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.

[Retroperitoneal mixed malignant mullerian tumor : exceptional localisation and prognosis]. / Une tumeur mullérienne mixte maligne rétropéritonéale de localisation et pronostic exceptionnels.

Primary retroperitoneal carcinosarcoma or mixed malignant mullerian tumor (MMMT) is an extremely rare clinical entity. These aggressive tumors arise most commonly from genital tract. The retroperitoneal location is exceptional. Here we report the case of a 63-years old female diagnosed with heterologous, extra-genital, retroperitoneal carcinosarcoma, with malignant cells in the ascitic fluid and extra-ovarian metastatic implants. She was treated with complete radical surgical treatment consisting of resection of the retroperitoneal tumor, with omentectomy, hysterectomy, bilateral salpingooophorectomy and lumbo-aortic and pelvic lymphadenectomy. She received adjuvant chemotherapy with 6 cycles of Carboplatin and Paclitaxel. She is in complete clinical and radiological remission since the end of chemotherapy, for a total of 113 months. To our knowledge, this is the longest reported disease free survival of the extra-genital retroperitoneal MMMT. This case and the review of the literature illustrate the importance of surgical treatment. However, there are no evidence-based guidelines for the systemic management of these tumors.

Le carcinosarcome rétropéritonéal ou tumeur mullérienne mixte maligne (TMMM) est une entité clinique extrêmement rare. Ce sont des tumeurs agressives du tractus génital provenant des tissus mullériens. Les localisations rétropéritonéale et extra-génitale sont exceptionnelles. Ce cas clinique concerne une patiente de 63 ans atteinte d'un carcinosarcome extra-génital, retropéritonéal, hétérologue associé à des cellules malignes dans le liquide péritonéal et des implants métastatiques extra-ovariens. Elle a bénéficié d'une laparotomie de résection de la masse rétropéritonéale avec hystérectomie, annexectomie bilatérale, omentectomie et lymphadénectomie pelvienne et lombo-aortique, et a reçu une chimiothérapie adjuvante par 6 cycles de Carboplatine et Paclitaxel. Elle est en rémission clinique et radiologique complète depuis la fin de sa chimiothérapie, soit une durée de 113 mois. A notre connaissance, il s'agit de la durée de suivi sans récidive la plus longue rapportée dans la littérature. Ce cas, ainsi que la revue de littérature, mettent en évidence l'importance du traitement chirurgical. Par contre, il n'existe pas de standard thérapeutique pour la prise en charge adjuvante ou systémique de ces tumeurs.

Interest of next-generation sequencing in BCG-treated high-risk bladder cancer.

OBJECTIVES: There are only few predictive factors for response of non-musculo-invasive bladder cancer (NMIBC) to Bacillus Calmette-Guérin (BCG) therapy. Our study analyzed the results of the sequencing of new generation (NGS) targeted on 50 genes of oncological interest obtained on bladder resection parts in high-risk NMIBC patients treated with BCG, to describe this population from a molecular point of view and try to correlate these results in patients who present or not recurrence after BCG. METHODS: We reviewed 63 patients with high grade NMIBC treated between 2014 and 2016 with BCG after endoscopic resection. Each one had NGS analysis. Association tests between mutations detected by NGS and recurrence or progression were realized. RESULTS: The 45 remaining patients were fully analysed. For 73% of cases a mutation has been found, most frequent one's being FGFR3, TP53 and PIK3CA. With a median follow-up of 24 months (4-40), recurrence was present in 15 patients (33.3%), with 10 NMIBC (22.2%) and 5 progressions to muscular-invasive cancer (11.1%). If some mutations were more frequent in different prognostic groups no significant association has been found. No patient presenting CIS had FGFR3 mutation (P<0.0001). CONCLUSION: Next generation sequencing in NMIBC could be a supplementary aid in treatment decision making in the future. In an area where personalized medicine is rapidly growing in importance we need larger studies to define molecular characteristics in tumours to detect genomic associations between clinical phenotypes and recurrence or progression of the disease. LEVEL OF EVIDENCE: 3.

[An unusual digestive occlusion]. / Une occlusion intestinale inhabituelle.

Malignant melanoma is a rapidly metastatic disease. Metastasis in the small intestine is as such not uncommon, whereas the clinical presentation of obstruction due to intussusception is very rare. We hereafter report the case of a 58-year-old female admitted with general degradation, syndrome of intestinal occlusion and a cervical mass. Imaging studies showed signs suggesting an invagination of the small intestine. A resection of the cervical mass and segmental small intestine resection were performed. Pathological findings revealed a cervical malignant melanoma spread into the small intestine. The diagnosis of intestinal metastasis should therefore be considered in patients with intestinal occlusion and history of melanoma and presenting gastrointestinal symptoms.

[Pemetrexed salvage chemotherapy for NSCLC: implementation study]. / Traitement de rattrapage par pémétrexed pour les CBNPC: étude rétrospective d'implémentation.

BACKGROUND: Registration trials demonstrated the activity of pemetrexed in non small cell lung carcinoma (NSCLC) either in combination with first-line agents or in monotherapy for salvage treatment. The aim of our implementation study was to verify, in an unselected population, the results obtained with pemetrexed salvage chemotherapy in clinical practice. PATIENTS AND METHODS: The charts of all patients diagnosed with NSCLC, receiving pemetrexed for progressive disease after at least one previous course of chemotherapy, were retrospectively reviewed in two academic institutions. Response was assessed according to WHO and toxicity using the CTCAE and WHO criteria. RESULTS: Between November 2004 and September 2009, 84 patients were given pemetrexed as second, third or greater than third line therapy (n=18/14/52). Intent-to-treat response rate was 11.9% (95% CI, 5%-18.8%). Median progression-free survival was 2.2 months and median survival time was 6.4 months. The most frequent grade 3-5 toxicity was neutropenia (23.9%). CONCLUSION: Salvage chemotherapy with pemetrexed for progressing NSCLC confirmed, in an unselected population, who had been extensively treated previously, the level of activity observed in registration trials although a significant toxicity was noted.