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Desmoid Tumor of the Mesentery: A Potential Mimic of Other GI Pathology

Desmoid Tumor of the Mesentery: A Potential Mimic of Other GI Pathology

Elliot K. Fishman M.D.
Professor of Radiology, Surgery, Oncology and Urology
Johns Hopkins Hospital

Click here to view this module as a video lecture.

 

"Primary tumors arising in the mesentery are relatively rare. On the other hand, the mesentery is a frequent avenue of spread for malignant neoplasms through the peritoneal cavity and between the peritoneal spaces and the retroperitoneum. Patients with mesenteric neoplasms usually present with nonspecific symptoms of abdominal pain, weight loss, a palpable abdominal mass, or diarrhea. CT plays a critical role in achieving an accurate diagnosis of these neoplasms to guide patient management."
Mesenteric Neoplasms: CT Appearances of Primary and Secondary Tumors and Differential Diagnosis
Sheth S, Horton KM, Garland MR, Fishman EK
RadioGraphics Mar 2003, Vol. 23, No. 2:457–473

 

"Desmoid tumors are rare, locally aggressive, nonencapsulated masses resulting from a benign proliferation of fibrous tissue. Abdominal desmoids can occur sporadically and develop anywhere in the abdomen, including the musculature of the abdominal wall, the retroperitoneum, and the pelvis. However, desmoids forming in the mesentery are especially common in patients with familial adenomatous polyposis (Gardner syndrome), occurring in 9%–18% of cases."
Mesenteric Neoplasms: CT Appearances of Primary and Secondary Tumors and Differential Diagnosis
Sheth S, Horton KM, Garland MR, Fishman EK
RadioGraphics Mar 2003, Vol. 23, No. 2:457–473

 

“Desmoid tumors (DTs), also known as aggressive fibromatosis, are fibroblastic neoplasms which are often locally aggressive but lack metastatic potential. They may occur sporadically or in association with familial adenomatous polyposis (FAP) syndrome. Among individuals with FAP, desmoids most frequently occur in intra-abdominal and abdominal wall locations with most arising from the peritoneum. These abdominal desmoids range in severity from indolent, asymptomatic lesions to highly invasive, sometimes fatal tumors. Although less common than abdominal desmoids and very rarely fatal, extra-abdominal desmoids are also a significant cause of morbidity in this population.”
"Extra-Abdominal Desmoid Tumors Associated with Familial Adenomatous Polyposis”
George T. Calvert, Michael J. Monument, Randall W. Burt, Kevin B. Jones, R. Lor Randall, 
Sarcoma, vol. 2012, Article ID 726537, 11 pages, 2012

 

RLQ Pain

RLQ Pain

 

Desmoid Tumor of the Mesentery

 

Desmoid Tumor of the Mesentery

 

Desmoid Tumor of the Mesentery

 

Desmoid Tumor of the Mesentery

 

Desmoid Tumor of the Mesentery

 

Desmoid Tumor of the Mesentery

 

Desmoid Tumor of the Mesentery

 

Desmoid Fibromatosis RLQ

Desmoid Fibromatosis RLQ

 

“The most common DTs are sporadic and extraabdominal ones (extremities, head-neck, and chest wall/ breast). The typical clinical presentation is a slow growing painless or minimally symptomatic soft tissue mass. Approximately 30% of patients have tumors related to familial adenomatous polyposis (FAP), and in these patients, intra-abdominal location is the most common type and can be also multifocal. Intra-abdominal DTs’ clinical presentation can be typically as a slow-growing mass but can have also acute presentation such as intestinal ischemia or obstruction.”
Multimodality imaging features of desmoid tumors: a head-to-toe spectrum
F. Rosa et al.
Insights into Imaging (2020) 11:103

 

Desmoid Tumors: Facts

  • 30% of familial adenomatous polyposis (FAP) patients have desmoid-type fibromatosis (also called Gardner syndrome); 7.5– 16% of patients with fibromatosis have FAP.
  • Mean age: 36–42 years.
  • Female predominance from puberty to age 40; younger and older patients have M:F = ~ 1:1.
  • Male predominance in FAP of 3:1.

 

“Intra-abdominal DTs’ incidence significantly differs between sporadic and FAP-related cases: only 5% of sporadic DTs are intra-abdominal ones, whereas 80% of cases of FAP-related DTs are intra-abdominal (especially mesenteric ones) . Abdominal DTs can be divided into pelvic or mesenteric lesions.”
Multimodality imaging features of desmoid tumors: a head-to-toe spectrum
F. Rosa et al.
Insights into Imaging (2020) 11:103

 

“Mesenteric DT is the most common primary tumor of the mesentery . The clinical presentations range from a painless palpable abdominal mass to bowel obstruction or perforation and chronic hydronephrosis due to ureteral infiltration. Mesentery is the most common DT location in the Gardner syndrome.”
Multimodality imaging features of desmoid tumors: a head-to-toe spectrum
F. Rosa et al.
Insights into Imaging (2020) 11:103

 

“DTs are usually visualized as a large mass (> 15 cm), isodense to the muscle. Uncommonly, it can entrap ureters or encase small bowel loops, leading to intestinal perforation or obstruction. Otherwise, in patients with FAP, lesions are smaller and multiple.”
Multimodality imaging features of desmoid tumors: a head-to-toe spectrum
F. Rosa et al.
Insights into Imaging (2020) 11:103

 

DTs are uncommon, locally aggressive tumors with a high risk of recurrence. There are several therapeutic options, including “wait-and-see policy,” surgery, radiotherapy, chemotherapy, and hormonal and molecular targeted drugs. A multidisciplinary approach for a “tailored therapy” is usually needed: in this clinical scenario, radiologists play a crucial role to make a correct diagnosis and to guide the proper management, depending on the location, imaging features, and clinical presentation of these kinds of masses.”
Multimodality imaging features of desmoid tumors: a head-to-toe spectrum
F. Rosa et al.
Insights into Imaging (2020) 11:103

 

Desmoid Fibromatosis

Desmoid Fibromatosis

 

Desmoid Tumor of the Mesentery

 

Desmoid Tumor of the Mesentery

 

Desmoid Tumor of the Mesentery

 

Desmoid Tumor of the Mesentery

 

Desmoid Tumor of the Mesentery

 

Desmoid Tumor of the Mesentery

 

Desmoid Tumor of the Mesentery

 

Desmoid Tumor of the Mesentery

 

Desmoid Tumor of the Mesentery

 

Desmoid Tumor of the Mesentery

 

Mesenteric Desmoid Tumor: Differential Diagnosis

  • Lymphoma
  • GIST tumor
  • Carcinoid tumor
  • Sclerosing mesenteritis
  • Adenocarcinoma including small bowel and appendix

 

RLQ Pain

RLQ Pain

 

Desmoid Tumor of the Mesentery

 

Desmoid Fibromatosis RLQ

Desmoid Fibromatosis RLQ

 

Desmoid Tumor of the Mesentery

 

Desmoid Tumor of the Mesentery

 

Desmoid Tumor of the Mesentery

 

Desmoid Tumor of the Mesentery

 

Desmoid Tumor of the Mesentery

 

Desmoid Tumor of the Mesentery

 

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