Primary biatrial cardiac Rhabdomyosarcoma

Abstract Primary malignant neoplasms of the heart are rare. Cardiac rhabdomyosarcoma is the second most common primary sarcoma. We report a rare case of a 49-year-old woman with a huge biatrial cardiac rhabdomyosarcoma treated by performing surgical resection followed by salvage chemotherapy for local recurrence. Cardiac sarcoma that occupy both atria are extremely rare. Although the prognosis of cardiac rhabdomyosarcoma is dismal, surgical resection should be recommended as a first line therapy to clarify the diagnosis and to relieve symptoms associated with the tumor.

Sarcomas de partes blandas en pacientes menores de 2 años. Serie de casos / Soft tissue sarcomas in infants. Case series

Los sarcomas de partes blandas en menores de 2 años son infrecuentes. Durante 2007-2017, 445 pacientes con sarcomas de partes blandas fueron tratados en nuestra Institución; 6 (el 0,5 %) eran menores de 2 años. Se analizaron los resultados clínicos y oncológicos en este grupo. La edad media de diagnóstico fue 15 meses. Cuatro eran varones y 2, mujeres. El seguimiento promedio fue 29 meses. El fibrosarcoma (n = 4) fue la variedad más frecuente. Cinco fueron tratados con cirugía de conservación del miembro; al restante se le realizó amputación. Todos realizaron tratamiento adyuvante con quimioterapia. La supervivencia a 24 meses fue del 100 %. Dos pacientes presentaron recidiva local; ambos casos, antes de los 24 meses.El tratamiento quirúrgico asociado a la quimioterapia impresiona ser la mejor opción terapéutica. La proporción de recurrencia local es alta para este grupo de pacientes luego de la cirugía de conservación del miembro.

Soft tissue sarcomas in children under 2 years of age are infrequent. During 2007-2017, a total of 445 patients diagnosed with soft tissue sarcomas were treated at our institution, 6 (0.5 %) were under 2 years. We analysed clinical and oncologic outcomes in this select group. The mean age of diagnosis was 15 months. Four patients were male and 2 female. The mean follow-up time was 29 months. Fibrosarcoma (n = 4) was the most frequent diagnosis. Five patients were treated with limb salvage surgery, and the remaining one had to undergo amputation. All patients received adjuvant treatment with chemotherapy. The 24-month survival rate was 100 %. Two patients presented a local recurrence before 24-months follow-up. Surgical treatment associated with chemotherapy seems to be the best therapeutic option. Local recurrence rate after limb salvage surgery is high for this group of patients

Autotrasplante cardiaco en el tratamiento quirúrgico de tumores cardiacos primarios: Caso clínico / Heart autotrasplantation for the treatment of a rhabdomyosarcoma of the left ventricle: Report of one case

Background: Heart autotrasplantation is an exceptional surgical technique used in the treatment of uncontrolled cardiac arrhythmias and primary unresectable cardiac tumors. We report a 28-year-old male with a rhabdomyosarcoma of the left ventricle, localized in the lateral and posterior wall, which involved the mitral valve and circumflex artery. After a complete study ruling out dissemination of the tumor, the patient was operated. Surgical exploration determined the unresectabilility of the tumor with the heart in situ. Therefore, the heart was explanted, preserving the right atrium and coronary sinus for re-implantation. Fifty percent of the mitral valve and the circumflex artery from its origin, were resected due to tumor infiltration. The heart was reconstructed with bovine pericardium and a mechanical valve was implanted in the mitral position. Afterward, the heart was implanted again following the same sequence as in bicaval transplantation, followed by a double bypass grafting to the distal circumflex territory. The patient had no significant complications and after nine months of follow up, there was no evidence of local recurrence. In the fourth postoperative month, a subcutaneous mass in the left thigh that was considered a metastasis without histological confirmation appeared. The lesion disappeared with radio and chemotherapy.

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...

Reconstrucción de partes blandas de la planta del pie: retrospective analysis of 12 patients / Soft tissue reconstruction of the sole: retrospective analysis of 12 patients

Background: An adequate reconstruction of foot soft tissue, specially in the sole, is crucial to restore functional capacity. Aim: To report the experience with reconstruction of sole soft tissues. Material and Methods: Retrospective analysis of 12 patients aged 19 to 72 years (seven males), subjected to reconstruction of soft tissue defects in the sole. Results: The lesions were secondary to excisions of malignant melanomas in seven patients, old traumatisms in two patients, a burn lesion in one patient and a diabetic ulcer in one patient. The reconstruction techniques used were skin grafts in one patient, local flaps in eight and free flaps in three patients. Five patients experienced complications and one of these required a new reconstructive surgery. Functional recovery was adequate in 11 patients. Conclusions: Reconstructive surgery of the sole can achieve an adequate functional recovery in most operated patients.

Se presenta una casuística de 12 pacientes que requirieron reconstrucción de la planta del pie en el Hospital de la Universidad Católica entre los años 2005 y 2008. La etiología principal fueron lesiones tumorales en 7 casos; las técnicas de reconstrucción fueron: injerto de piel (1 paciente), colgajos locales (8 pacientes) y colgajos libres (3 pacientes). Hubo complicaciones en 5 pacientes, uno de los cuales requirió una nueva cirugía reconstructiva. La recuperación funcional fue adecuada en 11 de los 12 pacientes.

Surgical treatment of primary cardiac rhabdomyosarcoma / Tratamento cirúrgico de rabdomiosarcoma primário do coração

The authors report a case of a 16-year-old man who presented progressive dyspnea. At that time the diagnosis of rheumatic fever with mitral valve involvement was performed. The bidimensional echocardiogram showed presence of mobile mass inside the left atrium. The tumor presented lobules, projecting into the left ventricle during the diastole and provoking turbulence. The patient underwent surgical resection with postoperative course needing re-operation for mitral valve replacement. Histopathology has proven that such tumor was a primary cardiac rhabdomyosarcoma and the early clinical diagnosis of rheumatic mitral valve disease was very difficult.

Os autores reportam caso de jovem de 16 anos, o qual apresentou dispnéia progressiva. No momento do atendimento foi feito diagnóstico de febre reumática com comprometimento da valva mitral. Ecocardiograma bidimensional demonstrou a presença de massa móvel dentro do átrio esquerdo. O tumor apresentava lobos, se projetando para o interior do ventrículo esquerdo durante a diástole, provocando turbulência. O paciente foi submetido a ressecção cirúrgica, complicada com reoperação e troca valvar mitral. A histopatologia demonstrou tratar-se de rabdomiosarcoma primário e o diagnóstico clínico diferencial com febre reumática e doença valvar mitral foi muito difícil desde o seu início.

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

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

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

Rabdomiosarcoma en la región cervicofacial: informe de dos casos / Cervicofacial rhabdomyosarcoma: report of two cases

Los rabdomiosarcomas (RMS) son los sarcomas de tejidos blandos más comunes en niños y 15 por ciento de los tumores sólidos tratados a nivel hospitalario en servicios pediátricos; se presentan con una amplia variedad morfológica, en diferentes órganos y con una gama de patrones histológicos, lo que puede dificultar su diagnóstico. Se reportan dos casos de rabdomiosarcoma, uno embrionario y otro alevolar, que se presentaron en la región preauricular y maseterina, respectivamente, en pacientes masculinos, diagnosticados y tratados en el Hospital Central Dr. Ignacio Morones Prieto SSA San Luis Potosí, SLP

Rabdomiosarcoma de vía biliar en la infancia, su tratamiento con cirugía conservadora video-laparóscopica / Biliary rhabdomyosarcoma in children: treatment with video assisted laparoscopic surgery

La escasa frecuencia del rabdomiosarcoma del árbol biliar extrahepátco,ha impedido la estandarización de su tratamiento.Se presenta en este trabajo la metodología diagnóstica y terapéutica aplicada a una paciente de 3 años de edad,portadora de divha patología.Se pone énfasis es que es el primer paciente tratado por cirugía videolaparóscopica,exponiéndose los resultados de la misma.Se discuten los protocolos de tratamiento

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

Rabdomiosarcoma de epididimo / Epididymal Rhabdomyosarcoma

Se presenta un tumor mesenquimatoso maligno originado en las estructuras paratesticulares, que corresponde a un radiomiosarcoma de epidismo. Se destaca su baja incidencia en la literatura mundial y su manejo terapeútico multidisciplinario (cirugia, radioterapia, quimioterapia). Fue intervenido en el Servicio de Urología de Antártida Hospital Privado, en el año 1993 y se presenta su evaluación y seguimiento hasta la fecha

Rabdomiossarcoma da órbita / Orbital rhabdomyossarcoma

Paciente de 59 anos de idade, sexo feminino, desenvolveu proptose súbita do olho direito, associada com dor e hiperemia. A tomografia computadorizada revelou a presença de uma massa sólida extraconal, na regiao medial-superior da órbita direita e opacificaçao dos seios etmoidais e maxilar direito. Foi submetida à remoçao, através do acesso de etmoidectomia externa, sendo o exame histológico consistente com rabdomiossarcoma embrionário, confirmado por avaliaçao imuno-histoquímica. A lesao recorreu 15 dias após o procedimento cirúrgico, quando foram identificadas células blásticas na medula óssea e envolvimento metastático da coluna lombar. A paciente morreu da doença dois meses após o procedimento cirúrgico inicial. De acordo com o nosso conhecimento, ela é paciente mais idosa com o diagnóstico de rabdomiosarcoma embrionário por imuno-histoquímica relatado na literatura.

Tratamiento quirúrgico de los tumores de la pared abdominal / Surgical treatment for tumors of the abdominal wall

Los tumores de la pared abdominal son poco frecuentes, predominan los tumores metastásicos a las vías de accesos a la cavidad peritoneal. Se presenta la técnica quirúrgica de extirpación de los tumores de la pared abdominal, así como la reparación con injerto libre o pediculado de fascia lata. En el lapso de 1970 a 1990, se han tratado 32 pacientes con esta técnica. 11 tumores de niños de tipo desmoide, 5 desmoides en mujees adultas y 6 tumores de tipo sarcoma en adultos y 10 tumores de tipo sarcoma en adultos y 10 tumores metastásicos implantados en las incisiones de laparotomía. Se comenta el posible aumento de implantes tumorales, por la información de la literatura de la presencia de este fenómeno en los puertos de entrada de los trócares de cirugía laparoscópica. Se informa de los posibles mecanismos de producción de este fenómeno y las posibles limitaciones que el manejo de las neoplasias de la cavidad abdominal por cirugía laparoscópica

Metástase linfonodal de sarcomade partes moles em adulto. Relato de caso / Lymph node metastasis from soft tissue sarcoma in adult. Case report

Os autores descrevem o caso de um paciente de 33 anos, branco, masculino, com rabdomiossarcoma de alto grau de malignidade da face anterior da coxa direita e metástase linfonodal na regiao ilíaca. Foi tratado com radioterapia pré-operatória, ressecçao ampla em monobloco com linfadenectomia iliinguinal e quimioterapia pós-operatória. Está vivo e assintomático sete anos e três meses após o tratamento. Teve como complicaçoes cirúrgicas imediatas alto débito de secreçao pelo dreno de sucçao e pequena área de deiscência com infecçao da ferida operatória. Como complicaçao tardia, apresentou fratura do colo do fêmur, que foi resolvida com cirurgia de Girdestone.

Sarcoma botrioide / Botrioide sarcoma

El sarcoma botrioides de localización uterina es muy poco frecuente, habiendo tenido su tratamiento y pronóstico una importante evolución a la fecha. Se presenta un caso clínico, analizando la experiencia al respecto