Stepwise Progression of Familial Adenomatous Polyposis-Associated Desmoid Precursor Lesions Demonstrated by a Novel CT Scoring System

Middleton, Simon B.; Clark, Susan; Matravers, Paul; Katz, David; Reznek, Rodney H.; Phillips, R. K. S.

doi:10.1007/s10350-004-6586-1

Cited by 30 publications

(12 citation statements)

References 17 publications

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“…15 Although it is assumed that there are desmoid precursor lesions progressing to a semimalignant state analogous to the adenomacarcinoma sequence in FAP, 16 the pathogenesis of desmoids remains poorly understood. It has been reported that somatic ␤-catenin mutations are found quite frequently in sporadic desmoids, occurring in up to 52 percent of analyzed cases.…”

mentioning

confidence: 99%

Genetic Testing for Germline Mutations of the APC Gene in Patients With Apparently Sporadic Desmoid Tumors but a Family History of Colorectal Carcinoma

Brückl

Ballhausen

Förtsch

et al. 2005

Diseases of the Colon &Amp; Rectum

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These results may suggest that patients with sporadic desmoids and no clinical signs of familial adenomatous polyposis detected on careful examination, esophagogastroduodenoscopy, and complete colonoscopy do not need to be tested routinely for germline mutations of the APC gene. However, as large studies dealing with this problem are absent, it might be more time and cost effective to perform an APC mutational analysis instead.

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mentioning

confidence: 99%

Genetic Testing for Germline Mutations of the APC Gene in Patients With Apparently Sporadic Desmoid Tumors but a Family History of Colorectal Carcinoma

Brückl

Ballhausen

Förtsch

et al. 2005

Diseases of the Colon &Amp; Rectum

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“…• Ultrasound (US): this tool shows an echogenic mass with well-defined and smooth margins; • CT: this tool allows finding precursors of lesions [12,14,25,26], assessing the quantity of water associated with rapid growth, and determining the extension and involvement of vital organs or anatomical structures; • RMN: this tool is more refined compared to CT, allowing the involvement of vascular structures and relapse following surgical treatment [27]. Treatment of such neoplasms is guided by the clinical and evolutive characteristics.…”

Section: Discussionmentioning

confidence: 99%

Abdominal wall reconstruction with intraperitoneal prosthesis in desmoid tumors surgery

Catania

Ruggeri²,

Iuppa

et al. 2011

Updates Surg

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Desmoid tumor (DT), also known as aggressive fibromatosis, is a rare soft tissue neoplasm. For those tumors localized in the anterior abdominal wall, radical resection and reconstruction with mesh are indicated. Due to the rarity of this disease, there have been no randomized trials, but in reported retrospective series, although it is considered a benign lesion, it is clear that local recurrence is not uncommon. Records from seven consecutive patients (1 man, 6 women; mean age 35 years, range 25-60 years) presenting with desmoid tumors of the anterior abdominal wall were analyzed. In all cases the surgical strategy was the same: wide surgical excision and immediate plastic reconstruction with ePTFE mesh after intraoperative confirmation by frozen section of disease-free margins >1 cm. No immediate postoperative complications were recorded, and no patients developed recurrence after a median follow-up period of 60 months. The long-term mean of global health status recorded was 100%. Radical resection aided by intraoperative margin evaluation by frozen sections, followed by immediate mesh reconstruction, is a safe procedure and can provide a definitive cure without functional limitations for patients with desmoid tumors of the anterior abdominal wall.

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“…The cumulative risk for developing desmoids approaches 29% [8] and recurrence rates around surgical sites are high. As treatment protocols are not well established [15][16][17], a graded approach to treating desmoids has been recommended including surgery for extra-abdominal tumors, benign therapies for intra-abdominal desmoids, and finally more aggressive chemotherapy agents, surgery and radiation therapy if needed [9,19,20].…”

Section: Discussionmentioning

confidence: 99%

Abdominal desmoid in familial adenomatous polyposis presenting as a pancreatic cystic lesion

2005

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A 17-year-old male with familial adenomatous polyposis (FAP) presented with chest pain and significant weight loss. An abdominal CT scan detected a cystic pancreatic lesion of unknown etiology. The patient therefore underwent surgical resection of the distal pancreas, which included the lesion, because of the known association of pancreatic cancer with FAP. Histopathological examination of the resected specimen showed a benign pancreatic cyst and fibrous plaque with desmoid fibromatosis adherent to the surface of the pancreas, serosa of the stomach, and colon. The fibrous plaque was histologically identical to the fibrous mesenteric plaque known to occur in FAP and associated mesenteric fibromatosis. We present pathologic evidence that the pancreatic cyst formation was induced by FAP-associated desmoid invasion. Desmoid growth should be considered in the differential diagnosis of a pancreatic cystic mass lesion in patients with FAP or its Gardner syndrome variant. This case report provides the first pathologic evidence for benign epithelial cyst formation in the pancreas caused by fibromatosis invasion of that organ as a part of FAP.

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Stepwise Progression of Familial Adenomatous Polyposis-Associated Desmoid Precursor Lesions Demonstrated by a Novel CT Scoring System

Abstract: A reliable CT scoring system for mesenteric fibromatosis and desmoid tumors in familial adenomatous polyposis is presented. Results with this system provide further evidence for a stepwise progression in the development of desmoids.

Cited by 30 publications

References 17 publications

Genetic Testing for Germline Mutations of the APC Gene in Patients With Apparently Sporadic Desmoid Tumors but a Family History of Colorectal Carcinoma

Genetic Testing for Germline Mutations of the APC Gene in Patients With Apparently Sporadic Desmoid Tumors but a Family History of Colorectal Carcinoma

Abdominal wall reconstruction with intraperitoneal prosthesis in desmoid tumors surgery

Abdominal desmoid in familial adenomatous polyposis presenting as a pancreatic cystic lesion

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