A recurrent pleuropneumonia revealing Erdheim-Chester Disease.

Erdheim-Chester disease (ECD) is a rare form of L group histiocytosis, accounting for up to 1500 cases to date worldwide, which mainly affects men between their 5th and 7th decade of life. The most frequent manifestations are bone involvement, perirenal infiltration with an evocating appearance of "hairy kidneys", and a "coated aorta" aspect. Lung involvement in ECD is less common and includes pleural infiltration and interstitial lung disease. Herein, we report the case of a 76-year-old woman with recurrent pleuropneumonia revealing ECD.

Cervical bone pain revealing a bone metastatic colon cancer: A case report.

Colorectal cancer (CRC) is one of the most common malignancies worldwide, with common sites of metastases including abdominal lymph nodes, the liver and lungs. Bone metastases are known to be relatively rare sites of metastasis. The present study reported a patient with advanced colorectal cancer and metastatic colorectal cancer (mCRC) with atypical metastases presentation. Bone mCRCs were not frequent and presented a poor prognosis in the present case. It was concluded that further studies are required to clarify the pathogenesis of bone metastases, to improve the management and treatment of patients.

Correction to: Prediction of pancreatic neuroendocrine tumour grade with MR imaging features: added value of diffusion-weighted imaging.

The original version of this article, published on 19 August 2016, unfortunately contained a mistake.

Different kinetics of infectious processes in vertebral osteomyelitis of pyogenic or tuberculous origin explain different timing of surgery.

Background: Whether surgery modalities vary according to kinetics of pathological processes responsible for vertebral osteomyelitis (VO) is unclear. We therefore compared surgical modalities in patients with haematogenous pyogenic VO (HPVO) or tuberculous VO (TVO).Methods: Patients who had surgery for HPVO or TVO between January 1997 and June 2018 in a university hospital were included. Surgical indications, timing, and procedures and outcomes were evaluated at the end of treatment.Results: Seventy-eight patients (50 men) were included: 39 with HPVO and 39 with TVO; median age was 64 and 41 years, respectively. In patients with HPVO, surgery was performed early: 17 (44%) had surgery within 72 h of admission; main indication for surgery was neurological deficit in 29 patients that persisted in 12 patients (27%). In patients with TVO, surgery was performed later (p<.001), after two weeks in 20 patients (51%), and was indicated by a neurological deficit in 23 patients; among them, only one (4%) had residual deficit.Conclusions: Different kinetic profiles of the infectious processes explain the more rapid indication for surgery in patients with HPVO and the more favourable neurological recovery in patients with TVO.

More complications in cervical than in non-cervical spine tuberculosis.

Purpose: Cervical spine tuberculosis (CST) is a rare disease that may lead to severe neurological complications. The goal of the study was to compare the characteristics of patients with CST with those of patients with non-cervical spine tuberculosis (NCST).Methods: Between 1997 and 2016, we reviewed all cases of proven tuberculosis from a cohort of spine infections in a tertiary care hospital. Clinical, biological, and imaging data were collected at baseline and after treatment.Results: Fifty-one cases of spine tuberculosis were included: 14 with CST on imaging (27%) and 37 with no cervical localization. Median age was 39 y. Demographic characteristics, duration of symptoms and neurological findings of spine compression were similarly present at presentation in CST and NCST patients. On imaging, lesions were more often multifocal in CST than in NCST patients (9/14 [64%] versus 10/37 [27%], p = .014). Spinal surgery was required in 32/51 (63%) patients. At the end of follow-up (median: 20 months), cure rates were similar in CST and NCST patients but motor and/or sensitive functional sequel were more frequent in CST than NCST patients (6/14 [43%] versus 2/37 [5%], p = .003).Conclusions: Cervical involvement is present in more than a quarter of patients with spinal tuberculosis. Patients with CST had more frequent neurological sequelae than patients with NCST. This was mainly due to a more multifocal disease at presentation. Screening for cervical localization should be systematic in patients with spinal tuberculosis even in the absence of cervical symptoms.

Splenic Artery Aneurysms, A Rare Complication of Type 1 Gaucher Disease: Report of Five Cases.

Type 1 Gaucher disease is a rare genetic lysosomal disorder due to acid betaglucosidase deficiency. The main features are thrombocytopenia, anemia, hepatosplenomegaly and complex skeletal disease. Complications include pulmonary hypertension, cirrhosis and splenic infarction; comorbidities, such as autoimmune phenomena, B-cell malignancies and Parkinson disease also occur. Visceral aneurysms have been only rarely noted in Gaucher disease. We report the retrospective data from patients with Gaucher disease type 1 and splenic arterial aneurysm. We describe the different outcomes of a giant splenic arterial aneurysm in five patients with type 1 Gaucher disease and discuss the main possible pathophysiological explanations. Aneurysms of the splenic artery are rare in Gaucher disease but are probably greatly under-reported.

Surgery is safe and effective when indicated in the acute phase of hematogenous pyogenic vertebral osteomyelitis.

BACKGROUND: The overall benefit of surgical management in the acute phase of hematogenous pyogenic vertebral osteomyelitis remains difficult to evaluate because of the balance between potential functional benefit versus complications of surgery. METHODS: Between 2000 and 2013, in a tertiary care hospital, we analyzed a cohort of patients with hematogenous pyogenic vertebral osteomyelitis treated surgically and compared them to those treated medically. Neurologic deficit (using the ASIA impairment scale) and pain (using the analgesic level required) 4 months later, recurrences and infection-related deaths 12 months later were evaluated. A propensity score was developed to adjust for nonrandomized allocation to surgery. RESULTS: Ninety patients were included (mean age 64 years, 63% male); 28 (31%) were treated surgically. After adjustment for the propensity score, the improvement in neurological deficit at 4 months did not differ between surgical and medical treatment (p = .82), but the reduction of pain tended to be greater in surgical versus medical treatment (p = .051). Recurrences of infection (5%) and infection-related deaths (12%) occurred at similar rates in both groups at 12 months (p = 1.00 for both). CONCLUSIONS: Patients with hematogenous pyogenic vertebral osteomyelitis requiring surgery improved equally as non-surgical patients in terms of neurological deficit and residual pain. This result was not hampered by increased complications related to surgery. When indicated, surgery is safe and effective in patients suffering from hematogenous pyogenic vertebral osteomyelitis.

Non-measurable infiltrative HCC: is post-contrast attenuation on CT a sign of tumor response?

OBJECTIVES: To evaluate the value of CT attenuation to assess the response to sorafenib in infiltrative/endovascular non-measurable advanced hepatocellular carcinoma (HCC). METHODS: From 2007 to 2014, patients with infiltrative HCC ± tumor-in-vein (TIV) were retrospectively included. Attenuation of tumors and TIV were measured at baseline and follow-up on arterial and portal venous phase CT by two independent radiologists. Attenuation changes (overall and as per Choi criteria) and Child-Pugh score were correlated to overall survival. RESULTS: Forty patients were included (38 men, 95%). Attenuation of both the tumors and TIV was significantly lower in follow-up CT than on baseline CT (p = 0.002 (arterial), and p = 0.001 (portal) for tumor, and p = 0.004 (arterial) and p < 0.001 (porta) for TIV). Median attenuation of TIV was significantly lower than that of the tumor in follow-up images (p = 0.010). Median OS for the entire cohort was 4 ± 1 months (95% CI: 2.1-5.9), with estimated OS rates at 6, 12, and 24 months of 43%, 29 and 12%, respectively. Baseline and follow-up CT attenuation in tumors and TVI were not correlated with survival. Survival was not significantly increased in patients with Choi criteria >15% CT HU decrease in the tumor and/or TIV during follow-up. Only Child-Pugh A (HR 4.9 (95%CI 2.3-10.7), p < 0.001) was identified as an independent factor of improved survival on multivariate analysis. CONCLUSION: Despite significant changes under sorafenib, tumor attenuation of infiltrative/endovascular non-measurable HCC may be of limited value to assess survival in this subgroup of patients with very poor prognosis. KEY POINTS: • Attenuation of both tumors and tumor-in-vein decreases after sorafenib. • Attenuation decrease is more marked in the tumor-in-vein than in the tumor. • Attenuation decrease is not associated with longer overall survival.

Uncommon evolutions and complications of common benign liver lesions.

Frequently encountered on abdominal imaging studies, the majority of common benign liver lesions are asymptomatic, confidently diagnosed by imaging, and do not require further workup, follow-up, or treatment. The increasing use of multimodality liver imaging, has allowed the recognition of uncommon evolutions of common benign liver lesions such as size changes, fibrotic regression, and content and vascularization changes, and their complications such as rupture, hemorrhage, thrombosis, extrinsic compression, and malignancy. The purpose of this pictorial review is to describe and illustrate the incidence and diagnostic features of these uncommon evolutions and complications on cross-sectional imaging, mainly on computed tomography and magnetic resonance imaging, with emphasis on those imaging clues which are helpful in the differential diagnosis or indicate the need for treatment.

Efficacy and safety of selective internal radiotherapy with yttrium-90 resin microspheres compared with sorafenib in locally advanced and inoperable hepatocellular carcinoma (SARAH): an open-label randomised controlled phase 3 trial.

BACKGROUND: Sorafenib is the recommended treatment for patients with advanced hepatocellular carcinoma. We aimed to compare the efficacy and safety of sorafenib to that of selective internal radiotherapy (SIRT) with yttrium-90 (90Y) resin microspheres in patients with hepatocellular carcinoma. METHODS: SARAH was a multicentre, open-label, randomised, controlled, investigator-initiated, phase 3 trial done at 25 centres specialising in liver diseases in France. Patients were eligible if they were aged at least 18 years with a life expectancy greater than 3 months, had an Eastern Cooperative Oncology Group (ECOG) performance status of 0 or 1, Child-Pugh liver function class A or B score of 7 or lower, and locally advanced hepatocellular carcinoma (Barcelona Clinic Liver Cancer [BCLC] stage C), or new hepatocellular carcinoma not eligible for surgical resection, liver transplantation, or thermal ablation after a previously cured hepatocellular carcinoma (cured by surgery or thermoablative therapy), or hepatocellular carcinoma with two unsuccessful rounds of transarterial chemoembolisation. Patients were randomly assigned (1:1) by a permutated block method with block sizes two and four to receive continuous oral sorafenib (400 mg twice daily) or SIRT with 90Y-loaded resin microspheres 2-5 weeks after randomisation. Patients were stratified according to randomising centre, ECOG performance status, previous transarterial chemoembolisation, and presence of macroscopic vascular invasion. The primary endpoint was overall survival. Analyses were done on the intention-to-treat population; safety was assessed in all patients who received at least one dose of sorafenib or underwent at least one of the SIRT work-up exams. This study has been completed and the final results are reported here. The trial is registered with ClinicalTrials.gov, number NCT01482442. FINDINGS: Between Dec 5, 2011, and March 12, 2015, 467 patients were randomly assigned; after eight patients withdrew consent, 237 were assigned to SIRT and 222 to sorafenib. In the SIRT group, 53 (22%) of 237 patients did not receive SIRT; 26 (49%) of these 53 patients were treated with sorafenib. Median follow-up was 27·9 months (IQR 21·9-33·6) in the SIRT group and 28·1 months (20·0-35·3) in the sorafenib group. Median overall survival was 8·0 months (95% CI 6·7-9·9) in the SIRT group versus 9·9 months (8·7-11·4) in the sorafenib group (hazard ratio 1·15 [95% CI 0·94-1·41] for SIRT vs sorafenib; p=0·18). In the safety population, at least one serious adverse event was reported in 174 (77%) of 226 patients in the SIRT group and in 176 (82%) of 216 in the sorafenib group. The most frequent grade 3 or worse treatment-related adverse events were fatigue (20 [9%] vs 41 [19%]), liver dysfunction (25 [11%] vs 27 [13%]), increased laboratory liver values (20 [9%] vs 16 [7%]), haematological abnormalities (23 [10%] vs 30 [14%]), diarrhoea (three [1%] vs 30 [14%]), abdominal pain (six [3%] vs 14 [6%]), increased creatinine (four [2%] vs 12 [6%]), and hand-foot skin reaction (one [<1%] vs 12 [6%]). 19 deaths in the SIRT group and 12 in the sorafenib group were deemed to be treatment related. INTERPRETATION: In patients with locally advanced or intermediate-stage hepatocellular carcinoma after unsuccessful transarterial chemoembolisation, overall survival did not significantly differ between the two groups. Quality of life and tolerance might help when choosing between the two treatments. FUNDING: Sirtex Medical Inc.

Risks factors for severe pain after selective liver transarterial chemoembolization.

BACKGROUND & AIMS: Post-procedural pain is frequent after transarterial chemoembolization (TACE) for hepatocellular carcinoma (HCC), and is only partially prevented by treatment selectivity. Our aim was to determine the risk factors of severe pain after selective TACE for HCC. METHODS: From January 2012 to June 2014, all treatment-naïve patients undergoing a first selective TACE were included. Risk factors for severe pain, that is, the need for opioid analgesics (grade II-III), were identified by uni- and multivariate analysis. Internal validation of a logistic regression model for prediction of opioid intake was done with bootstrapping. RESULTS: We analysed 335 tumours (mean 47 ± 37 mm) in 159 patients (131 men), mean 63.4 years old (20-92). Twenty-seven patients (17%) requested opioids. In univariate analysis, opioid intake was associated with young age (P=.021), doxorubicin dose received (P=.031), large HCC (P=.038), absence of chronic liver disease (P<.001) and alpha-foetoprotein levels (P=.03). In multivariate analysis, opioid intake was associated with young age (OR=0.65 per 10 years increment, P=.048), absence of chronic liver disease (OR=31.7, P<.001) and a higher fraction of the doxorubicin dose (OR=1.32 per 10% increment, P=.009). The optimism-corrected area under the curve of the prediction model for opioid intake using these three factors was 0.751. CONCLUSION: In patients with HCC treated with TACE, selective procedure does not always prevent from severe pain. Young patients without chronic liver disease may be more susceptible to severe pain.

Prediction of pancreatic neuroendocrine tumour grade with MR imaging features: added value of diffusion-weighted imaging.

OBJECTIVES: To evaluate the value of MR imaging including diffusion-weighted imaging (DWI) for the grading of pancreatic neuroendocrine tumours (pNET). MATERIAL AND METHODS: Between 2006 and 2014, all resected pNETs with preoperative MR imaging including DWI were included. Tumour grading was based on the 2010 WHO classification. MR imaging features included size, T1-w, and T2-w signal intensity, enhancement pattern, apparent (ADC) and true diffusion (D) coefficients. RESULTS: One hundred and eight pNETs (mean 40 ± 33 mm) were evaluated in 94 patients (48 women, 51 %, mean age 52 ± 12). Fifty-five (51 %), 42 (39 %), and 11 (10 %) tumours were given the following grades (G): G1, G2, and G3. Mean ADC and D values were significantly lower as grade increased (ADC: 2.13 ± 0.70, 1.78 ± 0.72, and 0.86 ± 0.22 10-3 mm2/s, and D: 1.92 ± 0.70, 1.75 ± 0.74, and 0.82 ± 0.19 10-3 mm2/s G1, G2, and G3, all p < 0.001). A higher grade was associated with larger sized tumours (p < 0.001). The AUROC of ADC and D to differentiate G3 and G1-2 were 0.96 ± 0.02 and 0.95 ± 0.02. Optimal cut-off values for the identification of G3 were 1.19 10-3 mm2/s for ADC (sensitivity 100 %, specificity 92 %) and 1.04 10-3 mm2/s for D (sensitivity 82 %, specificity 92 %). CONCLUSION: Morphological/functional MRI features of pNETS depend on tumour grade. DWI is useful for the identification of high-grade tumours. KEY POINTS: • Morphological and functional MRI features of pNETs depend on tumour grade. • Their combination has a high predictive value for grade. • All pNETs should be explored by MR imaging including DWI. • DWI is helpful for identification of high-grade and poorly-differentiated tumours.

Sequential transarterial chemoembolization and portal vein embolization before resection is a valid oncological strategy for unilobar hepatocellular carcinoma regardless of the tumor burden.

OBJECTIVE: To investigate the long-term oncological outcome of patients with resectable hepatocellular carcinoma (HCC) undergoing sequential transarterial chemoembolization (TACE) and portal vein embolization (PVE). METHODS: Analysis of all Child A HCC patients who underwent TACE-PVE before major liver resection from 2006 to 2012 was performed according to whether or not they underwent surgical resection as planned. RESULTS: 54 patients (50 men, 93% median 69-years (range 44-87)) were included. Thirty-nine (72%) patients underwent resection, including 19/25, 16/23, and 4/6 of patients with BCLC A, B, and C (p = 0.839). Twenty-two (56%) had tumor recurrence (median delay 10 months) including 9/19, 11/16, and 2/4 of the patients with BCLC A, B, and C (p = 0.430). Survival was significantly better in resected patients as compared to those who were not resected (median overall survival (OS): 44 vs. 18 months; p < 0.001). Recurrence was associated with a poorer prognosis as compared to patients without recurrence (median OS 43 months vs. not reached; p < 0.001). BCLC stage did not influence survival (p = 0.13). CONCLUSION: In patients with large unilobar HCC, TACE-PVE leads to resection in most patients, with a good oncological outcome regardless of the tumor burden. When this strategy fails, patients can be managed with TACE despite prior PVE.