Low-grade chondrosarcoma vs enchondroma: challenges in diagnosis and management.

A 28-year-old man presented with a swelling at the right thoracic wall. Computed tomography showed an aggressive process involving the cortex of the rib with concomitant soft tissue mass. However, a needle biopsy specimen revealed an enchondroma and consequently the physician decided to apply a "wait-and-see" strategy. After 3 years of careful follow-up by MR imaging, the patient complained of subtle enlargement of the lesion, which was later confirmed on repeated CT scan. Despite an aggressive appearance on control MR imaging, histopathological examination after incisional biopsy could not differentiate between enchondroma and low-grade chondrosarcoma. Wide excision including previous biopsy trajectory was performed. Diagnosis of a low-grade (grade I) chondrosarcoma was made on findings of the excisional specimen and seeding of cartilage tissue along the previous incisional biopsy trajectory was found.

Thyroid acropachy: correlation of imaging and pathology.

Thyroid acropachy is a rare manifestation of autoimmune thyroid disease, in the form of soft tissue swelling of the hands and feet with insidious onset, associated with clubbing and characteristic periosteal reactions. It is usually part of a syndrome consisting of a typical triad of thyroid acropachy, exophthalmos, and pretibial myxedema. The purpose of this case report is to demonstrate the imaging features of this typical triad in a 65-year-old-woman. This case is the first in which the MRI features of thyroid dermopathy are documented.

MR features of peripheral nerve sheath tumors: can a calculated index compete with radiologist's experience?

The aims of this study were, firstly, to provide a formula (neurogenic index) based on MR characteristics used in daily routine for predicting whether a soft tissue tumor is neurogenic or not, secondly, to test prospectively the performance of this formula, and thirdly, to compare this performance with that of radiologists experienced in MR imaging of soft tissue tumors. Retrospectively, MR images of 70 neurogenic and 70 non-neurogenic soft tissue tumors were evaluated in random order by two teams of two observers each. A neurogenic index (NI) was calculated based on those MR parameters that showed no or minor interobserver variability. Subsequently, three investigators in concert used the NI in a validation group of 15 neurogenic and 22 nonneurogenic soft tissue tumors. The same team, based on their own experience, tried to differentiate in the same validation group neurogenic from non-neurogenic soft tissue tumors. This was expressed in a subjective score (SS). Sensitivity, specificity, and predictive values were calculated. NI comprised spread (intra- or extracompartmental), distribution, fluid-fluid levels, homogeneity on T2-weighted images (WI), highest signal intensity (SI) on T1WI, lowest SI on T2WI, and delineation on T2WI. In the validation group, NI had a sensitivity of 88.6%, a specificity of 52.0%, a positive predictive value (PPV) of 54.1%, and a negative predictive value (NPV) of 84.6% for neurogenic tumors. The subjective score SS was superior and had a sensitivity of 93.3%, a specificity of 77.2%, a PPV of 73.7%, and a NPV of 94.4%. Our NI was less accurate than the SS; however, the low number of false-negative diagnoses for neurogenic tumors warrants continued efforts in development of neural networks.

Sclerosing bone dysplasias: genetic and radioclinical features.

Although knowledge of basic genetics in the field of sclerosing bone dysplasias is progressing, the radiologist still plays a pivotal role in the diagnosis of this relatively poorly understood group of disorders. Based on a target site approach, these anomalies are classified into three groups. Within each group, further differentiation can be made by distinctive clinical findings and by mode of inheritance: (a) dysplasias of endochondral bone formation: osteopetrosis (Albers-Schönberg disease), pycnodysostosis, enostosis, osteopoikilosis, osteopathia striata (Voorhoeve disease); (b) dysplasias of intramembranous bone formation: progressive diaphyseal dysplasia (Camurati-Engelmann disease) and variants, hyperostosis corticalis generalisata (Van Buchem disease) and variants; and (c) mixed sclerosing dysplasias: melorheostosis (Leri disease) and overlap syndromes.

MR imaging of clear cell sarcoma (malignant melanoma of the soft parts): a multicenter correlative MRI-pathology study of 21 cases and literature review.

OBJECTIVE: To evaluate MR imaging and pathology findings in order to define the characteristic features of clear cell sarcoma of the soft tissues (malignant melanoma of the soft parts). DESIGN AND PATIENTS: MR examinations of 21 patients with histologically proven clear cell sarcoma of the musculoskeletal system were retrospectively reviewed and assessed for shape, homogeneity, delineation, signal intensities on T1- and T2-weighted images, contrast enhancement, relationship with adjacent fascia or tendon, secondary bone involvement, and intratumoral necrosis. In 19 cases the pathology findings were available for review and for a comparative MR-pathology study. RESULTS: On T1-weighted images, lesions were isointense (n=3), hypointense (n=7) or slightly hyperintense to muscle (n=11). Immunohistochemical examination was performed in 17 patients. All 17 specimens showed positivity for HMB-45 antibody. In nine of 11 lesions with slightly increased signal intensity on T1-weighted images, a correlative MR imaging-pathology study was possible. All nine were positive to HMB-45 antibody. CONCLUSIONS: Clear cell sarcoma of the musculoskeletal system often has a benign-looking appearance on MR images. In up to 52% of patients, this lesion with melanocytic differentiation has slightly increased signal intensity on T1-weighted images compared with muscle. As the presence of this relative higher signal intensity on T1-weighted images is rather specific for tumors displaying melanocytic differentiation, radiologists should familiarize themselves with this rare entity and include it in their differential diagnosis when confronted with a well-defined, homogeneous, strongly enhancing mass with slightly higher signal intensity compared with muscle on native T1-weighted images.

Desmoplastic fibroma of bone: MRI features.

Desmoplastic fibroma of bone is a very rare benign tumor, which may be locally aggressive. In contrast to the well-documented radiological appearance, the literature on MR imaging features of this tumor is scarce. The MR imaging characteristics in our case are compared to those previously reported. Although there is a considerable overlap in the MR imaging features with other bone tumors, an interesting MR feature of desmoplastic fibroma is the presence of low to intermediate signal intensity foci on T2-weighted images, which radiographically does not correspond to calcifications. This feature may help narrow the differential diagnosis.

Proteus syndrome: MRI characteristics of plantar cerebriform hyperplasia.

Proteus syndrome is a rare congenital hamartomatous syndrome with a variety of abnormalities. It shares many features with other congenital hamartomatous disorders, but cerebriform hyperplasia of the soles and the palms is known as a quite distinctive characteristic in the dermatologic literature. The purpose of this case report is to demonstrate the MRI features of plantar cerebriform hyperplasia in a 9-year-old boy with known Proteus syndrome.

Metachronous eosinophilic granuloma of bone.

We present the case of a 4-year-old girl who developed a metachronous osseous lesion of the left distal femur five months after successful surgical treatment of an eosinophilic granuloma in the left proximal femur. After treatment of the second lesion with curettage, no further recurrence occurred during a follow-up interval of 17 months. Radiologists should be aware that additional eosinophilic granulomas of bone, occurring in up to four years after the initial diagnosis, should still be interpreted as a localized form of Langerhans'cell histiocytosis with a more favorable prognosis than the multifocal disseminated form, with involvement of organ systems other than the skeletal system.

Magnetic resonance imaging of cartilaginous tumors: is it useful or necessary?

OBJECTIVE: A retrospective study was undertaken to compare the diagnostic accuracy of plain radiography and magnetic resonance (MR) imaging in the diagnosis of cartilaginous tumors. PATIENTS: The study population consisted of 68 patients with a histologically proven cartilaginous tumor. DESIGN: For each lesion, a comparative analysis of "plain radiography" versus "plain radiography together with MR study" was performed by two radiologists together. Recently reported literature data were used as criteria by which to define accuracy rating. RESULTS AND CONCLUSIONS: MR imaging improves accuracy in diagnosing low-grade chondrosarcomas. Since osteochondromas have a characteristic appearance on plain films, MR imaging contributes only in the diagnostic workup of cases in which malignant transformation is suspected.

Magnetic resonance imaging of pelvic bone tumors.

The aim of our study was to determine the value of magnetic resonance (MR) imaging in the diagnostic workup of pelvic bone tumors. We retrospectively evaluated the MR findings in 60 pelvic bone tumors. Owing to its high contrast resolution and multiplanar imaging capabilities, MR offers a clear depiction of cortical, medullar or soft tissue involvement, intratumoral necrosis, and relationship to neurovascular structures, and may be considered as the modality of choice for the staging of pelvic bone tumors. Since grading of bone tumors reaches a high accuracy on conventional radiography (CR), the value of MR imaging is rather complementary. Although the role of MR imaging in tissue characterization is mostly limited to recognition of tumoral components, accurate tissue characterization if often possible (e.g. in low-grade chondrosarcoma, eosinophilic granuloma, aneurysmal bone cyst, giant cell tumor, and chordoma). MR imaging in osteochondromas, metastases, and fibrous dysplasia remains of limited value since most of these lesions are well recognized on CR and/or CT. CR remains the first choice examination in diagnosis and grading of bone tumors, but MR imaging has significantly improved staging and tissue characterization in bone tumor imaging. The aim of our study is to determine the value of magnetic resonance (MR) imaging in the diagnostic workup of pelvic bone tumors, i.e. in staging, in differentiating benign from malignant tumors (grading), and in further characterization of tumors or tumoral components.

Magnetic resonance imaging of pilomatricoma.

Two patients (a 16-month-old boy and a 53-year-old man) were referred for MR imaging of a small, subcutaneous nodule at the forearm. Plain films were available in one patient and showed a non-specific well-circumscribed lesion. Upon ultrasonography (1 patient), a nodular, well-circumscribed, hyperechoic lesion was seen. In both cases spin-echo (SE) T1-weighted images (T1WI) showed homogeneous, intermediate signal intensity (SI). On gadolinium-enhanced T1WI (1 patient), no enhancement was observed. Both lesions showed predominant low-to-intermediate SI on T2WI. At histological examination characteristic findings of pilomatricoma were observed.

Magnetic resonance imaging of cartilaginous tumors: a retrospective study of 79 patients.

OBJECTIVE: Hyaline cartilaginous tumors are characterized by extremely high signal intensity on T2-weighted images. Recently, some distinctive MR features of cartilaginous bone tumors were reported in small series. Low signal intensity septa surrounding high signal intensity cartilage lobules were seen on T2-weighted images in low-grade chondrosarcomas. On spin-echo T1-weighted images after Gd contrast injection, marked 'septal' or 'ring-and-arc' enhancement was observed in low-grade chondrosarcomas and enchondromas. The purpose of this study was to determine sensitivity and specificity of these MR findings in diagnosis of cartilaginous tumors, and to assess the value of MR in diagnostic workup of these lesions. MATERIALS AND METHODS: Retrospective evaluation of MR findings in 79 cartilaginous tumors and in 79 non-cartilaginous tumors. All lesions were biopsy proven. Each MR examination was independently reviewed by two experienced radiologists without knowledge of clinical data, radiographic and/or CT findings, or histological diagnosis. All lesions were evaluated for morphology (lobular or non-lobular), presence of a high signal intensity mass on T2-weighted images, presence of low signal intensity septa separating high signal intensity lobules on T2-weighted images, and evidence of septal ('ring-and-arc') enhancement. RESULTS: None of the reviewed parameters is useful in diagnosing osteochondromas. Since osteochondromas have a characteristic appearance on plain radiography, the value of MR imaging in the workup of these lesions remains limited. MR findings in enchondromas have a low specificity and a low sensitivity. Low-grade chondrosarcomas, often hard to diagnose on plain radiography and difficult to differentiate from enchondromas, are characterized by the MR tandem of 'low signal intensity septa on T2-weighted images' together with 'septal or ring-and-arc enhancement' (sensitivity 92.3%, specificity 76.5%). High-grade chondrosarcomas are easily recognized on plain radiography. CONCLUSIONS: In differentiating cartilaginous from non-cartilaginous tumors, MR features are highly specific but lack sensitivity. Grading potentials of MR parameters are promising due to the high accuracy in diagnosing low-grade chondrosarcomas.

Chondrosarcoma in a patient with relapsing polychondritis.

A case of a chondrosarcoma in a patient with relapsing polychondritis was presented. The association of these two unusual disorders of cartilaginous tissue has never been described before. The clinical presentation and radiologic and histologic features were discussed.