Epithelioid hemangioendothelioma, an ultra-rare cancer: a consensus paper from the community of experts.

Epithelioid hemangioendothelioma (EHE) is an ultra-rare, translocated, vascular sarcoma. EHE clinical behavior is variable, ranging from that of a low-grade malignancy to that of a high-grade sarcoma and it is marked by a high propensity for systemic involvement. No active systemic agents are currently approved specifically for EHE, which is typically refractory to the antitumor drugs used in sarcomas. The degree of uncertainty in selecting the most appropriate therapy for EHE patients and the lack of guidelines on the clinical management of the disease make the adoption of new treatments inconsistent across the world, resulting in suboptimal outcomes for many EHE patients. To address the shortcoming, a global consensus meeting was organized in December 2020 under the umbrella of the European Society for Medical Oncology (ESMO) involving >80 experts from several disciplines from Europe, North America and Asia, together with a patient representative from the EHE Group, a global, disease-specific patient advocacy group, and Sarcoma Patient EuroNet (SPAEN). The meeting was aimed at defining, by consensus, evidence-based best practices for the optimal approach to primary and metastatic EHE. The consensus achieved during that meeting is the subject of the present publication.

Osteoid osteoma treated with radiofrequency ablation in non-operating room anesthesia. A different way of approaching ablative therapy on osteoid osteoma.

OBJECTIVE: The purpose of this study is to verify the effectiveness and complications occurrence of radiofrequency ablation (RFA) in the treatment of osteoid osteoma (OO) in non-operating room anesthesia (N.O.R.A.). PATIENTS AND METHODS: From 2014 to 2017, 61 patients affected by OO (40 men and 21 women) with an age of 20.7 years on average (range, 4-51 years; 12 patients aged 20 years or younger) underwent computed tomography-guided percutaneous radiofrequency ablation (RFA) in N.O.R.A. (Non-Operating Room Anesthesia). Lesion sites treated were: femur (27), tibia (22), pelvis (2), talar bone (3), distal radius (1), and humerus (6). Mean follow-up time was 36 months. In each case, anesthesiologic support followed a new protocol (N.O.R.A. protocol), approved by our Institute. Primary success rate, complications, symptom-free intervals, and follow-up results were evaluated. RESULTS: Pain relief (evaluated with Visual Analogue Scale - VAS) was significant in 97% of patients; it disappeared within 24 hours of the procedure in 44 patients, within 3 days in 10 patients, and within 7 days in 7 patients. After 6 months of observation time, 60 of 61 patients were successfully treated and had no more complaints. In 2 patients, two major complications were found: infection of the site treated, healed with antibiotics, and a nerve lesion, healed with steroid therapy. No other complications were observed. CONCLUSIONS: RFA is a highly effective, efficient, minimally invasive and safe method for the treatment of OO following N.O.R.A.

The effectiveness of a standard drill connected to a core biopsy needle: How to obtain specimens in very strong bone tumors.

INTRODUCTION: Core biopsy is today recognized as the gold standard for the diagnosis of bone lesions; unfortunately, when the bone is too solid it can be very difficult to penetrate it; in case of failure, open biopsy is indicated but it is associated with greater contamination and complications. A possible solution is to connect a common orthopedic drill to the core biopsy needle. The aim of the presenting study was to present a technique useful for performing biopsies in case of very strong bone lesions and to evaluate the adequacy and quality of the obtained specimen. MATERIALS AND METHODS: A standard bone biopsy set was connected to a commercial drill to perform bone biopsies. Data was collected over a 2-year period (2015-2016). Information regarding technical success, diagnostic data and complication rates was all collated to assess the technical feasibility of this technique. RESULTS: Out of 357 bone biopsies, 34 patients underwent the procedure using a common orthopedic drill connected to a core biopsy needle. Diagnostic material was obtained in each patient and the artifacts were considered irrelevant. No major complications occurred in any patient. DISCUSSION: The use of a core biopsy needle connected to a common orthopedic drill facilitates the penetration of thick cortical bone by simply applying continuous speed and pressure; nevertheless, the biopsy needle we use is not designed for a drilling procedure and for this reason it can be damaged, but if the biopsy is performed with particular attention, the mechanical failure can be avoided CONCLUSIONS: Bone biopsy using a commercial hand drill has a technically high success rate with minimal complications. Further studies with more cases are necessary to verify our results.

The use of engineered biomaterial Bone Plexur M® in benign epiphyseal tumors: our experience at 20 months of follow-up.

The objective is to reconstruct the subchondral bone after curettage of benign tumors located in the epiphysis, a relevant topic in oncological orthopedics. Several bones substituted are commercially available, yet none of these are suitably moldable to repair or be placed in the bone defect; although autologous bone for little defects and homologous for bigger defects are still considered the standard in reconstruction, we verify the ability to adapt and support articular cartilage through the application of Plexur M (Registered Trademark), a newly engineered biomaterial bone. In the present study, we enrolled the first ten consecutive cases referred to our department, where patients were affected by a benign epiphyseal tumor destroying the subchondral bone through to the articular cartilage. Every patient underwent curettage of the disease, apposition of a newly engineered biomaterial bone and filling with homologous morselized bone. The quality of reconstruction was evaluated by two surgeons and by a radiologist based on the achievement of surgical objectives and comparing pre and postoperative imaging. In seven out of eight cases of lesions located in the lower limbs the quality of reconstruction was considered good, restoring an adequate support to the articular cartilage. The quality of the remaining case was considered poor probably due to the extent of the spread of the disease, which destroyed the entire proximal tibial epiphysis. In the two cases where the disease was located in the upper limbs, the Plexur M application restored support to the articular cartilage sufficiently well. However, in the case of a giant cell tumor of the distal radial epiphysis there was a slight reabsorption of the morselized homologous bone. Our series suggest that Plexur M should be considered a valid option for orthopedic surgeons in restoring adequate mechanical support to the articular cartilage; nevertheless, considering its high cost, its use might be reserved to selected cases until further studies can verify the integration process, the effects on the survival of the articular cartilage and on the prevention of premature osteoarthritis.

Pelvic sacral and hemi lumbar spine resection of low grade pelvic chondrosarcoma: a multistage procedure involving vascular bypass, spine fixation and vascular exclusion.

Peripheral chondrosarcoma is a rare tumor particularly insidious when arising from the pelvis, becoming symptomatic later in time when surgery may be too difficult and dangerous due to this complex area. In the present case, the tumor arose from an exostosis located on the medial surface of the left iliac wing. Its diameter was 25 cm × 20 cm × 15 cm, adhering to the last three vertebrae, involving the left iliac vein and artery, displacing the left ureter. In a similar case, a hindquarter amputation is indicated but, if the patient refuses, a resection remains possible. In this paper, we describe a multistage technique consisting of an extra-anatomic vascular bypass, a lumbar stabilization, a neurovascular bundles anterior isolation and a postero-lateral resection of this mass. After a five-year follow-up, the patient is alive and able to stand and walk with support, after undergoing twice lung metastasis removal.

Vertebral hemi-resection for bone tumor with wide invasion of the vertebral canal: modified surgical method.

The authors describe a variation in the method of vertebral hemi-resection used for the treatment of neoplasms that present a wide invasion of the vertebral canal. This is followed by a review of the literature on the subject.

Substitution in vertebral resections.

The authors discuss the reconstructive methods used after curettage and/or vertebral resection possibly associated with removal of surrounding muscular, visceral and nervous structures.

Stool antigen assay (HpSA) is less reliable than urea breath test for post-treatment diagnosis of Helicobacter pylori infection.

BACKGROUND: The diagnostic yield of the stool antigen test (HpSA) in evaluating the results of Helicobacter pylori eradication therapy is controversial, but many studies have used only the 13C-urea breath test (13C-UBT) as a gold standard which has greatly reduced their relevance. AIM: To compare the reliability of HpSA and 13C-UBT in patients post-treatment using biopsy-based methods as reference tests. METHODS: A total of 100 consecutive dyspeptic patients (42 male and 58 female; mean age, 56 +/- 18 years) were enrolled in our study. All patients were H. pylori positive on the basis of at least two biopsy-based methods, and underwent 1 week of treatment with various triple therapies. They were again endoscoped 4 weeks after completing therapy and six biopsy specimens were taken from the gastric antrum and corpus for rapid urease test, histology and culture. HpSA and 13C-UBT were also performed within 3 days of the second endoscopy. RESULTS: On the basis of biopsy-based tests, infection was eradicated in 77 patients but continued in 23. Three false negatives were observed with HpSA and two with 13C-UBT. In contrast, the number of false positives was significantly higher (P < 0.01) with HpSA than with 13C-UBT (nine vs. one), confirming the lower specificity of the former test. The overall accuracy of HpSA was 88% vs. 97% for 13C-UBT (P < 0.02). CONCLUSIONS: HpSA has lower diagnostic value than 13C-UBT in the evaluation of the outcome of anti-H. pylori therapy. 13C-UBT remains the first-line diagnostic method to monitor eradication results. The use of HpSA should be reserved for those settings in which 13C-UBT is not available.

Chondrosarcoma of the ribs and sternum. Considerations on 20 cases treated.

Over the last 20 years at the Rizzoli Orthopaedic Institute in Bologna 20 cases of chondrosarcoma (CS) of the thoracic wall (14 males, 6 females, mean age 49 years) have been submitted to surgery. Localization was costal in 11 cases, costosternal in 3, sternal in 3, costovertebral in 3. The most frequent histological variety was central with 15 observations. All of the cases were treated surgically. Exeresis was wide in 14 cases, marginal in 6. Reconstruction of the thoracic wall took place either by direct suturing or (14 cases) using prosthetic materials (Marlex mesh, 1 or 2 shapeable metal plates). Of the 20 cases treated, 16 patients are still alive (80%) with a mean survival rate of 33.5 months and a mean reduction in the postoperative ventilative index of function of less than 10%. The results obtained lead us to believe that surgical treatment involving wide exeresis is adequate, and the reconstruction method using Marlex mesh and metal plates is reliable.

Comparison of the main oesophageal pathophysiological characteristics between short- and long-segment Barrett's oesophagus.

AIM: To assess the oesophageal manometric characteristics and 24-h pH profiles of patients with both short-segment and long-segment Barrett's oesophagus and compare them with those of patients with reflux oesophagitis and controls. METHODS: Seventy-nine patients who had undergone upper digestive endoscopy were recruited: 16 had short-segment Barrett's oesophagus, 13 had long-segment Barrett's oesophagus, 25 had grade III oesophagitis according to the Savary-Miller classification and 25 were used as controls. The diagnosis of Barrett's oesophagus was based on the histological detection of specialized intestinal metaplasia, which extended < 3 cm into the oesophagus in patients with short-segment disease and > 3 cm in patients with long-segment disease. All subjects underwent oesophageal manometry and basal 24-h oesophageal pH monitoring. RESULTS: The lower oesophageal sphincter pressure was significantly lower in patients with reflux oesophagitis and short-segment and long-segment Barrett's oesophagus than in controls (P=0.0004-0.0001), but there was no difference among the three reflux groups. The peristaltic wave amplitude of patients with long-segment Barrett's oesophagus was significantly lower than that of controls (P=0.002) and patients with short-segment Barrett's oesophagus (P=0.02), but was no different from that of patients with reflux oesophagitis. The percentage of non-propagated wet swallows was significantly higher in patients with reflux oesophagitis and short-segment and long-segment Barrett's oesophagus when compared with that of controls (P=0.0004-0.0001). The total percentage of time the oesophagus was exposed to pH < 4.0 was significantly higher in patients with reflux oesophagitis and short-segment and long-segment Barrett's oesophagus (P=0.0001) than in controls, and was higher in patients with long-segment disease than in those with short-segment disease (P=0.01). CONCLUSIONS: Long-segment Barrett's oesophagus is characterized by a greater impairment of peristaltic wave amplitude and a higher oesophageal acid exposure than is short-segment Barrett's oesophagus. However, both forms are linked to increased acid reflux.