Multicentric desmoid tumors in psoas muscle

Clinical Cases 29.08.2007
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Section: Musculoskeletal system
Case Type: Clinical Cases
Patient: 22 years, female
Authors: Daniel Gulias, Rafaela Soler, Esther Rodríguez Department of Radiology
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AI Report

Clinical History

A 22-year-old woman presented a 2-month history of intermittent left upper abdominal pain. The patient was diagnosed of seronegative spondyloarthropathy two years previously and was treated with non-steroidal anti-inflammatory drugs. At admission, the patient was asymptomatic and both physical examination and blood chemistry were normal

Imaging Findings

An abdominal magnetic resonance (MR) examination was conducted on a 1.5 T unit (Gyroscan NT, Philips Medical System, Best, The Netherlands). A synergy body coil was used for all sequences. Abdominal turbo spin-echo T2-weighted sequences were obtained during breath-hold in the coronal and sagittal planes. Transverse T1-weighted gradient-echo in-phase, opposed-phase and contrast enhanced gradient echo T1-weighted images through psoas muscles were also performed. The coronal (Figure 1) turbo spin-echo T2-weighted image demonstrated two well defined masses involving the left psoas muscle, isointense to muscle and surrounded by a bright peripheral rim. On transverse T1-weighted gradient-echo in-phase and opposed-phase images, the masses were isointense to muscle and showed a peripheral rim of high signal intensity on in-phase image (Figure 2) and low signal intensity on opposed-phase image (Figure 3) which represents peritumoral fat. The transverse contrast enhanced gradient-echo T1-weighted image showed homogeneous pronounced enhancement of the masses (Figure 4). Surgical intervention through a left lumbar approach revealed two independent masses between the muscle cell bundles of the left psoas muscle. The post-operative course was uneventful. A follow-up contrast-enhanced abdominal CT scan one year later showed no signs of recurrence and the patient remains asymptomatic.

Discussion

Fibromatoses are soft-tissue tumors divided into superficial and deep lesions. According to the World Health Organization classification, the term desmoid-type fibromatosis includes extra-abdominal desmoid tumors [1]. Desmoid tumors are most common in the ages of 25 and 40 years [2], and females within the fertile age [3, 4]. A well-known association occurs in patients with a past history of abdominal or pelvic surgery. Other associations include trauma, pregnancy, estrogen therapy, and Gardner syndrome [5]. Extraabdominal desmoid tumors are typically solitary lesions originated from connective tissue in muscle, fascia, or aponeuroses, but multiple lesions can be seen [4]. In our case, desmoid tumors originated from connective tissue between the muscle cell bundles of the major left psoas muscle. Histologically, desmoid tumors consist of spindle cells of uniform appearance that are surrounded and separated from each other by collagen [3, 4]. Desmoid tumors evolve over time in three stages [5]. In the first stage, lesions are more cellular. 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 [5, 6]. These changes are reflected in the MR appearance of features of the lesions. In the first stage, desmoid tumors display nonspecific low signal intensity on T1-weighted images and predominantly high 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 [6]. After the administration of contrast material, desmoids tumors may show homogeneous, heterogeneous or no significant enhancement [3, 6]. In the final stage, as in our case, these tumors have low signal intensity on all sequences due to an increase in the fibrous composition [5, 6]. Also, our case showed a peripheral rim of fat surrounding the tumor. This finding is known as the split fat sign and it is seen more frequently in neoplasms of large nerves and benign peripheral nerve sheath tumors [7]. We believe that in our case, the split fat sign results from the origin of desmoid tumors between the psoas muscle cell bundles, which are surrounded by fat, and the fact that non infiltrating tumors arising in this site can maintain a rim of fat about them as they slowly enlarge. Margins of desmoid tumors vary widely [2] and the most aggressive lesions usually occur in patients younger than 20 years [8]. Differential diagnoses of primary psoas masses muscle include soft tissue sarcomas and neurogenic tumors arising from the nerves of the lumbar plexus that transverse the psoas muscle [9]. Soft tissue sarcomas usually have areas of high signal intensity on T2-weighted images [10]. On T2-weighted images, neurogenic tumors typically show high signal intensity with a variable degree of heterogeneity [7]. In our case, the combination of the MR imaging findings, the age and sex of the patient and the location within the psoas muscle, make desmoid tumors a strong primary diagnostic consideration. Definitive diagnosis should be established with histopathologic analysis.

Differential Diagnosis List

Histopathologic examination was consistent with desmoid tumors.

Final Diagnosis

Histopathologic examination was consistent with desmoid tumors.

Liscense

Figures

Coronal turbo spin-echo T2-weighted images obtained through abdomen shows two well-defined masses within the left psoas muscle (asterisks) with signal intensity similar to normal muscle and a peripheral rim of high signal intensity (arrows).

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Coronal turbo spin-echo T2-weighted images obtained through abdomen shows two well-defined masses within the left psoas muscle (asterisks) with signal intensity similar to normal muscle and a peripheral rim of high signal intensity (arrows).

Transverse T1-weighted gradient-echo in-phase image obtained through one of two masses involving the left psoas muscle demonstrates that the mass has similar signal intensity to normal muscle (asterisk) and the peripheral rim is hyperintense (arrow).

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Transverse T1-weighted gradient-echo in-phase image obtained through one of two masses involving the left psoas muscle demonstrates that the mass has similar signal intensity to normal muscle (asterisk) and the peripheral rim is hyperintense (arrow).

On transverse T1-weighted gradient-echo opposed-phase image, the mass has similar signal intensity to normal muscle (asterisk) and the peripheral rim is hypointense.

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On transverse T1-weighted gradient-echo opposed-phase image, the mass has similar signal intensity to normal muscle (asterisk) and the peripheral rim is hypointense.

Transverse contrast enhanced gradient echo T1-weighted image obtained through one of two masses involving the left psoas muscle shows homogeneous pronounced enhancement (arrows).

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Transverse contrast enhanced gradient echo T1-weighted image obtained through one of two masses involving the left psoas muscle shows homogeneous pronounced enhancement (arrows).