Pancreatic cystic desmoid tumor following metastatic colon cancer surgery: A case report

Shayesteh, Shahab; Salimian, Kevan J.; Fouladi, Daniel Fadaei; Blanco, Alejandra; Chu, Linda C.; Fishman, Elliot K.

doi:10.1016/j.radcr.2020.08.013

Cited by 7 publications

(1 citation statement)

References 9 publications

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“…These tumours in GS account for 15% of cases [29]. They can appear as sporadic tumours or be associated with FAP and GS [30,31]. Benign soft-tissue tumours associated with GS are Gardner-associated fibromas which may progress into desmoid fibromatosis or they co-occur with them [32,33].…”

Section: Discussionmentioning

confidence: 99%

Gardner syndrome with desmoid tumors – case report

Rycyk,

Knop-Chodyła,

Kasztelan-Szczerbińska

et al. 2024

J Pre Clin Clin Res.

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Introduction. Gardner syndrome (GS) is characterized as a type of familial adenomatous polyposis (FAP), an autosomal dominant inherited disease which, if left untreated, with 100% risk leads to the development of colorectal cancer. Case Report. The case is presented a of a 40-year-old man who was diagnosed with Gardner syndrome at the age of 12. During his hospitalization, the patient underwent gastroscopy, colonoscopy, and computed tomography (CT) scans of head, neck and abdomen. The examination revealed the presence of extra-intestinal manifestations of GS: desmoid tumours, osteomas, and dental cavities. At present, the patient is scheduled for enteroscopy. Conclusions. GS is a diagnosis of genetic testing, although clinicians should be aware of the fact that up to 30% of GS cases are detected as de novo mutations. The physical examination should always be performed with accuracy to avoid a too late diagnosis of FAP, including GS, which may result in death.

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Section: Discussionmentioning

confidence: 99%

Gardner syndrome with desmoid tumors – case report

Rycyk,

Knop-Chodyła,

Kasztelan-Szczerbińska

et al. 2024

J Pre Clin Clin Res.

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Radiological features of pancreatic desmoid-type fibromatosis: a case series and systematic review

Tanishima,

Kurokawa,

Sone

et al. 2024

Abdom Radiol

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Purpose This retrospective study aimed to investigate the radiological features of pancreatic desmoid-type fibromatosis (PDF) and systematically review the previous publications and two new cases. Methods We searched PubMed, Cochrane Library, and Web of Science Core Collection and included 31 patients with pathologically proven PDFs with analyzable preoperative computed tomography (CT) and magnetic resonance imaging, including two patients from our institution and 29 patients from 28 publications. Two board-certified radiologists reviewed all images. Results The median age of the patients was 39 years, with a male dominance observed (male, 54.8% vs. female, 45.2%). Abdominal pain was the most frequent symptom, occurring in 58.1% of cases. Surgical resection was performed in all cases of PDFs, resulting in a recurrence rate of 8.3% (2/24). The tumors were most commonly located in the pancreatic tail (23/31, 74.2%). In terms of morphology, a “solid” shape was most prevalent (14/31, 45.2%), followed by a “solid and cystic” shape (9/31, 29.0%) and a “cystic” shape (8/31, 25.8%). Characteristic radiological features included heterogeneous enhancement of the solid portion of the tumors on CT scans (13/20, 65%), moderate-to-weak enhancement in the late phase on CT (16/17, 94.1%), and a presence of cystic components in the tumors (17/31, 54.8%). In 16.1% (5/31) of PDFs, the cystic component was pathologically confirmed to be a dilated pancreatic duct. Conclusion We summarized the clinical and imaging characteristics of PDF. Although the incidence may not be high, cystic components suggesting a dilated pancreatic duct within the tumor are unique imaging features in PDF.

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