Imaging presentation of extraskeletal osteosarcomas on CT and MRI and correlation with patients outcome: A two-center retrospective study of 54 patients.

PURPOSE: The purpose of this study was to analyze the imaging features of extraskeletal osteosarcomas (ESOS) on computed tomography (CT) and magnetic resonance imaging (MRI) and to investigate their associations with overall survival (OS) using uni- and multivariable survival analyses. MATERIALS AND METHODS: This two-center retrospective study included all consecutive adult patients between 2008 and 2021 with histopathologically-proven ESOS who underwent pre-treatment CT and/or MRI. Clinical and histological characteristics, ESOS presentation on CT and MRI, treatment and outcomes were reported. Survival analyses were performed using Kaplan-Meier analysis and Cox regressions. Associations between imaging features and OS were searched using uni- and multivariable analyses. RESULTS: Fifty-four patients were included (30/54 [56%] men, median age: 67.5 years). Twenty-four died of ESOS (median OS: 18 months). ESOS were mostly deep-seated (46/54, 85%) in the lower limb (27/54, 50%) with a median size of 95 mm (interquartile range: 64, 142; range: 21-289 mm). Mineralization was seen on 26/42 (62%) patients, mainly gross-amorphous (18/26; 69%). ESOS were generally highly heterogeneous on T2-weighted images (38/48; 79%) and contrast-enhanced (CE) T1-weighted images (29/40; 72%), with necrosis (39/40; 97%), well-defined or focally infiltrative margins (39/47; 83%), with moderate peritumoral edema (39/47; 83%) and rim-like peripheral enhancement (17/40; 42%). Size, location, mineralization on CT, signal intensity heterogeneity on T1-, T2- and CE-T1-weighted images and hemorragic signal on MRI were associated with poorer OS (range of log-rank P = 0.0069-0.0485). At multivariable analysis, hemorragic signal and signal intensity heterogeneity on T2-weighted images remained predictive for poorer OS (hazard ratio [HR] = 2.68, P = 0.0299; HR = 9.85, P = 0.0262, respectively) CONCLUSION: ESOS typically presents as mineralized heterogeneous and necrotic soft tissue tumor with a possible rim-like enhancement and limited peritumoral abnormalities. MRI may help estimate outcome of patients with ESOS.

Spectrum of Skeletal Imaging Features in Osteopetrosis: Inheritance Pattern and Radiological Associations.

Osteopetrosis (from the Greek "osteo": bone; "petrosis": stone) is a clinically and genetically heterogeneous group of rare diseases of the skeleton, sharing the same main characteristic of an abnormally increased bone density. Dense bones in radiological studies are considered the hallmark of these diseases, and the reason for the common term used: "Marble bone disease". Interestingly, a radiologist, Dr. Albers-Schonberg, described this disease for the first time in Germany in 1904. Indeed, radiology has a key role in the clinical diagnosis of osteopetrosis and is fundamental in assessing the disease severity and complications, as well as in follow-up controls and the evaluation of the response to treatment. Osteopetrosis includes a broad spectrum of genetic mutations with very different clinical symptoms, age onset, and prognosis (from mild to severe). This diversity translates into different imaging patterns related to specific mutations, and different disease severity. The main recognized types of osteopetrosis are the infantile malignant forms with autosomal recessive transmission (ARO-including the rarer X-linked recessive form); the intermediate autosomal recessive form (IAO); and the autosomal dominant ones ADO, type I, and type II, the latter being called 'Albers-Schonberg' disease. Imaging features may change among those distinct types with different patterns, severities, skeletal segment involvement, and speeds of progression. There are several classical and well-recognized radiological features related to osteopetrosis: increased bone density (all types with different degrees of severity assuming a 'Marble Bone Appearance' especially in the ARO type), different metaphyseal alterations/enlargement including the so-called 'Erlenmeyer flask deformity' (particularly of femoral bones, more frequent in ADO type 2, and less frequent in ARO and IAO), 'bone in bone' appearance (more frequent in ADO type 2, less frequent in ARO and IAO), and 'rugger-jersey spine' appearance (typical of ADO type 2). After conducting an overview of the epidemiological and clinical characteristic of the disease, this review article aims at summarizing the main radiological features found in different forms of osteopetrosis together with their inheritance pattern.

Atlantoaxial rotatory subluxation/fixation and Grisel's syndrome in children: clinical and radiological prognostic factors.

Atlantoaxial rotatory subluxation/fixation (AARS/F) is a rare cause of torticollis in children. The aim of our study is to investigate all possible clinical and radiological prognostic factors in children with AARS/F. We retrospectively reviewed all cervical spine CT scans of children with AARS/F treated in our Hospital over the last 15 years. AARS/F was classified according to Fielding and Hawkins classification and C1-C2 rotation-degree was calculated. Moreover, two orthopedic surgeons reviewed all clinical reports of these children. All patients were conservatively treated (cervical traction/neck collar). An early recovery was considered in patients with complete clinical/radiological healing at 3 months follow-up, while a late recovery was considered in patients with disease persistence or relapse at 3 months follow-up or earlier. Fifty-five patients with diagnosis of AARS/F were included in the study (mean age = 8.5 years old - 25F, 30M). In 9/55 subjects (16.4%), a late recovery was observed. The presence of a concomitant infection or inflammation in the head and neck region (Grisel's syndrome) was significantly associated with a late recovery (p < .001). Also, the type of AARS/F (p = .019), according to the Fielding and Hawkins classification, and C1-C2 rotation-degree (p = .027) were significantly correlated with the recovery time.Conclusion: In patients with AARS/F, the presence of a concomitant infection/inflammation in the head and neck region is the most important prognostic factor and it is associated with a late recovery. The Fielding and Hawkins classification and C1-C2 rotation-degree well correlate with patients' recovery time. What is Known: • Atlantoaxial rotatory subluxation/fixation (AARS/F) is a rare cause of torticollis in children and CT is the most useful imaging tool for diagnosis and classification of AARS/F. • Conservative treatments are effective in the majority of pediatric patients with AARS/F. What is New: • The presence of a concomitant infection/inflammation in the head and neck region associated with AARS/F (Grisel's syndrome) is the most important prognostic factor and it is associated with a late recovery. • C1-C2 rotation-degrees, as well as Fielding and Hawkins classification system, well correlate with patients' recovery time.

Non-surgical Treatments for Lung Metastases in Patients with Soft Tissue Sarcoma: Stereotactic Body Radiation Therapy (SBRT) and Radiofrequency Ablation (RFA).

BACKGROUND: Radio-frequency ablation (RFA) and Stereotactic Body Radiation Therapy (SBRT) are two emerging therapies for lung metastases. INTRODUCTION: Aliterature review was performed to evaluate the outcomes and complications of these procedures in patients with lung metastases from soft tissue sarcoma (STS). METHODS: After selection, seven studies were included for each treatment encompassing a total of 424 patients: 218 in the SBRT group and 206 in the RFA group. RESULTS: The mean age ranged from 47.9 to 64 years in the SBRT group and from 48 to 62.7 years in the RFA group. The most common histologic subtype was, in both groups, leiomyosarcoma. In the SBRT group, median overall survival ranged from 25.2 to 69 months and median disease- free interval was from 8.4 to 45 months. Two out of seven studies reported G3 and one G3 toxicity, respectively. In RFA patients, overall survival ranged from 15 to 50 months. The most frequent complication was pneumothorax. Local control showed a high percentage for both procedures. CONCLUSION: SBRT is recommended in patients unsuitable to surgery, in synchronous bilateral pulmonary metastases, in case of deep lesions and patients receiving high-risk systemic therapies. RFA is indicated in case of a long disease-free interval, in oligometastatic disease, when only the lung is involved, in small size lesions far from large vessels. Further large randomized studies are necessary to establish whether these treatments may also represent a reliable alternative to surgery.

Efficacy of selective transarterial chemoembolization in inducing tumor necrosis in small (<5 cm) hepatocellular carcinomas.

UNLABELLED: Transarterial chemoembolization (TACE) is commonly used as a bridge therapy for patients awaiting liver transplantation (LT) and for downstaging patients initially not meeting the Milan criteria. The primary aim of this study was to analyze whether a difference exists between selective/superselective and lobar TACE in determining tumor necrosis by a pathological analysis of the whole lesion at the time of LT. The secondary aim was to investigate the relationship between the tumor size and the capacity of TACE to induce necrosis. Data were extracted from a prospective database of 67 consecutive patients who underwent LT for hepatocellular carcinoma and cirrhosis from 2003 to 2009 and were treated exclusively with TACE as a bridging (n = 53) or downstaging therapy (n = 14). We identified 122 nodules; 53.3% were treated with selective/superselective TACE. The mean histological necrosis level was 64.7%; complete tumor necrosis was obtained in 42.6% of the nodules. In comparison with lobar TACE, selective/superselective TACE led to significantly higher mean levels of necrosis (75.1% versus 52.8%, P = 0.002) and a higher rate of complete necrosis (53.8% versus 29.8%, P = 0.013). A significant direct relationship was observed between the tumor diameter and the mean tumor necrosis level (59.6% for lesions < 2 cm, 68.4% for lesions of 2.1-3 cm, and 76.2% for lesions > 3 cm). Histological necrosis was maximal for tumors > 3 cm: 91.8% after selective/superselective TACE and 66.5% after lobar procedures. Independent predictors of complete tumor necrosis were selective/superselective TACE (P = 0.049) and the treatment of single nodules (P = 0.008). Repeat sessions were more frequently needed for nodules treated with lobar TACE (31.6% versus 59.3%, P = 0.049). CONCLUSION: Selective/superselective TACE was more successful than lobar procedures in achieving complete histological necrosis, and TACE was more effective in 3- to 5-cm tumors than in smaller ones.