DOI: 10.1148/radiol.2361031038
(Radiology 2005;236:81-84.)
© RSNA, 2005
Case 84: Desmoid Tumor of the Abdominal Wall1
Harvey E. L. Teo, MBBS, FRCR,
Wilfred C. G. Peh, MD, FRCP, FRCR and
Tony W. H. Shek, MBBS, FRCPA
1 From the Department of Diagnostic Imaging, Kandang Kerbau Women's and Children's Hospital, Singapore (H.E.L.T., W.C.G.P.); and Department of Pathology, University of Hong Kong, Hong Kong SAR, China (T.W.H.S.). Received July 1, 2003; revision requested September 10; revision received September 22; accepted October 14.
Address correspondence to W.C.G.P., Programme Office, Singapore Health Services, 7 Hospital Drive, #02-09, Singapore 169611 (e-mail: wilfred.peh{at}singhealth.com.sg).
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HISTORY
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A 29-year-old woman presented with a 4-month history of a painless mass in the left lower abdomen. The patient stated that the mass was gradually increasing in size. A review of systems was unremarkable except for a history of left oophorectomy 4 years earlier. At physical examination, the mass was firm, nontender, and fixed to the left lower abdominal wall. Computed tomographic (CT) scans and magnetic resonance (MR) images were obtained.
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IMAGING FINDINGS
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The unenhanced transverse CT image obtained through the pelvis shows a focal but nondemarcated mass involving the left inferior rectus abdominis muscle and indenting the left anterolateral aspect of the bladder (Fig 1). The transverse T1-weighted MR image shows the mass to be well delineated with respect to the adjacent adipose tissue. The margin of the mass and the adjacent skeletal muscle is not well defined. The mass is predominantly isointense to muscle, with small areas of low signal intensity within it (Fig 2a). The transverse fat-saturated contrast material–enhanced MR image shows mild enhancement in the lateral and posterior aspects of the mass. The other parts of the mass show no enhancement (Fig 2b). The transverse fast spin-echo T2-weighted MR image also shows the mass to be poorly defined and isointense to muscle, with small areas of low signal intensity within it (Fig 2c).
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Figure 1. Unenhanced transverse CT image obtained through the pelvis shows a solid anterior abdominal wall mass (arrow) in the left rectus muscle. The margins of the mass are not well delineated.
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Figure 2a. (a) Transverse spin-echo T1-weighted MR image (repetition time msec/echo time msec, 620/8) obtained through the pelvis shows the focal soft-tissue mass (arrow) in the left rectus muscle. Several small areas of decreased signal intensity (arrowheads) are noted within the mass. (b) Fat-saturated gadopentetate dimeglumine–enhanced transverse spin-echo T1-weighted MR image (680/20) obtained through the pelvis shows the mass (arrow) within the left rectus sheath, with only mild enhancement in the lateral and posterior aspects of the mass. The other parts of the mass do not show enhancement. (c) Fat-saturated transverse fast spin-echo T2-weighted MR image (3920/80) obtained through the pelvis shows the mass to be isointense to muscle, with several small areas of low signal intensity (arrowheads) within the mass, similar to the appearance on T1-weighted images.
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Figure 2b. (a) Transverse spin-echo T1-weighted MR image (repetition time msec/echo time msec, 620/8) obtained through the pelvis shows the focal soft-tissue mass (arrow) in the left rectus muscle. Several small areas of decreased signal intensity (arrowheads) are noted within the mass. (b) Fat-saturated gadopentetate dimeglumine–enhanced transverse spin-echo T1-weighted MR image (680/20) obtained through the pelvis shows the mass (arrow) within the left rectus sheath, with only mild enhancement in the lateral and posterior aspects of the mass. The other parts of the mass do not show enhancement. (c) Fat-saturated transverse fast spin-echo T2-weighted MR image (3920/80) obtained through the pelvis shows the mass to be isointense to muscle, with several small areas of low signal intensity (arrowheads) within the mass, similar to the appearance on T1-weighted images.
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Figure 2c. (a) Transverse spin-echo T1-weighted MR image (repetition time msec/echo time msec, 620/8) obtained through the pelvis shows the focal soft-tissue mass (arrow) in the left rectus muscle. Several small areas of decreased signal intensity (arrowheads) are noted within the mass. (b) Fat-saturated gadopentetate dimeglumine–enhanced transverse spin-echo T1-weighted MR image (680/20) obtained through the pelvis shows the mass (arrow) within the left rectus sheath, with only mild enhancement in the lateral and posterior aspects of the mass. The other parts of the mass do not show enhancement. (c) Fat-saturated transverse fast spin-echo T2-weighted MR image (3920/80) obtained through the pelvis shows the mass to be isointense to muscle, with several small areas of low signal intensity (arrowheads) within the mass, similar to the appearance on T1-weighted images.
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DISCUSSION
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The occurrence of a lesion in the rectus abdominis muscle and the adjacent musculoaponeurosis should alert the radiologist to the possibility of a desmoid tumor. The diagnosis of a desmoid tumor should be strongly considered in this case because of the patient's age, sex, history of previous surgery, and imaging findings (1). The occurrence of desmoid tumors peaks between the ages of 25 and 40 years (2). Cases have been reported in pediatric patients, but these are rare (1). In the fertile age group, desmoid tumors are 1.8 times more common in female subjects than in male subjects (3). A well-known association occurs in patients with a past history of abdominal or pelvic surgery. Other associations include trauma, pregnancy, estrogen therapy, familial adenomatous polyposis, and Gardner syndrome (4). In patients with familial adenomatous polyposis, 9%–18% of patients develop desmoid tumors. Most of these tumors are situated within the mesentery of the small bowel. Death occurs as a result of progressive cachexia and complications of small bowel obstruction (5). Desmoid tumors are rare, with approximately 3.7 new cases occurring per 1 million persons per year (3). Desmoid tumors are also known as aggressive fibromatoses (4). The fibromatoses are a diverse group of soft-tissue tumors that occur at different ages and anatomic locations and have common histopathologic features. According to the World Health Organization (WHO) classification of tumors of soft tissue and bone, the term desmoid-type fibromatosis includes extraabdominal desmoid tumors and aggressive fibromatosis.
Desmoid tumors do not metastasize, but they invade locally and have a tendency to recur (4). They are classified as intra- or extraabdominal types, but the histologic findings in these lesions are identical. Those occurring in the anterior abdominal wall arise from the musculoaponeurosis and occasionally cross the midline (6).
Histologically, desmoid tumors consist of spindle cells of uniform appearance that are surrounded and separated from each other by collagen (4). They have been shown to evolve over time (4). Vandevenne et al (4) followed multicentric desmoid tumors in two patients over a 6-year period and described three stages of evolution of the lesions during this time. However, no time frame associated with each stage of evolution was described. In the first stage, lesions are more cellular, and they have larger extracellular spaces and fewer areas of hyalinized collagen. In the second stage, there is an increasing amount of collagen deposition in the central and peripheral areas of the tumor. In the third stage, there is an increase in the fibrous composition, with a decrease in cellularity and volume in the extracellular spaces and water content (4). These changes are reflected in the MR appearance of features of the lesions. In the first stage, desmoid tumors are low in signal intensity on T1-weighted images and are predominantly high in signal intensity on T2-weighted images. In the next stage, there is increasing heterogeneity on the T2-weighted images due to the increasing collagen deposition within the tumor. These areas of low signal intensity may have a bandlike configuration (1). The remaining areas of high signal intensity on the T2-weighted images are the same areas that show enhancement on the T1-weighted images after the administration of contrast material. In the final stage, desmoid tumors have low signal intensity on both T1- and T2-weighted images due to an increase in the fibrous composition within the tumor (4). This is the stage seen in our patient.
The MR imaging features of desmoid tumors show wide variability depending on the stage at which they are imaged. Characteristic but nonspecific MR imaging features of desmoid tumors include a soft-tissue mass with heterogeneous signal intensity approximating that of fat on T2-weighted images and that of skeletal muscle on T1-weighted images (2). Malignant tumors usually have areas of signal intensity that are greater than that of fat on T2-weighted images (7). Tumor margins vary greatly in desmoid tumors (2). Encasement of the neurovascular bundles may occur. Desmoid tumors occurring in the extremities may occasionally erode adjacent bone. Most lesions demonstrate moderate to marked enhancement after the administration of gadopentetate dimeglumine, but approximately 10% of lesions show no substantial enhancement (2).
Radiographs are not helpful in imaging abdominal desmoid tumors. Ultrasonography is useful in the initial evaluation of patients with soft-tissue masses. Desmoid tumors appear as well-defined lesions with varying echogenicity (8). Radiotracer accumulation has been reported within lesions seen on blood pool and delayed static images obtained with bone scintigraphy (9). Similar uptake has also been reported in recurrent lesions.
CT and MR imaging are useful techniques in the evaluation of the size, site, extent, and relationship of the lesion to adjacent structures. The CT appearance of desmoid tumors varies, depending on their composition. Lesions may appear hypo-, iso-, or hyperintense when compared with the attenuation of muscles. The attenuation of the lesions may be homogeneous or heterogeneous. After administration of contrast material, the degree of enhancement of the lesions is also variable (10). Attempts to correlate the CT appearance of tumors with the underlying histologic findings, collagen content, or microscopic or angiographic vascularity have been unsuccessful (2).
The differential diagnoses for rectus abdominis lesions include acute hematoma and other soft-tissue tumors, such as fibrosarcoma, lymphoma, rhabdomyosarcoma, neurofibroma, benign fibrous tumor, and primitive neuroectodermal tumor (6). A definitive diagnosis based solely on imaging features is not possible, as the MR imaging features of stage I and II desmoid tumors and the previously mentioned lesions are nonspecific. In this case of a stage III lesion, the combination of features, such as the history of previous surgery, the age and sex of this patient, the location of this mass within the anterior abdominal wall, and the MR imaging features, make desmoid tumor a strong primary diagnostic consideration (1). Definitive diagnosis must still be established with histopathologic analysis. In our patient, the mass was excised, and histopathologic analysis showed the lesion extending irregularly into adjacent skeletal muscle (Fig 3a). Fascicles of fibroblastic spindle cells with abundant intercellular collagen, a finding that is typical of fibromatosis, were noted (Fig 3b).
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Figure 3a. (a) Photomicrograph of the excised rectus desmoid tumor shows that the lesion (arrow) extends irregularly into the adjacent muscle. (Hematoxylin-eosin stain; original magnification, x20.) (b) Photomicrograph of the excised rectus desmoid tumor shows fascicles of fibroblastic spindle cells with abundant intercellular collagen, which is typical of fibromatosis. (Hematoxylin-eosin stain; original magnification, x200.)
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Figure 3b. (a) Photomicrograph of the excised rectus desmoid tumor shows that the lesion (arrow) extends irregularly into the adjacent muscle. (Hematoxylin-eosin stain; original magnification, x20.) (b) Photomicrograph of the excised rectus desmoid tumor shows fascicles of fibroblastic spindle cells with abundant intercellular collagen, which is typical of fibromatosis. (Hematoxylin-eosin stain; original magnification, x200.)
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The definitive treatment of desmoid tumors is wide local excision (2). The recurrence rate of desmoid tumors is 20%–30% depending on the location, extent, and completeness of the initial resection (6). The rate of recurrence is reduced if there is sufficient normal tissue surrounding the resected tumor (6). Radiation therapy is used in patients with inoperable tumors, local recurrences, or incompletely excised lesions. Chemotherapy and endocrine therapy have also been used successfully to treat desmoid tumors (11,12). Fatalities may occur secondary to direct invasion of the chest wall, neck, or vital organs (2). The mortality rate may be as high as 10% due to the local behavior of these tumors (13).
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FOOTNOTES
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Part one of this case appeared 4 months previously and may contain larger images.
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References
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- Kransdorf MJ. Fibrous tumors. In: Kransdorf MJ, Murphey MD, eds. Imaging of soft tissue tumors. Philadelphia, Pa: Saunders, 1997; 143–186.
- Quinn SF, Erickson SJ, Dee PM, et al. MR imaging in fibromatosis: results in 26 patients with pathologic correlation. AJR Am J Roentgenol 1991; 156:539–542.[Abstract/Free Full Text]
- Reitamo JJ, Scheinin TM, Hayry P. The desmoid syndrome: new aspects in the cause, pathogenesis and treatment of the desmoid tumor. Am J Surg 1986; 151:230–237.[CrossRef][Medline]
- Vandevenne JE, De Schepper AM, De Beuckeleer L, et al. New concepts in understanding evolution of desmoid tumors: MR imaging of 30 lesions. Eur Radiol 1997; 7:1013–1019.[CrossRef][Medline]
- Jones IT, Jagelman DG, Fazio V, et al. Desmoid tumors in familial polyposis coli. Ann Surg 1986; 204:94–97.[Medline]
- Casillas J, Sais GJ, Greve JL, et al. Imaging of intra- and extraabdominal desmoid tumors. RadioGraphics 1991; 11:959–968.[Abstract]
- Hartman TE, Berquist TH, Fetsch JF. MR imaging of extraabdominal desmoids: differentiation from other neoplasms. AJR Am J Roentgenol 1992; 158:581–585.[Abstract/Free Full Text]
- Mantello MT, Haller JO, Marquis JR. Sonography of abdominal desmoid tumors in adolescents. J Ultrasound Med 1989; 8:467–470.[Medline]
- Terui S, Terauchi T, Abe H, et al. Role of technetium-99m pertechnetate scintigraphy in the management of extra-abdominal fibromatosis. Skeletal Radiol 1995; 24:331–336.[Medline]
- Brooks AP, Reznek RH, Nugent K, et al. CT appearances of desmoid tumours in familial adenomatous polyposis: further observations. Clin Radiol 1994; 49:601–607.[CrossRef][Medline]
- Schnitzler M, Cohen Z, Blackstein M, et al. Chemotherapy for desmoid tumors in association with familial adenomatous polyposis. Dis Colon Rectum 1997; 40:798–801.[CrossRef][Medline]
- Bauernhofer T, Stoger H, Schmid M, et al. Sequential treatment of recurrent mesenteric desmoid tumor. Cancer 1996; 77:1061–1065.[CrossRef][Medline]
- Nursal TZ, Abbasoglu O. Sporadic hereditary pancreatic desmoid tumor: a new entity? J Clin Gastroenterol 2003; 37:186–188.[CrossRef][Medline]
Congratulations to the 78 individuals and two resident groups who submitted the most likely diagnosis (desmoid tumor of the abdominal wall) for Diagnosis Please, Case 84. Credit was given only if "desmoid" or "aggressive fibromatosis" was included in the submitted diagnosis. The names and locations of the individuals and resident groups, as submitted, are as follows:
- Jose Luis Abbona, MD, Mendoza, Argentina
- Pablo Jose Abbona, MD, Mendoza, Argentina
- Hisashi Abe, Osaka, Japan
- Gholamali Afshang, MD, Tinley Park, Ill
- Alexandra Araújo, Lisbon, Portugal
- Robert Berkenblit, MD, Briarcliff, NY
- Eliana Bonfante, MD, Houston, Tex
- Ghislain Brousseau, MD, Charlesbourg, Quebec, Canada
- Michael P. Buetow, MD, Okemos, Mich
- Marcio Bustamante, Rio de Janeiro, Brazil
- Paloma Cebrian Villar, Salamanca, Spain
- Marcos Nogueira Chagas, MD, Brasilia, Brazil
- Haris Chrysikopoulos, MD, Kerkyra, Greece
- Pablo Cikman, MD, Cordoba, Argentina
- Neal R. Conti, MD, Seattle, Wash
- Marco Cura, MD, San Antonio, Tex
- J. F. K. de Villiers, Gisborne, New Zealand
- Dr Johannes Demharter, Augsburg, Germany
- Thaworn Dendumrongsup, MD, Songkla, Thailand
- T. Dhurairaj, Pennsauken, NJ
- Morgan G. Dunne, San Antonio, Tex
- Dra Marta Fité i Grau, Barcelona, Spain
- Francis Flaherty, MD, Ridgefield, Conn
- Jordi Catala Forteza, Barcelona, Spain
- Marcelo B. G. Funari, São Paulo, Brazil
- William Gawman, Waterloo, Ontario, Canada
- Paulo Gil Agostinho, Coimbra, Portugal
- Alvaro Gomez Naar, Salta, Argentina
- Gowthaman Gunabushanam, New Delhi, India
- Dr Jean Pierre Gurret, Annecy, France
- Ferris M. Hall, MD, Boston, Mass
- Dr Raúl Hernández Muñiz, Madrid, Spain
- Ronald Homer, Los Angeles, Calif
- Suzanne Homer, MD, Los Angeles, Calif
- Alfred L. Horowitz, MD, Asheville, NC
- Tim Humes, Moline, Ill
- Eric Kakinami, São Paulo, Brazil
- Hung-Wen Kao, MD, Taipei, Taiwan, Republic of China
- Nurettin Katranci, MD, Antalya, Turkey
- Takuji Kiryu, MD, Gifu, Japan
- David S. Klein, MD, Fairfield, Conn
- Mitchell A. Klein, MD, Milwaukee, Wis
- Takeshi Kubo, Boston, Mass
- Yu-Ting Kuo, MD, Kaohsiung, Taiwan
- Mark Kutler, MD, Dallas, Tex
- Mario Laguna, West Allis, Wis
- John T. Lim, MD, Newport Coast, Calif
- Patricia Lowry, MD, Richmond, Va
- Walter Mak, MD, Peoria, Ill
- Bernardo Martins, Brasilia, Brazil
- Carla Giannine P. Medina, MD, Brasilia, Brazil
- Koen Mermuys, MD, Heverlee, Belgium
- Ari Mintz, MD, Lake Forest, Ill
- Dr Moataz Montasser, Alexandria, Egypt
- Tammam Nehme, East Wenatchee, Wash
- Chris Ng, MD, Nashville, Tenn
- Mizuki Nishino, MD, Boston, Mass
- Ahmet Oguz, Istanbul, Turkey
- Michael T. O'Loughlin, MD, West Hartford, Conn
- David M. Panicek, MD, New York, NY
- Victor A. Pérez-Candela, MD, Las Palmas de Gran Canaria, Spain
- Ryan Rebello, MD, Dundas, Ontario, Canada
- Mario G. Santamarina, MD, Mendoza, Argentina
- Steven M. Schultz, MD, Fort Worth, Tex
- Stanford R. Schwimer, MD, Beverly Hills, Calif
- Mustafa Secil, MD, Izmir, Turkey
- Matt Shapiro, MD, Charlottesville, Va
- Kenneth Sheah, Singapore
- Gilberto Szarf, São Paulo, Brazil
- Norio Takahashi, MD, Fukui, Japan
- Luis Tata, MD, Amadora, Portugal
- Dr Ravikumar B. Thapar, Maharashtra, India
- Eugene Tong, MD, Austin, Tex
- William C. Torreggiani, MB, Dublin, Ireland
- Meriç Tüzün, Ankara, Turkey
- F. M. Vanhoenacker, MD, PhD, Edegem, Belgium
- Piet K. Vanhoenacker, MD, Aalst, Belgium
- Joan C. (Kai) Vilanova, MD, Girona, Spain
- Oregon Health and Science University Radiology Residents, Portland, Ore
- Hospital of the University of Pennsylvania Radiology Residents, Philadelphia, Pa
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K. I. Stanek
Intra-abdominal Desmoid Tumor
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A. V. Proto
Diagnosis Please Certificate of Recognition Awarded to Taro Shimono, MD, PhD
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TOP
HISTORY
IMAGING FINDINGS
