Extramammary Paget disease: a report of 10 cases.

Extramammary Paget disease (EMPD) is a very rare form of intraepithelial adenocarcinoma. We report the cases of EMPD diagnosed in a referral hospital over a 5-year period from 2009 to 2013. Ten cases of EMPD were diagnosed in 4 men and 6 women aged between 50 and 79 years. Erroneous clinical diagnoses led to diagnostic delays of 1 to 5 years in all cases. Six patients had in situ lesions and dermal invasion was observed in the other 4 cases. Four patients had a second neoplasm and 2 of the patients with invasive EMPD died. Because EMPD is rare and its clinical presentation is nonspecific, a high degree of clinical suspicion is required to avoid delays in diagnosis and treatment.

Skin infection caused by Scedosporium apiospermum in immunocompromised patients. Report of two cases.

Scedosporium apiospermum is a filamentous fungus that can cause cutaneous or extracutaneous disease. A large number of cases have been published over the last decades, mainly in patients immunocompromised as a result of their disease or treatment. These kinds of infections can progress rapidly and become disseminated, leading to very serious or even fatal complications. We report two new cases of skin infection by Scedosporium apiospermum from our hospital.

[Circumscript myositis ossificans in a four-year-old boy]. / Miositis osificante circunscrita en un niño de cuatro años.

We present the case of a 4 year old boy with no previous personal or family history of interest, who attended the Paediatric Department of our hospital after a physical examination revealed a painful induration in the left arm which had increased in size; imaging tests were consistent with a calcified mass in soft tissues, without cortical involvement, suspected of being malignant. Two-phase bone scintigraphy was requested in which a soft tissue lesion, not suggestive of malignancy, was detected. The biopsy was negative for malignant cells. However, in view of the progressive increase in size of the lesion, FDG positron emission tomography (FDG-PET) was performed, showing a hypermetabolic mass consistent with malignancy in the left arm, for which the patient underwent surgery for suspected possible parosteal osteosarcoma.

Hibernoma as an incidental finding on 18F-FDG-PET/CT in lymphoma patient follow-up / Hibernoma como hallazgo incidental en la 18F-FDG-PET/TC en paciente en seguimiento por linfoma

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Enfermedad de Paget extramamaria: 10 casos / Extramammary Paget Disease: A Report of 10 Cases

La enfermedad de Paget extramamaria (EPEM) es una forma extremadamente rara de adenocarcinoma intraepitelial. Presentamos los casos de EPEM diagnosticados en los últimos 5 anos en un hospital de tercer nivel (2009-2013). Se trata de 10 casos, 4 varones y 6 mujeres, con edades comprendidas entre los 50-79 anos. Todos ellos sufrieron un retraso diagnóstico de uno a 5 anos debido a sospechas diagnósticas erróneas. Seis casos correspondieron a EPEM in situ y en 4 existía invasión dérmica. Se localizó una segunda neoplasia en 4 pacientes y 2 con EPEM invasiva fallecieron. La baja incidencia de esta enfermedad y su clínica inespecífica nos debe alertar sobre la necesidad de establecer una alta sospecha clínica para evitar retrasos diagnósticos y terapéuticos (AU)

Extramammary Paget disease (EMPD) is a very rare form of intraepithelial adenocarcinoma. We report the cases of EMPD diagnosed in a referral hospital over a 5-year period from 2009 to 2013. Ten cases of EMPD were diagnosed in 4 men and 6 women aged between 50 and 79 years. Erroneous clinical diagnoses led to diagnostic delays of 1 to 5 years in all cases. Six patients had in situ lesions and dermal invasion was observed in the other 4 cases. Four patients had a second neoplasm and 2 of the patients with invasive EMPD died. Because EMPD is rare and its clinical presentation is nonspecific, a high degree of clinical suspicion is required to avoid delays in diagnosis and treatment (AU)

Miositis osificante circunscrita en un niño de cuatro años / Circumscript myositis ossificans in a four-year-old boy

Se presenta el caso de un niño de 4 años de edad sin antecedentes personales ni familiares de interés, que acude al Servicio de Pediatría de nuestro hospital por presentar en la exploración física una induración dolorosa en el brazo izquierdo que ha aumentado de tamaño, junto con pruebas de imagen compatibles con masa calcificada en partes blandas, sin afectación cortical sospechosa de malignidad. Se le solicita una gammagrafía ósea en dos fases en la cual se aprecia una lesión de partes blandas no sugerente de malignidad. La biopsia realizada fue negativa para células malignas. Pero ante el progresivo aumento de tamaño de la lesión se le realiza una tomografía por emisión de positrones con fluordesoxiglucosa (PET -FDG), donde se evidencia una masa hipermetabólica compatible con malignidad en el brazo izquierdo, por lo que, ante la sospecha de un posible osteosarcoma parostal, se lleva a cabo una intervención quirúrgica

We present the case of a 4 year old boy with no previous personal or family history of interest, who attended the Paediatric Department of our hospital after a physical examination revealed a painful induration in the left arm which had increased in size; imaging tests were consistent with a calcified mass in soft tissues, without cortical involvement, suspected of being malignant. Two-phase bone scintigraphy was requested in which a soft tissue lesion, not suggestive of malignancy, was detected. The biopsy was negative for malignant cells. However, in view of the progressive increase in size of the lesion, FDG positron emission tomography (FDG-PET) was performed, showing a hypermetabolic mass consistent with malignancy in the left arm, for which the patient underwent surgery for suspected possible parosteal osteosarcoma

A propósito de un caso: histiocitosis de células de Langerhans en adolescente / Langerhan’s cell histiocytosis in an adolescent. Report of a case

Propósito: El objetivo del artículo ha sido realizar una revisión de la Histiocitosis de células de Langerhans (HCL) a través de la presentación de un caso en nuestro centro. Material y métodos: A partir del caso clínico presentado en un paciente de 15 años se procedió a la revisión exhaustiva de la bibliografía encontrada de una enfermedad poco frecuente para los oncólogos. Para la realización de esta revisión se solicitó ayuda a la Sociedad Española de Oncología Pediatría. Conclusiones: La HCL es una patología propia de niños y adolescentes con una alta tasa de curabilidad de ahí la importancia en realizar un correcto estadiaje y tratamiento adecuado siguiendo las recomendaciones de la Sociedad Española de Oncología Pediátrica; asimismo se ha de hacer hincapié en el seguimiento de estos pacientes dada la posibilidad de recaída y tratamiento de rescate. (AU)