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CASE SERIES
Sonographic Appearances of Desmoid Tumors Li Lou, PhD, Jianbo Teng, MD, Hengtao Qi, PhD, Yongguang Ban, PhD
The medical records of 16 patients (9 female and 7 male; age range, 5–66 years) with 24 lesions that had a histologic diagnosis of desmoid tumors were reviewed at our institution. Six cases were extra-abdominal, 4 intra-abdominal, and 6 in the abdominal wall. Lesions ranged from 1.5 to 18.0 cm in diameter (mean, 6.8 cm). All lesions were solid masses, which appeared hypoechoic, isoechoic, or hyperechoic with homogeneous or heterogeneous echogenicity. Posterior acoustic enhancement was seen in 18 lesions. No lesions showed central necrosis. Most lesions had substantial flow and high resistive index values (>0.70). Sonography can show a desmoid tumor’s site, size, contour, margin, echogenicity, homogeneity, vascularity, and resistive index value in detail. Key words—aggressive fibromatosis; color Doppler sonography; desmoid tumor; musculoskeletal ultrasound; sonography; spectral Doppler sonography
Received August 19, 2013, from the Department of Ultrasound, Shandong Medical Imaging Research Institute, Shandong University, Jinan, China. Revision requested September 27, 2013. Revised manuscript accepted for publication December 3, 2013. This work was supported by grant 2013WS0183 from the Medical Science and Technology Development Program of Shandong Province. Address correspondence to Jianbo Teng, MD, Department of Ultrasound, Shandong Medical Imaging Research Institute, Shandong University, 324 Jing 5 Rd, 250021 Jinan, China. E-mail:
[email protected] Abbreviations
MRI, magnetic resonance imaging; RI, resistive index doi:10.7863/ultra.33.8.1519
D
esmoid tumors are rare mesenchymal tumors characterized histologically by proliferation of fibroblasts and myofibroblasts that arise from the fascia or aponeurosis of muscles. They constitute a diverse group of soft tissue tumors that occur at different ages and anatomic locations and also known as aggressive fibromatoses.1 They are classified according to their intraabdominal, abdominal (wall), and extra-abdominal locations,2 but the pathologic findings in these lesions are identical. Computed tomography and magnetic resonance imaging (MRI) are useful modalities for evaluating the size and extension of desmoid tumors and the involvement of adjacent structures.3 Given its easy accessibility, high resolution, quick scan time, and low cost, sonography has gained more popularity as a helpful technique for diagnosing soft tissue tumors.4 In diagnosing a desmoid tumor, sonography can show the tumor’s site, size, margin, contour echogenicity, and homogeneity in detail.5 However, little emphasis has been placed on the color Doppler and pulsed Doppler features of desmoid tumors. In this series, we evaluated the grayscale and duplex Doppler sonographic appearances of desmoid tumors to improve the diagnostic efficacy of sonography for this disease.
©2014 by the American Institute of Ultrasound in Medicine | J Ultrasound Med 2014; 33:1519–1525 | 0278-4297 | www.aium.org
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Materials and Methods The medical records of 16 patients with surgically proven cases of desmoid tumors from January 2009 to January 2013 were reviewed at our institution. Medical records, sonograms, and pathologic reports were retrospectively reviewed for each patient. Institutional Review Board approval was obtained. A waiver of informed consent was granted for this retrospective study. Sonography was performed preoperatively with various scanners (LOGIQ 9 and Vivid 7; GE Healthcare, Milwaukee, WI; and ProSound α10; Aloka Co, Ltd, Tokyo, Japan) using 3.5–10-MHz convex and linear array transducers, depending on the location and size of the tumor. All patients underwent color and pulsed Doppler sonography to assess the tumor’s vascularity. Color Doppler sonography was performed with optimized color Doppler parameters. Two sonologists who specialized in musculoskeletal sonography with knowledge of the diagnosis evaluated the imaging findings by consensus. The sonographic appearances were evaluated for features including tumor site, size, shape, contour, margin, echogenicity, and homogeneity. The vascularity of the tumor was subjectively graded as no flow (no flow in the tumor), minimal flow (sparse flow in the tumor), or substantial flow (abundant flow in the tumor) on the basis of color Doppler images. If adequate and persistent intratumoral vascularization was detected, then pulsed Doppler sonography was performed to obtain spectral waveforms, and the resistive index (RI) values were calculated for the waveforms.
Results Sixteen patients (9 female and 7 male; age range, 5–66 years) with 24 histologically confirmed desmoid tumors had preoperative sonograms for analysis. Fifteen were new patients with primary tumors, and 1 was a recurrent patient.
The most common finding at presentation was a mass in all 16 patients. Table 1 summarizes the patient demographic data and the anatomic locations of the 24 tumors. Of the 16 cases, 6 were extra-abdominal, 4 intra-abdominal, and 6 in the abdominal wall. Lesions ranged from 1.5 to 18.0 cm in diameter (mean, 6.8 cm). All patients underwent surgical resection, and the surgical explorations were consistent with the sonographic findings. The sonographic appearances of the tumors are shown in Table 2. All lesions were solid masses with variable echogenicity. Posterior acoustic enhancement was seen in 18 of 24 lesions (75.0%). No lesions showed central necroses or calcifications. Fourteen lesions (58.3%) had substantial flow; 8 (33.3%) had minimal flow; and 2 (8.3%) that were smaller than 2.0 cm had no flow. Spectral Doppler imaging yielded high RI values (>0.70; range, 0.73–1.00) in all lesions showing intratumoral flow, except 1 lesion, whose RI value was 0.54. Six patients had abdominal wall lesions. All had single lesions, which measured 4.0 to 15.0 cm in diameter. Of the 2 lesions located in the rectus muscle, 1 remained at the site of origin, and the other crossed the midline of the abdomen and involved both rectus muscles. Three patients with histories of abdominal surgery had lesions in proximity to the areas of the previous incisions. The lesions were oval with smooth contours (Figures 1 and 2); both ends of the lesions were contiguous with the muscle bundle from which the tumors arose, and a fibrotic bandlike configuration (Figure 2) was observed inside 4 lesions. All lesions had substantial flow and high RI values (>0.70), except 1 lesion, which had minimal flow, with an RI of 0.54. The extra-abdominal group included patients with thigh, popliteal fossa, and foot involvement. Lesions appeared as masses with variable echogenicity and smooth, sharply defined margins (Figure 3). The lateral borders of 4 lesions appeared poorly defined. In this group, 2 patients had multiple lesions. One had surgical resection of a Baker
Table 1. Patient Demographics and Anatomic Locations of the Desmoid Tumors Female
Male
Age Range, y
Abdominal wall
5
1
24–43
Extra abdominal
3
3
5–46
Intra abdominal
1
3
39–66
Group
1520
Site Rectus muscle Internal oblique muscle External oblique muscle Thigh Popliteal fossa Foot Mesentery Retroperitoneum Pelvis
Patients
Lesions
3 2 1 2 1 3 2 1 1
3 2 1 3 2 3 2 1 7
Size Range, cm 4.0–15.0
2.2–9.5
1.5–18.0
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cyst 6 years previously, and 2 lesions were found close to the incision. The other was a 5-year-old girl who had 2 adjacent thigh lesions at a distance of 1.2 cm, which measured 4.4 and 2.4 cm in diameter, respectively. The larger one presented as a lobulated heterogeneous mass with poorly defined lateral borders, which had substantial flow. The smaller one appeared as an oval well-defined homogeneous mass with a smooth contour, which had minimal flow. Both had high RI values (0.78 and 0.76, respectively). The intra-abdominal group included mesentery, retroperitoneal, and pelvic cases. The lesion situated within the retroperitoneum was a recurrent tumor. The pelvic case had multiple lesions (7 lesions altogether), which were located in the left illiac fossa, inguinal region, and left upper medial thigh. The largest lesion was in the illiac fossa and measured 9.4 cm in diameter, with substantial flow and a high RI (0.73). The smallest one was in the upper medial thigh and measured 1.5 cm in diameter, with no flow. All lesions appeared as oval hypoechoic or isoechoic fairly homogeneous masses, except the largest lesion in the illiac Table 2. Sonographic Appearances of the Desmoid Tumors
Sonographic Feature
Lesions Abdominal ExtraIntraWall abdominal abdominal (n = 6) (n = 8) (n = 10) Total (%)
Shape Oval Infiltrative Contour Smooth Lobulated Irregular Margin Well defined Poorly defined Echogenicity Hypoechoic Isoechoic Hyperechoic Homogeneity Homogeneous Heterogeneous Acoustic enhancement Present Absent Vascularity Substantial flow Minimal flow No flow RI >0.70 0.70; range, 0.73–1.00) in all lesions showing intratumoral flow (Figure 2C), except 1 lesion, whose RI value was 0.54. We observed that there were no particular differences between the lesion with a low RI and the others, but only 1 lesion with a low RI was included in this series, making a meaningful analysis of this subgroup difficult. To our knowledge, this observation of high RI values in desmoid tumors has not been reported previously. Regarding the RI, Klijanienko20 stated that it has a poor predictive value for malignancy, with a substantial overlap between benign and malignant soft tissue tumors. A previous study also concluded that the RI cannot be used to distinguish benign from malignant musculoskeletal soft tissue masses.21 A review of literature revealed no information on the RI values of desmoid tumors. However, we hope that this finding will be helpful for diagnosis, although owing to the limitation of the small number cases, further study is needed to determine whether high RI values should raise a high level of suspicion for desmoid tumors. The main differential diagnosis of desmoid tumors is abdominal wall endometriosis and malignant soft tissue sar-
comas. The typical clinical presentation of abdominal wall endometriosis is a palpable nodule near a cesarean delivery scar accompanied by cyclic pain and swelling during menses, which appears as a discrete hypoechoic and heterogeneous subcutaneous nodule with speculated margins infiltrating the muscularis fascia, circumscribed by a hyperechoic ring caused by a perilesional inflammatory reaction. Small cystic areas may be detected in lesions larger than 3 cm.22 Most malignant soft tissue sarcomas grow as intramuscular masses with an irregular shape, which have complicated components and therefore manifest as lesions with heterogeneous echogenicity. Sarcomas usually infiltrate local structures, including bone structures, and show poorly defined margins. As they enlarge further, they may outstrip their blood supply, resulting in central necrosis.23 In conclusion, desmoid tumors frequently appeared as an oval well or poorly defined solid soft tissue mass with variable echogenicity. Posterior acoustic enhancement was seen in most lesions, and no lesions showed central necrosis or calcifications. Most lesions had substantial flow and high RI values (>0.70). Awareness of the sonographic features, in combination with a history of surgery or trauma, the age and sex of the patient, and the location of the mass, should make a desmoid tumor a primary diagnostic consideration. However, a definitive diagnosis based mainly on imaging features is not possible, and the final diagnosis is obviously established by histopathologic evaluation.
Figure 3. Images from a 35-year-old woman with a desmoid tumor of the right foot. Sonograms show a solid hypoechoic homogeneous mass (A) with substantial flow (B). A
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