Non-inferior efficacy of non-surgical treatment to surgical treatment in patients with nonmetastatic head and neck rhabdomyosarcoma: a SEER-based study

Purpose Head and neck rhabdomyosarcoma (HNRMS) is a rare but aggressive malignant neoplasm. Given the young patient age and critical anatomy of the head and neck, performing surgery on the primary tumor still remains debatable. This study aimed to evaluate the impact of the non-surgery-based treatment versus surgery-based treatment on patients with nonmetastatic HNRMS. Methods Patients diagnosed with nonmetastatic HNRMS between 2004 and 2015 from the Surveillance, Epidemiology, and End Results (SEER) database were enrolled in our study. Inverse probability treatment weighting (IPTW) method was employed to balance confounding factors between surgery and non-surgery groups. Kaplan–Meier methods and COX regression analyses were used to analyze survival outcomes of overall survival (OS) and cancer-specific survival (CSS). Prognostic nomogram was established to predict survival. Results A total of 260 eligible patients were extracted from the SEER database. Kaplan–Meier survival curves revealed that there was no significant difference in OS and CSS between the surgery and non-surgery groups both before and after IPTW (p > 0.05). Cox regression analyses and IPTW-adjusted Cox regression analyses for both OS and CSS showed similar survival between the two groups. Prognostic factors were explored and a nomogram for patients in the surgery group was constructed. Risk stratification based on the nomogram indicated that patients in surgery-high-risk group did not benefit from primary surgery. While those in surgery-low-risk group had an equal survival outcome to those in non-surgery group. Conclusions Our study revealed that compared to patients receiving surgery, those not receiving surgery had similar survival outcomes for nonmetastatic HNRMS. Our established nomogram may serve as a practical tool for individual prognostic evaluations (AU)

Rhabdomyosarcoma of the head and neck in pediatric patients: a systematic review

Background: Rhabdomyosarcoma (RMS) is a soft tissue malignant tumor of mesenchymal cell origin, which usually shows variable differentiation of muscle cells. It is the most common solid sarcoma in children. The most usual site of occurrence are the head and neck regions. RMS presents a variety of histologic features, and so differential diagnosis with other small round cell tumors is needed. Hence, it has been very useful to the field to undertake additional immunohistochemical studies to determine the diagnosis and, on occasions, to assign subtype tumors. Material and methods: A systematic review of three databases (Medline, Biological Science Collection and Health & Medical Collection) was carried out with the purpose of analyzing rhabdomyosarcoma cases reported in the literature, specifically with localization in the head and neck regions in children. This strategy allowed us to identify the main anatomical site of appearance, the subtype of RMS, average age, histologic characteristics and immunohistochemistry markers used in a usual and any additional way. Results: According to the selection criteria in this systematic review, twelve articles, and fourteen cases were identified that highlight that the histological diagnosis usually presents cellular heterogeneity. Therefore, immunohistochemistry is needed to confirm the diagnosis. Conclusions: Histologic characterization is not always sufficient for a conclusive diagnosis of RMS. Therefore, immunohistochemistry is helpful to determine the subtype and consequently, sometimes the behavior, treatment and prognosis. Additional markers may vary according to the institution and the need of particular cases. (AU)

Clinical manifestations of head and neck cancer in pediatric patients, an analysis of 253 cases in a single Brazilian center

Background: Pediatric head and neck cancer (PHNC) is rare and its nonspecific clinical manifestations may often lead to delayed diagnosis. We aimed to describe the signs, symptoms, and clinicopathological characteristics of PHNC. Material and Methods: Medical records were retrospectively reviewed for all PHNC cases diagnosed from 1986 to 2016 affecting patients aged 19-years and younger from a tertiary referral center in Brazil. Demographic variables, anatomical site of primary tumors, histopathological diagnoses, signs and symptoms, and patterns of misdiagnosis were collected and interpreted by statistical and descriptive analysis. Results: A total of 253 PHNC cases were included. The mean age was 9.3 years and male patients were more frequently affected (60.9%). Burkitt lymphoma (23.7%), nasopharyngeal carcinoma (15.8%), and rhabdomyosarcoma (15.4%) were the most common cancer types. The nasopharynx (28.9%), cervical/lymph node region (25.3%), and craniofacial bones (8.3%) were the predominant anatomical sites. Tumor/swelling (68.4%), was the clinical finding often presented. The univariable analysis showed association between tumor histology and clinical variables such as sex (p=0.022), age (p<0.0001), anatomical location (p<0.0001) tumor/swelling (p=0.034), pain (p=0.031), systemic/general manifestations (p=0.004), nasal/breathing alterations (p=0.012), orbital/ocular alterations (p<0.0001). Misdiagnosis such as tonsillitis, otitis, and abscess were frequent. Conclusions: Although the clinical findings of PHNC are often unspecific, this study provided signs and symptoms with significant correlations between tumor histology. The suspicion of malignancy should be considered when the main signs and symptoms reported here appear and persist, in order to conduct a timely diagnosis.(AU)

GEIS-SEHOP clinical practice guidelines for the treatment of rhabdomyosarcoma

Rhabdomyosarcoma (RMS) is the most frequent soft tissue sarcoma (STS) in children and adolescents. In Spain the annual incidence is 4.4 cases per million children < 14 years. It is an uncommon neoplasm in adults, but 40% of RMS are diagnosed in patients over 20 years of age, representing 1% of all STS in this age group. RMS can appear anywhere in the body, with some sites more frequently affected including head and neck, genitourinary system and limbs. Assessment of a patient with suspicion of RMS includes imaging studies (MRI, CT, PET-CT) and biopsy. All patients with RMS should receive chemotherapy, either at diagnosis in advanced or metastatic stages, or after initial resection in early local stages. Local control includes surgery and/or radiotherapy depending on site, stage, histology and response to chemotherapy. This guide provides recommendations for diagnosis, staging and treatment of this neoplasm (AU)

Abordaje multidisciplinario de un rabdomiosarcoma embrionario orbitario: a propósito de un caso / Multidisciplinary approach to orbital embryonic rhabdomyosarcoma: about a case

De las lesiones tumorales de cabeza y cuello en niños y adolescentes, un 5 a un 10% son tumores malignos primarios. Dentro de estos tumores destaca el rabdomiosarcoma orbitario infantil, que es el sarcoma primario de tejido blando más común en niños. Su diagnóstico requiere de un alto grado de sospecha clínica, que puede corroborarse con una serie de exámenes, para así estadificarlo e indicar el tratamiento adecuado. Actualmente se utiliza de primera intención cirugía y quimioterapia; el uso de la radioterapia convencional se limita a casos en los que fracasan los tratamientos anteriores o existe riesgo de recurrencia. El siguiente trabajo tiene como propósito exponer el cuadro clínico, diagnóstico, la estadificación y tratamiento integral de un rabdomiosarcoma embrionario orbitario, así como el manejo interdisciplinario que se realizó para mejorar el pronóstico de la paciente (AU)

Of the head and neck tumoral lesions in children and adolescents, 5 to 10% are primary malignant tumors. Among these tumors, orbital rhabdomyosarcoma stands out, which is the most common primary soft tissue sarcoma in children. Its diagnosis requires a high degree of clinical suspicion, and it can be corroborated with a series of examinations, in order to stage it and carry out the appropriate treatment. Currently, surgery and chemotherapy are the primary treatments, and the use of conventional radiotherapy is limited to cases where previous treatments fail or there is a risk of recurrence. The following case report aims to expose the clinical picture, diagnosis, staging and integral treatment of orbital rhabdomyosarcoma, as well as the interdisciplinary management that was performed to improve the patient's prognosis (AU)

Carcinoma urotelial sarcomatoide de vejiga en el adulto: histología, sintomatología, tratamientos y supervivencia / Sarcomatoid urothelial bladder carcinoma in adults: Histology, symptomatology, treatments and survival

Objetivo: El carcinoma urotelial sarcomatoide vesical representa el 0,3% de los tumores de vejiga y es considerado unos de los tumores más agresivos del tracto urinario. Nuestro objetivo es analizar las características del carcinoma urotelial sarcomatoide de vejiga en la edad adulta, tratamientos realizados y supervivencia. Método: Estudio retrospectivo entre el año 2000 y el 2017 de todos los pacientes con diagnóstico de carcinoma urotelial sarcomatoide de vejiga en un solo centro. Se analizan características anatomopatológicas, sintomatología en el momento del diagnóstico, tratamiento realizado y supervivencia según tratamiento llevado a cabo. Resultados: Dieciséis pacientes fueron diagnosticados de carcinoma sarcomatoide, 11 sin componente heterólogo, uno con componente rabdomiosarcoma, 2 con componente condrosarcoma y 2 con componente osteosarcoma. La edad media fue de 74 años (± 20) y el 88% eran fumadores. El síntoma principal fue la hematuria y el peor tolerado la disuria conjuntamente con el dolor hipogástrico. El 94% de los pacientes presentaron infiltración de la capa muscular y el 18% metástasis en el momento del diagnóstico. En el 37% de los pacientes se realizó tratamiento mediante cistectomía radical, en el 13% mediante cistectomía radical más quimioterapia adyuvante y en el 50% mediante resección transuretral de forma paliativa para conseguir el control de los síntomas. Se realizó una curva de supervivencia con los diferentes tratamientos realizados, presentando una supervivencia media global de 7 meses y sin observar diferencias estadísticamente significativas en cuanto a la supervivencia según el tratamiento realizado. Conclusiones: El carcinoma urotelial sarcomatoide es una enfermedad agresiva, de evolución rápida y tórpida que se produce en pacientes de edad avanzada y fumadores. No existen pautas de tratamiento establecidas y no parece que ningún tratamiento influya en un aumento de la supervivencia de los pacientes. Se debe de valorar la cistectomía como alternativa de tratamiento en aquellos pacientes de difícil control sintomatológico. Los diferentes componentes heterólogos no parecen influir en la evolución de la enfermedad ni en la supervivencia del paciente

Objective: Sarcomatoid urothelial bladder carcinoma comprises 3% of the tumours of the bladder and is considered one of the most aggressive tumours of the urinary tract. Our aim is to analyse the characteristics of sarcomatoid urothelial bladder carcinoma in adults, its treatments and survival. Method: A retrospective study performed between 2000 and 2017 of all the patients with a sarcomatoid urothelial bladder carcinoma in a single centre. We studied the anatomopathological characteristics, symptoms at time of diagnosis, treatment given and survival according to the treatment given. Results: Sixteen patients were diagnosed with sarcomatoid carcinoma, 11 with no heterologous component, one with rhabdomyosarcomatous components, 2 with chondrosarcomatous components and 2 with osteosarcomatous components. The mean age was 74 years (± 20) and 88% were smokers. The primary symptom was haematuria, and the least well-tolerated was dysuria together with hypogastric pain. Ninety-four percent of the patients had muscle layer infiltration and 18% had metastases at the time of diagnosis. Thirty-seven percent of the patients were treated by radical cystectomy, thirteen percent by radical cystectomy plus adjuvant chemotherapy, and 50% were treated by palliative transurethral resection to control their symptoms. A survival curve was made with the different treatments given, which showed a mean global survival of 7 months and no statistically significant differences in terms of survival according to the treatment given. Conclusions: Sarcomatoid urothelial carcinoma is an aggressive disease, of rapid and torpid onset which occurs in patients of advanced age and smokers. There are no established treatment guidelines, and it appears that no treatment influences increased survival. Cystectomy should be evaluated as a treatment alternative for patients whose symptoms are difficult to control. The various heterologous components do not appear to influence the progression of the disease or patient survival

Oncología quirúrgica pediátrica / Pediatric surgical oncology

Las neoplasias suponen la primera causa de muerte en niños mayores de 1 año en países desarrollados. la cirugía pediátrica juega un importante papel terapéutico en el cáncer infantil a distintos niveles: extirpación tumoral, implantación de accesos vasculares, manejo de metástasis y complicaciones. La oncología pediátrica es una especialidad muy dinámica con actualizaciones y cambios en protocolos constantemente. el objetivo de este artículo es la revisión actualizada de los tumores sólidos más frecuentes y con características específicas en relación con la cirugía pediátrica: neuroblastoma, tumor de Wilms, hepatoblastoma, tumores ováricos, testiculares y de partes blandas. El neuroblastoma es el tumor sólido extracraneal más común en la infancia. tanto el neuroblastoma como el tumor de Wilms suelen presentarse como una masa abdominal asintomática. la combinación del grupo de riesgo, edad, factores biológicos y resultados histológicos permite asignar a cada paciente un estadio de riesgo con valor pronóstico, y establecer una estrategia terapéutica específica en cada caso de neuroblastoma. el nefroblastoma o tumor de Wilms es el tumor renal maligno más frecuente en niños. en nuestro medio el nuevo protocolo establecido por la Siop se conoce como UMBrella que se basa en la quimioterapia preoperatoria con el objetivo de reducir masa tumoral seguido de cirugía. En los casos de hepatoblastoma es importante la clasificación preteXt (Pretreatment Extent of Disease) porque define la extensión de parénquima hepático afectada y enfermedad extrahepática dando información sobre la resecabilidad del tumor y su respuesta a la quimioterapia. Ante una masa de tejido blando debe plantearse en primer lugar que se trate de procesos reactivos y tumores benignos, los tumores malignos de partes blandas son raros. dentro de estos, el rabdomiosarcoma se caracteriza por su buena respuesta a quimioterapia, por lo que está en desuso la cirugía con resecciones agresivas o mutilantes. Respecto a los tumores gonadales en la infancia, a diferencia de los adultos, predominan los germinales, en concreto el teratoma. la cirugia tiende a ser conservadora para respetar la fertilidad futura. en las últimas décadas, los avances en tratamientos oncológicos han logrado un aumento de la supervivencia en la mayoría de tumores infantiles, en parte gracias al enfoque multidisciplinar necesario desde el diagnóstico de cada caso

Malignant neoplasms constitute the first cause of death in children over 1 year of age in developed countries. Pediatric surgery plays an important therapeutic role in childhood cancer on different sides: removing tumors, placing vascular access devices, metastasis management and complications. Pediatric oncology is a dinamyc specialty with constant updates and changes in protocols. the principal aim of this report is an updated review of more common solid tumors and their specific surgical aspects: neuroblastoma, Wilms' tumor, hepatoblastoma, ovarian tumor, testicular tumor and rhabdomyosarcoma. Neuroblastoma is the most common extracranial solid tumor in chidhood. Wilms' tumor and neuroblastoma usually appears as an asyntomatic abdominal mass. the risk group, age, biological factors and histologic analysis allow to assign a risk stage to each patient, that has prognostic value and determines the specific treatment for each case of neuroblastoma. Nephroblastoma or Wilms' tumor is the most frequent malignant kidney tumor in children. UMBrella is the new protocol of Siop based on preoperative chemotherapy to reduce the size of the tumor and surgery after. The classification PRETEXT (Pretreatment Extent of Disease) is important in cases of hepatoblastoma because defines the extent of hepatic infiltrated parenchyma, so we can know about its resectability and the response to chemotherapy. A soft-tissue mass is probably a reactive process or a benign tumor, since malignant soft-tissue tumors are rare. rhabdomyosarcoma has a good response to chemotherapy, so wide resections and radical surgery are not current techniques. With regard to gonadal tumors in childhood, unlike adults, germ tumors predominate, in particular teratoma. Sparing surgery is the current treatment to preserve future fertility. over the last several decades, the advances in cancer treatment have achieved an increased survival in most of childhood tumors, thanks to multidisciplinary approach from diagnosis

Linicopathological analysis of head and neck rhabdomyosarcoma: A series of 10 cases and literature review

Background: To describe the clinicopathological characteristics of a series of head and neck rhabdomyosarcoma (RMS) and to review the literature. Material and Methods: Cases diagnosed as RMS affecting the head and neck region were retrospectively retrieved from the files of two Brazilian institutions from January 2006 to January 2017. Data on clinical features (sex, age and affected site), microscopic subtype, immunohistochemical results, treatment employed and follow-up status were obtained from the patient's medical charts. Results: During the period considered, 10 cases of RMS were identified. Females predominated (4M:6F), the mean age at diagnosis was 16.5 years-old and the orbit was the most affected site (4 cases). Microscopically, most cases were classified as embryonal RMS (6 cases) and the Desmin/Myogenin/Myo-D1 immunohistochemical positivity was useful to confirm the diagnosis. Chemotherapy and radiotherapy were applied to 9 and 8 patients respectively, whereas 2 patients were treated by surgery. Recurrences occurred in 3 patients and distant metastasis in 2 cases. Nine patients were alive in their last follow-up, 3 of them with disease, whereas 1 patient died due to the disease. Conclusions: Head and neck RMS is an aggressive malignant neoplasm which demands especial concern to achieve early diagnosis and successful treatment (AU)

No disponible

Rabdomiosarcoma mediastínico diagnosticado mediante punción aspiración guiada por ecografía endobronquial / Mediastinal rhabdomyosarcoma diagnosed by endobronchial ultrasound-guided transbronchial needle aspiration

El rabdomiosarcoma es una neoplasia mesenquimal maligna y, además, es la segunda neoplasia cardiaca después del angiosarcoma, y su incidencia global es inferior al 0,07%. Otras denominaciones que se le han dado son rabdomioblastoma, miosarcoma y rabdomioma maligno. Habitualmente la clínica de presentación es inespecífica, y el diagnóstico se alcanza con el estudio histológico e inmunohistoquímico de la lesión obtenida mediante biopsia intracavitaria o bien abierta. Presentamos el caso de un paciente de 35 años, con clínica de disnea y alteraciones electrocardiográficas al que se le diagnostica de un rabdomiosarcoma mediante una ultrasonografía endobronquial (EBUS). Revisada la bibliografía existente este es el primer caso descrito de diagnóstico de rabdomiosarcoma mediante la punción de una adenopatía mediastínica por EBUS (AU)

Rhabdomyosarcoma is a malignant mesenchymal neoplasm with an incidence of less than than 0.07%, although it is the second most frequent cardiac neoplasia after cardiac angiosarcoma. It has also been named rhabdomyoblastoma, myosarcoma and malignant rhabdomyoma. Its clinical presentation is usually non-specific and histological and immunohistochemical studies of the lesion obtained by intracavitary or open biopsy are required for diagnosis. We report a case of a 35 year old patient, with dyspnea and electrocardiographic changes, diagnosed with rhabdomyosarcoma by endobronchial ultrasound (EBUS). To our knowledge, this is the first case of a rhabdomyosarcoma diagnosed by puncturing a mediastinal adenopathy using EBUS (AU)

Un tumor genitourinario infrecuente: sarcomas paratesticulares / Paratesticular sarcoma: An infrequent genitourinary tumor

OBJETIVO: Exponer los aspectos relacionados con el diagnóstico, terapéutica y seguimiento de los sarcomas paratesticulares, a partir de la presentación de tres casos con diferente histología. MÉTODO: Descripción de los casos clínicos, el manejo quirúrgico, y los resultados anatomopatológicos de las piezas quirúrgicas. RESULTADOS: Presentamos tres casos de pacientes con sarcomas paratesticulares, un caso de rabdomiosarcoma y dos de liposarcoma. Dos pacientes tuvieron una primera y única cirugía exitosa, mientras que el tercero precisó de reintervención tras recidiva. Actualmente los tres se encuentran libre de enfermedad. CONCLUSIONES: Los sarcomas paratesticulares son neoplasias infrecuentes en urología. Es esencial que el urólogo sospeche esta posibilidad ante un tumor paratesticular, pues la radicalidad en la cirugía será el factor más determinante en el éxito del tratamiento. Las terapias adyuvantes deben individualizarse en cada caso, y el seguimiento tras la cirugía conviene que sea estrecho, dada la mala evolución de estos tumores en muchos casos

OBJECTIVE: To expose the features related to the diagnosis, therapy and follow-up of paratesticular sarcomas, through the presentation of three cases with different histologies. METHODS: Description of the clinical cases, surgical management, and pathological results of the surgical specimens. RESULTS: We present three cases of paratesticular sarcomas, one case being a rhabdomyosarcoma and two liposarcomas. Two patients underwent a single successful surgery, while the third one required a second intervention after recurrence. Today all three patients are free of disease. CONCLUSIONS: Malignant paratesticular sarcomas are infrequent neoplasias in urology. It is essential that the urologist is aware of this possibility when faced with a paratesticular tumor, since radicalness of surgery will be the most decisive factor in the success of the treatment. Adjuvant therapies must be individualized in each case, and the follow-up after surgery should be close, given the poor evolution of these tumors in many cases

Unilateral breast metastasis from alveolar rhabdomyosarcoma in adult detected by 18F-FDG PET/CT / Metástasis mamaria unilateral de rabdomiosarcoma alveolar en un adulto, detectada por 18F-FDG PET/TAC

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Rabdomiosarcoma pleomórfico de mama en el varón. Descripción de un caso y revisión de la literatura / Pleomorfic rhabdomyosarcoma of the male breast. Report of a case and review the literature

El cáncer de mama en el varón tiene una incidencia estimada del 0.5-1% representado el sarcoma menos del 1%. Su presentación en la edad adulta es excepcional, siendo más frecuente en la infancia. Los hallazgos en la mamografía son inespecíficos, la ecografía parece tener mayor utilidad y el Tac y la RMN se utilizan para la estadificación. Se recomienda la realización de una biopsia con aguja gruesa. El tratamiento de elección es la mastectomía simple sin linfadenectomía ya que no suele existir afectación ganglionar. El tratamiento adyuvante aporta pocos beneficios en adultos. El pronóstico suele ser desfavorable

Male breast cancer has an estimated incident of 0.5-1% and sarcoma represent less than 1%. Adult presentation is exceptional, being most common in childhood. Mammography findings are nonspecific, ultrasound appears to have greater utility and the CT and MRI are used for staging. Core needle biopsy is recommended. The treatment of choice is simple mastectomy without lymphadenectomy because usually there is not lymph node involvement. Adjuvant therapy has poor benefits in adults. The prognosis is usually unfavorable

Rabdomiosarcoma genitourinario en la infancia, nuestra experiencia / Genital-urinary rhabdomyosarcoma in childhood: our experience

Introducción. El rabdomiosarcoma (RMS) constituye el tumor de tejidos blandos más frecuente en la edad pediátrica, representando el 2-3% de los tumores infantiles. La localización genitourinaria es la segunda en frecuencia tras la cabeza y cuello. El tratamiento suele ser médico, quedando la cirugía como coadyuvante, excepto en casos no controlados en que se precisan cirugías muy agresivas. El objetivo del estudio es analizar los casos de RMS de localización genitourinaria tratados en nuestro Centro y el papel que la cirugía tiene en su tratamiento. Material y métodos. Estudio retrospectivo de 20 pacientes (7 niñas y 13 niños) con una mediana de edad de 24 meses (rango de 1 mes a 12 años) con RMS del tracto urogenital tratados en nuestro Hospital desde 1990 hasta 2012. Se describen variables demográficas, localización del tumor primario, estadio al diagnóstico, tratamiento recibido, tanto médico como quirúrgico, con especial atención al tipo de cirugía realizada y seguimiento en términos de supervivencia. Resultados. La localización del tumor primario fue: vejiga (6), paratesticular (5), vagina (3), retroperitoneo (3), pelvis menor (2) y próstata (1). Todos recibieron tratamiento médico con quimioterapia y radioterapia según protocolo de la Sociedad Internacional de Oncología Pediátrica (SIOP) previa biopsia diagnóstica. La cirugía, practicada en todos los casos como coadyuvante fue: reevaluación por biopsia (1), orquiectomía (5), resección tumoral (8) y cirugía radical (cistoprosta-tectomía o exanteración pélvica) en 6 pacientes. Hubo 3 fallecimientos, 2 por progresión de la enfermedad y 1 por sepsis postoperatoria. Los 17 restantes están vivos, lo que supone una supervivencia del 80% con una mediana de seguimiento de 14 años. Conclusiones. El RMS es el tumor de tejidos blandos más frecuente en la infancia y la localización genitourinaria la segunda en frecuencia tras las parameníngeas. El tratamiento es multidisciplinar y la cirugía tiene un papel coadyuvante en casos de no respuesta al tratamiento médico o de tumor residual aunque hay pacientes que no responden al tratamiento médico y precisan de cirugía radical para su curación

Introduction. Rhabdomyosarcoma (RSM) becomes the most common tumour of the soft tissues during the paediatric age. It represents among 2-3% of child tumours. The genital-urinary location is the second most common location, only after head and neck. The treatment is usually medical, being the surgery a mere contribution, except for the cases in which the situation is not under control, when very aggressive surgery is necessary. The aim of this study is to analyse the cases of genial-urinary RMS that have been treated in our centre and the role that surgery has in their treatment. Material and methods. Retrospective study of 20 patient (7 girls and 13 boys) with a median age of 24 months (range from 1 month to 12 years) with RMS in the aurochs-genial tract who have been treated in our hospital from 1990 to 2012. The variables described are demographic, location of the primary tumour, state at diagnosis, received treatment, both medical and surgical, with greater emphasis on the kind of surgery applied and monitoring in terms of survival. Results. The location of the primary tumour was: bladder (6), par-atesticular (5), vagina (3) retroperitoneal space (3), lesser pelvis (2) and prostate (1). All of them received medical treatment with chemotherapy and radiotherapy following International Society of Pediatric Oncology protocol after diagnostic biopsy. Surgery, which was always used as help, was: reappraisal of biopsy (1), orchiectomy (5), tumoral resection (8) and radical surgery (cystoprostatectomy or pelvic exenteration) in 6 patients. There were 3 deaths, 2 because of the evolution of the disease and 1 because of postoperative sepsis. The survival rate is 80% with a median follow - up of 14 years. Conclusions. The RMS is the most common tumour of soft tissues in childhood and the genital-urinary location is the second most common after the parameningeal one. The treatment is multidisciplinary and the surgery has a contributing role when there is no answer to the medical treatment or when there is a residual tumour even if some patients do not respond to medical treatment and they need a radical surgery for recovery

Analgesia caudal continua guiada por ultrasonido en una paciente de 4 años / No disponible

La epidural caudal es la técnica más popular en anestesia y analgesia regional pediátrica. El empleo de una guía ecográfica en este procedimiento, aunque aún no es un estándar, podría disminuir los riesgos inherentes a la técnica tradicional y ofrecer algunas ventajas. Nosotros describimos el caso de una niña de 4 años sometida a una resección de un rabdomiosarcoma en muslo izquierdo con metástasis ganglionar inguinal e implantación de catéteres para braquiterapia; a quien se le colocó un catéter caudal para analgesia postoperatoria continua, usando la ultrasonografía (US) como método para guiar la colocación de dicho catéter. Después de inducir anestesia general, se realizó un escaneo ecográfico previo de la zona sacra identificando la anatomía, posteriormente después de implementar las medidas antisépticas y asépticas se colocó un catéter caudal guiado por US en tiempo real y con modo Doppler color se confirmó la posición en el espacio epidural caudal al inyectar una dosis en bolo de mezcla anestésica. Se presentó un adecuado control del dolor postoperatorio. El uso de US es una excelente alternativa a las técnicas clásicas fundamentadas en anatomía para la inserción de catéteres epidurales continuos en pediatría y permite ciertas ventajas que las técnicas a ciegas no pueden brindar (AU)

Caudal epidural is the most popular regional analgesia and anesthesia technique in pediatrics. The use of ultrasound (US) guidance in this procedure, is not yet the standard, but could reduce the risks related with the traditional approach and offer some advantages. We described a case of a 4-years-old patient undergoing a resection of a rabdomyosarcome on the left thigh plus inguinal metastatic nodes and implantation of brachitherapy catheters, in whom a continous caudal epidural catheter was placed under US guidance. After general anesthesia induction, a scout scanning identified the anatomy and afterwards, using strict aseptic techniques a caudal catheter was indwelling under the US guidance on real time and using the Doppler mode confirm the position of it inside the caudal epidural space with a local anesthetic bolus. There was an optimal pain control after surgery. The use of US as a guidance tool for caudal epidural catheter placement is an excellent alternative to the classic anatomical landmarks and give some advantages compare with those blind techniques (AU)

Tumores testiculares y paratesticulares en la infancia y adolescencia / Testicular and paratesticular tumors during childhood and adolescence

Introducción: Los tumores testiculares y paratesticulares constituyen el 1-2% de los tumores sólidos en la infancia. Se presenta una serie retrospectiva de 15 casos en menores de 18 años. Resultados: La edad media de los pacientes fue de 9,7 años, siendo prepuberales 6 (media 2,08 ± 1 año) y puberales 9 (media 15,1 ± 1,3 años). La forma de presentación clínica más frecuente fue una masa testicular indolora. Los niveles de alfa-fetoproteína estaban elevados en 5 pacientes (tumores de saco vitelino y carcinomas embrionarios).El estudio anatomopatológico demostró 11 tumores primarios testiculares y 4 paratesticulares (rabdomiosarcomas), siendo el 60% tumores germinales y el resto de células no germinales. El 60% de ellos fueron tumores malignos (2 tumores de saco vitelino, 2 carcinomas embrionarios, un seminoma y 4 rabdomiosarcomas). Entre los tumores benignos, el más frecuente fue el teratoma quístico maduro. La cirugía fue el tratamiento inicial en todos los casos (orquidectomía radical en 13 tumores y enucleación en 2 teratomas, con linfadenectomía retroperitoneal en 4 casos). En 11 de los pacientes el tumor se encontraba en estadio I y en 4 casos (2 carcinomas embrionarios y 2 rabdomiosarcomas), en estadio IV con metástasis pulmonares. Recibieron tratamiento adyuvante con quimioterapia asociada o no a radioterapia 7 de los pacientes (4 rabdomiosarcomas, 2 carcinomas embrionarios y un seminoma). Conclusiones: Los tumores testiculares y paratesticulares en niños prepuberales son un grupo con unas características epidemiológicas, histológicas, evolutivas y terapéuticas, bien diferenciadas respecto de las encontradas en pacientes pospuberales o adultos(AU)

Introduction: Testicular and paratesticular tumors represent 1-2% of the solid tumors in children. We present a retrospective series of 15 cases in patients less than 18 years of age. Results: The mean age of the patients was 9.7 yrs, 6 of them prepubertal (mean age: 2.08 ± 1 yrs) and 9 pubertal (mean age: 15.1 ± 1.3 yrs). The most common clinical form of presentation was a painless testicular mass. The alpha-fetoprotein levels were high in 5 patients (yolk-sac tumors and embryonal carcinomas).The pathological study showed 11 primary testicular tumors and 4 paratesticular tumors (rhabdomyosarcomas), with 60% being germinal tumors and the rest non-germinal. Around 60% were malignant tumors (2 from the yolk-sac tumors, 2 embryonal carcinomas, one seminoma and 4 rhabdomyosarcomas). Among the benign tumors, the most common was the mature cystic teratoma. Surgery was the initial treatment in all of the cases (radical orchiectomy in 13 tumors and enucleation in 2 teratomas, with retroperitoneal lymphadenectomy in 4 cases). In 11 patients the tumor was in stage I, while 4 cases (2 embryonal carcinomas and 2 rhabdomyosarcomas) were in stage IV with pulmonary metastasis. Chemotherapy whether or not combined with radiotherapy was applied in 7 patients (4 rhabdomyosarcomas, 2 embryonal carcinomas and one seminoma). Conclusions: Testicular and paratesticular tumors in prepubertal children show epidemiological, histological, therapeutical and evolutional characteristics well differentiated from postpubertal or adult subjects(AU)

Tumores mesenquimales: sarcomas de partes blandas / Mesenchymal tumors: soft tissue tumors

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Translocaciones cromosómicas en los sarcomas de partes blandas: de la biología molecular a la aplicación clínica / Chromosomal translocations in soft tissue sarcomas: from molecular biology to clinical application

Los avances recientes en el campo de la biología molecular de los sarcomas pediátricos, en especial el descubrimiento y caracterización de translocaciones cromosómicas específicas, han sentado las bases para la implantación de nuevas herramientas diagnósticas. En esta revisión se repasan las principales translocaciones asociadas a tumores pediátricos y se resumen sus características moleculares en relación a su capacidad oncogénica, su posible utilidad como herramientas de diagnóstico diferencial así como su posible relación con parámetros clínicos(AU)

Recent advances in the knowledge of the molecular biology of paediatric sarcomas, especially the characterisation of chromosomal translocations associated specifically with particular types of cancer, have established bases for the introduction of new diagnostic tools. This article reviews the main chromosomal translocations associated with paediatric tumours, and summarises their molecular characteristics regarding their oncogenic capabilities, possible usefulness as a differential diagnostic tools and possible correlation with clinical parameters(AU)