Gran tumor cardiaco de cavidades derechas con infiltración pulmonar en una mujer joven / Large right-cavity cardiac tumor with pulmonary infiltration in a young woman

Introducción: El diagnóstico diferencial de una masa intracavitaria incluye las variantes normales, los trombos, las vegetaciones y las neoplasias. Los tumores cardíacos primarios son poco frecuentes, con una incidencia que varía del 0,0017 por ciento al 0,28 por ciento y las metástasis aparecen entre el 1,5 por ciento al 20,6 por ciento de las necropsias de los enfermos neoplásicos. Objetivo: Presentar una paciente con masa en ventrículo derecho y vértice pulmonar derecho, con diagnóstico de rabdomiosarcoma cardíaco con metástasis pulmonar. Caso clínico: Paciente femenina de 46 años sin enfermedad previa ni hábitos tóxicos, que ingresa en el servicio de Medicina Interna del Hospital Militar Dr. Carlos J. Finlay, por falta de aire y síntomas dispépticos. Evoluciona de forma tórpida en 48 días, con progresión de la disnea, asociada a hipotensión y taquicardia. Hubo una sospecha inicial de tromboeembolismo pulmonar y luego de enfermedad neoplásica, intracavitaria o pulmonar. Conclusiones: El rabdomiosarcoma cardíaco es poco frecuente y se caracteriza por su crecimiento rápido que conduce a la muerte en semanas o meses, a partir del momento de su presentación clínica(AU)

Introduction: The differential diagnosis of an intracavitary mass includes normal variants, thrombi, vegetations and neoplasms. Primary cardiac tumors are rare, with an incidence ranging from 0.0017 percent to 0.28 percent and metastases appear in 1.5 percent to 20.6 percent of necropsies in neoplastic patients. Objective: To present a patient with a right ventricular and right lung apex mass diagnosed with cardiac rhabdomyosarcoma with pulmonary metastases. Clinical case: A 46-year-old female patient with no previous illness or toxic habits was admitted to the Internal Medicine Service of Military Hospital Dr. Carlos J. Finlay due to lack of air and dyspeptic symptoms. Torpid evolution in 48 days with progression of dyspnea associated with hypotension and tachycardia. Initial suspicion of pulmonary thromboeembolism and then neoplastic, intracavitary or pulmonary disease. Conclusions: Cardiac rhabdomyosarcoma is rare and characterized by rapid growth leading to death within weeks or months from the time of clinical presentation(AU)

Rabdomiosarcoma botrioide del aparato genital femenino en una adolescente virgen / Botryoid Rhabdomyosarcoma of the Female Genital Tract in a Virgin Adolescent

El rabdomiosarcoma es un tumor complejo y de gran malignidad que se origina en las células de la mesénquima embrionaria con capacidad para diferenciarse en células musculares esqueléticas. Este es el tumor maligno de tejido blando más frecuente. Representa aproximadamente 3,5 por ciento de los casos de cáncer en niños de 0 a 14 años de edad. Se presenta una paciente adolescente y virgen en la que se diagnostica histológicamente rabdomiosarcoma botrioide de la vagina. La presentación clínica del rabdomiosarcoma embrionario variedad botrioide es, en general, una masa que protruye por la uretra o el introito vaginal, o por la presencia de flujo fétido o sangrado vaginal en niñas menores de 2 años. En estas lesiones el apoyo diagnóstico con inmunohistoquímica es de vital importancia. El pronóstico de esta enfermedad está determinado por variables como el tamaño tumoral, órgano comprometido, edad del paciente, resultado quirúrgico (R0/R1) y presencia de metástasis. La evaluación médica multidisciplinaria precoz y oportuna permitirá siempre establecer un diagnóstico y tratamiento adecuados(AU)

Rhabdomyosarcoma is a complex and highly malignant tumor that originates in the cells of the embryonic mesenchyme with the ability to differentiate into skeletal muscle cells. This is the most common malignant soft tissue tumor. It represents approximately 3.5 percent of cancer cases in children from zero to 14 years of age. A case of a virgin adolescent patient is presented. A botryoid rhabdomyosarcoma of the vagina was diagnosed histologically. The clinical presentation of embryonic botryoid rhabdomyosarcoma variety is, in general, a mass that protrudes through the urethra or vaginal introitus, or the presence of fetid flow or vaginal bleeding in girls under 2 years. In these lesions, diagnostic support with immunohistochemistry is of vital importance. The prognosis of this disease is determined by variables such as tumor size, compromised organ, age of the patient, surgical result (R0 / R1) and presence of metastasis. Early and timely multidisciplinary medical evaluation will always allow an adequate diagnosis and treatment to be established(AU)

Rabdomiosarcoma botrioide del aparato genital femenino en paciente adolescente virgen / Botryoid rhabdomyosarcoma of the female genital apparatus in a virgin adolescent

El rabdomiosarcoma es un tumor complejo y de gran malignidad que se origina en las células del mesénquima embrionario con capacidad para diferenciarse en células musculares esqueléticas. Este es el tumor maligno de tejido blando más frecuente. Representa aproximadamente 3,5 por ciento de los casos de cáncer en niños de 0 a 14 años de edad. La presentación clínica del rabdomiosarcoma embrionario variedad botrioides es, en general, una masa que protruye por la uretra o el introito vaginal, o por la presencia de flujo fétido o sangrado vaginal en niñas menores de 2 años. Se presenta el caso de un rabdomiosarcoma botrioides de la vagina diagnosticado es una paciente de 16 años y virgen. El apoyo diagnóstico con inmunohistoquímica es de vital importancia y la evaluación médica multidisciplinaria precoz y oportuna permitirá siempre establecer un diagnóstico y tratamiento adecuados que mejoren el pronóstico de quienes padecen esta enfermedad(AU)

Rhabdomyosarcoma is a complex tumor of great malignity that originates in the embryonary mesenchymal cells with the capacity of differentiating into skeletal muscle cells. This is the most frequent malignant tumor in the soft tissue. It roughly represents 3.5 percent of cancer in children aged 0 to 14 years. Generally, the clinical presentation of botryoid-type embryonary rhabdomyosarcoma is a mass that protrudes from the urethra or the vaginal introit or the presence of fetid fluid or vaginal bleeding in girls under 2 years-old. This is the case of a 16 years-old virgin female patient diagnosed with botryoid rhabdomyosarcoma of the vagina. The diagnostic support with immunohistochemistry is of vital importance in addition to the early and timely multidisciplinary medical assessment for setting adequate diagnosis and treatment that improve the prognosis of persons suffering this disease(AU)

Le rhabdomyosarcome de l'oreille chez l'enfant. Etude d'une observation

Nous rapportons un cas de rhabdomyosarcome de l'oreille localement étendu diagnostiqué chez un enfant de 5 ans. Le retard diagnostique observé peut s'expliquer par le caractère aspécifique de la symptomatologie avec une localisation qui simule au début une otite moyenne chronique. A la lumière de cette observation et au regard d'une revue de la littérature, nous soulignons l'intérêt du diagnostic précoce afin d'améliorer le pronostic de cette pathologie

Rhabdomyosarcoma of Orbit: A Rare Case Report and Review.

Rhabdomyosarcoma (RMS) is a highly fulminant, mesenchymal malignant tumor and is considered to be one among life-threatening disease in the present decades. It is considered to be most common malignant neoplasm of the head and neck region with 10% of cases occurring in orbit. Th ough it is common in sixth and seventh decades, it can also occur in early childhood. In this article, we present a rare case of 9-yearold boy who was diagnosed with histopathologically proven RMS of orbit who had undergone a salvage left orbital exenteration following a chemotherapy. Th e main aim of this article is also to provide an overview of RMS of orbit, clinical features, investigations required, staging and various treatment modalities.

Rabdomiosarcoma de la vía biliar: Presentación de un caso / Rhabdomyosarcoma of the biliary tree: Case report

Se presenta el caso de una paciente de 21 meses con ictericia recurrente. La consulta inicial se había realizado debido a la aparición de febre de origen desconocido, pero después de 10 días se observó ictericia, acolia y prurito. Se efectuó una colangipancreatografía retrógrada endoscópica con esfnterotomía amplia, con lo que se logró la desaparición de los síntomas. Un mes más tarde, estos reaparecieron, por lo que, con sospecha de que se trataba de un quiste del colédoco, se realizó una nueva colangipancreatografía retrógrada endoscópica para confrmar el diagnóstico y colocar un stent para drenaje de la vía biliar. El material obtenido en el estudio se envió a anatomía patológica y se diagnosticó rabdomiosarcoma embrionario de la vía biliar. Se inició tratamiento con quimioterapia según el protocolo EpSSGRMS 2005. La niña no presentaba metástasis en el momento del diagnóstico. Completó el tratamiento y hasta la fecha de redacción de este trabajo, se encontraba libre de enfermedad.

We present a girl 21 months old with recurrent jaundice. Initially she presented fever of unknown origin but jaundice, white coloured stools and pruritus were observed 10 days later. She underwent endoscopic retrograde cholangiopancreatography with sphincterotomy; symptoms dissapeared. One month later, symptoms came back and, suspecting choledochal cyst the patient underwent endoscopic retrograde cholangiopancreatography for diagnostic confrmation and for placement of a biliary stent. The material obtained was sent for histopathology study and embryonal rhabdomyosarcoma of the biliary tree was diagnosed. The patient started chemotherapy following EpSSGRMS 2005 protocol. There was no evidence of metastasis. She completed treatment and to the day of this report she is free of illness.

Rabdomiossarcoma de cabeça e pescoço: 24 casos e revisão da literatura / Rhabdomyosarcoma of the head and neck: 24 cases and literature review

O rabdomiossarcoma (RMS) é o tumor maligno de partes moles mais comum na infância, localizando-se principalmente na cabeça e pescoço. Apresenta comportamento clínico-biológico variado, devendo receber terapia individualizada. OBJETIVO: Descrever os dados de pacientes portadores de RMS de cabeça e pescoço diagnosticados e tratados em um hospital comparando-os aos da literatura. Forma de Estudo: Descritivo retrospectivo. MATERIAL E MÉTODO: Análise retrospectiva de dados de prontuários de 24 pacientes portadores de RMS de cabeça e pescoço diagnosticados e tratados em um hospital no período de 1994 a 2008. RESULTADOS: A média de idade foi de 7,79 anos. Quanto ao sexo, encontramos 54,17 por cento do sexo masculino e 45,83 por cento do sexo feminino. Todos os pacientes foram submetidos a quimioterapia (QT), sendo que 62,5 por cento destes também realizaram radioterapia (RT) e 16,67 por cento foram submetidos a cirurgia. Dos 24 pacientes, 8 (33,3 por cento) foram a óbito, 6 (25 por cento) encontravam-se livres de neoplasia e 2 ( 8,3 por cento) apresentaram recidiva do tumor. CONCLUSÃO: O RMS de cabeça e pescoço frequentemente se apresenta com sintomas inespecíficos. Terapia multimodal individualizada deve ser realizada, incluindo cirurgia, quimioterapia e radioterapia.

Rhabdomyosarcoma (RMS) is a malignant tumor of soft tissues, more common in childhood, mainly located in the head and neck. It presents varied clinical and biological behavior and requires individualized management. AIM: To describe information on patients with head and neck RMS diagnosed and treated in a hospital, and to compare them to results in the literature. Study design: Descriptive and retrospective. MATERIALS AND METHODS: A retrospective analysis of data from 24 patients with head and neck rhabdomyosarcoma diagnosed and treated in a hospital from 1994 to 2008. RESULTS: The mean age was 7.79 years. According to gender, 54.17 percent were males and 45.83 percent were female. All patients underwent chemotherapy (CT), 62.5 percent of them also underwent radiotherapy (RT) and 16.67 percent were submitted to surgery. Of the 24 patients, 8 (33.3 percent) died, 6 (25 percent) were found free of neoplasia and 2 (8.3 percent) experienced tumor recurrence. CONCLUSION: The RMS of the head and neck often presents with nonspecific symptoms. Individualized multimodal therapy should be performed for these patients, including surgery, chemotherapy and radiotherapy.

Síndrome de Gradenigo como primeira manifestação de rabdomiossarcoma / Gradenigo's syndrome as a first manifestation of rhabdomyosarcoma

Introdução: Rabdomiossarcoma é uma neoplasia maligna com origem em músculos estriados e é responsável por aproximadamente 50% de todos os tumores de partes moles em crianças. O subtipo embrionário é o mais comum na região da cabeça e pescoço, sua localização na orelha média e mastoide é rara e de pior prognóstico devido à alta incidência de extensão intracraniana.Os sinais e sintomas dependem da localização do tumor. Objetivo: O objetivo desse relato de caso foi realizar uma breve revisão sobre a associação Rabdomiossarcoma e Síndrome de Gradenigo e elucidar a importância do diagnóstico diferencial nesse tipo de patologia. Relato do Caso: Nesse trabalho é relatado o caso de um menino de cinco anos de idade com o diagnóstico de Rabdomiossarcoma de osso temporal que se apresentou com o quadro de Síndrome de Gradenigo (paralisia do abducente, otorreia/otalgia, dor retro-orbitária) e paralisia do nervo facial.

Introduction: Rhabdomyosarcoma is a malignant neoplasm with origin from voluntary muscles and causes approximately 50% of all tumors of soft part in children. The embryonal subtype is the most common in the head and neck region, its location in the middle ears and mastoid is uncommon and of the worst prognosis due to the high incidence of intracranial extension. the signs and symptoms depend on the location of the tumor. Objective: The objective of this case report was to carry out a brief review on the Rhabdomyosarcoma and Gradenigo's Syndrome association and clarify the importance of the differential diagnosis in this kind of pathology. Case Report: In this work we report the case of a five-year-old boy with diagnosis of temporal bone Rhabdomyosarcoma that presented with a case of Gradenigo's Syndrome (abducens paralysis, otorrhea/otalgia, retroorbitary pain) and facial nerve paralysis.

Clinical characteristics and outcome of surgical treatment of childhood rhabdomyosarcoma: a 7-year experience

Background: The aim of this study was to describe the outcome and determine the prognostic factors of outcome of childhood rhabdomyosarcoma in a tertiary hospital in a developing country. Patients and Methods: This was a retrospective review of the clinical presentation; investigation; intervention; and treatment outcomes of children with rhabdomyosarcoma in our hospital over a 7-year period. Statistical analysis was performed using Chi-square test. Results: A total of 18 patients were identified with two-thirds being males (n = 12) with median age of 7 years. Most of the children were below 10 years of age. Lower limbs tumour predominated (n = 6) followed by the upper limbs and head and neck (n = 4 each). Other sites included perianal/perineal (n = 3) and the orbit (n = 1). Two patients were Intergroup Rabdomyosarcoma Study (IRS) group I; four group II; five group III; and seven group IV. Lymph node involvement was the commonest site of metastasis. Clinical group and stage was significantly more advanced in patients older than 10 years compared to younger than 10 years (P = 0.010; P = 0.008; respectively). There were 12 patients with alveolar disease while six had embryonal type of rhabdomyosarcoma. Treatment was by combination chemotherapy; and surgical excision which was done primarily in 11; after chemotherapy in four; and after radiotherapy in one. Two had biopsy only. Five patients are alive; two of them without evidence of disease at average follow-up period of 2 years. Conclusion: Mortality from rhadomyosarcoma in our setting is still unacceptably high. Late presentation may be the major contributor to high mortality. A more aggressive multimodality treatment approach may improve the outcome

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.

Bone marrow involvement at presentation in pediatric non-haematological small round cell tumours.

Pediatric small round cell tumors (SRCT) are a group of neoplasms occurring in children, which have in common a cytomorphology of groups of small round cells with scanty cytoplasm. The common SRCT encountered are neuroblastoma, retinoblastoma, Ewing's sarcoma/peripheral neuroectodermal tumor (PNET), rhabdomyosarcoma and lymphoma which show varying degrees of bone marrow involvement and bone marrow evaluation forms a part of the initial staging procedure. This study was undertaken to evaluate marrow involvement at presentation in pediatric non hematological SRCT. 7833 bone marrow aspirates done over a period of three years in different malignancies were analysed and of these 180 aspirates were performed in patients of pediatric non hematological SRCT at presentation. These cases were evaluated in detail for incidence of marrow involvement. Thirty two (17.7%) cases showed marrow involvement and these cases have been analysed with respect to the primary tumor. The SRCT showing involvement of bone marrow included neuroblastoma (48.8%), retinoblastoma (11.1%), Ewing's sarcoma/PNET (8.6%) and rhabdomyosarcoma (3.2%).These findings are discussed in the light of available world literature.

Rabdomiosarcoma embrionario parameníngeo del oido medio: a propósito de un caso: servicio de otorrinolaringología y oncología pediátrica / Rhabdomysarcoma embryonal parameningeo of ear middle: a purpose case: otolaryngology and oncology pediatric Hospital

El rabdomiosarcoma embrionario es un tumor altamente maligno, más frecuente en niños, que requiere un diagnóstico temprano para un mejor pronóstico de sobrevida. La presentación en el oído medio y mastoides es rara (8 por ciento), el tipo histológico más frecuente es el embrionario. La terapia multimodal con cirugía, quimioterapia y radioterapia se realiza actualmente con mejor pronóstico en sobrevida a tres años, pero sigue siendo un tumor altamente agresivo con extensión a meninges y pulmón. Presentamos el caso de una paciente de 6 años de edad, sexo femenino con diagnóstico de rabdomiosarcoma de oído medio mastoides con extensión parameníngea a quien realizó quimioterapia con respuesta adecuada. Por ser una patología muy poco frecuente queremos publicar este caso y realizar con una revisión de la literatura.

Orbital rhabdomyosarcoma: a case series.

Orbital rhabdomyosarcoma is the most common orbital malignancy of childhood with the common presentation of rapidly evolving unilateral proptosis. We studied six patients who were diagnosed and treated for rhabdomyosarcoma between January 1999 and June 2004. The age of the patients ranged from 4 to 29 years. Four patients presented with acute onset proptosis associated with signs of inflammation, mimicking orbital cellulitis. One patient presented with lid mass. Another patient presented with a soft, blind eye that was pushed superotemporally by a large inflammed, vascularised mass. Embryonal rhabdomyosarcoma was the commonest histopathological type in our series found in five patients. One patient was completely cured with chemotherapy alone whereas two patients were treated with a combination of chemotherapy and radiotherapy. Three patients in our series required exenteration.