Figure 1
Plain X-ray film. Bilateral hips.
Bone tumour in the centromedular intertrochanteric region of the left femur.
Liposclerosing myxofibrous tumour
SectionMusculoskeletal system
Case TypeClinical Cases
AuthorsConstanza Martínez Stocker, Iván García Duitama, Anna Agustí Claramunt, Albert Solano López, Irene Espallargas Giménez.
Connected authors
68 years, male
Clinical History
A 68-year-old male patient with known hypertension, dyslipemia, auricular fibrillation and peripheral vasculopathy, presented with left sciatic pain and non-symptomatic hips.
Imaging Findings
Radiographs showed a bone tumour, in the centromedular intertrochanteric region of the left femur (Figs 1 and 2). It has well-defined limits, doubtful chondroid matrix and absence of aggressive signs (Fig 3). CT confirmed the presence of a geographic hypodense lesion with sclerotic borders and dystrophic internal mineralised matrix (Fig 4). On MRI (Figs 5, 6 7), the non-calcified component was hypointense to muscle on T1 and very hyperintense on T2-FATSAT. Note is made on a small fatty nodule in the non-calcified tumoural matrix.
Discussion
Liposclerosing myxofibrous tumour (LSMFT) is a rare fibro-osseous benign tumour [2]. It is formed by the combination of several elements with variable proportions: lipoma, fibroxanthoma, myxoma, myxofibroma, fibrous tissue, cyst formation, fat necrosis, ischaemic bone tissue, and rarely cartilaginous tissue [1]. LSMFT affects men and women equally in the fourth decade of life [3], without ethnical predilection [2]. Histological and genetic analysis suggested that it may represent a variant or evolution of fibrous dysplasia or intraosseous lipoma, probably because of stress or fatigue suffered by the initial lesion [2].
The frequent presentation is an incidental finding in asymptomatic patients. In cases of being symptomatic, pain and pathologic fractures are the most common presentations[2].
Bone biopsy may be hard to interpret and the results may be confounding because of the heterogeneity of the tumour matrix. Nowadays one is discouraged unless with imaging demonstration of aggressive features [2].
The radiologic appearance and the skeletal distribution of LSMFT are typical and establish the diagnosis. It has an important predilection for the femur (85%) and for the intertrochanteric region (91%) [1, 2]. CT and MRI show a well-defined geographic lesion, often with a thick and extensive sclerotic margin, reflecting its slow growing pattern, sometimes with globular and irregular mineralised matrix. After venous contrast administration, the myxoid matrix may show some enhancement.
MR and/or CT are essential to differentiate from intraosseous lipoma because they offer the possibility of documenting intralesional fat, which is minimal if any in LSMFT. Fatty areas demonstrate low attenuation values (<-60HU) on CT, high SI on T1/T2WI and low SI on FS/STIR on MRI. The differentiation from intraosseous lipoma with involutional change may not be possible through imaging studies but the intertrochanteric location favours the diagnosis of LSMFT [2].
Fibrous dysplasia (FD) reveals less sclerosis, usually with the characteristic “ground glass” pattern and endosteal scalloping, less frequent in LSMFT [2].
In bone scintigraphy both FD and LSMFT may show low tracer uptake [3].
Malignant transformation to osteosarcoma or malignant fibrous histiocytoma occurs approximately in 10-16% [1]. It is suggested in imaging when aggressive signs are developed, such as cortical disruption, rapid growing and soft-tissue extension. Treatment is considered in symptomatic patients, which includes curettage, bone-grafting and fixation. Cases with malignant transformations or pathological fracture, usually require arthroplasty [3]. The non-surgical treatment includes analgesics and follow-up with plain X-ray every six months and MR or CT every year during the first two years [2].
Radiology can lead to the diagnosis.
Histology can cause confusion.
Written informed patient consent for publication has been obtained.
The frequent presentation is an incidental finding in asymptomatic patients. In cases of being symptomatic, pain and pathologic fractures are the most common presentations[2].
Bone biopsy may be hard to interpret and the results may be confounding because of the heterogeneity of the tumour matrix. Nowadays one is discouraged unless with imaging demonstration of aggressive features [2].
The radiologic appearance and the skeletal distribution of LSMFT are typical and establish the diagnosis. It has an important predilection for the femur (85%) and for the intertrochanteric region (91%) [1, 2]. CT and MRI show a well-defined geographic lesion, often with a thick and extensive sclerotic margin, reflecting its slow growing pattern, sometimes with globular and irregular mineralised matrix. After venous contrast administration, the myxoid matrix may show some enhancement.
MR and/or CT are essential to differentiate from intraosseous lipoma because they offer the possibility of documenting intralesional fat, which is minimal if any in LSMFT. Fatty areas demonstrate low attenuation values (<-60HU) on CT, high SI on T1/T2WI and low SI on FS/STIR on MRI. The differentiation from intraosseous lipoma with involutional change may not be possible through imaging studies but the intertrochanteric location favours the diagnosis of LSMFT [2].
Fibrous dysplasia (FD) reveals less sclerosis, usually with the characteristic “ground glass” pattern and endosteal scalloping, less frequent in LSMFT [2].
In bone scintigraphy both FD and LSMFT may show low tracer uptake [3].
Malignant transformation to osteosarcoma or malignant fibrous histiocytoma occurs approximately in 10-16% [1]. It is suggested in imaging when aggressive signs are developed, such as cortical disruption, rapid growing and soft-tissue extension. Treatment is considered in symptomatic patients, which includes curettage, bone-grafting and fixation. Cases with malignant transformations or pathological fracture, usually require arthroplasty [3]. The non-surgical treatment includes analgesics and follow-up with plain X-ray every six months and MR or CT every year during the first two years [2].
Radiology can lead to the diagnosis.
Histology can cause confusion.
Written informed patient consent for publication has been obtained.
Differential Diagnosis List
Liposclerosing myxofibrous tumour (LSMFT)
Intraosseous lipoma
Fibrous dysplasia
Final Diagnosis
Liposclerosing myxofibrous tumour (LSMFT)
References
[1] Mark J. Kransdorf, MD, Mark D. Murphey, MD, and Donald E. Sweet, MD. (1999) Liposclerosing Myxofibrous Tumor: A Radiologic-Pathologic-Distinct Fibro-osseous Lesion of Bone with a Marked Predilection for the Intertrochanteric Region of the Femur. Radiology Sept;212(3): 693-8 (PMID: 10478234)
[2] Regado ER, Garcia PBL, Caruso AC, de Almeida ALB, Aymoré IL, Meohas W, et al. (2016) Liposclerosing myxofibrous tumor: A series of 9 cases and review of the literature. Journal of Orthopaedics Sep;13(3):136–9 (PMID: 27222619)
[3] Nieto A, Pérez-Andrés R, Lorenzo JC, Vilanova JC. (2010) Diagnóstico por imagen del fibromixoma lipoesclerosante óseo. Radiología May;52(3):251-4
Case information
| URL: | https://www.eurorad.org/case/15865 |
| DOI: | 10.1594/EURORAD/CASE.15865 |
| ISSN: | 1563-4086 |
License
This work is licensed under a Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International License.
Figure 2
Plain X-ray film. Left hip.
Bone tumour in the centromedular intertrochanteric region of the left femur.
Figure 6
Coronal and axial MR of lesion in left hip
Axial T1 FS WI of the left hip, pre-contrast administration.
Axial T1 FS WI of the left hip, post-contrast administration. Arrow: spot of enhancement.
Coronal T1 FS WI of the left hip, pre-contrast administration.
Coronal T1 FS WI of the left hip, post-contrast administration. Arrow: spot of enhancement.
Plain X-ray film. Bilateral hips.
Bone tumour in the centromedular intertrochanteric region of the left femur.
Radiology Department, Hospital de Mar, Parc de Salut Mar, 2017.
Radiology Department, Hospital de Mar, Parc de Salut Mar, 2017.
Plain X-ray film. Left hip.
Bone tumour in the centromedular intertrochanteric region of the left femur.
Radiology Department, Hospital de Mar, Parc de Salut Mar, 2017.
Radiology Department, Hospital de Mar, Parc de Salut Mar, 2017.
Coronal and axial TC of lesion in left hip.
Coronal TC of lesion in left hip. Thin arrow: dystrophic matrix calcification.
Radiology Department, Hospital de Mar, Parc de Salut Mar, 2017.
Radiology Department, Hospital de Mar, Parc de Salut Mar, 2017.
Axial TC of lesion in left hip. Dotted line: no extra-osseous extension.
Radiology Department, Hospital de Mar, Parc de Salut Mar, 2017.
Radiology Department, Hospital de Mar, Parc de Salut Mar, 2017.
Axial TC of lesion in left hip. Thin arrow: fatty matrix.
Radiology Department, Hospital de Mar, Parc de Salut Mar, 2017.
Radiology Department, Hospital de Mar, Parc de Salut Mar, 2017.
Axial and coronal MR of lesion in the left hip
Axial T1WI, of left hip. Arrow: fatty matrix. Arrowhead: myxoid matrix.
Department of Radiology, Hospital de Mar, Parc de Salut Mar, 2017.
Department of Radiology, Hospital de Mar, Parc de Salut Mar, 2017.
Axial T2FS WI, of left hip. Arrow: fatty matrix. Arrowhead: myxoid matrix.
Department of Radiology, Hospital de Mar, Parc de Salut Mar, 2017.
Department of Radiology, Hospital de Mar, Parc de Salut Mar, 2017.
Coronal T2FS WI, of left hip. Arrow: fatty matrix. Arrowhead: myxoid matrix.
Department of Radiology, Hospital de Mar, Parc de Salut Mar, 2017.
Department of Radiology, Hospital de Mar, Parc de Salut Mar, 2017.
Coronal and axial MR of lesion in left hip
Axial T1 FS WI of the left hip, pre-contrast administration.
Radiology Department, Hospital de Mar, Parc de Salut Mar, 2017.
Radiology Department, Hospital de Mar, Parc de Salut Mar, 2017.
Axial T1 FS WI of the left hip, post-contrast administration. Arrow: spot of enhancement.
Radiology Department, Hospital de Mar, Parc de Salut Mar, 2017.
Radiology Department, Hospital de Mar, Parc de Salut Mar, 2017.
Coronal T1 FS WI of the left hip, pre-contrast administration.
Radiology Department, Hospital de Mar, Parc de Salut Mar, 2017.
Radiology Department, Hospital de Mar, Parc de Salut Mar, 2017.
Coronal T1 FS WI of the left hip, post-contrast administration. Arrow: spot of enhancement.
Radiology Department, Hospital de Mar, Parc de Salut Mar, 2017.
Radiology Department, Hospital de Mar, Parc de Salut Mar, 2017.