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.

The sacral chordoma margin.

OBJECTIVE: Aim of the manuscript is to discuss how to improve margins in sacral chordoma. BACKGROUND: Chordoma is a rare neoplasm, arising in half cases from the sacrum, with reported local failure in >50% after surgery. METHODS: A multidisciplinary meeting of the "Chordoma Global Consensus Group" was held in Milan in 2017, focusing on challenges in defining and achieving optimal margins in chordoma with respect to surgery, definitive particle radiation therapy (RT) and medical therapies. This review aims to report on the outcome of the consensus meeting and to provide a summary of the most recent evidence in this field. Possible new ways forward, including on-going international clinical studies, are discussed. RESULTS: En-bloc tumor-sacrum resection is the cornerstone of treatment of primary sacral chordoma, aiming to achieve negative microscopic margins. Radical definitive particle therapy seems to offer a similar outcome compared to surgery, although confirmation in comparative trials is lacking; besides there is still a certain degree of technical variability across institutions, corresponding to different fields of treatment and different tumor coverage. To address some of these questions, a prospective, randomized international study comparing surgery versus definitive high-dose RT is ongoing. Available data do not support the routine use of any medical therapy as (neo)adjuvant/cytoreductive treatment. CONCLUSION: Given the significant influence of margins status on local control in patients with primary localized sacral chordoma, the clear definition of adequate margins and a standard local approach across institutions for both surgery and particle RT is vital for improving the management of these patients.

IMRiS phase II study of IMRT in limb sarcomas: Results of the pre-trial QA facility questionnaire and workshop.

INTRODUCTION: Soft tissue sarcomas of the extremities (STSE) are rare malignancies. We report current UK practice for immobilisation of soft tissue sarcoma of STSE, as part of the initial study set-up within the IMRiS trial, a phase II study of intensity modulated radiotherapy (IMRT) in primary bone and soft tissue sarcoma. METHODS: A facility questionnaire (FQ) was circulated to 29 IMRiS centres investigating the variation in immobilisation devices, planning techniques, and imaging protocols. A workshop was held to address concerns raised by centres. It focused on STSE immobilisation and patient set-up. Robustness of patient set-up at each centre was evaluated based on the following criteria: evidence of local set-up audit, calculation of margins based on set-up audit results, imaging frequency, and number of patients treated per centre per annum. RESULTS: Twenty-seven (93%) questionnaires were returned. 30% (8/27) of responders routinely treated STSE with IMRT. The remaining 70% (19/27) had little or no experience with IMRT for STSE. Vacuum bags were the most frequent immobilisation device (9/27), followed by thermoplastic shells (7/27). Nine centres had audited their local set-up; however, only 4 had calculated margins in response to the results. Ten centres were classified as having high level of robustness. CONCLUSIONS: Immobilisation devices and planning techniques for STSE are inconsistent across centres. Robustness of set-up is an important tool to ensure quality of results in a multicentre trial setting with such different levels of experience. The IMRiS trial Quality Assurance programme encourages centres to assess robustness of set-up through local audit and subsequent calculation of treatment margins. IMPLICATIONS FOR PRACTICE: This is the first study that used robustness criteria to tailor QA support to individual centres.

Defining the target in cancer of the oesophagus: direct radiotherapy planning with fluorodeoxyglucose positron emission tomography-computed tomography.

AIMS: Target definition in radiotherapy treatment planning (RTP) of oesophageal cancer is challenging and guided by a combination of diagnostic modalities. This planning study aimed to evaluate the contribution of single positron emission tomography-computed tomography (PET-CT) in the treatment position to RTP. MATERIALS AND METHODS: Nineteen patients referred for radiotherapy from April to December 2008 were retrospectively identified. Two sets of target volumes were delineated using the planning CT and the (18)F-fluoro-deoxy-D-glucose ((18)F-FDG) PET-CT data sets, respectively. Target volumes were compared in length, volume and geographic conformality. Radiotherapy plans were generated and compared for both data sets. RESULTS: PET-CT planning target volume (PET-CT(PTV)) was larger than the CT target (CT(PTV)) in 12 cases and smaller in seven. The median PTV conformality index was 0.82 (range 0.44-0.98). Radiotherapy plans conforming to normal tissue dose constraints were achieved for both sets of PTV in 16 patients (three patients could not be treated to the prescription dose with either technique due to very large target volumes and significant risk of normal tissue toxicity). Previously undetected locoregional nodal involvement seen on PET-CT in three cases was localised and included in the PTV. In nine cases, the CTPTV plan delivered less than 95% dose to 95% of the PET-CT(PTV), raising concern about potential for geographical miss. CONCLUSION: A single scan with diagnostic PET-CT in the treatment position for RTP allows greater confidence in anatomical localisation and interpretation of biological information. The use of PET-CT may result in larger PTV volumes in selected cases, but did not exclude patients from radical treatment within accepted normal tissue tolerance.

Tumour volume changes following pre-operative radiotherapy in borderline resectable limb and trunk soft tissue sarcoma.

AIMS: To evaluate tumour volume changes after preoperative radiotherapy (PRT) for borderline operable soft tissue sarcomas (STS). MATERIALS AND METHODS: A retrospective review was performed of 68 patients who received PRT between December 2004 and July 2011. Endpoints were radiological response, surgical margins, local control and survival. RESULTS: Median tumour size was 12.5 cm. Tumour location was extremity (87%), trunk (12%), and neck (1%). Commonest histological subtypes were myxoid liposarcoma (32%) and myxofibrosarcoma (16%). The majority of patients (88%) received 50 Gy in 25 fractions. Post-radiotherapy imaging was available in 55 cases. By RECIST there was stable disease in 89%, partial response in 7% and progressive disease in 4%. Tumour volumes reduced in 80%. Median change in maximal tumour dimension was -13.6%; median change in volume was greater, at -33.3%. Tumour volumes increased in 11 cases (20%). However, surgical margins were clear in all 11 cases, with no local recurrences in this group. For the entire group, surgical margins were clear in 93%, and microscopically positive in 7%. Eight patients (12%) had local relapse at 2-24.8 months after surgery. Two year local relapse free survival was 87.5%; 2 year overall survival was 74.7%. CONCLUSION: The majority of tumours showed reduction in volume. A small number of tumours increased in volume, but there was no definite relationship between volume increase and poor surgical outcomes or lower local control rates. Local control was equivalent to published series' of PRT. PRT is a reasonable approach in patients with borderline resectable tumours.

Mandibular spine: a case report.

This is a brief review of the origin and normal variations of the genial tubercles, and specifically, a mandibular spine as an anatomical variation or abnormality. The case presented is that of abnormal genial tubercles observed during dissection of the floor of the mouth. A review and description on the morphology of the genial tubercles are given.

A tube system for the management of ruptured oesophagus.

In a patient who has ruptured his oesophagus a major objective should be to prevent access of swallowed material or refluxed gastric contents to the area of the rupture. We describe a tube system, introduced into the oesophagus via a cervical oesophagostomy, which prevents swallowed material from getting to the lower oesophagus and allows a tube to the passed into the stomach for continuous aspiration of gastric contents. To discontinue the diversion, a surgical procedure is not required. The successful use of this tube system in 3 patients is described.