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Cited by 2Soft tissue tumor case studiesPancreatic and Hepatic Oncology ResearchNeurofibromatosis and Schwannoma Cases

The DOI matches its Crossref record; 45 of the 45 references it cites were found in a registry or an index.

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

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Tanishima, Tomoya, Kurokawa, Ryo, Sone, Miyuki, Nakai, Yudai, Kusumoto, Masahiko·2024·Abdominal Radiology
DOI 10.1007/s00261-024-04570-8PMC PMC11794332

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Abstract

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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Crossref · 10.1007/s00261-024-04570-8

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Figures

Fig. 1 Flow diagram of study identification
Fig. 1 Flow diagram of study identificationCC BY · PubMed Central
Fig. 2 Pancreatic desmoid-type fibromatosis in a 22-year-old female presenting with abdominal bloating, constipation, and abdominal pain (case 1). CT shows a 27-cm well-delineated tumor with a solid and cystic morphology (A, plain; B, arterial-phase; C, delayed-phase). The solid portion shows delayed enhancement on contrast-enhanced CT (A, B, C) and MRI (H), high intensity on fat-suppressed T1WI (D) and coronal T1WI (E), intermediate intensity on coronal T2WI (F), high intensity on fat-suppressed coronal T2WI (G), and homogeneous enhancement (H). The cystic portion shows high intensity on coronal T2WI (F) and fat-suppressed coronal T2WI (G), and high intensity on fat-suppressed T1WI (D) and coronal T1WI (E). Most of the cystic portions are continuous (white arrows), some with hemorrhage (white arrowheads). Diffusion restriction is not observed with the mean ADC value of the solid portion of 1.50 × 10−3mm2/s (I, J). Low-power magnification with hematoxylin and eosin stain shows desmoid-type fibromatosis with prominent pancreatic ductal dilation (K, white arrow). High-power magnification with hematoxylin and eosin stain shows pancreatic ductal epithelium showing reactive changes without evidence of dysplasia (L). Corresponding β-catenin nuclear positivity is observed (M). CT Computed tomography, MRI magnetic resonance imaging, T1WI T1-weighted imaging, T2WI T2-weighted imaging, ADC apparent diffusion coefficient
Fig. 2 Pancreatic desmoid-type fibromatosis in a 22-year-old female presenting with abdominal bloating, constipation, and abdominal pain (case 1). CT shows a 27-cm well-delineated tumor with a solid and cystic morphology (A, plain; B, arterial-phase; C, delayed-phase). The solid portion shows delayed enhancement on contrast-enhanced CT (A, B, C) and MRI (H), high intensity on fat-suppressed T1WI (D) and coronal T1WI (E), intermediate intensity on coronal T2WI (F), high intensity on fat-suppressed coronal T2WI (G), and homogeneous enhancement (H). The cystic portion shows high intensity on coronal T2WI (F) and fat-suppressed coronal T2WI (G), and high intensity on fat-suppressed T1WI (D) and coronal T1WI (E). Most of the cystic portions are continuous (white arrows), some with hemorrhage (white arrowheads). Diffusion restriction is not observed with the mean ADC value of the solid portion of 1.50 × 10−3mm2/s (I, J). Low-power magnification with hematoxylin and eosin stain shows desmoid-type fibromatosis with prominent pancreatic ductal dilation (K, white arrow). High-power magnification with hematoxylin and eosin stain shows pancreatic ductal epithelium showing reactive changes without evidence of dysplasia (L). Corresponding β-catenin nuclear positivity is observed (M). CT Computed tomography, MRI magnetic resonance imaging, T1WI T1-weighted imaging, T2WI T2-weighted imaging, ADC apparent diffusion coefficientCC BY · PubMed Central
Fig. 3 Pancreatic desmoid-type fibromatosis in a 20-year-old asymptomatic female (case 2). CT shows a 4-cm well-delineated tumor with a solid and cystic morphology (A, plain; B, arterial-phase; C, delayed-phase). The solid portion shows delayed enhancement on contrast-enhanced CT (A, B, C) and MRI (G), intermediate intensity on coronal T2 True Fast Imaging with Steady State Precession (True FISP) (D) and T1WI (F), and homogeneous enhancement (G). The cystic portion shows high intensity on coronal True FISP (D) and MRCP (E), and low intensity on T1WI (F). Most of the cystic portions are continuous (A–E, white arrows). Low-power magnification with hematoxylin and eosin stain shows desmoid-type fibromatosis with prominent pancreatic ductal dilation (H, white arrows). High-power magnification with hematoxylin and eosin stain shows pancreatic ductal epithelium showing reactive changes without evidence of dysplasia (I). Corresponding β-catenin nuclear positivity is observed (not shown). CT Computed tomography, MRI magnetic resonance imaging, T2WI T2-weighted imaging, T1WI T1-weighted imaging, MRCP magnetic resonance cholangiopancreatography
Fig. 3 Pancreatic desmoid-type fibromatosis in a 20-year-old asymptomatic female (case 2). CT shows a 4-cm well-delineated tumor with a solid and cystic morphology (A, plain; B, arterial-phase; C, delayed-phase). The solid portion shows delayed enhancement on contrast-enhanced CT (A, B, C) and MRI (G), intermediate intensity on coronal T2 True Fast Imaging with Steady State Precession (True FISP) (D) and T1WI (F), and homogeneous enhancement (G). The cystic portion shows high intensity on coronal True FISP (D) and MRCP (E), and low intensity on T1WI (F). Most of the cystic portions are continuous (A–E, white arrows). Low-power magnification with hematoxylin and eosin stain shows desmoid-type fibromatosis with prominent pancreatic ductal dilation (H, white arrows). High-power magnification with hematoxylin and eosin stain shows pancreatic ductal epithelium showing reactive changes without evidence of dysplasia (I). Corresponding β-catenin nuclear positivity is observed (not shown). CT Computed tomography, MRI magnetic resonance imaging, T2WI T2-weighted imaging, T1WI T1-weighted imaging, MRCP magnetic resonance cholangiopancreatographyCC BY · PubMed Central

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Research works printed but not linked: 1

  1. Fritchie KJ, Crago AM, Van de Rijn M (2020) Soft Tissue and Bone Tumours, 5th. World Health Organization, pp 93-95

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Metadata

Authors
Tanishima, T., Kurokawa, R., Sone, M., Nakai, Y., & Kusumoto, M.
Affiliations
  • National Cancer Centre Japan (JP)
  • The University of Tokyo (JP)
ORCID
Venue
Abdominal Radiology
Publisher
Springer Science and Business Media LLC
Published
2024-09-15
Volume
50
Issue
2
Pages
839-850
ISSN
  • 2366-004X (ISSN-L)
  • 2366-0058
DOI
10.1007/s00261-024-04570-8
PMC
PMC11794332
OpenAlex
W4402549875
Cited by
2
References
45
Citations by year
2025: 2
FWCI
0.15
Type
journal article
Language
en
Open access
Licence
Locations
  • Abdominal Radiology (publishedVersion, cc-by)
  • Research Square (acceptedVersion, cc-by)
  • PubMed Central (publishedVersion, cc-by)
  • PubMed (publishedVersion)
Funders
The University of Tokyo
Topics
  • Health Sciences › Medicine › Oncology › Soft tissue tumor case studies
  • Health Sciences › Medicine › Oncology › Pancreatic and Hepatic Oncology Research
  • Health Sciences › Medicine › Clinical Neurology › Neurofibromatosis and Schwannoma Cases
Keywords
aggressive fibromatosis, radiological features, computed tomography, magnetic resonance imaging, cystic tumor components, pancreatic duct dilatation, tumor morphology, tumor recurrence, surgical resection, pancreatic tail
MeSH terms
Adult, Female, Humans, Magnetic Resonance Imaging, Male, Middle Aged, Pancreatic Neoplasms, Retrospective Studies, Tomography, X-Ray Computed, Desmoid Tumors

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