Rabdomiosarcoma laríngeo en un adulto, descripción de un caso y revisión de la literatura / Adult laryngeal rhabdomyosarcoma. Case report and literature review

Resumen El rabdomiosarcoma laríngeo es un cáncer infrecuente en cabeza y cuello, y aún más en adultos. Describimos el caso de un varón de 55 años con un rabdomiosarcoma del músculo cricoaritenoideo posterior izquierdo tratado mediante laringectomía total y linfadenectomía funcional bilateral.

Abstract Laryngeal rhabdomyosarcoma is an uncommon cancer in head and neck, especially in adults. We report a 55 years old male with a rhabdomyosarcoma from the left posterior cricoarytenoid muscle treated with a total laryngectomy and double functional cervical lymphadenectomy.

Bacteremia due to Weeksella virosa in a pediatric patient with embryonal rhabdomyosarcoma / Bacteriemia por Weeksella virosa en un paciente pediátrico con rabdomiosarcoma embrionario

Abstract Background: Weeksella virosa is one of the two species of the genus Weeksella. Clinical disease due to this bacterium in humans is rare, for which only nine cases have been reported in the literature. Case report: A 4-year-old male patient was diagnosed with a left orbit rhabdomyosarcoma Stage III and was admitted to a northeast third level referral center in Mexico. Aerobic, non-pigmented, Gram-negative rod was isolated from a blood culture. W. virosa was identified by Sensititre™ ARIS. This organism has been described in cases of spontaneous bacterial peritonitis, sepsis, pneumonia, ventriculitis, and urinary tract infection. Conclusions: Clinicians should consider the diagnosis of W. virosa bacteremia in cases involving immunocompromised patients with oral lesions, although it is infrequent. To the best of our knowledge, this is the first clinical report of W. virosa bacteremia described in an immunocompromised pediatric patient.

Resumen Introducción: Weeksella virosa es una de las dos especies del género Weeksella. En los humanos, los reportes de infección por este microorganismo son raros. Solo se han reportado nueve casos en la literatura. Caso clínico: Paciente masculino de 4 años con diagnóstico de rabdomiosarcoma embrionario de órbita izquierda en estadio III, que fue atendido en un hospital de tercer nivel en el noreste de México. Se realizó un hemocultivo, a partir del cual se aisló un bacilo gramnegativo aerobio, no pigmentado. Se identificó W. virosa mediante Sensititre™ ARIS. Este microorganismo ha sido descrito en casos de peritonitis bacteriana espontánea, sepsis, neumonía, ventriculitis e infección del tracto urinario. Conclusiones: Aunque los casos de bacteriemia por W. virosa son raros, los clínicos deben considerar este agente en pacientes inmunocomprometidos con lesiones orales. Hasta donde se conoce, este es el primer caso que se describe de bacteriemia por W. virosa en un paciente pediátrico inmunocomprometido.

Rhabdomyosarcoma of the common bile duct mimicking choledochal cyst: a rare cause of obstructive jaundice / 中国当代儿科杂志

Extrahepatic biliary tract tumors are rare and among them rhabdomyosarcoma is most common. Rhabdomyosarcoma is a soft tissue malignant musculoskeletal tumor and is a very rare malignancy of the common bile duct in children. It usually presents as obstructive jaundice and/or pruritus. If there is no local invasion to the adjacent tissues, the radiological appearance of the tumor lesion is like a choledochal cyst. So the diagnosis is usually made at surgery or by preoperative biopsy. It is important to diagnose early and differentiate it from choledochal cyst and start treatment as early as possible for long time survival of the patient. This case report presented a case of a 10-year-old boy with recurrent onset of obstructive jaundice and fever preoperatively who was diagnosed as choledochal cyst and postoperatively as embryonal rhabdomyosarcoma of the common bile duct. After surgical resection and postoperative chemotherapy, the child had a good prognosis. So it is crucial to know that this rare tumor can mimic congenital choledochal cyst and it should be considered in the differential diagnosis of obstructive jaundice in children.

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

Rabdomiosarcoma embrionario de cérvix uterino en mujer adulta / Embryonal rhabdomyosarcoma of uterine cervix in adult female

El rabdomiosarcoma (RMS) del tracto genital inferior es una patología maligna relativamente frecuente en la infancia aunque muy poco prevalente en la edad adulta. Tan solo suponen el 2-4 por ciento de todos los sarcomas de partes blandas. Se trata de una neoplasia derivada de células progenitoras de miocitos de músculo estriado en distinto grado de diferenciación. En un elevado número de casos, el cuadro se presenta como un pólipo endocervical de apariencia benigna, lo cual retrasa el diagnóstico. El correcto manejo del RMS de tracto genital es controvertido. Un esquema agresivo de tratamiento con cirugía, poliquimioterapia y radioterapia en pacientes seleccionadas, ha demostrado aumentar la supervivencia e incluso conseguir la curación en estadios precoces.

Rhabdomyosarcoma (RMS) of the lower genital tract is a common childhood malignancy but a rare tumor in female adults. It accounting for around 2-4 percent of soft-tissue sarcomas. It is a malignant neoplasm originating from myogenic progenitors cells that shows variable stages of skeletal muscle differentiation. In many cases, the tumor appears as a benign endocervical polyp and this delays the correct diagnosis. Optimal management of adult genital tract RMS is uncertain. Aggressive primary therapy with local excition, poliche-motherapy and radiotherapy in selected patients may result in prolonged survival and cure in early stages.

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.

Embryonal rhabdomyosarcoma of the chest wall: a case report and review of the literature.

Embryonal rhabdomyosarcoma is a soft-tissue sarcoma which has a predilection for the head and neck area, genitourinary tract and the extremities. We report a rare case of embryonal rhabdomyosarcoma of the chest wall in an 8-year-old girl, presenting as a destructive tumor in the rib and clinically and radiologically mimicking Ewing's sarcoma. Histopathological examination showed a small round cell tumor. Immunohistochemically, the positivity for muscle markers desmin and myogenin in the tumor cells proved to be useful for making a definitive diagnosis of embryonal rhabdomyosarcoma. Cytogenetic analysis revealed a high level of aneuploidy in the tumor cells, with double-minutes and additional chromosomal structural aberrations. The patient is responding well to chemotherapy.

Embryonal rhabdomyosarcoma of the biliary tree mimicking a choledochal cyst.

Embryonal rhabdomyosarcoma (ERMS) of biliary tree is a rare type of mesenchymal neoplasm diagnosed at surgery or by preoperative liver biopsy. We present a one year eight months old child who mimicked a choledochal cyst and was eventually treated with surgery, chemotherapy with IRS IV protocol and adjuvant postoperative radiotherapy to surgical bed with 6 MV photons to a dose of 5040 cGy in 28 fractions.

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)

Leiomiossarcoma, rabdomiossarcoma embrionário e tumor maligno da bainha de nervo periférico: relato de três casos de sarcomas retroperitoneais atípicos / Leiomyosarcoma, embrionary rhabdomyosarcoma and malignant peripheral nerve sheath tumor: report of three cases of atypical retroperitoneal sarcomas

São relatados três casos de sarcomas retroperitoneais primários atípicos: leiomiossarcoma, rabdomiossarcoma embrionário e tumor maligno da bainha de nervo periférico (antigamente neurofibrossarcoma ou neurossarcoma). São lesões incomuns e geralmente diagnosticadas tardiamente, caracterizadas por volumosas e heterogêneas massas no retroperitônio. A tomografia computadorizada com o uso de contraste endovenoso é um método adequado na avaliação destas entidades e de sua relação com órgãos e estruturas adjacentes.

Botryoid rhabdomyosarcoma of vagina--two case reports.

Botryoid rhabdomyosarcoma, previously considered a type of embryonal rhabdomyosarcoma, has clinicopathological features distinctive enough to warrant its classification as a separate entity. It almost always occurs in children under 5 years of age. Two cases of botryoid rhabdomyosarcoma are reported here because of their relative rarity.

Tumores parafaríngeos en niños: descripción de dos casos clínicos / Parapharyngeal tumors in pediatrics: 2 clinical cases

Se presentan dos casos clínicos de tumores parafaríngeos de niños tratados en el Servicio de ORL del Hospital Regional Valdivia. El primero corresponde a un coristoma que histológicamente constituye tejido neuroglial maduro el cual fue tratado con cirugía exclusiva. El segundo caso es un Rabdomiosarcoma embrionario que fue sometido a una resección con quimioterapia y radioterapia según protocolo y a una cirugía de rescate por una recidiva local. Se revisa la literatura con especial énfasis a la anatomía, técnica quirúgica e histología de los tumores que afectan el espacio parafaríngeo

Embryonal rhabdomyosarcoma of prostate in an adult--a diagnostic dilemma.

Embryonal rhabdomyosarcoma of the prostate is a rare. Highly malignant tumour. The median age of occurrence is five years, but sporadic cases have been reported in adults' To the best of our knowledge, till date, fewer than ten cases have been reported of which only two are above the age of sixty years. We report a case of embryonal rhabdomyosarcoma of prostate in a patient more than sixty years of age. If one is not aware of this entity, one can make a mistake in the diagnosis as well as treatment.

Rabdomiosarcoma como tumor secundario en la órbita / Rabdomyosarcoma as a secundary orbital tumor

El rabdomiosarcoma de la órbita es un tumor altamente maligno que se presenta en la órbita como tumor primario, y menos frecuentemente como neoplasia secundaria. Presentamos el caso clínico de una paciente de 18 años de edad, con diagnóstico de rabdomiosarcoma secundario de la órbita, con origen en senos paranasales. Se revisan los hallazgos clínicos y paraclínicos de esta neoplasia