Rabdomiossarcoma embrionário de colo uterino: relato de caso em adolescente / Embryonic rabdomyosarcoma of the cervix: case report in a teenager

O rabdomiossarcoma embrionário, variante botrioide, é uma neoplasia maligna dos tecidos moles que deriva de células musculares mesenquimais embrionárias. Alguns fatores de risco genéticos são conhecidos, mas a doença geralmente se apresenta de forma esporádica. É raro manifestar-se em adolescentes, assim como é raro ser primário do colo uterino. Cursa com a presença de pólipos e até massas que se sobressaem na vagina com casos de sangramento vaginal anormal. O diagnóstico é realizado essencialmente pela história e exame anatomopatológico. Quanto maior o tempo para confirmação do diagnóstico, pior o prognóstico. Há várias modalidades de tratamento ­ que deve ser individualizado e envolver uma equipe multidisciplinar ­, que, basicamente, incluem quimioterapia, radioterapia e cirurgia. Os resultados geralmente são menos favoráveis em adolescentes, quando comparados com os de crianças com a mesma neoplasia.(AU)

Embryonic rhabdomyosarcoma, a botryoid variant, is a malignant neoplasm of soft tissues that derives from embryonic mesenchymal muscle cells. Some genetic risk factors are known, but the disease usually presents itself sporadically. It's rarely manifested in adolescents, just as it is rare to be primary in the cervix. It occurs with the presence of polyps and even masses that protrude in the vagina with cases of abnormal vaginal bleeding. The diagnosis is made essentially by history and anatomopathological examination. The longer the time to confirm the diagnosis, the worse the prognosis. There are several treatment modalities ­ involving a multidisciplinary team ­ that must be individualized and basically include chemotherapy, radiotherapy and surgery. The results are generally less favorable in adolescents, when compared with those of children with the same neoplasia.(AU)

Rabdomiossarcoma Embrionário: Relato de Caso com 15 Anos de Sobrevida e Revisão de Literatura / Embryonal Rhabdomyosarcoma: Case Report with a 15-year Survival and Literature Review

O rabdomiossarcoma de cabeça e pescoço é o sarcoma mais comum de tecido mole em crianças. O planejamento do tratamento depende da localização do tumor, extensão da doença e presença ou não de metástases.O tratamento pode causar diversas sequelas tardias na região de cabeça e pescoço, principalmente na cavidade oral. A prevenção e o controle dessas sequelas proporcionam uma melhor qualidade de vida para o paciente. Relato de caso: Este relato descreve um caso de rabdomiossarcoma embrionário parameníngeo em região de parótida diagnosticadoem um paciente do sexo masculino aos 3 anos de idade, tratado com quimioterapia e radioterapia. Esse paciente recebeu atendimento odontológico como parte integrante do tratamento multidisciplinar. Apresentou boa resposta ao tratamento, permanecendo em controle clínico sem evidência de doença por 15 anos. As sequelas tardias em face e cavidade oral do tratamento oncológico foram: fechamento precoce das raízes; rizogênese incompleta em todos os elementos dentários; agenesias dentárias; múltiplos dentes inclusos; hipoplasia dos ossos da face e trismo. Entretanto,teve qualidade de vida satisfatória, com manutenção da capacidade mastigatória, tendo frequentado a escola edesenvolvido um bom convívio social. Conclusão: O rabdomiossarcoma de cabeça e pescoço está associado a sequelas tardias decorrentes tratamento. A abordagem multidisciplinar é importante para a prevenção e o controle das sequelas e obtenção de uma melhor qualidade de vida para os pacientes...

Primary embryonal rhabdomyosarcoma of the liver in a young male

Rhabdomyosarcoma [RMS] occurs infrequently in the liver. Rhabdomyosarcomas are malignant tumours that display features of striated muscle differentiation. They are the most common soft-tissue sarcomas among children. In adults however, these are very rare. We report a case of a primary embryonal rhabdomyosarcoma of the liver in a 17 years old boy. This was confirmed by histological examination using immunohistochemical analysis [LCA negative, desmin positive, myogenin focally positive and cytokeratin negative] and site was confirmed by PET CT scan. He received multiple chemotherapies including [doxorubicin, ifosfamide, dacarbazine; gemcitabine, paclitaxel; vincristine, actinomycin D, cyclophosphamide] but longest sustained stable disease was seen with gemcitabine-paclitaxel regimen. The patient died 31 months after the first presentation, secondary to complicated abundant abdominal progressive disease. The poor prognosis and early death of most previously reported cases imply the need for investigation of a more effective treatment method of this uncommon tumour

Embryonal Rhabdomyosarcoma Arising from a Mediastinal Teratoma: An Unusual Case Report

We report an unusual case of 9.5-cm-sized embryonal rhabdomyosarcoma arose from a mediastinal mature teratoma in a 46-yr-old man. A man presented with chest trauma as a result of an accident at 10 September 2011. On chest X-ray, an anterior mediastinal mass was detected. To obtain further information, chest computed tomography (CT) with contrast enhancement was performed, revealing an anterior mediastinal mass. Complete surgical excision was performed and entire specimen was evaluated. Pathologic diagnosis was embryonal rhabdomyosarcoma arising in mature cystic teratoma. After surgical excision, two cycles of dactinomycin-based chemotherapy were performed. Lung metastasis was detected on follow up CT in September 2012, and wedge resection was performed. Pathological finding of the lung lesion showed same feature with that of primary rhabdomyosarcoma.

Rabdomiosarcoma embrionario nasal: actualización / Nasal embryonal rhabdomyosarcoma: update

El rabdomiosarcoma no es sólo el sarcoma de tejidos blandos más común en los niños menores de 15 años, sino también en adolescentes y adultos jóvenes. Presentamos el caso de un paciente de sexo masculino de 12 años de edad, quien desarrolló un tumor polipoideo que protruía en la fosa nasal derecha, diagnosticándose rabdomiosarcoma embrionario. Se realiza tratamiento quirúrgico y quimioterápico con muy buen resultado. El paciente después de dos años de su diagnóstico se encuentra libre de enfermedad. Realizamos una revisión de esta variante histopatológica de rabdomiosarcoma, su pronóstico y tratamiento

Case report of rhabdomyosarcoma in the maternity hospital-Damascus university

Rhabdomyosarcoma is the commonest soft tissue cancer of childhood accounting for 5% of all childhood malignancies. The tumor arises from embryonic mesenchymal tissue in any of various sites, the most frequent being head and neck [40%], GU tract [20%], and extremities [18%], the trunk [7%], retroperitoneum [7%] and perineum and other sites [8%]. Rhabdomyosarcomas have many presentations with widely different prognoses and the classification is controversial. Multivariate analysis of prognostic factors agree that the presence or absence of metastasis is the most important prognostic factor. The addition of chemotherapy to radiation therapy and surgery has dramatically improved survival in rhabdomyosarcoma in the last 20 years. R. Kh., a 15 - year old patient, referred to the Maternity Hospital with huge mass in perineum that extended to the left pelvic wall and metastasized to left paraortic lymph nodes compressing left ureter. There was also lung metastasis. Under general anaesthesia the huge mass was removed. Biopsies were taken from left paraortic lymph nodes and left pelvic mass. Left ureter was freed and reinserted in the urinary bladder and colostomy was done. The patient received 6 courses of [VAC], chemotherapy [3] weeks after surgery. At the end of chemotherapy, the rest of perineal mass, pelvic mass and left paraortic nodes disappeared. Only lung metastasis remained stationary. After 4 months a new lung metastasis appeared. The patient was put on Etopside and Ifosfamide chemotherapy