Sarcoma mieloide intraoral: forma poco común de debut de la leucemia aguda mieloide / Intraoral myeloid sarcoma. An uncommon debut of acute myeloid leukemia

La leucemia aguda mieloide (LAM) es una neoplasia maligna con manifestaciones sistémicas, pudiendo presentar lesiones a nivel intraoral. Es el cáncer más frecuente en la infancia y constituye la primera causa de muerte por cáncer en la edad pediátrica. El sarcoma mieloide es un tumor maligno extramedular compuesto por células precursoras mieloides inmaduras. Este tumor se asocia fundamentalmente con la leucemia mieloide aguda y síndromes mielodisplásicos y, generalmente, debuta después del diagnóstico de la enfermedad primaria. La aparición del sarcoma mieloide dentro de la cavidad oral como manifestación inicial de la LAM es extremadamente rara (3-6 % de los casos según las series). Se presenta el caso de una lactante de 19 meses de edad que llevaron a Urgencias por fiebre y una tumoración gingival en el cuarto cuadrante refractaria a tratamiento antibiótico. Se solicitó un estudio ecográfico para valorar la existencia de un absceso, donde se identificó una lesión que, tras realizar una biopsia para estudio anatomopatológico, resultó ser un sarcoma mieloide. La paciente fue diagnosticada de LAM y se inició tratamiento quimioterápico. Tras tres ciclos, actualmente la paciente se encuentra libre de enfermedad

Acute myeloid leukemia (AML) is a malignant neoplasm with systemic manifestations, which may present intraoral lesions. It is the most common cancer in childhood and the leading cause of death from cancer in pediatric population. Myeloid sarcoma is a malignant extramedullary tumor composed of immature myeloid precursor cells. This tumor is mainly associated with AML and myelodysplastic syndromes and, generally, shows up after the diagnosis of the primary disease. The appearance of myeloid sarcoma within the oral cavity as an initial manifestation of AMK is extremely rare (3-6% of cases according to the series). We present a case of a 19-month infant who was taken to the Emergency Department due to fever and a gingival lesion that did not respond to antibiotic treatment. An ultrasound study was requested to assess the presence of an abscess. However, a lesion was identified and after performing a biopsy, pathologist found it was a myeloid sarcoma. The patient was diagnosed with AML and chemotherapy treatment was started. After three cycles of treatment, the patient is currently free of disease

Screening for symptoms of obstructive sleep apnea in children with severe craniofacial anomalies: assessment in a multidisciplinary unit.

OBJECTIVE: To assess the incidence of airway obstruction symptoms and the presence of obstructive sleep apnea in children with severe craniofacial anomalies by a proactive screening program using a standard questionnaire and cardiorespiratory polygraphy. PATIENTS AND METHODS: Children with severe craniofacial anomalies referred to our paediatric airway unit from February 2001 to June 2011 were eligible to be included in this retrospective, single centre study. Symptoms of airway obstruction were proactively investigated using the shorter version of the Pediatric Sleep Questionnaire (PSQ). Obstructive sleep apnea was assessed by means of cardiorespiratory polygraphy. Demographic data and reason for referral were also recorded. Primary outcomes were the prevalence of symptoms of airway obstruction and OSA. RESULTS: 44 children (24 girls) with severe craniofacial anomalies (15 Crouzon, 13 Apert, 9 Goldenhar, 5 Treacher-Collins, 2 Pfeiffer) were included, at a mean age of 5 years (range 8 months to 14 years). Reason for referral was routine follow up in 30 patients and overt OSA symptoms and signs in the remaining 14. PSQ results showed symptoms of airway obstruction in 82% of patients, being snoring the most frequent symptom (64.1%) followed by apneas (33.3%). Polygraphic studies showed inconclusive results in 8 children (18.2%), normal apnea-hypopnea index (AHI) in 16 (36.4%), mild obstructive sleep apnea in 9 (20.4%), moderate in 4 (9.1%) and severe obstructive sleep apnea in 7 (15.9%). CONCLUSIONS: Children with craniofacial anomalies have a high prevalence of symptoms of airway obstruction and obstructive sleep apnea that support a proactive screening strategy in this highly selected population.