Evaluación urodinámica en pacientes sometidos a braquiterapia por rabdomiosarcomas genitourinarios / Urodynamic evaluation in patients undergoing brachytherapy for genitourinary rhabdomyosarcoma

Introducción: El rabdomiosarcoma (RMS) es uno de los tumores sólidos pediátricos más frecuentes. Se ha descrito que la braquiterapia es una herramienta prometedora para tratar esta patología, presentando menos secuelas en los tejidos vecinos. Este estudio tiene como propósito objetivar las secuelas de la dinámica vesical, a través de evaluación con estudios urodinámicos (EUD) a pacientes pediátricos sometidos a cirugía conservadora más braquiterapia por RMS de origen uroginecológico.P acientes y Métodos: Revisión de casos de RMS uroginecológicos desde 2004 a 2011, manejados con braquiterapia pre y/o post operatoria, más quimioterapia preoperatoria. Se realiza estudio urodinámico invasivo y/o no invasivo para evaluar secuelas en función miccional. Resultados: Serie de 4 casos de pacientes con RMS genitourinarios sometidos a braquiterapia sola o asociada a cirugía resectiva: 2 de próstata, 1 vaginal y 1 vesical. Los estudios urodinámicos muestran una capacidad vesical disminuida en 1 paciente, mínima disminución en otro y 2 presentan capacidad normal para la edad. La acomodación del detrusor es adecuada en los 4 pacientes y no hay alteraciones en la función esfinteriana. Clínicamente todos los pacientes son continentes y no presentan sintomatología de disfunción miccional. Conclusiones: El RMS genitourinario es un tumor altamente invasivo y secuelante. La braquiterapia ha logrado un nivel de radiación óptimo que se enfoca en el área tumoral sin afectar mayormente tejidos vecinos. Esto implicaría menos probabilidades de producir efectos secundarios en la dinámica vesical. Hemos demostrado en esta serie, con evaluación urodinámica, que estos pacientes con RMS presentan mínimas alteraciones en la evaluación urodinámica y no tienen repercusión clínica post tratamiento. Trabajos futuros están orientados a evaluación urodinámica pre y post braquiterapia.

Introduction: Rhabdomyosarcoma (RMS) is one of the most common pediatric solid tumors. It has been described that brachytherapy is a promising tool to treat this pathology, presenting less sequelae in neighboring tissues. This study aims to objectify alterations in bladder function through evaluation with urodynamic studies (UDS) in pediatric patients undergoing conservative surgery plus brachytherapy due to urogynecological RMS. Patients and Methods: A review of cases of RMS urogynecological from 2004-2011, managed with pre and / or post-operative brachytherapy, and preoperative chemotherapy. Invasive and / or noninvasive Urodynamic Studies are performed to assess sequelae in voiding function. Results: A series of 4 cases of genitourinary RMS patients undergoing brachytherapy alone or combined with resection surgery: 2 prostate cases, 1 vaginal and 1 bladder case. Urodynamic studies show a decreased bladder in 1 patient, minimal decrease in another and 2 show normal capacity for the patients age. Detrusor compliance is adequate in the 4 patients and there are no alterations in sphincter function. Clinically all patients are continent and show no symptoms of voiding dysfunction. Conclusions: Genitourinary RMS are a highly invasive and invalidating tumor. Brachytherapy achieves optimal radiation level that focuses on the tumor area without greatly affecting neighboring tissues.This could imply less likelyhood to cause side effects in bladder dynamics. We have shown in this series, with urodynamic evaluation, that these patients with RMS have minimal alterations in urodynamic Studies and have no post-treatment clinical impact. Future work is aimed at evaluating urodynamics before and after brachytherapy.

Novos conceitos e revisão atualizada sobre sarcomas uterinos / New concepts and update review of uterine sarcomas

Realizou-se uma revisão sistemática das publicações dos últimos dez anos sobre os sarcomas uterinos. Este artigo traz novos conceitos, como a mudança do carcinossarcoma para carcinoma metaplásico. Evidencia-se a necessidade de individualizar o tratamento dos sarcomas, pois existem muitas diferenças de comportamento clínico, padrão metastático e prognóstico entre os diferentes tipos histológicos. Também foram enfocadas as controvérsias existentes quanto a extensão cirúrgica, a necessidade de ooforectomia e de esvaziamento ganglionar e o papel da adjuvância

It was performed a systematic review of publications for the past 10 years about uterine sarcomas. This article introduces new concepts, such as the change of carcinosarcoma to metaplastic carcinoma. This study highlights the need to individualize the treatment of uterine sarcomas, as there are many differences in clinical behavior, prognosis and metastatic pattern between the different histological types. Controversies have also been focused on the surgical extension, the need for lymph node dissection and oophorectomy and the role of adjuvant therapy

Braquiterapia en urología pediátrica: ¿una mejor alternativa con menos secuelas? / Brachytherapy in pediatric urology: a better alternative with les sequels?

El rabdomiosarcoma (RMS) representa el 3° tumor sólido extracraneal pediátrico. El uso de braquiterapia nos ha dado una nueva herramienta en el tratamiento de esta patología. En este trabajo queremos reportar la experiencia del uso de cirugía más conservadora asociada a braquiterapia en pacientes con RMS urológicos. Método: Revisión de todos los casos del año 2004-2011 de RMS urológicos manejados con braquiterapia postoperatoria y/o intraoperatoria, asociado a quimioterapia (QMT) preoperatoria. Resultados: En los 8 años de estudio hubo 6 RMS; se incluyen 5pacientes dado que uno fue RMS testicular que no requirió radioterapia. Caso 1: Masculino de 2 años RMS embrionario Grupo IV Estadio 4 de próstata, con QMT según protocolo y braquiterapia en la semana 24 de QMT. Lleva 76 meses libre de enfermedad. Caso 2: Masculino de 5 años, RMS embrionario Grupo lll Estadio 2 en vejiga, con QMT preoperatoria (12 semanas), cirugía con resección de tumor en cara anterior de vejiga y braquiterapia; completó esquema QMT. Lleva 30 meses libre de enfermedad, sin alteraciones miccionales. Caso 3: Masculino de 7 años, RMS embrionario Grupo lll Estadio 3 de próstata, con QMT según protocolo y braquiterapia en la semana 22 de QMT. Lleva 17meses libre de enfermedad, función vesical e intestinal normal. Caso 4: Femenino de 4 años, RMSbotroide Grupo lll Estadio 1 de vagina, con quimioterapia preoperatoria (12 semanas), cirugia y braquiterapia postquirúrgica, completó esquema de QMT. Lleva 4 meses libre de enfermedad, sin alteración miccional ni intestinal. Caso 5: Femenino de 2 años, RMS embrionario Grupo lll Estadio 3de psoas con compromiso de vejiga, con cirugía, QMT según protocolo y radioterapia externa; pre-sentó recidiva local, por lo que inicia QMT, cirugía resectiva del tumor en cara posterior de vejiga y uréter derecho + radioterapia intraoperatoria con cono. Lleva 2 meses libre de enfermedad. Sin alteración miccional ni intestinal...

The rhabdomyosarcoma (RMS) represents the 3m’ extracraneal solid tumor in children. Brachytherapy use has given a new tool in this disease treatment. In this investigation we want to report the experience of conservative surgery associated to brachytherapy in patients with urological RMS. Method: Retrospective review of all the urological RMS cases between the years 2004-2011managed with postoperative and or intraoperative brachytherapy, associated to preoperative chemotherapy ( CM T). Results: ln a 8 year period there were 6 RMS; 5 are included because one case was a testicular RMS that did not required radiotherapy. Case 1: Two years old male children, prostatic group l V stage 4 embryonal RMS, with CMT according to protocol and brachytherapy in the 24”’ week of CMT Has been 76 months free of disease. Case 2: Five years old boy, bladder group lll stage 2embryonal RMS, with preoperative CMT (12 weeks), surgery including resection of tumor in the bladder anterior wall and brachytherapy,‘ Completed CMT protocol. Has been 30 months free of disease, without voiding disorders. Case 3: Seven years old boy, prostatic group lll stage 3 embryonal RMS, with CMT according to protocol and brachytherapy in 22'“ CMT week. Has been 17 months free of disease, normal bladder and intestinal function. Case 4: Four years old girl, vagina group lll stage 1botyroid RMS, with preoperative CMT (12 weeks), resective surgery and post-operative brachytherapy, completed CMT according to protocol. Has been 4 months free of disease, without voiding or intestinal disorders. Case 5: Two years old girl, psoas compromising bladder group llI stage 3 embryonal RMS, with surgery, CMT according to protocol and external radiotherapy. Presented local recurrence reason why initiates new CMT protocol, resective posterior wall bladder and right ureter surgery intraoperative radiotherapy cone. Has been 2 months free of disease. Without voiding or intestinal...

Spinal Cord Glioblastoma Induced by Radiation Therapy of Nasopharyngeal Rhabdomyosarcoma with MRI Findings: Case Report

Radiation-induced spinal cord gliomas are extremely rare. Since the first case was reported in 1980, only six additional cases have been reported.; The radiation-induced gliomas were related to the treatment of Hodgkin's lymphoma, thyroid cancer, and medullomyoblastoma, and to multiple chest fluoroscopic examinations in pulmonary tuberculosis patient. We report a case of radiation-induced spinal cord glioblastoma developed in a 17-year-old girl after a 13-year latency period following radiotherapy for nasopharyngeal rhabdomyosarcoma. MRI findings of our case are described.

Rabdomiossarcoma prostático em adulto: relato de caso / Prostatic rhabdomyosarcoma in an adult patient: a case report

O rabdomiossarcoma prostático é um tumor agressivo, encontrado predominantemente na infância, sendo bastante raro na vida adulta. Apresentamos o caso de um paciente de 27 anos de idade que tinha lesão extensa, com invasão dos planos periprostáticos e metástases a distância no momento do diagnóstico. Estudamos os achados aos exames de ultra-som, tomografia computadorizada, ressonância magnética e tomografia por emissão de pósitrons/tomografia computadorizada, correlacionando-os com os casos já descritos na literatura.

Prostatic rhabdomyosarcoma is an aggressive tumor predominantly found in children. The present paper reports a case of a 27-year-old-patient who had an extensive lesion invading periprostatic planes, with distant metastases found at the moment of the diagnosis. Imaging findings on ultrasound, computed tomography, magnetic resonance imaging, and positron emission tomography/computed tomography were evaluated and correlated with the ones already described in the literature.

Rhabdomyosarcoma of the larynx

Rhabdomyosarcoma of the larynx is a very unusual neoplasm and delays in diagnosis are common because the presenting symptoms are often mistaken for inflammatory or benign laryngeal disease, therefore a high index of suspicion is necessary to make diagnosis. We report a 13 years old girl with diagnosis of laryngeal Rhabdomyosarcoma. The optimum treatment of head and neck rhabdomyosarcoma has not been defined. Therapeutic modalities include an aggressive surgery without major morbidity. Radiotherapy and chemotherapy is also preferred for the treatment of rhabdomyosarcoma

Rhabdomyosarcoma: the experience of the pediatric Unit of Kasr El Aini Center of radiation oncology and nuclear medicine [NEMROCK] [from January 1992 to January 2001]

Our present study is a retrospective analysis of the treatment results of new rhabdomyosarcoma pediatric patients who had attended the pediatric unit clinic of Kasr El Aini Center of Radiation Oncology and Nuclear Medicine [NEMROCK] from January 1992 to January 2001]. Fifly-five new cases of pediatric rhabdomyosarcoma attended the pediatric unit outpatient clinic of [NEMROCK] from the period of January 1992 until January 2001. Patients were divided into 4 stages and classified into low-risk patients and high-risk patients according to the extent of resection. Stage I, II orbital and stage I para-testicular embryonal rhabdomyosarcomas received 32 weeks of vincristine and actinomycin-D [vincristine 1.5 mg/m2 weekly, actinomycin-D 0.015 mg/ Kg/day day ito day 5]. Other pathologies, sites and stages received 52 weeks of chemotherapy. Chemo-therapy regimens included VAC [vincristine 1.5 mg/m2 weekly, actinomycin-D 0.015 mg/Kg/day day ito day 5 and endoxan 2.2 gm/m2 I.V with mesna every 21 days], VAI [vincristine, actinomycin-D and ifosfamide 1.8 gmIm2 I.V day 1 to day 5 with mesna] or VIE [vincristine, ifosfamide and vepesid 100 mg/m2 I.V day 1 to day 5] 111, 12]. Stages I and II received conventional fractionation radiotherapy 414 OcGy on week 13, stages III and IV received conventional fractionation radiation therapy 5O4OcGy also, on week 13. The radiation volume included the tumor bed with a 2cm safety margin at least. Relapsing cases received palliative radiation therapy and chemotherapy [cisplatinum I.V 100 mg/m2 divided over 2 days and vepesid i00mg/m2 I.V day ito day 3 to be recycled every 21 days]. Patients were followed-up for 5 years, with a median follow-up of 36 months. Overall survival, disease free survival, treatment response, and complications of treatment were assessed and statistically analyzed. Fifty-five new cases of pediatric rhabdomyosarcoma attended the pediatric unit outpatient clinic of [NEMROCK] and were evaluated. Males constituted about 63.6% of the cases [35 cases] and females 36.4% [20 cases]. The median age was 6 years and the ages of the patients ranged from 1 to 9 years. Most of the cases were in early stages [40/55 i.e. 72.7%] versus late stages [15/55, i.e. 27.3%]. Pathologically, embryonal type was the commonest statistically [48/55, i.e. 87.3%] compared to the alveolar type [7/55, i.e. 12.7%]. Concerning site of the primary tumor it was found to be highest in the head and neck [20/55, i.e. 36.4%] followed by abdominal site [23.6%] excluding the genitourinary system which was classified separately because it included pelvis and abdomen [13/55, i.e. 23.6%]. The estimated 5-year Failure Free actuarial Survival [FFSR] for the entire study is 68% [n=55; 95% confidence interval [CI], 63% to 73%], and the estimated 5-year overall actuarial survival [OS] rate is 74% [95% CI, 69% to 79%]. Twenty cases experienced relapse during the 5 years follow up [i.e. 36.4%]. There was no lost follow-up in the selected group of children studied. In addition, only 3 cases showed distant metastasis at the onset of the study. Complete remission [CR] was achieved in 50.9% of the cases. Despite the advances in the therapy of rhabdomyosarcoma. Nearly 30% of the pediatric cases with rhabdomyosarcoma experience progressive or relapsing disease, which has a fatal end. The factors determining the 5-year survival after relapse at the time of initial diagnosis include histological subtype, and disease clustel. These findings will form the basis of a multi-institutional risk adapted relapse protocol for childhood rhabdomyosarcoma patients

Adult colonic rhabdomyosarcoma: a case report with review of literature

We report a 62-year-old male who presented with altered bowel habit, right hypochondriac pain, anorexia, vomiting and significant weight loss. A hard mass was palpable in the right lumbar region and iliac fossa on clinical examination. Colonoscopic biopsy revealed a malignant neoplasm of ascending colon. The patient underwent right radical hemicolectomy and histopathological examination of the resected specimen revealed alveolar rhabdomyosarcoma which is extremely rare in adults and very uncommon in the gastrointestinal tract

Sarcomas de partes moles / Soft tissue sarcomas

Definido o conceito de sarcomas e relacionando os principais tipos que ocorrem na infância, a autora destaca a importância e frequência do rabdomiossarcoma e estuda o quadro clínico desse tumor, em suas diferentes localizaçöes, a ocorrência de metástases, o diagnóstico clínico, radiológico e anatomopatológico, seu estadiamento, tratamento cirúrgico, radio e quimioterápico e os fatores prognósticos

Sarcoma de partes moles (rabdomiossarcoma embrionário): relato de um caso / Soft tissue sarcoma (embrionary rabdomiossarcoma): a case report

Os autores apresentam um caso de Rabdomiossarcoma embrionário, em criança de 5 meses de idade, incontrolável. As várias formas de terapêutica sao discutidas

Rabdomiosarcoma pleomórfico de cavidad oral: a propósito de un caso / Rhabdomyosarcoma of the oral cavity: a purpose of a case

Se describe un caso de Rabdomiosarcoma Pleomórfico en cavidad oral. Paciente masculino de 32 años, quién ingreso al Hospital General del Este "Dr. Domingo Luciani" del IVSS, en septiembre de 1992, presentando una tumoración que crecía progresivamente en cavidad oral, limitando apertura de la misma, la ingesta alimentaria y que recidiva posterior a la exéresis, no hubo precisión diagnóstica histopatológica, sino hasta el momento de haber sido iniciada la radioterapia, el diagnóstico histopatológico de certeza se realizó en EEUU. El tratamiento final fue éficaz, aunado a la radioterapia. La evolución postoperatoria es satisfactoria y actualmente se mantiene asintomático

Efectos de las radiaciones y de los quimioterápicos antimitóticos sobre la dentición (trad) / Implact of radiation and antimitotic chemotherapy on the teeth. Apropos of 3 cases

Los autores muestran la desorganización del desarrollo dentario en tres niños que han recibido radiaciones y una quimioterapia antimitótica. Ellos piensan que, en esos casos, la quimioterapia induce anomalías dentarias y, en un caso, un efecto acumulativo o de potenciación de radiaciones por la quimioterapia que no puede ser excluido. Los efectos secundarios de las irradiaciones sobre el sistema dentario son frecuentemente incorporados en los estudios de larga duración en niños que sobreviven a un cáncer. Las incidencias sobre el desarrollo de la dentición son varias: agenesia(s) dentarias(s), microdoncia, interrupción en el crecimiento de las raíces, raíces cortas y en V, cierre prematuro de los ápices, hipoplasia adamantina. Por el contrario, los efectos producidos por los quimioterápicos sobre el desarrollo de la dentición son mucho menos conocidos y son objeto de pocas publicaciones. La casi totalidad de los estudios han sido hechos sobre animales. Nos ha parecido por lo tanto interesante incluir tres observaciones de niños tratados muy tempranamente con quimoterápicos (un caso) y radioterapi + quimioterapia (dos casos). Estos pacientes, de 10 años y medio, 11 años y 16 años de edad, son actualmente seguidos en consulta ortodóncica y presentan daños en su sistema dentario que puden ser atribuidos principalmente a la quimioterapia

Rabdomiossarcoma ocular: estudo clínico de 12 casos / Ocular rabdomiossarcoma: clinical study of 12 cases

Foram avaliados 12 pacientes com rabdomiossarcoma ocular em relaç*o aos seguintes dados: sexo, idade, raça, tempo de história, localizaçäo do tumor, exame ocular e estudo anatomopatológico. Os resultados mostraram-se concordantes com a literatura. 8 casos tiveram um seguimento adequado tendo sido analisados em relaçäo ao tratamento, evoluçäo e complicaçöes. Os 2 pacientes que foram a óbito tinham sido submetidos a radioterapia isoladamente. A paciente que recebeu radioterapia e quimioterapia associada a exenteraçäo respondeu bem à terapêutica e pode ser considerada curada. Os demais pacientes foram tratados com quimioterapia e radioterapia, näo tendo apresentado recorrências até o momento, sendo que os autores concluem ser este o melhor esquema terapêutico