papadopoulos ◽  
S. Panidis ◽  
I. Tzeveleki ◽  
E. Karamanlis ◽  
...  
Keyword(s):  
Case Report ◽  
Familial Adenomatous Polyposis ◽  
Desmoid Tumor ◽  
Adenomatous Polyposis
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