Tumor de Askin: un tumor infrecuente de la pared torácica / Askin tumor: a rare tumor of the chest wall / Tumor de Askin: um tumor raro da parede torácica

El tumor de Askin o tumor primitivo neuroectodérmico es una neoplasia de células pequeñas redondas que se origina de los tejidos blandos de la pared torácica, probablemente a partir de células embrionarias que migran de la cresta neural. Son tumores muy agresivos que metastatizan rápidamente y de forma diseminada. Clínicamente, los pacientes presentan una masa de tejidos blandos en la pared del tórax que puede cursar o no con dolor. Otras manifestaciones incluyen disnea, tos, pérdida de peso, síndrome de Horner y adenopatías regionales. La radiografía de tórax muestra una masa heterogénea extrapulmonar, por lo general de gran tamaño, que puede opacificar completamente el hemitórax. El pronóstico del tumor de Askin es pobre; sin embargo, el uso combinado de quimioterapia, cirugía y radiación ha mejorado el resultado de forma drástica.

Askin tumor or primitive neuroectodermal tumor is a small round cells' neoplasia, which originates in the chest's soft tissues probably from embryonic cells that migrate from the neural crest. They are very aggressive tumors that metastasize and disseminate quickly. Clinically, patients show a soft tissue mass in the chest that may or may not be accompanied by pain. Other manifestations include dyspnea, cough, weight loss, Horner syndrome and regional lymphadenopathy. Chest radiographies show a usually large extrapulmonary heterogeneous mass, which can completely opacify the hemithorax. The prognosis is poor; however, the combined use of chemotherapy, surgery and radiation has improved results dramatically.

O tumor de Askin ou tumor neuroectodérmico primitivo é uma neoplasia de pequenas células redondas que se origina dos tecidos moles da parede torácica, provavelmente de células embrionárias que tem migrado da crista neural. São tumores muito agressivos que metastatizam e se disseminam rapidamente. Clinicamente, os pacientes apresentam uma massa de partes moles na parede torácica que pode ou não causar dor. Outras manifestações incluem dispneia, tosse, perda de peso, síndrome de Horner e linfadenopatia regional. A radiografia de tórax mostra uma massa extrapulmonar heterogênea, geralmente grande, que pode opacar completamente o hemitórax. O prognóstico do tumor de Askin é ruim; no entanto, o uso combinado de quimioterapia, cirurgia e radiação tem melhorado drasticamente o resultado.

Spontaneous pneumomediastinum and subcutaneous emphysema as a complication of asthma in children: case report and literature review.

BACKGROUND: Spontaneous pneumomediastinum (SPM) is an uncommon disorder. It is rarely reported in paediatric patients and may be accompanied by subcutaneous emphysema. It is usually benign and self-limiting, with only supportive therapy being needed, but severe cases may require invasive measures. Asthma exacerbations have classically been described as a cause of SPM. However, detailed descriptions in asthmatic children are scarce. We aimed at improving the current understanding of the features of SPM and subcutaneous emphysema, and outcomes, by means of a case report and a systematic review. METHODS: For the systematic review a literature search was performed in PubMed to identify reported cases of SPM in asthmatic children. RESULTS: The case a 10-year-old asthmatic girl with SPM is reported. The patient received an inhaled corticosteroid and long-acting beta2 agonist, in addition to sublingual immunotherapy (SLIT) with eventual control of asthma symptoms. REVIEW: A total of 114 published cases were found since 1995, most of them in teenagers; no sex differences were observed. Clinical presentation was associated with an asthma exacerbation in a number of cases. Other presenting features were chest pain, dyspnoea, cough, and particularly acute swelling of the face, neck, and upper chest. Subcutaneous emphysema was present in most patients. Overall, three cases of pneumothorax and two cases of pneumorrhachis were reported. Therapy was mainly based on supportive care, rest, oxygen therapy, analgesics, steroids, and bronchodilators. All patients recovered spontaneously, in spite of a small initial increase in SPM in a few cases. CONCLUSIONS: Early identification of patients at risk of SPM would avoid the high number of under-diagnosed cases. Patients should be treated not only with supportive therapy but also with measures to achieve control of the underlying cause (such as poorly controlled asthma).