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1.
Ann Surg Oncol ; 30(7): 4515-4526, 2023 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-37160805

RESUMEN

BACKGROUND: The safety of multivisceral resection of retroperitoneal sarcoma is an issue. Previous reports have investigated its associations with the pattern of resection and factors recognized mostly per operatively. METHODS: All consecutive RPS resections from May 2015 to April 2022 were studied retrospectively with respect to adverse events. Two univariate and multivariate logistic regression analyses were performed to investigate the associations between severe adverse events and factors recognized pre- and per operatively. Associations of adverse events with overall survival (OS) and local recurrence (LR) were investigated. RESULTS: A total of 265 surgical interventions corresponding to 251 patients were recorded (38 RPS surgeries/year). Severe postoperative adverse events (Clavien-Dindo ≥ 3) occurred in 50 patients (18.9%), 15 (5.6%) patients underwent an iterative laparotomy, and 6 patients (2.3%) died within 90 days. On multivariate analysis including all parameters known preoperatively, male sex, performance status, dedifferentiated liposarcoma histology, and low serum albumin level were found to be significant predictors of major complications, whereas the timing of surgery and preoperative treatment were not. On univariate analysis including all per operative parameters, transfusion requirement, operative time, number of digestive anastomoses, and pancreas and/or major arterial resection were found to entail higher operative risk. On multivariate analysis, only transfusion requirement was significant. There was no impact of postoperative adverse events on OS or LR. CONCLUSIONS: The recognition of preoperative parameters that impact safety could mitigate the extent of the surgery, specifically the resection of adherent organs not overtly invaded. For the best decision, this surgery should be performed in referral centers.


Asunto(s)
Neoplasias Retroperitoneales , Sarcoma , Humanos , Masculino , Estudios Retrospectivos , Sarcoma/patología , Morbilidad , Neoplasias Retroperitoneales/patología , Recurrencia Local de Neoplasia/patología
2.
Pediatr Blood Cancer ; 70(11): e30627, 2023 11.
Artículo en Inglés | MEDLINE | ID: mdl-37580901

RESUMEN

PURPOSE: Three-dimensional conformal RT (3D-RT) techniques are gold standard for post-operative flank radiotherapy (RT) in paediatric renal tumours. Recently, highly conformal RT (HC-RT) techniques have been implemented without comparative clinical data. The main objective of this multicentre study was to compare locoregional control (LRC) in children treated either with HC-RT or 3D-RT techniques. METHODS: Patients treated with post-operative flank RT for renal tumour registered in the national cohort PediaRT between March 2013 and September 2019 were included. Treatment and follow-up data, including toxicities and outcomes, were retrieved from the database. LRC was calculated, and dose reconstruction was performed in case of an event. RESULTS: Seventy-nine patients were included. Forty patients were treated with HC-RT and 39 with 3D-RT. Median follow-up was 4.5 years. Three patients had locoregional failure (LRF; 4%). HC-RT was not associated with a higher risk of LRF. Three-year LRC were 97.4% and 94.7% in the HC-RT and 3D-RT groups, respectively. The proportion of planning target volumes receiving 95% or more of the prescribed dose did not significantly differ between both groups (HC-RT 88%; 3D-RT 69%; p = .05). HC-RT was better achieving dose constraints, and a significant mean dose reduction was observed in the peritoneal cavity and pancreas associated with lower incidence of acute gastrointestinal toxicity. CONCLUSION: LRF after post-operative flank RT for renal tumours was rare and did not increase using HC-RT versus 3D-RT techniques. Dose to the pancreas and the peritoneal cavity, as well as acute toxicity, were reduced with HC-RT compared to 3D-RT.


Asunto(s)
Neoplasias Renales , Radioterapia Conformacional , Niño , Humanos , Neoplasias Renales/radioterapia , Neoplasias Renales/cirugía , Dosificación Radioterapéutica , Planificación de la Radioterapia Asistida por Computador/métodos , Radioterapia Conformacional/efectos adversos , Radioterapia Conformacional/métodos
3.
Strahlenther Onkol ; 197(8): 690-699, 2021 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-33914102

RESUMEN

BACKGROUND: Treatment of extremity rhabdomyosarcomas (RMS) includes chemotherapy, surgery, and radiotherapy. Lymph node irradiation is recommended in the presence of regional node involvement at diagnosis. The aim of this study was to analyze the correlation between the pattern of relapse of non-metastatic extremity RMS and the initial therapies delivered. METHODS: All patients with localized extremity RMS prospectively treated in France in the MMT-95 and RMS-05 protocols were selected. Extent of disease and pattern of relapse were evaluated by clinical examination and imaging. RESULTS: We identified 59 patients with clinical characteristics corresponding to unfavorable prognostic factors. Twenty patients (34%) were considered to have lymph node involvement at diagnosis. Regional node biopsy was performed in 32 patients (54%) and modified the lymph node stage in 8 of the 59 patients (14%). Seventy-three percent of patients received radiotherapy. Fifty-two patients achieved first remission. Overall, 26 patients underwent complete tumor resection, 17 had R1 margins, and 5 were not operated due to early tumor progression. With a median follow-up of 82 months (range: 5-287), 18 relapses had occurred, at least locoregional in 12 cases. The 5­year local and nodal control rates were 73% (63-86%) and 86% (77-95%), respectively. Five-year progression-free and overall survival were 57% (95%CI [45-72%]) and 70% (95%CI [58-84%]), respectively. CONCLUSION: The main sites of extremity RMS relapse are locoregional. Nodal failures in non-irradiated fields are not uncommon. We recommend systematic biopsy of in-transit nodes, especially in alveolar RMS and/or RMS with regional positive nodes at diagnosis to ensure their negativity.


Asunto(s)
Extremidades/patología , Recurrencia Local de Neoplasia/patología , Rabdomiosarcoma/patología , Rabdomiosarcoma/terapia , Adolescente , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Niño , Preescolar , Femenino , Humanos , Lactante , Ganglios Linfáticos/patología , Masculino , Estadificación de Neoplasias , Estudios Retrospectivos , Rabdomiosarcoma/radioterapia , Rabdomiosarcoma/cirugía
4.
Pediatr Blood Cancer ; 68 Suppl 4: e29045, 2021 06.
Artículo en Inglés | MEDLINE | ID: mdl-33826235

RESUMEN

Pleuropulmonary blastoma (PPB) is a rare cancer occurring mainly during early childhood and often associated with germline DICER1 mutations. It is classified by the macroscopic appearance into three interrelated clinico-pathologic entities on a developmental continuum. Complete tumor resection is a main prognostic factor and can be performed at diagnosis or after neoadjuvant treatment that includes chemotherapy and in some cases radiotherapy. Optimal modalities of neo- or adjuvant treatments can be challenging taking into account potential long-term toxicities in this young population. This paper presents the recommendations for diagnosis and treatment of children and adolescents with PPB elaborated by the European Cooperative Study Group for Pediatric Rare Tumors (EXPeRT) within the European Union-funded project PARTNER (Paediatric Rare Tumours Network - European Registry).


Asunto(s)
Neoplasias Pulmonares , Blastoma Pulmonar , Adolescente , Niño , Preescolar , ARN Helicasas DEAD-box/genética , Humanos , Neoplasias Pulmonares/diagnóstico , Neoplasias Pulmonares/genética , Neoplasias Pulmonares/terapia , Terapia Neoadyuvante , Blastoma Pulmonar/diagnóstico , Blastoma Pulmonar/genética , Blastoma Pulmonar/terapia , Sistema de Registros , Ribonucleasa III
5.
Acta Oncol ; 60(2): 245-251, 2021 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-33095672

RESUMEN

OBJECTIVE: Radiation therapy (RT) is used for the treatment of sacral chordoma, in combination with surgery or alone for unresected tumours, to improve local control (LC) and potentially overall survival (OS). The purpose of the present study was to evaluate efficacy and toxicity of proton therapy (PT), and/or intensity modulated radiation therapy (IMRT), particularly Tomotherapy, for sacral chordoma treatment. Material: Between November 2005 and June 2018, 41 consecutive patients who were not included in clinical trials, received sacral chordoma radiation treatment in Institut Curie with Tomotherapy alone in 13 patients, and combined PT and Tomotherapy boost (Proton - Tomo) in 28 patients. RT was delivered as the exclusive local treatment in 11 patients, and as a post-operative complementary treatment in 30 patients. RESULTS: After a median follow-up of 46 months (range, 0-125 months), eight local relapses were observed, and seven patients developed distant metastasis (particularly bone and lung). The 2- and 5- year local relapse rates were 11.4% CI (0.65-22.2%) and 29% (10.5-47.4%), respectively. Over the follow-up period, ten patients died (24.4%). The estimated 2- and 5-year OS rates were 91.4% CI (82.5-100%) and 74.5% (59.4-93.5%), respectively. Fibrosis, cauda equina syndrome, and pain were the most common late toxicities. The comparison between Tomotherapy alone and Proton - Tomo revealed that acute and late cystitis were significantly more frequent in the Tomotherapy group: SHR = 0.12 IC95% (0.01-0.90 [p = .04]), as well as late proctitis. A dosimetric comparison confirmed the interest of PT to spare rectum and bladder in this context. CONCLUSION: RT remains essential to improve local control in sacral chordoma. The combination of proton and photon seems to improve organ at risk sparing, resulting in a decreased rate of reported late toxicities.


Asunto(s)
Cordoma , Terapia de Protones , Radioterapia de Intensidad Modulada , Cordoma/radioterapia , Humanos , Recurrencia Local de Neoplasia , Terapia de Protones/efectos adversos , Dosificación Radioterapéutica , Radioterapia de Intensidad Modulada/efectos adversos , Estudios Retrospectivos , Sacro , Resultado del Tratamiento
6.
Pediatr Blood Cancer ; 67(9): e28526, 2020 09.
Artículo en Inglés | MEDLINE | ID: mdl-32618059

RESUMEN

Testicular transposition (TT) before scrotal external radiotherapy (RT) is poorly reported in children with cancer, with only rare case reports published. TT surgical techniques, dosimetric parameters, and testicular functions are retrospectively reported in 12 children, median age 12.8 years, after scrotal RT for sarcomas. TT has low morbidity and allows a dramatic RT dose decrease in the healthy testicle. Endocrine functions seem preserved while more follow-up is needed to assess fertility. Though a rare situation, TT should be discussed in children and young adult cases when a scrotal high-dose RT is needed.


Asunto(s)
Preservación de la Fertilidad/métodos , Tratamientos Conservadores del Órgano/métodos , Escroto/efectos de la radiación , Neoplasias Testiculares/radioterapia , Neoplasias Testiculares/cirugía , Testículo/cirugía , Adolescente , Niño , Preescolar , Estudios de Seguimiento , Humanos , Masculino , Pronóstico , Estudios Retrospectivos
7.
Lancet Oncol ; 20(8): 1148-1159, 2019 08.
Artículo en Inglés | MEDLINE | ID: mdl-31296491

RESUMEN

BACKGROUND: Pathological complete response to preoperative treatment in adults with soft-tissue sarcoma can be achieved in only a few patients receiving radiotherapy. This phase 2-3 trial evaluated the safety and efficacy of the hafnium oxide (HfO2) nanoparticle NBTXR3 activated by radiotherapy versus radiotherapy alone as a pre-operative treatment in patients with locally advanced soft-tissue sarcoma. METHODS: Act.In.Sarc is a phase 2-3 randomised, multicentre, international trial. Adults (aged ≥18 years) with locally advanced soft-tissue sarcoma of the extremity or trunk wall, of any histological grade, and requiring preoperative radiotherapy were included. Patients had to have a WHO performance status of 0-2 and a life expectancy of at least 6 months. Patients were randomly assigned (1:1) by an interactive web response system to receive either NBTXR3 (volume corresponding to 10% of baseline tumour volume at a fixed concentration of 53·3 g/L) as a single intratumoural administration before preoperative external-beam radiotherapy (50 Gy in 25 fractions) or radiotherapy alone, followed by surgery. Randomisation was stratified by histological subtype (myxoid liposarcoma vs others). This was an open-label study. The primary endpoint was the proportion of patients with a pathological complete response, assessed by a central pathology review board following European Organisation for Research and Treatment of Cancer guidelines in the intention-to-treat population full analysis set. Safety analyses were done in all patients who received at least one puncture and injection of NBTXR3 or at least one dose of radiotherapy. This study is registered with ClinicalTrials.gov, number NCT02379845, and is ongoing for long-term follow-up, but recruitment is complete. FINDINGS: Between March 3, 2015, and Nov 21, 2017, 180 eligible patients were enrolled and randomly assigned and 179 started treatment: 89 in the NBTXR3 plus radiotherapy group and 90 in the radiotherapy alone group. Two patients in the NBTXR3 group and one patient in the radiotherapy group were excluded from the efficacy analysis because they were subsequently discovered to be ineligible; thus, a total of 176 patients were analysed for the primary endpoint in the intention-to-treat full analysis set (87 in the NBTXR3 group and 89 in the radiotherapy alone group). A pathological complete response was noted in 14 (16%) of 87 patients in the NBTXR3 group and seven (8%) of 89 in the radiotherapy alone group (p=0·044). In both treatment groups, the most common grade 3-4 treatment-emergent adverse event was postoperative wound complication (eight [9%] of 89 patients in the NBTXR3 group and eight [9%] of 90 in the radiotherapy alone group). The most common grade 3-4 adverse events related to NBTXR3 administration were injection site pain (four [4%] of 89) and hypotension (four [4%]) and the most common grade 3-4 radiotherapy-related adverse event was radiation skin injury in both groups (five [6%] of 89 in the NBTXR3 group and four [4%] of 90 in the radiotherapy alone group). The most common treatment-emergent grade 3-4 adverse event related to NBTXR3 was hypotension (six [7%] of 89 patients). Serious adverse events were observed in 35 (39%) of 89 patients in the NBTXR3 group and 27 (30%) of 90 patients in the radiotherapy alone group. No treatment-related deaths occurred. INTERPRETATION: This trial validates the mode of action of this new class of radioenhancer, which potentially opens a large field of clinical applications in soft-tissue sarcoma and possibly other cancers. FUNDING: Nanobiotix SA.


Asunto(s)
Hafnio/uso terapéutico , Nanopartículas/uso terapéutico , Óxidos/uso terapéutico , Fármacos Sensibilizantes a Radiaciones/uso terapéutico , Sarcoma/terapia , Neoplasias de los Tejidos Blandos/terapia , Adulto , Anciano , Anciano de 80 o más Años , Femenino , Humanos , Masculino , Persona de Mediana Edad , Radioterapia/métodos , Adulto Joven
8.
Strahlenther Onkol ; 195(6): 504-516, 2019 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-30963203

RESUMEN

PURPOSE: The aim of this study was to retrospectively study survival and long-term morbidities of children with nasopharyngeal carcinoma (NPC) treated by induction chemotherapy and concurrent chemoradiation (CRT). The total dose of radiation was adapted to the response following neoadjuvant chemotherapy. METHODS: Children with non-metastatic NPC treated in France between 1999 and 2015 were retrospectively included in the study. The strategy combined neoadjuvant platinum-based chemotherapy, followed by adapted CRT to tumor response. RESULTS: In total, 95 patients (median age 15 years [range, 7-23 years], male-to-female ratio 1.8) with undifferentiated NPC were included; 59% of patients had TNM stage IV. Intensity-modulated radiotherapy (IMRT) was delivered to 57 patients (60%), while the other patients were treated with conformal RT (3D-RT). After a median follow-up of 4.5 years [range, 3.6-5.5 years], 13 relapses and seven deaths had occurred. The 3­year overall and relapse-free survival (RFS) were 94% [95% CI, 85-97%] and 86% [77-92%], respectively. The locoregional failure rate was 6% [95% CI, 2-14]. Long-term treatment-related sequelae of grade 2+ were reported by 37 (50%) patients; odynophagia was significantly reduced treated by IMRT vs. conventional 3D-RT (7% vs. 55%, p = 0.015). Using a reduction dose of 59.4 Gy, 54 Gy, and 45 Gy, respectively, to the primary, involved, and uninvolved neck nodes, after a favorable tumor response, was not associated with an increased locoregional failure rate. CONCLUSIONS: The survival rates for NPC have been considerably improved by means of multimodal therapy, but long-term locoregional morbidity remains common. Use of IMRT may induce less residual odynophagia. Radiation dose reduction adapted to chemotherapy response does not have a negative impact on outcome. These findings support the use of an RT protocol adapted to the tumor response to neoadjuvant chemotherapy for a long-lasting improvement in the patient's quality of life.


Asunto(s)
Quimioradioterapia/métodos , Quimioterapia de Inducción , Carcinoma Nasofaríngeo/terapia , Neoplasias Nasofaríngeas/terapia , Adolescente , Niño , Terapia Combinada , Supervivencia sin Enfermedad , Femenino , Estudios de Seguimiento , Francia , Humanos , Masculino , Carcinoma Nasofaríngeo/mortalidad , Carcinoma Nasofaríngeo/patología , Neoplasias Nasofaríngeas/mortalidad , Neoplasias Nasofaríngeas/patología , Estadificación de Neoplasias , Radioterapia de Intensidad Modulada , Estudios Retrospectivos , Insuficiencia del Tratamiento , Adulto Joven
10.
BMC Cancer ; 17(1): 326, 2017 05 12.
Artículo en Inglés | MEDLINE | ID: mdl-28499444

RESUMEN

BACKGROUND: Survival of childhood, adolescent and young adult (CAYA) cancers has increased with progress in the management of the treatments and has reached more than 80% at 5 years. Nevertheless, these survivors are at great risk of second cancers and non-malignant co-morbidities in later life. DeNaCaPST is a non-interventional study whose aim is to organize a national screening for thyroid cancer and breast cancer in survivors of CAYA cancers. It will study the compliance with international recommendations, with the aim, regarding a breast screening programme, of offering for every woman living in France, at equal risk, an equal screening. METHOD: DeNaCaPST trial is coordinated by the INSERM 1018 unit in cooperation with the LEA (French Childhood Cancer Survivor Study for Leukaemia) study's coordinators, the long term follow up committee and the paediatric radiation committee of the SFCE (French Society of Childhood Cancers). A total of 35 centres spread across metropolitan France and la Reunion will participate. FCCSS (French Childhood Cancer Survivor Study), LEA and central registry will be interrogated to identify eligible patients. To participate, centers agreed to perform a complete "long-term follow-up consultations" according to good clinical practice and the guidelines of the SFCE (French Society of Children Cancers). DISCUSSION: As survival has greatly improved in childhood cancers, detection of therapy-related malignancies has become a priority even if new radiation techniques will lead to better protection for organs at risk. International guidelines have been put in place because of the evidence for increased lifetime risk of breast and thyroid cancer. DeNaCaPST is based on these international recommendations but it is important to recognize that they are based on expert consensus opinion and are supported by neither nonrandomized observational studies nor prospective randomized trials in this specific population. Over-diagnosis is a phenomenon inherent in any screening program and therefore such programs must be evaluated.


Asunto(s)
Neoplasias de la Mama/diagnóstico , Detección Precoz del Cáncer/métodos , Tamizaje Masivo/métodos , Neoplasias Primarias Secundarias/diagnóstico , Neoplasias de la Tiroides/diagnóstico , Mama/patología , Femenino , Francia , Humanos , Glándula Tiroides/patología
11.
Int Orthop ; 41(11): 2401-2405, 2017 11.
Artículo en Inglés | MEDLINE | ID: mdl-28842782

RESUMEN

PURPOSE: Local control of soft tissue sarcomas frequently involves adjuvant radiation to the surgical resection. When opting for post-operative radiation, care should be taken that radiation is started within some reasonable time after the surgery. We were interested to the proportion of patients who did not recieve optimal post-operative radiation and the variables associated. METHODS: We retrospectively analyzed a series of 77 patients operated on for an extremity soft-tissue sarcoma and due for post-operative radiotherapy. Patients were considered to have received radiation optimally if radiation was started within 12 weeks of surgery. Variables associated with not receiving radiation optimally were looked for using univariable and multivariable regression models. RESULTS: Overall, 26 patients (34%; 95% CI: 23-45%) did not receive radiation optimally. Twenty (26%) did not start radiation within the 12-weeks mark and six (8%) could not have radiation at all. The main reason identified for not receiving radiation on time was the occurrence of a wound complication (14 (54%) patients). An increased body mass index (OR: 1.14; 95% CI: 1.02-1.26; p = 0.02) and an older age (OR: 1.55; 95% CI: 1.18-2.14; p = 0.04; of note, the OR are for a 10 year change) were significantly associated with not receiving radiation optimally. Patients with a social fragility (p = 0.04), metastatic spread at presentation (p = 0.04), and increased duration of surgery (p = 0.02) were more likely to develop a wound complication. CONCLUSIONS: About 34% of patients do not receive optimal post-operative radiation treatment. Older and obese patients have a higher risk of not receiving radiation optimally. The decision for pre- or post-operative radiation should account for these findings.


Asunto(s)
Radioterapia Adyuvante/estadística & datos numéricos , Sarcoma/cirugía , Adulto , Anciano , Extremidades/patología , Femenino , Humanos , Masculino , Persona de Mediana Edad , Complicaciones Posoperatorias , Periodo Posoperatorio , Estudios Retrospectivos , Sarcoma/radioterapia
13.
Strahlenther Onkol ; 191(7): 604-12, 2015 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-25896312

RESUMEN

INTRODUCTION: Neuroblastoma (NB) is the most frequent indication for extracranial pediatric radiotherapy. As long-term survival of high-risk localized NB has greatly improved, we reviewed treatment-related late toxicities in pediatric patients who received postoperative radiotherapy (RT) for localized NB within two French prospective clinical trials: NB90 and NB94. PATIENTS AND METHODS: From 1990-2000, 610 children were enrolled. Among these, 35 were treated with induction chemotherapy, surgery, and RT. The recommended RT dose was 24 Gy at ≤ 2 years, 34 Gy at > 2 years, ± a 5 Gy boost in both age groups. RESULTS: The 22 patients still alive after 5 years were analyzed. The median follow-up time was 14 years (range 5-21 years). Late effects after therapy occurred in 73 % of patients (16/22), within the RT field for 50 % (11/22). The most frequent in-field effects were musculoskeletal abnormalities (n = 7) that occurred only with doses > 31 Gy/1.5 Gy fraction (p = 0.037). Other effects were endocrine in 3 patients and second malignancies in 2 patients. Four patients presented with multiple in-field late effects only with doses > 31 Gy. CONCLUSION: After a median follow-up of 14 years, late effects with multimodality treatment were frequent. The most frequent effects were musculoskeletal abnormalities and the threshold for their occurrence was 31 Gy.


Asunto(s)
Neuroblastoma/radioterapia , Traumatismos por Radiación/etiología , Radioterapia Adyuvante , Adolescente , Niño , Preescolar , Fraccionamiento de la Dosis de Radiación , Femenino , Francia , Amplificación de Genes , Humanos , Lactante , Masculino , Proteína Proto-Oncogénica N-Myc , Neoplasia Residual/mortalidad , Neoplasia Residual/radioterapia , Neoplasias Inducidas por Radiación/etiología , Neuroblastoma/genética , Neuroblastoma/mortalidad , Proteínas Nucleares/genética , Proteínas Oncogénicas/genética , Estudios Prospectivos , Dosificación Radioterapéutica , Análisis de Supervivencia
14.
Clin Exp Ophthalmol ; 43(1): 12-9, 2015.
Artículo en Inglés | MEDLINE | ID: mdl-24923774

RESUMEN

BACKGROUND: Orbital rhabdomyosarcoma (ORMS) is associated with an excellent survival rate greater than 85%, and is considered to be a favourable site for this tumour. Treatment is based on combination chemotherapy together with best local therapy, sometimes surgery but more often radiation therapy. Local therapy is associated with frequent and potentially severe late sequelae. DESIGN: Retrospective hospital single-centre analysis. PARTICIPANTS: Eighty-two patients treated in Institut Curie, Paris. METHODS: To define long-term status of survivors after localized ORMS, patients treated between 1975 and 2010 were analysed. MAIN OUTCOME MEASURES: Clinical structural and functional orbital, and general sequelae. RESULTS: Median age at diagnosis was 6 years (range: 8 months-19 years), and median follow up was 8.5 years (range: 7 months-24 years). The 5-year globe conservation rate was 90.4%. Ophthalmic dysfunction was present in 79% of patients. Impaired visual acuity (VA), was present in 62% of patients; 38% of them had severe visual disability with VA < 6/60. Late effects on orbitofacial structure were present in 39.8% of patients. Ocular or palpebral sequelae were present in 79% of survivors, mainly cataract (42%), ocular surface lesions such as keratoconjunctivitis (40%) and eyelid abnormalities (29%). General late effects were rare. CONCLUSIONS: These data suggest that ocular and orbital late effects are frequent after treatment of ORMS, indicating the need for systematic long-term ophthalmologic follow up of these patients. Radiation therapy is an important part of the total burden of therapy.


Asunto(s)
Neoplasias Orbitales/patología , Rabdomiosarcoma/patología , Adolescente , Antineoplásicos/uso terapéutico , Niño , Preescolar , Femenino , Estudios de Seguimiento , Humanos , Lactante , Masculino , Neoplasias Orbitales/mortalidad , Neoplasias Orbitales/terapia , Complicaciones Posoperatorias , Radioterapia , Estudios Retrospectivos , Rabdomiosarcoma/mortalidad , Rabdomiosarcoma/terapia , Tasa de Supervivencia , Adulto Joven
15.
J Pediatr Hematol Oncol ; 36(8): 605-12, 2014 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-25171453

RESUMEN

BACKGROUND: Orbital rhabdomyosarcoma (ORMS) treatment is based on combination chemotherapy associated with best local therapy, sometimes surgery but more often radiation therapy. A retrospective single-center analysis was conducted to more clearly define the long-term outcome of patients with ORMS, to identify patients in whom aggressive first-line local therapy can be avoided. POPULATION: A total of 95 patients with localized parameningeal (PM) or nonparameningeal (NPM) ORMS, treated at the Institut Curie between 1975 and 2010, were analyzed. RESULTS: Median age at diagnosis was 6 years (range, 8 mo to 19.5 y), and median follow-up was 8.5 years (range, 7 mo to 24 y). A total of 25 patients presented PM extension. Radiation therapy was part of primary therapy for 78 patients. Five-year event-free survival and overall survival rates were 65.4%±5.2% and 85.6%±3.9%, respectively. On multivariate analysis, initial tumor size was identified as a significant prognostic factor. Event-free survival was similar for PM and NPM tumors (60.3%±10.4% vs. 62.7%±5.9%, P=0.57), whereas there was a trend for overall survival to be better for NPM tumors (90%±3.9% vs. 72.7%±9.6%, P=0.07). CONCLUSIONS: Localized ORMS has a favorable outcome despite the current trend toward less aggressive and more limited indications of local therapy. Patients with a favorable pattern of strictly ORMS can be treated without first-line radiation therapy.


Asunto(s)
Quimioradioterapia/métodos , Neoplasias Orbitales/terapia , Rabdomiosarcoma/terapia , Adolescente , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Niño , Preescolar , Ciclofosfamida/uso terapéutico , Dactinomicina/uso terapéutico , Supervivencia sin Enfermedad , Epirrubicina/uso terapéutico , Estudios de Seguimiento , Humanos , Ifosfamida/uso terapéutico , Lactante , Estadificación de Neoplasias , Neoplasias Orbitales/diagnóstico por imagen , Neoplasias Orbitales/patología , Pronóstico , Cintigrafía , Estudios Retrospectivos , Rabdomiosarcoma/diagnóstico por imagen , Rabdomiosarcoma/patología , Resultado del Tratamiento , Vincristina/uso terapéutico , Adulto Joven
16.
J Pediatr Hematol Oncol ; 36(4): 257-62, 2014 May.
Artículo en Inglés | MEDLINE | ID: mdl-24633301

RESUMEN

Synovial sarcoma (SS) is a high-grade soft tissue sarcoma characterized by local invasiveness and a propensity to metastasize, affecting pediatric, adolescent, and adult populations. The peak incidence is observed in the third decade of life and SS is the most common nonrhabdomyosarcoma soft tissue sarcoma in childhood and adolescence. Although pediatric and adult SS appear clinically and radiologically identical, treatment modalities may differ according to the patient's age. For many years, pediatric oncologists have treated SS as a chemosensitive tumor according to the "rhabdomyosarcoma philosophy." In contrast, adult oncologists generally treat this tumor as a poorly chemosensitive tumor and focus on local control. The authors propose an update of SS in the pediatric population and analyze their results to those obtained in adults.


Asunto(s)
Sarcoma Sinovial/patología , Sarcoma Sinovial/terapia , Adolescente , Adulto , Niño , Preescolar , Femenino , Humanos , Incidencia , Lactante , Masculino , Invasividad Neoplásica , Sarcoma Sinovial/epidemiología , Factores de Tiempo
17.
Lancet Oncol ; 14(13): e601-8, 2013 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-24275133

RESUMEN

Ovarian transposition was the first procedure proposed to preserve fertility in girls with cancer and is indicated for patients with tumours requiring pelvic radiation at doses of 42·0-58·4 Gy, much higher doses than those that can induce loss of ovarian function (4-20 Gy). Ovarian transposition is usually done after neoadjuvant chemotherapy and is completed by minimally invasive surgery or open surgery in case of concomitant resection of the abdominal tumour. According to the type of tumour, the ovaries are moved and placed in the paracolic gutters when the radiation field reaches the midline (for medulloblastoma or urogenital rhabdomyosarcoma), contralaterally to the tumour (for pelvic sarcomas), or in line with the iliac crests (for Hodgkin's lymphoma). However, in 10-14% of cases the procedure can fail to protect the ovaries. Although few long-term results in adults are available, normal hormonal function and pregnancies have been reported in a few long-term follow-up studies. In view of the continued development of fertility preservation techniques, ovarian transposition should be discussed at a multidisciplinary meeting at the time of cancer diagnosis.


Asunto(s)
Preservación de la Fertilidad/métodos , Fertilidad/efectos de la radiación , Procedimientos Quirúrgicos Ginecológicos/métodos , Infertilidad Femenina/etiología , Infertilidad Femenina/prevención & control , Neoplasias/radioterapia , Ovario/cirugía , Adolescente , Neoplasias Cerebelosas/patología , Neoplasias Cerebelosas/radioterapia , Niño , Femenino , Estudios de Seguimiento , Enfermedad de Hodgkin/radioterapia , Humanos , Meduloblastoma/patología , Meduloblastoma/radioterapia , Procedimientos Quirúrgicos Mínimamente Invasivos , Neoplasias Pélvicas/radioterapia , Embarazo , Resultado del Embarazo , Radioterapia/efectos adversos , Dosificación Radioterapéutica , Rabdomiosarcoma/radioterapia , Neoplasias Urogenitales/radioterapia
18.
Eur J Cardiothorac Surg ; 66(1)2024 Jul 01.
Artículo en Inglés | MEDLINE | ID: mdl-38960727

RESUMEN

We report the case of a 5-year-old girl who underwent left pneumonectomy for Ewing sarcoma of the lung. Two expandable prostheses were placed in the left hemi-thorax to prevent post-pneumonectomy syndrome and to protect the heart from radiotherapy. With a follow-up of 10 years, the procedure proved to be effective both on post-pneumonectomy syndrome and on cardiac protection.


Asunto(s)
Neoplasias Pulmonares , Neumonectomía , Sarcoma de Ewing , Humanos , Sarcoma de Ewing/cirugía , Femenino , Neumonectomía/métodos , Preescolar , Neoplasias Pulmonares/cirugía , Implantación de Prótesis/métodos , Estudios de Seguimiento , Prótesis e Implantes , Tomografía Computarizada por Rayos X
19.
Eur J Surg Oncol ; 50(1): 107298, 2024 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-38086314

RESUMEN

BACKGROUND: Histologic subtype of cancer guides treatment sequencing and the extent of surgery for retroperitoneal tumours (RPTs) but concerns persist regarding percutaneous core needle biopsy (CNB). OBJECTIVE: Endpoints were the incidence of early complications, needle tract seeding (NTS) after CNB, diagnostic accuracy. METHODS: Between 2015 and 2022, data from patients with RPT who underwent a CNB and who operated on at Institut Curie were collected. We retrospectively reviewed the medical records and microscopic analysis of both CNB and surgical specimens to evaluate the diagnostic accuracy of CNB (quantified using positive and negative predictive values, PPV/NPV). RESULTS: 313 patients underwent CNB. In 10/326 (3 %) procedures, minor complications were observed. One of 212 (0.47 %) resected RPSs exhibited a local recurrence compatible with NTS. Microscopic analysis of CNB specimens allowed the classification of tumours between groups of cancers and benign/intermediate mesenchymal tumours in 307/313 (98 %) patients. Among the 204 patients with retroperitoneal sarcoma, the overall concordance between CNB and final pathology following resection was 178/204 (87.2 %). The respective PPVs of solitary fibrous tumour, dedifferentiated liposarcoma, leiomyosarcoma and well-differentiated liposarcoma were 100 %, 98 %, 97 % and 68 %, respectively. The diagnosis of a high-grade (G 2-3) sarcoma resulted in a high specificity (97 %) and PPV (98 %) but low sensitivity (76 %). CONCLUSIONS: CNB allowed the classification of RPT in the vast majority of patients with a low morbidity rate. Concordance with final diagnosis was high for sarcomas with the exception of well-differentiated liposarcoma. As a result, CNB results should be integrated with imaging/radiomics by multidisciplinary tumour boards.


Asunto(s)
Liposarcoma , Neoplasias Retroperitoneales , Sarcoma , Neoplasias de los Tejidos Blandos , Humanos , Biopsia con Aguja Gruesa/métodos , Neoplasias Retroperitoneales/cirugía , Neoplasias Retroperitoneales/patología , Estudios Retrospectivos , Sarcoma/cirugía , Liposarcoma/patología , Neoplasias de los Tejidos Blandos/patología
20.
Cancer Treat Rev ; 124: 102684, 2024 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-38278078

RESUMEN

BACKGROUND: Radiation therapy (RT) is essential in treating advanced lung cancer, but may lead to radiation pneumonitis (RP). This systematic review investigates the use of pulmonary function tests (PFT) and other parameters to predict and mitigate RP, thereby improving RT planning. METHODS: A systematic review sifted through PubMed and on BioMed Central, targeting articles from September 2005 to December 2022 containing the keywords: Lung Cancer, Radiotherapy, and pulmonary function test. RESULTS: From 1153 articles, 80 were included. RP was assessed using CTCAEv.4 in 30 % of these. Six studies evaluated post-RT quality of life in lung cancer patients, reporting no decline. Patients with RP and chronic obstructive pulmonary disease (COPD) generally exhibited poorer overall survival. Notably, forced expiratory volume in one second (FEV1) and diffusing capacity of the lung for carbon monoxide (DLCO) declined 24 months post-RT, while forced vital capacity (FVC) stayed stable. In the majority of studies, age over 60, tumors located in the lower part of the lung, and low FEV1 before RT were associated with a higher risk of RP. Dosimetric factors (V5, V20, MLD) and metabolic imaging emerged as significant predictors of RP risk. A clinical checklist blending patient and tumor characteristics, PFT results, and dosimetric criteria was proposed for assessing RP risk before RT. CONCLUSION: The review reveals the multifactorial nature of RP development following RT in lung cancer. This approach should guide individualized management and calls for a prospective study to validate these findings and enhance RP prevention strategies.


Asunto(s)
Carcinoma de Pulmón de Células no Pequeñas , Neoplasias Pulmonares , Neumonitis por Radiación , Humanos , Carcinoma de Pulmón de Células no Pequeñas/patología , Pulmón/patología , Neoplasias Pulmonares/radioterapia , Neoplasias Pulmonares/patología , Estudios Prospectivos , Calidad de Vida , Neumonitis por Radiación/etiología , Neumonitis por Radiación/prevención & control , Neumonitis por Radiación/patología , Medición de Riesgo
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