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)

Uterine cavity embryonal rhabdomyosarcoma

Rhabdomyosarcoma (RMS) is a rare solid tumor in childhood and adolescence. The higher incidence is predominant during the first two decades of life. According to the Intergroup RMS Study Group, the embryonal RMS (ERMS), botryoidal variant, constitutes a histological subtype characterized as a "grape-like" lesion of 2.0 cm to 9.5 cm. The treatment involves chemotherapy, surgery, and/or radiotherapy. We present the case of a 14-year-old female patient diagnosed with ERMS, botryoidal variant, which originated in the uterine cervix with vaginal externalization. The initial therapeutic approach comprised an initial prolapsed mass excision followed by Wertheim­Meigs surgery due to the tumor extension. No consensual protocol to ERMS treatment is found in the medical literature; however, a combined approach seems to offer a better result. The postoperative time period was uneventful and the patient followed an adjuvant therapy with vincristine, d-actinomycin, and cyclophosphamide. A comprehensive evaluation of the therapeutic options preserving the reproductive function­unfortunately not always possible­is part of a multi-disciplined care team concerning the pediatric patients.

Embryonal rhabdomyosarcoma of the caruncle in a 4 year-old boy: case report / Rabdomiossarcoma embrionário da carúncula em um menino de 4 anos: relato de caso

Rhabdomyosarcoma is a rare tumor, with an annual incidence of 4.3 cases per million children. Even thought, it is the most common soft tissue sarcoma in childhood, with a mean age of 6 to 8 years at diagnosis. A 4 year-old boy presented with a history of a fast growing (1-month) nodular lesion in the caruncle of his left eye. Slit lamp examination showed a vascularized solid nodular lesion in the semilunar fold. The lesion was surgically removed obtaining infiltrated edges with tumoral cells. A second surgery was performed with free tumour edges. The diagnosis of embryonal rhabdomyosarcoma, botryoid type, of intermediate differentiation was made. The treatment for botryoid rhabdomyosarcoma is basically surgical with the combined use of adjuvant polychemotherapy. In adolescent or adult patients (not in infants where growth bone disturbances can occur) external beam radiotherapy can be combined with chemotherapy. Rhabdomyosarcomas of the conjunctiva without orbital extension are rarely reported. We presented a case of a child with a rare tumor which we had a high suspicion of malignancy and early diagnosis and treatment and child is free of systemic disease 6 and half years later. Our research group believes that the key in these tumors is the high index of suspicion and early treatment.

Rabdomiossarcoma é um tumor raro, com uma incidência anual de 4,3 casos por milhão de crianças. É o sarcoma de partes moles mais comum na infância, com uma idade média de 6 a 8 anos no momento do diagnóstico. Um menino de 4 anos apresentou-se com uma história de um crescimento rápido (1 mês) lesão nodular em carúncula de seu olho esquerdo. O exame biomicroscópico mostrou uma lesão vascularizada sólida nodular na prega semilunar. A lesão foi removida cirurgicamente com obtenção de bordas infiltradas com células tumorais, segunda cirurgia foi feita com bordas tumorais livres. O diagnóstico de rabdomiossarcoma embrionário, tipo botrióide, de diferenciação intermediária foi feita. O tratamento para a rabdomiossarcoma botrióide é basicamente cirúrgico com o uso combinado de poliquimioterapia adjuvante. Em pacientes adolescentes e adultos (e não em lactentes onde os distúrbios do crescimento ósseo pode ocorrer) radioterapia externa pode ser combinada com a quimioterapia. Rabdomiossarcomas da conjuntiva sem extensão orbital são raramente relatadas. Apresentamos um caso de uma criança com um tumor raro que tivemos uma alta suspeita de malignidade e diagnóstico precoce e tratamento e criança é livre de doença sistêmica 6 anos e meio depois. Nosso grupo de pesquisa acredita que a chave para esses tumores é o alto índice de suspeita e tratamento precoce.

Rabdomiosarcoma embrionario uterino: aspectos morfológicos e inmunohistoquímicos / Embryonal rhabdomyosarcoma of the corpus uterine: morphological and immunohistochemical characteristics

Los sarcomas uterinos son relativamente raros. El tipo histológico más frecuente es el leiomiosarcoma, seguido por el sarcoma del estroma endometrial. Los rabdomiosarcomas (RMS) son neoplasias malignas con diferenciación muscular esquelética. El rabdomiosarcoma embrionario (RMSE) tipo botrioide es el sarcoma más común de la infancia; con escasos reportes en adultos, afectando la región de cabeza y cuello, tracto genitourinario y extremidades más frecuentemente. En el tracto genitourinario, la vagina es la localización mas frecuente, comprometiendo en raras ocasiones el cuello o fondo uterino. Se presentan las características clínicas y morfológicas de un caso de RMSE uterino tipo botrioide diagnosticado en una paciente de 58 años en la Unidad de Anatomía Patológica del Hospital Hernán Henríquez Aravena de Temuco.

Uterine sarcomas are relatively rare. The most common histological type is leiomyosarcoma, followed by endometrial stromal sarcoma. The rhabdomyosarcoma (RMS) are malignant neoplasms with skeletal muscle differentiation. Embryonal rhabdomyosarcoma (RMSE) type botryoides is the most common in childhood, with few reports in adults, affecting the head and neck region, genitourinary tract and extremities more frequently. In the genitourinary tract, the vagina is the most common location, rarely involving cervix and fundus uterine. Clinical and morphological characteristics are presented of a case of uterine RMSE diagnosed in a 58 year-old woman in the Pathology Unit of the Hernán Henríquez Aravena Hospital in Temuco.

Embryonal rhabdomyosarcoma of the adult soft palate.

Embryonal rhabdomyosarcoma is the most common soft tissue sarcoma in children. We report a rare case of embryonal rhabdomyosarcoma of the soft palate in a 32-year-old Caucasian female. Detailed histology of the tumor is described. Positive staining with desmin, myogenin and myoD1 confirmed the tumor to be embryonal rhabdomyosarcoma. A genetic association between rhabdomyosarcoma, polycystic ovary syndrome and the FEM1A gene on the human chromosome is speculated upon.

Ectomesenquimoma maligno: a propósito de un caso / Malignant ectomesenchymoma: a purpose of a case

Es un tumor raro que puede surgir en el cerebro o los tejidos blandos, se define como una forma ectodermal con componentes represantados por neuroblastos, células ganglionares y mesenquimales. El componente mesenquinal la mayoría de las veces es rabdomiosarcoma. Caso de una lactante mayor femenina de 23 meses de edad, quien ingresa al Hospital "J.M de Los Ríos" con enfermedad de 18 meses de evolución caracterizada por lesión nodular de 5 cm de diámetro en un tercio próximal de antebrazo derecho de consistencia blanda, no dolorosa y de crecimiento progresivo por lo cual es referida a nuestro centro donde se realizan estudios, y biopsia que reporta: hallazgos histológicos e inmunohistoquímicos compatibles con rabdomiosarcoma embrionario con diferenciación ganglioneuromatosa (Ectomesenquimoma maligno). Inicia protocolo de quimioterapia, para sarcoma de Ewing/tumor neuroectodérmico primitivo óseo y extra óseo no metastásico y metastásico, cumple 3 ciclos con mejoría clínica de la lesión tumoral.

Is rare tumor arise in brain or soft tissue, is defined as form including ectodermal components represented by neuroblasts, ganglion cells, differentiated mesenchymal structures of various types. The mesenchymal component is most often rhabdomyosarcoma. The present study goes directed to discussion of case of a feminine greater suckling baby of 23 months of age, that enters Hospital “J.M of Rios” with disease of 18 months of evolution characterized by injury to nodular of 5 cm of diameter in a proximal third of right forearm of soft, no painful consistency and of progressive growth thus is referred our center where studies are made biopsy that reports: Compatible histological and immunohistochemestry findings with rabdomiosarcoma embryonic with ganglioneuromatosa differentiation(malignant ectomesenquimoma). It initiates protocol of chemotherapy for sarcoma of Ewing/tumor nonmetastasic and metastatic bony and extra bony neuroectodermic primitive, it fulfills three cycles with clinical improvement of the tumor like injury.

Congenital embryonal rhabdomyosarcoma with prenatal onset

Rhabdomyosarcoma [RMS] is the single most common type of soft tissue sarcoma in children and adolescents but it is extraordinarily rare in neonates. Extremity RMS comprises 20% of all sites, occurs more commonly in the leg than in the arm and accounts for 9% of all RMS cases. According to our review, this is the second case of RMS on day one of life with congenital, antenatal feature, and postnatal progressive clinical course of a large tumor of the hand [pretreatment staging T2bN1M0] with embryonic histological subtype and unfavorable prognosis. The patient is a term newborn boy with huge mass in the right hand and palpable lymph node in subaxillar region. Congenital embryonal rhabdomyosarcoma is a rare form of sarcomas with congenital in nature, antenatal feature and post natal progressive clinical course of sarcomas of extremities in newborn infants

Spinal and bilateral breast metastases of embryonal rhabdomyosarcoma.

A case of spinal and bilateral breast metastasis was reported The primary tumor was an embryonal rhabdomyosarcoma of the left hand. Intralesional resection of metastatic spinal sarcoma was done with concurrence chemotherapy and radiotherapy. The literatures of these rare conditions were reviewed.

Rabdomiosarcoma embrionario hepatico: a propósito de un caso / Hepatic embrionary Rhabdomysarcoma: presentation of a case

El Rabdomiosarcoma es el sarcoma de tejidos blandos de origen musculoesqueléticos más frecuente en niños menores de 15 años y uno de los más comunes en adolescentes y adultos jóvenes. (1) Las áreas del cuerpo más comunes donde puede alojarse este tumor son la cabeza, el cuello, la vejiga, la vagina, los brazos, las piernas y el tronco. Aunque también puede encontrarse en la próstata, el oído medio y el sistema de conductos biliares. (2) En los niños con rabdomiosarcoma embrionario, esta anomalía se encuentra en el cromosoma 11, mientras el alveolar en los cromosomas 2 y 13. (1) Se reconocen tres subtipos: embrionario, alveolar y pleomórfico. El embrionario se observa en niños y adolescentes menores de 15 años y se localiza principalmente en la cabeza y en el cuello, tracto urogenital, retroperitoneo y extremidades. Los síntomas pueden incluir una masa visible o palpable que puede ser doloroso o no, parestesia, dolor. (2) El diagnóstico se establece a través del examen físico, historia médica completa, así como estudios imagenológicos, biopsia y punción de medula ósea. (3) El tratamiento específico será dado por la localización del tumor primario y el estadio del tumor, en algunos pacientes se administra quimioterapia preoperatorio en un intento de reducir la extensión de la intervención y de preservar órganos vitales. El pronóstico se relaciona con la edad del paciente, sitio de origen, resecabilidad. (4)

Botryoid rhabdomyosarcoma of mesentery--a case report.

Botryoid rhabdodmyosarcomas are described predominantly in hollow visceral organs. We present case of an embryonal (botryoid) rhabdomyosarcoma arising in mesentery of a two year old male child.

Primary pulmonary rhabdomyosarcoma; a case report

Primary rhabdomyosarcoma is a rare entity. To date, only 29 cases have been reported in the world literature. The current article presents another case of this disease, in which a 3-year-old boy was referred to our service with a one-week history of productive cough and fever. Imaging studies revealed a huge mass on the right lung as well as plural effusion. He underwent a right posterolateral thoracotomy and lobectomy. Pathologic studies revealed a primary pulmonary rhabdomyosarcoma of embryonic type. He has no other symptoms of disease on a 6-month follow-up

Tumores con rabdomioblastos y neuroblastos / Tumors with rhabdomyoblasts and neuroblasts

Se evalúa uma técnica de impregnación metálica, doble impregnación de Del Río Hortega, para evidenciar rabdomioblastos y neuroblastos, con recomendaciones sobre los tiempos de impregnación para obtener mejores resultados. Las imágenes obtenidas son muy demostrativas, tanto de los elementos embrionarios del mesénquima primitivo desde mioblasto, miotubo a célula acintada rabdomiobástica con estraciones transversales, hasta los elementos neopláticos de esta estripe. Y de los neuroblastos con sus prolongaciones. El material de estudio incluye un Tumor de Wilms renal con rabdomioblastos y neuroblastos, y un Neuroblastoma de cerebelo con componente rabdomioblástico. A estas lesiones se las considera desembrioplasias. Además se estudiaron 2 Rabdomiosarcomas embrionarios botrioides, uno de ellos de presentación inusual en una mujer menopáusica, 2 Tumores müllerianos mixtos de útero y trompa de Falopio, un Rabdomioma de faringe, y 3 embriones humanos de material de aborto entre 5 y 13 semanas. Destacamos la utilidad de la doble impregnación para estudiar rabdomioblastos y neuroblastos. En los Rabdomiosarcomas se pueden ver estructuras y elementos comparables con los de la etapa embrionaria: células raquetoides, acintadas, miotubos, rabdomioblastos. El Tumor de Wilms es un tumor disembrioplásico y está constituido por el blastema renal. En él hemos encontrado rabdomioblastos y neuroblastos. En el Neuroblastoma del cerebelo servamos rabdomioblastos con cierta organicidad (ectomeséquima). Y por último describimos un caso poco frecuente de Ragdomiosarcoma botrioide de cuello uterino en una mujer menopáusica.

Sarcoma Botrióide de Bexiga: relato de caso / Botryoid sarcoma of the bladder

O sarcoma botrióide é uma neoplasia maligna que acomete os órgäos pélvicos. Os autores relatam o caso mais recente de sarcoma botrióide de bexiga, diagnosticado no Hospital do Câncer do Maranhäo (Hospital Aldenora Bello), discutindo seu diagnóstico, tratamento e aspectos anatomopatológicos.

Rabdomiosarcoma embrionario de la próstata. Informe de cuatro casos y revisión de la literatura / Embrionary rabdomyosarcoma of the prostate gland: report of four cases and literature review

El rabdomiosarcoma embrionario prostático es el más frecuente de los sarcomas de tejidos blandos. Estos tumores son altamente malignos. El tratamiento consiste en quimioterapia, radioterapia y cirugía radical. Se presentan cuatro casos, los cuales han sido tratados en 12 años, entre 1981 y 1992, con todas las modalidades en conjunto, que resultaron en una tasa de supervivencia de 50 por ciento