B. Boulet

Feasibility of metronomic oral cyclophosphamide plus prednisolone in elderly patients with inoperable or metastatic soft tissue sarcoma

BACKGROUND The number of elderly people with soft tissue sarcoma (STS) is increasing. A sizeable population of elderly patients with STS is unfit for conventional doxorubicin- or ifosfamide-based chemotherapy. We assessed the feasibility of metronomic oral cyclophosphamide (CPM) in this population. PATIENTS AND METHODS Patients aged 65 years or older with unresectable STS received CPM 100mg twice daily plus prednisolone 20mg daily, the first week of a 2-week cycle in the outpatient setting. Main evaluation criterion was safety. Secondary evaluation criteria were objective response rate and progression-free survival. RESULTS Twenty-six patients (median age: 72, range 66-88) received a total of 330 cycles (median per patient: 10, range 2-41) as first (n=19) or second-line chemotherapy (n=7). The most frequent histological subtypes were poorly differentiated sarcoma (n=8), leiomyosarcoma and liposarcoma (n = 5 each) and angiosarcoma (n=3). Grade ≥3 lymphopenia was observed in 81% of pts but no opportunist infection occurred. Grade 3 anaemia and thrombocytopenia occurred in 2 pts (8%) each. No other grade 3-4 toxicity was seen. The response rate was 26.9% (95%CI: 9.9-44.0) and the disease control rate (responses and stable disease >12 weeks) was 69.2% (95%CI: 51.5-87.0). One complete (hepatic epithelioid haemangio-endothelioma) and 6 partial responses (including 5pts with radiation-induced sarcomas) were seen. Progression-free survival ranged from 0 to 20.6 months (median: 6.8 months) and was significantly longer in patients with radiation-induced sarcomas (median: 7.8 versus 5.2 months, p=0.02). CONCLUSION Metronomic CPM showed good safety results for this frail population, with promising activity in patients with radiation-induced sarcoma. Toxicity profile was favourable, allowing prolonged home staying and rare treatment discontinuations. A larger prospective study is warranted to confirm these encouraging results in elderly with STS.

Accuracy of Diffusion-Weighted Echo-Planar MR Imaging and ADC Mapping in the evaluation of residual Cervical Carcinoma after radiation therapy

OBJECTIVES The impact of diffusion-weighted imaging (DWI) and apparent diffusion coefficients (ADCs) of MR imaging on the evaluation of residual Uterine Cervical Carcinoma after Radiation Therapy, in addition to conventional MR images. METHODS Fourty-nine women presenting with a uterine cervical cancer were examined with 1.5 T MRI and DWI, 8 (4-20) weeks after treatment. Treatment response was determined based on the histopathological results after therapy and was classified as a complete response (CR) or residual disease (RD). Post-treatment DWI and ADC results were compared. RESULTS Five (11%) and 44 (89%) patients were considered as having histologically-proven RD or a CR respectively. The mean ADC of cervical tissue for all patients was 1.74±0.324×10(-3) mm(2)/s and the SD was 1.94±1.11×10(-4). The mean ADC was 1.62±0.21×10(-3) mm(2)/s (SD=1.45×10(-4)) for the 5 patients with RD versus 1.76±0.33×10(-3) mm(2)/s (SD=1.99×10(-4)) for the 44 patients with a CR (p=0.09). Using 1.7×10(-3) mm(2)/s as a radiological cut-off value for the ADC, all patients classified as having histologically-proven RD had a mean ADC of ≤1.7×10(-3). In 12 (25%) cases, RD was suspected on T2-weighted MRI images alone. Eight of these cases were considered as false positives compared to the histological results. Their mean ADC was 1.98×10(-3) mm(2)/s and none of them had an ADC of <1.7×10(-3) mm(2)/s. CONCLUSION Although our results were not statistically significant, ADC values could potentially be used to predict and monitor the response of uterine cervical cancer.

Evaluation of response after pre-operative radiotherapy in soft tissue sarcomas; the European Organisation for Research and Treatment of Cancer – Soft Tissue and Bone Sarcoma Group (EORTC – STBSG) and Imaging Group recommendations for radiological examination and reporting with an emphasis on magnetic resonance imaging

At present, there is no standardised approach for the radiological evaluation of soft tissue sarcomas following radiotherapy (RT). This manuscript, produced by a European Organisation for Research and Treatment of Cancer-Soft Tissue and Bone Sarcoma Group (EORTC-STBSG) and Imaging Group endorsed task force, aims to propose standardisation of magnetic resonance imaging techniques and interpretation after neoadjuvant RT for routine use and within clinical trials.

Approche didactique de la moelle osseuse en IRM

The marrow contains a variable amount of yellow or fatty marrow and red or cellular marrow creating the signal intensity observed on MRI. Marrow replacement (by cells not normally present in bone marrow) typically is T1W hypointense. Marrow proliferation (by cells normally present in bone marrow) may be T1W hypointense (pseudo marrow replacement) or show intermediate T1W signal intensity due to red marrow redistribution. Marrow edema (reaction to an external process) show intermediate T1W hypointensity (mixture of water and marrow). Location will allow correct diagnosis. Bone marrow ischemia usually results in a necrotic fragment surrounded by a thin T1W hypointense rim.

Vaginal reconstruction with pedicled vertical deep inferior epigastric perforator flap (diep) after pelvic exenteration. A consecutive case series

Vaginal reconstruction after pelvic exenteration (PE) represents a challenge for the oncologic surgeon. Since the introduction of perforator flaps, using pedicled vertical DIEP (deep inferior epigastric perforator) flap allows to reduce the donor site complication rate. From November 2012 to December 2014, 27 PEs were performed in our institution. 13 patients who underwent PE with vaginal reconstruction and programmed DIEP procedure for gynecologic malignancies were registered. Nine patients underwent PE for recurrent disease and four for primary treatment. Six of the 13 patients have a preoperative fistula. Anterior PE was performed in 10 patients, and total PE in 3 patients. A vertical DIEP flap was performed in 10 patients using one or two medial perforators. The reasons for abortion of vertical DIEP flap procedure were: failure to localizing perforator vessels in two cases, and unavailability of plastic surgeon in one case. A vertical fascia-sparring rectus abdominis myocutaneous flap was then harvested. Median length of surgery was 335min, and 60min for DIEP harvesting and vaginal reconstruction. No flap necrosis occurred. One patient in the VRAM (vertical rectus abdominis myocutaneous) group experienced a late incisional hernia and one patient in the DIEP flap group required revision for vaginal stenosis. In our experience, DIEP flap represents our preferred choice of flap for circumferential vaginal reconstruction after PE. To achieve a high reproducibility, the technically demanding pedicled vertical DIEP flap has to be harvested by a trained surgeon, after strict evaluation of the preoperative imaging with identification and localization of perforator vessels.

Left Lateral Endosurgical Extraperitoneal Total Hysterectomy with Para-Aortic and Pelvic Lymphadenectomy. A Novel Approach for the Obese Patient with Endometrial Cancer

STUDY OBJECTIVE To describe the left lateral extraperitoneal approach to perform complete para-aortic and pelvic lymphadenectomy and transverse total hysterectomy from left to right as a novel approach to treat obese patients with endometrial cancer. Laparoscopic management of obese patients represents a challenge for the gynecologic surgeon. The extraperitoneal approach is technically easier in the obese patient because it naturally creates a bowel-free operative field. DESIGN A prospective pilot bicentric and descriptive study (Canadian Task Force classification III) evaluating the feasibility and reproducibility of the transverse total hysterectomy and complete lymphadenectomy through left endoscopic extraperitoneal approach in obese patients with endometrial cancer. SETTING A comprehensive cancer center in Toulouse and a teaching university hospital in Madrid. PATIENTS Sixteen consecutive overweight or obese patients (body mass index > 25 kg/m2) with high-risk endometrial cancer. INTERVENTIONS Currently, the left extraperitoneal approach is routinely used to perform complete para-aortic and pelvic lymphadenectomy. It provides direct access to the left ureter and uterine pedicle. This access can be extended to the right side when performing a transverse extrafascial hysterectomy from left to right. The procedure starts from the left extraperitoneal space, where the left uterine artery is sectioned and the vesicovaginal and rectovaginal septa are developed, without opening the peritoneum. Colpotomy is performed from the left to the right side. Once the right ureter is identified, the right uterine artery can be safely transected. Alternatively, the right uterine artery can be sealed and sectioned during the right pelvic lymphadenectomy. At the end of the procedure the peritoneum is opened to complete the surgery. MEASUREMENTS AND MAIN RESULTS Between May 2015 and February 2016, 16 consecutive obese patients were successfully treated using this technique. Median patient age was 62 years (range, 44-78), and median body mass index was 32.5 kg/m2 (range, 26-42). In 3 cases the right uterine artery was sealed during the right pelvic lymphadenectomy, in 11 cases after completing vaginal opening, and in 2 cases after peritoneal opening. The median operative time was 137.5 minutes (range, 66-260). The median blood loss was 85 mL (range, 0-260), and no blood transfusion was required in any of our 16 patients. No significant complications occurred. CONCLUSION The full extraperitoneal approach represents an interesting alternative strategy for the surgical treatment of obese patients with high-risk endometrial cancer.

A 14-year-old boy with mandibular tumor, spontaneous spinal fractures. Chronic recurrent multifocal osteomyelitis.

Editor’s note: Each month, this department features a discussion of an unusual diagnosis in genetics, radiology, or dermatology. A description and images are presented, followed by the diagnosis and an explanation of how the diagnosis was determined. As always, your comments are welcome via e-mail at editor@pediatricsupersite.com. A 14-year-old Boy with Mandibular Tumor, Spontaneous Spinal Fractures

ORL-WP-33 Lecture facile des foramens de la base du crane en imagerie

Objectifs pedagogiques Savoir reconnaitre ces foramens (topographie et forme). Faciliter la memorisation de leur contenu. Detecter leur extension de maniere precoce et cibler l’imagerie en cas de symptomatologie localisatrice. Messages a retenir Les foramens ne sont pas accessibles cliniquement, le scanner et l’IRM (surtout) sont necessaires a leur exploration. Les nerfs crâniens ont ete denommes de un a douze au fur et a mesure qu’ils sortent de la base du crâne de haut en bas et d’avant en arriere. Les foramens sont des canaux dont l’axe est soit parallele soit perpendiculaire au palais osseux, facilitant leur exploration en imagerie. L’obturation de la graisse (au scanner en en Tl en IRM) ou la prise de contraste en IRM sont les signes les plus precoces de leur envahissement.

Apport des nouveaux traitements a visee anti-angiogenique et imagerie

Objectifs Connaitre les differents types de therapies anti-angiogeniques testees dans le CHC. Connaitre les modifications morphologiques et les images pieges induites par ces nouveaux traitements. Connaitre les criteres d’echappement therapeutique. Savoir l’interet des techniques d’imagerie de perfusion de de l’IRM de diffusion pour l’evaluation therapeutique des therapies ciblees. Messages a retenir Le Sorafenib, inhibiteur de proteine kinases, a l’AMM pour le traitement du CHC au stade avance ou recidivant apres traitement local. Les traitements antiangiogeniques entrainent rarement une regression de taille des tumeurs. Les nouveaux criteres semiologiques a prendre en compte sont l’evaluation de la necrose et de la vascularisation tumorale. L’echappement therapeutique se traduit par l’apparition d’un bourgeon tumoral ou d’un epaississement parietal vascularise au sein de la zone de necrose. Les techniques d’imagerie de perfusion et d’IRM de diffusion apparaissent des techniques prometteuses pour l’evaluation de ces traitements. Resume Les therapies anti-angiogeniques sont particulierement indiquees dans le carcinome hepatocellulaire (CHC) qui est une tumeur le plus souvent hypervascularisee. Actuellement une drogue, le Sorafenib a l’AMM pour le traitement des CHC de stade avance ou en rechute apres traitement local chez des patients Child-Pugh A. Les habituels criteres d’evaluation therapeutiques (RECIST), sont partiellement adaptes a l’evaluation des therapies antiangiogeniques n’entrainant que rarement et tardivement une regression de la taille tumorale. Il est necessaire d’associer d’autres criteres tels que l’evaluation de la necrose tumorale et, ou l’etude de la perfusion tumorale. Des techniques d’imagerie de perfusion en echographie, scanner ou IRM peuvent etre utiles pour evaluer de facon objective la devascu-larisation des tumeurs sous traitement. L’IRM de diffusion apparait prometteuse pour l’evaluation de la necrose tumorale.

Approche diagnostique de la moelle osseuse en IRM

Objectifs Connaitre le role, la distribution et les variantes de la moelle normale. Connaitre l’aspect normal de la moelle en IRM. Reconnaitre les pathologies affectant la moelle osseuse : lesions de remplacement medullaire (tumeurs osseuses primitives et secondaires), lesions du tissu hematopoietique : proliferation (gammapathies monoclonales, syndrome myeloproliferatifs et leucemies) ou depletion, oedeme medullaire secondaire a un traumatisme, une infection, une tumeur ou une affection articulaire, ischemie medullaire. Messages a retenir La meilleure sequence pour etudier la moelle est le Tl. Les anomalies de signal ne sont pas specifiques. Necessite de bien differencier le cadre pathologie locale de celui pathologie generale. Resume La moelle contient une proportion variable de moelle jaune graisseuse et de moelle rouge cellulaire aboutissant au signal visible en IRM. Le remplacement medullaire (cellules non physiologique-ment presentes dans la moelle) apparait en hyposignal Tl franc. La proliferation medullaire (cellules physiologiquement presentes dans la moelle), apparait en hyposignal Tl franc (pseudo-remplacement medullaire) ou en hyposignal intermediaire par anomalies de distribution de la moelle rouge. L’œdeme medullaire (reaction a une agression externe) apparait en hyposignal intermediaire Tl (melange eau et moelle). C’est la topographie qui permettra une approche de son etiologie. L’ischemie medullaire aboutit typiquement a un fragment necrose delimite en Tl par un lisere en hyposignal.