Search

Background: Incisional hernias can be associated with pain or discomfort. Surgical repair especially mesh reinforcement, may likewise induce pain. The primary objective was to assess the incidence of pain after hernia repair in patients with and without pre-operative pain or discomfort. The secondary objectives were to determine the preferred mesh type, mesh location and surgical technique in minimizing postoperative pain or discomfort. Materials and methods: A registry-based prospective cohort study was performed, including patients undergoing incisional hernia repair between September 2011 and May 2019. Patients with a minimum follow-up of 3–6 months were included. The incidence of hernia related pain and discomfort was recorded perioperatively. Results: A total of 1312 patients were included. Pre-operatively, 1091 (83%) patients reported pain or discomfort. After hernia repair, 961 (73%) patients did not report pain or discomfort (mean follow-up = 11.1 months). Of the pre-operative asymptomatic patients (n = 221), 44 (20%, moderate or severe pain: n = 14, 32%) reported pain or discomfort after mean follow-up of 10.5 months. Of those patients initially reporting pain or discomfort (n = 1091), 307 (28%, moderate or severe pain: n = 80, 26%) still reported pain or discomfort after a mean follow-up of 11.3 months postoperatively. Conclusion: In symptomatic incisional hernia patients, hernia related complaints may be resolved in the majority of cases undergoing surgical repair. In asymptomatic incisional hernia patients, pain or discomfort may be induced in a considerable number of patients due to surgical repair and one should be aware if this postoperative complication.

Surgery demonstration (SD) is considered to be a mainstay of surgical education, but controversy exists concerning the patient’s safety. Indeed, the presence of visiting surgeons is a source of distraction and may have an impact on surgeon’s performance. This study’s objective was to evaluate possible differences in outcomes between robotic sphincter-saving rectal cancer surgery (RRCS) performed during routine surgical practice versus in the presence of visiting surgeons in the operating room (OR) with direct access to the surgeon. Retrospective case-matched studies were conducted from a prospectively collected database. 114 patients (38 with the presence of visiting surgeons) who underwent RRCS between January 2013 and September 2018 were included. Patients were matched in a 1:2 basis after propensity score analysis using five criteria: gender, body mass index, preoperative chemoradiation, type of mesorectum excision, and synchronous liver metastasis. There was no difference between the two groups with regard to mean operating time, estimated blood loss, conversion, and hospital stay. Also, overall (44% vs. 40%; P = 0.6), major morbidity (26% vs. 19%; P = 0.5), and unplanned reoperation (17% vs. 15%; P = 1.0) rates were not statistically different. No difference was noted with regard to the quality of mesorectum excision, or positive rate of circumferential and distal longitudinal resection margins. The mean number of harvested lymph nodes (17 vs. 14.5; P = 0.04) was lower in the SD group and the number of patients with

International audience; Background: Although minimally invasive rectal surgery (MIRS) for cancer provides better recovery for similar oncologic outcomes over open approach, conversion is still required in 10% and its impact on short-term and long-term outcomes remains unclear. The aim of our study was to evaluate the impact of conversion on postoperative and oncologic outcomes in patients undergoing MIRS for cancer. Methods: From June 2011 to March 2020, we reviewed 257 minimally invasive rectal resections for cancer recorded in a prospectively maintained database, with 192 robotic and 65 laparoscopic approaches. Patients who required conversion to open (Conversion group) were compared to those who did not have conversion (No conversion group) in terms of short-term, histologic, and oncologic outcomes. Univariate and multivariate analyses of the risk factors for postoperative morbidity were performed. Results: Eighteen patients (7%) required conversion. The conversion rate was significantly higher in the laparoscopic approach than in the robotic approach (16.9% vs 3.6%, p < 0.01). Among the 4 reactive conversions, 3 (75%) were required during robotic resections. Patients in the Conversion group had a higher morbidity rate (83.3% vs 43.1%, p = 0.01) and more severe complications (38.9%, vs 18.8%, p = 0.041). Male sex [HR = 2.46, 95%CI (1.41–4.26)], total mesorectal excision [HR = 2.89, 95%CI (1.57–5.320)], and conversion (HR = 4.87, 95%CI [1.34–17.73]) were independently associated with a higher risk of overall 30-day morbidity. R1 resections were more frequent in the Conversion group (22.2% vs 5.4%, p = 0.023) without differences in the overall (82.7 ± 7.0 months vs 79.4 ± 3.3 months, p = 0.448) and disease-free survivals (49.0 ± 8.6 months vs 70.2 ± 4.1 months, p = 0.362). Conclusion: Conversion to laparotomy during MIRS for cancer was associated with poorer postoperative results without impairing oncologic outcomes. The high frequency of reactive conversion due to intraoperative complications in robotic resections confirmed that MIRS for cancer is a technically challenging procedure.

Aims. Minimally invasive liver resection is a complex and challenging operation. Although authors have reported robotic liver resection shows improved safety and efficacy compared with open liver resection, robotic major liver resections for malignant liver lesions treatment remain inadequately evaluated. The aims of the present study were to evaluate the feasibility and safety of transitioning from open to robotic liver resection in a nonuniversity hospital. Patients and Methods. From December 2015 to March 2020, 46 patients underwent totally robotic-assisted liver resections out of 446 robotic procedures. Also, we retrospectively reviewed the last 27 open right hepatectomies (ORHs) and compared then with the first 25 anatomic robotic-assisted right hepatectomies (RRHs). Results. Mean operative time, mean blood lost, rate of complications, and mean hospital stay were associated with the complexity of the procedure. The comparison between ORH and RRH showed that intraoperative complications were less frequently observed during ORH whereas RRH showed a trend in favor of less blood loss. ORH had a trend toward smaller surgical margins and higher rate of R1 resections. Recurrence occurred in 31 (59%) patients and was more frequently observed after ORH. However, the mean follow-up was significantly shorter after RRH. Conclusion. Our study demonstrated the technical feasibility and safety of transitioning from open to robotic liver resection (including major hepatectomies) in a nonuniversity setting. Higher costs remain an important drawback for robotic surgery.

Background and objectives The number of robotic colorectal procedures performed has rapidly increased, but there are only sparse data available about the robotic learning curve of expert laparoscopic colorectal surgeons. Methods In this retrospective study, we reviewed 101 minimally invasive right colectomies consecutively performed by a single surgeon with 20 years of clinical practice fully dedicated to laparoscopic surgery. Thus, the last 59 laparoscopic resections were compared with the first 42 robotic resections. Results The duration of the procedure was longer in the robotic group, but the conversion rate was the same in both groups. There was no difference between groups in rates of overall and severe postoperative complications, reoperation, hospital length of stay, and readmission. Number of harvested lymph nodes and oncological quality of resection defined by the pathologist were the same. Conclusions This study suggests that the transition from the right laparoscopic colectomy with extracorporeal anastomosis to the robot-assisted right colectomy with intracorporeal anastomosis when performed by a surgeon with experience in laparoscopic colorectal surgery may not entail any increase on the morbidity rate or reduce the oncologic quality of the resection.

Timing of major elective operations is a potentially important outcome variable. This study examined the impact of operative start time (OST) on pathologic and short-term outcomes of minimally invasive rectal surgery (MIRS).All rectal tumors patients who underwent MIRS from May 2012 to April 2016 were identified. Peroperative outcomes and the oncological quality of surgical excision were compared between patients with OST before 13.00h and after.A total of 137 patients were included in the study (71 Romarobot-assisted and 66 conventional laparoscopic). Ninety-nine (72%) patients were operated before 13.00h and 38 after 13.00h. The majority of cases were low/middle rectal tumors (69%). Patient's baseline characteristics were quite similar in both groups. The rate of severe complication (p=0.460) or reoperation (p=0.614) was the same. Pathologic criteria (T or N stage, number of harvested lymph nodes, and presence of any positive margin) were the same between groups except for the quality of mesorectal excision (ME) that was significantly poorer for cases beginning after 13.00h (complete 91% vs 74%; p=0.016). The OST was found to be the only parameter associated with a poor quality of ME [OR 2.55 (1.08 - 6.36)].Perioperative outcome after MIRS does not appear to be influenced by OST. Poorer quality of ME was observed and may thus raise important questions about the timing and sequence of case scheduling.

Although several studies have compared totally robot-assisted gastric bypass (RA-GB) to laparoscopic gastric bypass (L-GB), the clinical benefit of the robotic approach remains unclear. We compared perioperative outcomes of 82 consecutive patients undergoing RA-GB between 2013 and 2016 to 169 consecutive patients having undergone L-GB between 2009 and 2016. Secondary endpoints included duration of hospitalization, readmission rate, weight loss at 1 year, and the learning curve of RA-GB, assessed by operation times and complication rates. There were no statistically significant differences between groups concerning age (43.5 ± 11.2 vs. 42.2 ± 12.4 years), body mass index (42.4 ± 5.0 vs. 43.6 ± 7.2 kg/m2), or comorbidities. The rate of revision surgery was higher in L-GB group without reaching statistical significance. No statistically significant difference was observed for duration of operation (134 ± 35 vs. 135 ± 37 min), readmission rate at 90 days (4.9% vs. 8.9%), or percentage of excess weight loss at 1 year (RA-GB vs. L-GB) (76.8% ± 20.5 vs. 73.1% ± 23.5). There were fewer statistically significant complications overall in RA-GB (9.8% vs. 21.9%, p = 0.019). Median duration of hospital stay was shorter for RA-GB (3 vs. 4 days, p

Resume L’appendicite et la diverticulite du colon sont les deux principales causes d’abdomen aigu febrile chez l’adulte. Le diagnostic en imagerie (echographie et TDM) est le plus souvent aise. Cependant, la realisation d’une imagerie inadaptee a la situation clinique et un examen pratique avec un mauvais protocole sont des sources d’erreur a eviter. Aussi, les variantes anatomiques, les cancers inflammatoires, les formes compliquees (perforation, occlusion du grele secondaire, abces peripheriques, fistules, pylephlebite, abces hepatiques) et les signes associes lies a une reaction inflammatoire peritoneale (ileus reflexe, ileite ou salpingite reactionnels) peuvent conduire a un diagnostic errone.

Resume L’atteinte digestive profonde de l’endometriose est caracterisee par un epaississement fibreux et retractile de la paroi digestive. La localisation la plus frequente est rectale haute, en contiguite d’une atteinte du torus. Dans le cadre d’un bilan preoperatoire, il est primordial d’etablir une cartographie precise et exhaustive des lesions digestives afin que le chirurgien puisse programmer son geste. L’echographie endovaginale et l’IRM analysent correctement les atteintes pelviennes et rectales. Devant la frequence des localisations digestives multiples, notamment sigmoidiennes et ileocaecales associees, il convient de faire un coloscanner. La place de l’echoendoscopie rectale est discutee.

Una masa ovarica detectada en la infancia corresponde en alrededor del 50% de los casos a una enfermedad no tumoral, como un quiste funcional. Entre los tumores de ovario, los tumores benignos son mas frecuentes que los canceres, por lo que una masa ovarica tendra un 80% de posibilidades de ser benigna. Independientemente de las circunstancias en las que estos tumores son descubiertos, es importante efectuar, antes de la intervencion quirurgica, aunque sea urgente, pruebas complementarias: radiografia simple de abdomen, ecografia y determinacion de marcadores tumorales (alfafetoproteina y gonadotropina corionica humana [hCG]), que orientan el diagnostico. Los tumores malignos o potencialmente malignos son casi siempre tumores de origen germinal o del estroma, al contrario que los tumores epiteliales observados en el adulto. Estos tumores presentan una evolucion rapida y cierto potencial metastatico, pero su pronostico es excelente siempre que se controlen correctamente, en estrecha colaboracion entre cirujanos y oncologos pediatricos. Los factores pronosticos antes de la era de la quimioterapia inicial eran el estadio de extension de la enfermedad y la histologia. Los tumores compuestos de tejido del saco vitelino o de coriocarcinoma eran los mas malignos, con una tasa de supervivencia, para los tumores diseminados en la pelvis o el abdomen, de menos del 20%. Estos factores pronosticos han desaparecido por completo despues del uso de quimioterapias mas eficaces, sobre todo con cisplatino y bleomicina. Los tumores de estirpe genital de la nina son excepcionales y reagrupan los rabdomiosarcomas, los tumores germinales y los adenocarcinomas de celulas claras. Su tratamiento es diferente, aunque las tasas de curacion son excelentes si el diagnostico es precoz. El tratamiento permite conservar utero, vagina y ovarios funcionales en la mayoria de los casos.

Une prise en charge multidisciplinaire est necessaire pour les patients atteints de sarcomes d’Ewing qui sont en frequence la seconde tumeur maligne osseuse de l’enfant et de l’adolescent. La chimiotherapie associee au traitement local permet la guerison de 65 a 70 % des patients ayant des tumeurs localisees. La chirurgie a vu ses indications augmenter avec les annees, d’une part pour ameliorer le controle local, d’autre part pour limiter la radiotherapie. Celle-ci demeure cependant importante dans les localisations inoperables ou en association avec la chirurgie. Il existe maintenant des facteurs pronostiques pour ajuster la chimiotherapie a la gravite de la maladie. Les formes initialement metastatiques demeurent d’un pronostic grave et necessitent l’evaluation de therapeutiques experimentales.

Malignant pheochromocytomas are rare in childhood and the prognosis of children with this tumor is not well known. We present 2 pediatric observations of malignant pelvic pheochromocytoma. Symptoms in both cases were headache and hypertension. The tumor invaded the sacral bone. Angiogram helped to localize the tumor and metastases, and allowed preoperative embolization of the tumor in 1 case. The first child underwent incomplete surgical resection, 131I-MIBG therapy and radiotherapy, and is still alive 2 years after diagnosis. The second child died from metastatic invasion a few weeks after discovery of the tumor. We reviewed previous reports of children with malignant pheochromocytomas (30 cases). Primary tumor was extraadrenal in 50% of cases. The 3-year survival rate was 73 ± 9% (mean ± SD). Apart from surgical resection, no particular treatment appeared to be more effective than others in reducing mortality.

Resume Les tumeurs malignes des tissus mous constituent un groupe tres heterogene compose de plus de 50 entites differentes derivees soit du mesenchyme, soit du neuroectoderme. Les difficultes diagnostiques concernent, d'une part, l'etablissement d'un diagnostic de malignite et, d'autre part, la determination precise du type de sarcome. Le pronostic est en grande partie determine par l'extension locale et a distance de la maladie. L'etude du genotype des cellules tumorales a permis de mettre en evidence des alterations genetiques. Ces alterations, frequemment des translocations chromosomiques, constituent des marqueurs specifiques a certains sous-groupes tumoraux et presentent ainsi un interet diagnostique. Ces marqueurs peuvent etre detectes par la cytogenetique, l'hybridation in situ ou la biologie moleculaire. La specificite de ces alterations pour les cellules tumorales et la possibilite de les mettre en evidence par la technique sensible de reaction en chaine a la polymerase ( polymerase chain reaction [PCR]) ont permis de developper des tests visant a rechercher les cellules tumorales au niveau de sites potentiellement metastatiques.

Appendicitis and diverticulitis of the colon are the two main causes of febrile acute abdomen in adults. Diagnosis from imaging (ultrasound and CT) is usually easy. However, an imaging procedure which is not suitable for the clinical situation and an examination performed with the wrong protocol are sources of error and must be avoided. Anatomical variants, inflammatory cancers, complicated forms (perforation, secondary occlusion of the small intestine, peripheral abscesses, fistulae, pylephlebitis, liver abscesses) and associated signs related to a peritoneal inflammatory reaction (reflex ileus, reactive ileitis or salpingitis) can also lead to a wrong diagnosis.

Deep gastrointestinal involvement in endometriosis is characterised by fibrous, retractile thickening of the intestinal wall. The most common location is the upper rectum, in contiguity with a lesion of the torus uterinus. As part of a preoperative assessment, it is essential to establish an accurate and exhaustive map of intestinal lesions so that the surgeon can plan his actions. Transvaginal sonography and MRI correctly analyse pelvic and rectal involvement. Given the frequency of multiple intestinal sites, particularly sigmoid and associated ileo-caecal lesions, water enema CT should be performed. The role of rectal endoscopic sonography is debated.

Primary heart tumors are uncommon in children. The majority of them are benign, with only 10% malignant. Among malignant cardiac tumors, sarcoma (rhabdomyosarcoma, angiosarcoma, synovial sarcoma) and lymphoma (Burkitt's lymphoma, large B-cell lymphoma, lymphoblastic lymphoma) predominate. There are few published pediatric series on malignant primary cardiac tumors. We report here 3 observations of primary malignant cardiac tumors, 2 cases of sarcoma (angiosarcoma and synovial sarcoma) and 1 case of Burkitt's lymphoma. A precise pathological diagnosis is necessary for the proper management of these patients. For sarcoma, treatment associates surgery and chemotherapy. Surgery should be as complete as possible because of the lack of chemotherapy sensitivity of some sarcomas, mainly angiosarcoma and synovial sarcoma. Therefore, the prognosis of cardiac sarcoma remains poor. For primary cardiac lymphoma, management should not be different from lymphoma in other locations. Chemotherapy is the main treatment, and surgery has to be used only when complications occur. Prognosis depends on histology and not lymphoma location, and so is better than the prognosis for sarcoma.

Soft tissue tumors account for approximately 25% of neonatal tumors and are most often benign (more than 2/3 of cases). Vascular tumors are the most frequent benign tumors and infantile hemangioma accounts for 32% of these tumors, affecting 1 out of 200 children at birth. Kaposiform hemangioendothelioma (KH) is a rare vascular tumor with locally aggressive behavior. More than 50% of KH are associated with the Kasabach-Merritt phenomenon, a condition characterized by thrombocytopenia and consumptive coagulopathy. Malignant soft tissue tumors are, after neuroblastoma, the second cause of cancer in neonates. Infantile fibrosarcoma (IF) is a rare tumor that most often affects the extremities of children aged 4 years or younger. A recurrent t(12;15) (p13;q25) rearrangement fusing the ETV6 gene with the NTRK3 neurotrophin-3 receptor gene has been identified in IF. Complete conservative surgical resection is usually curative. Chemotherapy is indicated when initial surgical removal cannot be accomplished without unacceptable morbidity. Prognosis of IF is excellent, with reported overall survival rates ranging from 80 to 100%. Neonatal rhabdomyosarcoma (RMS) is a rare tumor (0.5-1% of RMS). The primary tumor predominantly involves the limbs and the genitourinary tract. Treatment is based on age-adapted chemotherapy and surgery. Prognosis of RMS in children less than 1 year old appears to be comparable with that of older children.

Ifosfamide is an alkylating agent used in the treatment of germ-cell tumors, sarcomas and lymphomas. One of its main side effects is the encephalopathy of which the incidence may reach 30% in the literature, in adults and children just as well.Based on both our experience and a review of the literature, we propose some recommendations for the management of this complication.We report 15 encephalopathy cases in non-brain tumor patients, which occurred between January 1987 and March 2002 in children from 2 to 17 years old, treated for solid tumors at the Institut Gustave Roussy. Ifosfamide was administered at a posology between 5.4 and 15 g/m(2)/course, associated with other antimitotics such as actinomycin D, etoposide or vincristine.Six patients experienced a grade III neurological toxicity according to the NCI classification, which developed as excess drowsiness lasting up to 36 hours. Six other patients developed grade IV neurotoxicity, including two comas resolving within 4 days and four short generalized convulsions. Three other children experienced grade II drowsiness. Brain MRIs were normal and EEG showed an aspecific encephalopathy tracing. This early central neurotoxicity appeared right from the first administration, and occurred immediately after the first injection or during the second or third day of treatment. It was most often reversible, usually 3 to 5 days after the last ifosfamide administration. Five patients were administered a treatment with Methylene Blue with a demonstrable efficacy in only one case. No death or neurological sequelae have been noted. Ifosfamide has been renewed after the neurological accident in 7 of those patients. Only 1 of those 7 patients developed grade IV neurotoxicity during the next course of treatment. In 2 of those 7 children, Methylene Blue was used in a prophylactic way. No neurological disorders have been noted during the next courses of treatment.In the literature, the following are described as risk factors for ifosfamide encephalopathy: advanced pelvic disease, previous cisplatyl treatment and renal failure. We have not found any of these predisposing factors in our series, but three of the fifteen patients had severe neurotoxicity associated with Vincristin during previous treatments.Facing a clinical diagnosis of ifosfamide encephalopathy, it is recommended to discontinue administration of ifosfamide and inject by intravenous route 50 mg Methylene Blue every 4 hours until the symptomatology recedes. The re-challenge of Ifosfamide is not contra-indicated and should be performed under prophylactic treatment with Methylene Blue by intravenous route at the dose of 50 mg every 6 hours.

Purpose Final results are presented from two consecutive European studies for patients with metastatic rhabdomyosarcoma (RMS) to identify prognostic variables and determine the value of high-dose chemotherapy (HDCT) in complete remission. Patients and Methods A total of 174 patients aged 3 months to 18 years participated. From 1989 to 1991, patients received four cycles of intensive multiagent chemotherapy. From 1991 to 1995, patients achieving complete remission received consolidation with HDCT. All received local therapy (surgery, radiation therapy) according to response. Results At a median follow-up of 8 years, 5-year overall survival (OS) and event-free survival (EFS) for the whole group were 24% and 20%, respectively. No statistical difference was found between HDCT and standard chemotherapy (5-year OS, 36% v 27%; EFS 29% v 23%). Univariate analysis identified primary tumor in parameningeal, extremity, or other sites; age younger than 1 year and older than 10 years; bone or bone marrow metastases; multiple metastases; and multiple sites of metastases as unfavorable prognostic factors for OS and EFS. Multivariate analysis identified unfavorable site, bone or bone marrow involvement, and unfavorable age as independently unfavorable factors. Two subgroups were identified. Those with fewer than two unfavorable factors had 5-year EFS and OS of 40% and 47%, respectively. Patients with ≥ two unfavorable factors had 5-year EFS and OS of 7.5% and 9%, respectively. Conclusion A minority of patients with metastatic RMS have better survival than overall results for this population suggest. Those in the highest risk group have such poor survival that they are candidates for first-line novel therapies. There is no evidence that consolidation with HDCT improves outcome.

Prognosis of rhabdomyosarcoma of the infratemporal fossa is generally poor. We report our experience.Twenty-three children with rhabdomyosarcoma of the infratemporal fossa were treated at the Gustave Roussy and Curie Institutes between 1984 and 1999. Seventeen children received radiotherapy (group 1), four children were treated surgically with or without postoperative radiotherapy (group 2), and two children received no further treatment after chemotherapy (group 3).In group 1, there were 10 local recurrences and one metastatic failure; all 11 children died from their disease. Nodal recurrence was salvaged successfully in one other with chemotherapy and surgery. In group 2, three children were disease free at 3 years and one died of local recurrence. In group 3, one child experienced a local recurrence successfully salvaged with radiotherapy. This child was disease free at 3 years. The other child developed local recurrence and died.Overall survival rate in our patients was 50%. Local control remained the main prognostic factor for survival. Surgery has already been shown to improve local control in other localizations of rhabdomyosarcoma. If residual tumor tissue remains after neo-adjuvant chemotherapy, surgery should be considered in a multidisciplinary discussion of therapeutic options. Indications for postoperative radiotherapy depend on age and histological features of the surgical specimen.

From 1984 to 1997, 57 consecutive patients with non-metastatic Ewing's sarcoma of the ribs were treated according to multimodal French Society of Pediatric Oncology (SFOP) protocols EW 84, EW 88 and EW 93. The results of treatment were reviewed and analysed. Median age was 12 years. 34 patients had large tumours (greatest tumour dimensionor = 8 cm); pleural effusion was noted in 26. A tumour-positive margin after surgery was noted in 15 patients. Histological response after chemotherapy was assessed in 34 patients. 34 patients received radiation therapy. With a median follow-up of 5 years, the projected overall and relapse-free survival rates were 69 and 62%, respectively. The major site of relapse was local. None of the following was significant in predicting relapse: tumour size, gender, age at diagnosis, existence of pleural effusion, level of rib tumour, rib component, type of local control, surgical margin (positive or negative). Response to chemotherapy was the sole significant prognostic factor (P=0.004). Patients with pleural effusion had a higher percentage of relapse if they were treated without local radiation therapy. Our study confirms the prognostic significance of response to initial chemotherapy. Radiation therapy may be withheld in selected cases, but seems necessary in patients with pleural effusion.

The occurrence of secondary chromosome changes is frequent in Ewing tumors, in particular trisomies for chromosomes 8 and 12, and unbalanced (1;16) translocations leading to gains of 1q and losses of 16q. The prognostic value of these secondary aberrations has not been statistically demonstrated. We report here a CGH analysis of a series of 43 primary tumors corresponding to 21 localized and 22 metastatic tumors. For five of them, a sufficient amount of DNA for the CGH analysis was available from the frozen samples. For 19 samples, a preliminary step of DOP-PCR amplification of the DNA was necessary. For the last 19 tumors, DNA was obtained after DOP-PCR amplification of small amount of DNA contaminating the RNA. As a whole, the main chromosome imbalances previously described, such as trisomies for 1q, 8, and 12, were observed. It is noteworthy that the mean number of imbalances was more frequent in localized versus metastatic tumors. Gain of 1q was more frequent in metastatic than in localized tumors. Nevertheless, these two results do not reach statistical significance. Conversely, a statistically significant excess of copy number of chromosome 2 was observed in non-metastatic tumors, suggesting that this imbalance, which has never been previously reported, could be associated with more favorable tumor behavior.

The MINE regimen (mitoguazone, ifosfamide, vinorelbine and etoposide) is a salvage chemotherapy for relapsed and refractory Hodgkin's disease.We report the cases of a 16-year-old girl and a 17-year-old boy who had Hodgkin's disease and developed painful and massive subcutaneous inflammatory edema after MINE chemotherapy. Morphine was unable to control pain leading to major functional disability of joint movement. One patient had an elevated creatine kinase level, hypoalbuminemia, hypodermic and muscular edema at magnetic resonance imaging and diffuse hemorrhagic hypodermic edema at skin biopsy. The other patient was found to have only hypoalbuminemia. The clinical course was favorable in both cases within a few weeks, but with recurrent episodes of pain and localized areas of fat necrosis five months later in one case.This side effect of MINE chemotherapy - subcutaneous inflammatory edema, myalgia and skin pain - has not been described previously for the different components of the regimen. Three clinicopathological hypotheses could be put forward: capillary leak syndrome, panniculitis, toxic fasciitis. The causal drug remains undetermined, but the most likely would be vinorelbine because of the chronology of the eruptions during the first and last days of chemotherapy and because of the known vascular toxicity of vinorelbine which could explain a capillary leak syndrome.

Brachytherapy has been widely used at the Institut Gustave-Roussy since 1972 in pediatric oncology. In genitourinary rhabdomyosarcoma, because of its ballistic and physical characteristics, it represents the optimal treatment whenever irradiation is required and brachytherapy feasible. Between 1976 and 1998, 23 children with bladder or prostate rhabdomyosarcoma were treated with a protocol including brachytherapy, with five of them treated with a salvage brachytherapy. All but one brachytherapy was performed during the surgery. Among the 18 brachytherapies performed as a first-line treatment, eight presented a tumoral evolution: five presented a local evolution, one a local and nodal evolution and two a nodal evolution. Brachytherapy allowed a conservative treatment among ten out of 11 children alive with no evidence of disease. Among the five patients with salvage brachytherapy, two presented a second recurrence. Sequelae were minimal, consisting of one grade I rectitis and one asymptomatic vesical and ureteral reflux. These results are consistent with the published data using more radical treatment. Brachytherapy can represent an alternative to radical surgery, when indications are clearly defined in bladder or prostate rhabdomyosarcoma. This type of treatment can be performed only integrated with other treatments, more particularly with surgery. This approach requires a close cooperation between the different specialists: pediatricians, surgeons and brachytherapists.

Ewing sarcoma is an uncommon malignancy that has been frequently managed differently in childhood and adults. This is a review of the international experience that conducted to a common European study called Euro Ewing. This study was launched in 1999.

The significant progress made in pediatric oncology during recent years has been due to a major breakthrough in the field of molecular biology and the introduction of new therapeutic strategies that take into account both the quality and the duration of life. Molecular biology has already been instrumental in more fully categorizing the 'small round-cell tumor' group, and in reclassifying the 'Ewing family' tumors. It also provides a valuable tool for the prognostic evaluation of neuroblastomas through the analysis of the N-myc oncogene. In addition, it has permitted the identification of the Li-Fraumeni syndrome of predisposition to cancer in the child, thereby raising the problematical ethical issue of communicating relevant information to subjects at risk. Two examples illustrate innovative strategic concepts: 1) Burkitt's lymphoma, or an example of the successful de-intensification of treatment; and 2) brain tumors in young children, regarding which the desire to improve the quality of life has led to innovative attempts to replace radiotherapy by chemotherapy. Considerable progress has been made in the field of neuropsychology, thereby permitting an improved assessment of disorders and a better management of rehabilitation programs. New anti-cancer agents and also chemo- and radiotherapy that spare healthy tissue are also being developed. Gene therapy and molecular biology will play a major role in future therapeutic strategies; and are now at the preclinical trial stage. This significant overall progress leads to a reconsideration of the organizational approach toward treatment of the pediatric patient population suffering from cancer, and a critical assessment of disease management, which should take into account not only the technical aspects of the disease but also familial and social considerations.

Malignant pheochromocytomas are rare in childhood and the prognosis of children with this tumor is not well known. We present 2 pediatric observations of malignant pelvic pheochromocytoma. Symptoms in both cases were headache and hypertension. The tumor invaded the sacral bone. Angiogram helped to localize the tumor and metastases, and allowed preoperative embolization of the tumor in 1 case. The first child underwent incomplete surgical resection, (131)I-MIBG therapy and radiotherapy, and is still alive 2 years after diagnosis. The second child died from metastatic invasion a few weeks after discovery of the tumor. We reviewed previous reports of children with malignant pheochromocytomas (30 cases). Primary tumor was extraadrenal in 50% of cases. The 3-year survival rate was 73 +/- 9% (mean +/- SD). Apart from surgical resection, no particular treatment appeared to be more effective than others in reducing mortality.

The high chemosensitivity of pediatric tumors along with their natural propensity for an early distant dissemination have stimulated the interest for chemo-radiation combinations in children since the mid 50s. Following the early experiments in nephroblastomas on the interaction of Actinomycin-D and radiotherapy, multiple national and international studies have been conducted since the mid 70s with considerable success: nowadays most pediatric tumors enjoy a long term survival in excess of 70%. Like their adult counterparts, these associations aim to induce an early control of the primary tumor and distant spreading (spatial cooperation) but also, more specifically in children, to limit the toxicity on normal tissues when treatment intensity can be further reduced. The association of an initial chemotherapy followed by local radiation at a dose and in a volume adapted to the response to chemotherapy along with associated prognostic factors has become widely tested in national and international studies conducted in Hodgkin's disease, Ewing's sarcoma, medulloblastomas, and brain tumors in the very young. Conversely, concomitant associations have remained limited to high-risk subgroups (parameningeal rhabdomyosarcomas for example) due to their potential hazards.

A cohort of 4,400 persons treated for various cancers of childhood in France and the UK was followed up over an extended period to assess risks of subsequent brain tumour in relation to the radiotherapy and chemotherapy that the children received for their first cancer. Elevated risks of subsequent brain tumours were associated with first central nervous system (CNS) tumour (two-sided p = 0.0002) and neurofibromatosis (two-sided p = 0.001). There was also elevated brain tumour risk (two-sided p = 0.003) associated with ionising radiation exposure, the risk being concentrated among benign and unspecified brain tumours. The radiation-related risk of benign and unspecified brain tumours was significantly higher than that of malignant brain tumours (two-sided por =0.05); there was no significant change of malignant brain tumour risk with ionising radiation dose (two-sided p0.2). In general, there were no strong associations between alkylating agent dose and brain tumour risk. The only significant association between brain tumour risk and alkylating agent dose was in relation to compounds used (bleomycin, chloraminophen) that are thought not to deliver substantial doses to the brain; the statistical significance of the trend with dose depended on a single case, and thus must be considered a weak result.

Although most pediatric tumors can be cured with lower doses of radiation than their adult counterparts, long-term radiation-induced complications and sequelae remain a major concern both in terms of frequency and intensity. Most of them have been extensively documented in the pre-chemotherapeutic era like those affecting bone, cartilage and soft tissue growth or CNS and endocrine glands. More recently the emphasis has been put on the apparent increasing incidence of reported second malignancies. This could have been favored by the chemo-radiation combinations used in most children but also has been made possible mainly by the extensive follow-up of the increasing cohort of cured children.

Malignant tumors of soft tissue constitute a very heterogeneous group of tumors which is composed of more than 50 different entities derived from either the mesenchyme or the neuroectoderm. Diagnostic problems can be linked to the difficulty to firmly establish the diagnosis of malignancy and to the precise determination of the type of sarcoma. The prognostic is largely dependent on local and distant extension of the cancer. The study of the genotype of tumor cells has enabled the detection and characterization of genetic alterations. These alterations, frequently chromosome translocations, are specific markers for subgroups of tumors and can thus be of clinical relevance. These markers can be detected by cytogenetic, in situ hybridization or molecular biology techniques. The specificity of these alterations for tumor cells and the possibility to detect them with the highly sensitive polymerase chain reaction (PCR) technique have enabled development of tests aimed at the detection of tumor cells within potential metastatic sites.