ABSTRACT
Of the head and neck tumoral lesions in children and adolescents, 5%-10% are primary malignant tumors. Among these tumors, orbital rhabdomyosarcoma stands out, which is the most common primary soft tissue sarcoma in children. Its diagnosis requires a high degree of clinical suspicion, and it can be corroborated with a series of examinations, in order to stage it and carry out the appropriate treatment. Currently, surgery and chemotherapy are the primary treatments, and the use of conventional radiotherapy is limited to cases where previous treatments fail or there is a risk of recurrence. The following case report aims to expose the clinical picture, diagnosis, staging and integral treatment of orbital rhabdomyosarcoma, as well as the interdisciplinary management that was performed to improve the patient's prognosis.
Subject(s)
Head and Neck Neoplasms , Rhabdomyosarcoma , Adolescent , Humans , Neoplasm Recurrence, Local , Prognosis , Rhabdomyosarcoma/diagnosisABSTRACT
De las lesiones tumorales de cabeza y cuello en niños y adolescentes, un 5 a un 10% son tumores malignos primarios. Dentro de estos tumores destaca el rabdomiosarcoma orbitario infantil, que es el sarcoma primario de tejido blando más común en niños. Su diagnóstico requiere de un alto grado de sospecha clínica, que puede corroborarse con una serie de exámenes, para así estadificarlo e indicar el tratamiento adecuado. Actualmente se utiliza de primera intención cirugía y quimioterapia; el uso de la radioterapia convencional se limita a casos en los que fracasan los tratamientos anteriores o existe riesgo de recurrencia. El siguiente trabajo tiene como propósito exponer el cuadro clínico, diagnóstico, la estadificación y tratamiento integral de un rabdomiosarcoma embrionario orbitario, así como el manejo interdisciplinario que se realizó para mejorar el pronóstico de la paciente (AU)
Of the head and neck tumoral lesions in children and adolescents, 5 to 10% are primary malignant tumors. Among these tumors, orbital rhabdomyosarcoma stands out, which is the most common primary soft tissue sarcoma in children. Its diagnosis requires a high degree of clinical suspicion, and it can be corroborated with a series of examinations, in order to stage it and carry out the appropriate treatment. Currently, surgery and chemotherapy are the primary treatments, and the use of conventional radiotherapy is limited to cases where previous treatments fail or there is a risk of recurrence. The following case report aims to expose the clinical picture, diagnosis, staging and integral treatment of orbital rhabdomyosarcoma, as well as the interdisciplinary management that was performed to improve the patient's prognosis (AU)
Subject(s)
Humans , Female , Child , Head and Neck Neoplasms/diagnosis , Head and Neck Neoplasms/therapy , Rhabdomyosarcoma/diagnosis , Rhabdomyosarcoma/therapy , Neoplasm Staging , Patient Care Team , Treatment OutcomeABSTRACT
Of the head and neck tumoral lesions in children and adolescents, 5 to 10% are primary malignant tumors. Among these tumors, orbital rhabdomyosarcoma stands out, which is the most common primary soft tissue sarcoma in children. Its diagnosis requires a high degree of clinical suspicion, and it can be corroborated with a series of examinations, in order to stage it and carry out the appropriate treatment. Currently, surgery and chemotherapy are the primary treatments, and the use of conventional radiotherapy is limited to cases where previous treatments fail or there is a risk of recurrence. The following case report aims to expose the clinical picture, diagnosis, staging and integral treatment of orbital rhabdomyosarcoma, as well as the interdisciplinary management that was performed to improve the patient's prognosis.
ABSTRACT
Introducción. La familia de sarcomas de Ewing incluye el sarcoma de Ewing óseo, el tumor neuroectodérmico primitivo, el sarcoma de Ewing extraóseo y el tumor de Askim (sarcoma de Ewing de la pared torácica). Los sitios más comunes de estos tumores son tórax y extremidades. Estos tumores malignos surgen del hueso o de tejidos blandos. Histológicamente, se caracterizan por alta celularidad de pequeño tamaño, redondas, azules y con tendencia a formar estructuras rosetoides. Caso clínico. Se presentó el caso de un adolescente de 14 años de edad que, tres meses previos a su ingreso, inició con cefalea fronto-parietal izquierda con irradiación hacia el globo ocular. Al examen neurológico se encontró proptosis y disminución de la agudeza visual, además de atrofia papilar. Se realizó una tomografía computarizada de cráneo, donde se identificó una lesión intraorbitaria izquierda y otra parietotemporal ipsilateral. Se realizó la exéresis tumoral, reportando positividad para marcadores de tumor neuroectodérmico primitivo, vimentina y CD99. Conclusiones. El sarcoma de Ewing es extremadamente raro a nivel de la órbita. Sin embargo, es prudente considerarlo como diagnóstico diferencial en lesiones de esta región, ya que el pronóstico depende de la precisión diagnóstica temprana.
Background. The Ewing sarcoma family includes Ewing bone sarcoma, primitive neuroectodermal tumor, extraosseous Ewing's sarcoma, and Askim tumor (Ewing sarcoma of the chest wall). The most common sites of these tumors are chest and limbs. These malignancies arise from bone or soft tissues. Histologically, it is characterized by high cellularity, small, round, blue and with a tendency to form rosette structures. Case report. We report the case of a 14-year-old patient whose evolution began 3 months before admission with left frontoparietal headache radiating to the eyeball. During neurological examination, proptosis and decreased visual acuity were found, as well as papillary atrophy. Cranial computed tomography identifed a left intraorbital injury and another ipsilateral parietotemporal. Tumor exeresis was performed, reporting positive for primitive neuroectodermal tumor markers, vimentin and CD 99. Conclusions. Ewing sarcoma is extremely rare at the orbital level: however, it is prudent to consider this tumor as a differential diagnosis in lesions of this region. Prognosis depends on early diagnostic accuracy.
ABSTRACT
Los linfomas no hodgkinianos (LNH) representan la mitad de los tumores de la órbita, sin embargo, su incidencia en nuestro medio es poco frecuente. Se presenta una paciente de 52 años de edad, femenina, blanca, con antecedentes patológicos personales de hipertensión arterial, sin otros datos relevantes al interrogatorio, que acude a consulta por epífora y discreta ptosis palpebral del ojo derecho acompañada de tumoración dura y fija en zona ínfero medial de la órbita derecha. Las principales pruebas de laboratorio no mostraron nada relevante. En la resonancia magnética se comprobó tumoración de 2 × 1 cm, de contornos irregulares en relación con la pared posteroinferior de la órbita derecha. Se realizó biopsia de la lesión que se concluyó como un LNH con inmunofenotipo compatible con proceso linfoproliferativo de linfocitos B kappa. En la biopsia de médula ósea se informó infiltración nodular por proceso linfoproliferativo de bajo grado. Se concluyó el caso como un LNH primario de la órbita tipo zona marginal con expresión leucémica de células B monocitoides en estadio IVA. Se realizaron 8 ciclos de CHOP y se obtuvo respuesta parcial. Posteriormente se pasó a un esquema de segunda línea consistente en rituximab-fludarabina-ciclofosfamida-dexametasona, que se mantiene(AU)
The no-Hodgkin lymphomas (NHL) accounted for the half of orbit tumor; however, its incidence in our environment is very uncommon. This is the case of a white female patient aged 52 with personal pathological backgrounds of high blood pressure without other relevant data in questioning, referred to consultation due to epiphora and a discrete palpebral ptosis of right eye accompanied by hard and fixed tumor in inferior medial of right orbita. Main laboratory tests were normal. In magnetic resonance (MR) it was demonstrated the presence of a 2 x 1 cm tumor with irregular borders in relation to posteroinferior wall of right orbit. A lesion biopsy was carried out with diagnosed as a primary NHL con immunophenotype compatible with a lymphoproliferative process. We conclude that this case is a primary orbit NHL of principal zone with leukemic expression of monocytic B cells in IVA state. We performed 8 cycles of CHOP with a partial response. Subsequently, we passed to a second-line maintained scheme consistent in Rituximab-Fludarabine-Cyclophosfamide-Dexamethasone(AU)
Subject(s)
Humans , Middle Aged , Lymphoma, Non-Hodgkin/diagnosis , Orbital Neoplasms/diagnosis , Orbital Neoplasms/drug therapy , Antibodies, Monoclonal, Murine-Derived/therapeutic use , Lymphoma, Non-Hodgkin , Cyclophosphamide/therapeutic use , Dexamethasone/therapeutic useABSTRACT
Los linfomas no hodgkinianos (LNH) representan la mitad de los tumores de la órbita, sin embargo, su incidencia en nuestro medio es poco frecuente. Se presenta una paciente de 52 años de edad, femenina, blanca, con antecedentes patológicos personales de hipertensión arterial, sin otros datos relevantes al interrogatorio, que acude a consulta por epífora y discreta ptosis palpebral del ojo derecho acompañada de tumoración dura y fija en zona ínfero medial de la órbita derecha. Las principales pruebas de laboratorio no mostraron nada relevante. En la resonancia magnética se comprobó tumoración de 2 × 1 cm, de contornos irregulares en relación con la pared posteroinferior de la órbita derecha. Se realizó biopsia de la lesión que se concluyó como un LNH con inmunofenotipo compatible con proceso linfoproliferativo de linfocitos B kappa. En la biopsia de médula ósea se informó infiltración nodular por proceso linfoproliferativo de bajo grado. Se concluyó el caso como un LNH primario de la órbita tipo zona marginal con expresión leucémica de células B monocitoides en estadio IVA. Se realizaron 8 ciclos de CHOP y se obtuvo respuesta parcial. Posteriormente se pasó a un esquema de segunda línea consistente en rituximab-fludarabina-ciclofosfamida-dexametasona, que se mantiene.
The no-Hodgkin lymphomas (NHL) accounted for the half of orbit tumor; however, its incidence in our environment is very uncommon. This is the case of a white female patient aged 52 with personal pathological backgrounds of high blood pressure without other relevant data in questioning, referred to consultation due to epiphora and a discrete palpebral ptosis of right eye accompanied by hard and fixed tumor in inferior medial of right orbita. Main laboratory tests were normal. In magnetic resonance (MR) it was demonstrated the presence of a 2 x 1 cm tumor with irregular borders in relation to posteroinferior wall of right orbit. A lesion biopsy was carried out with diagnosed as a primary NHL con immunophenotype compatible with a lymphoproliferative process. We conclude that this case is a primary orbit NHL of principal zone with leukemic expression of monocytic B cells in IVA state. We performed 8 cycles of CHOP with a partial response. Subsequently, we passed to a second-line maintained scheme consistent in Rituximab-Fludarabine-Cyclophosfamide-Dexamethasone.
