Secuelas endocrinológicas en niños y adolescentes supervivientes de tumores del sistema nervioso central tras 5 años de seguimiento / Endocrinological outcome in children and adolescents survivors of central nervous system tumours after a 5 year follow-up

INTRODUCCIÓN: Con las terapias actuales, la supervivencia de los tumores del sistema nervioso central (TSNC) es cada vez mayor y, con ello, las complicaciones a largo plazo.ObjetivoEvaluar las secuelas endocrinológicas en niños con TSNC en relación con el tipo de neoplasia y el tratamiento recibido. Sujetos y métodos: Se revisaron retrospectivamente los datos clínicos, auxológicos, analíticos y radiológicos de 38 pacientes (36,8% mujeres y 63,2% varones) con antecedente de TSNC y seguimiento mínimo de 5 años. RESULTADOS: La media ± desviación estándar de edad al diagnóstico fue de 5,34 ± 3,07 años. El 76,3% de los casos presentó al menos un déficit hormonal, siendo el más prevalente el de hormona de crecimiento (GH) (73,7%), seguido de los déficits de tirotropina (TSH) (68,4%), corticotropina (31,6%), hormona antidiurética (28,9%) y gonadotropinas (LH/FSH) (21,1%). El 21,1% de los pacientes presentaron pubertad precoz. A los 5 años de seguimiento, el 28,9% presentaba obesidad. El craneofaringioma fue el tipo tumoral que registró mayor número de casos con deficiencias hormonales, obesidad y tasa de recurrencia. El tratamiento más frecuentemente administrado fue la combinación de cirugía + quimioterapia + radioterapia, empleado en el 47,4% de los pacientes. La talla final media ± desviación estándar (20 pacientes) fue −1,2 ± 1,6; con una disminución media de −0,53 DE respecto de su talla diana. CONCLUSIONES: 1) El tipo tumoral y el tratamiento recibido influyen sobre las secuelas endocrinológicas; 2) las deficiencias hormonales más frecuentes de todos los tipos de TSNC, independientemente del tratamiento recibido, fueron GH y TSH; 3) el diagnóstico precoz y la intervención temprana sobre la disfunción endocrina, reducen la morbilidad y mejoran la calidad de vida a largo plazo

INTRODUCTION: Given the successful increase in survival rates with the current treatments forcentral nervous system tumours (CNST), survivors are at high risk for late adverse effects. PURPOSE: To evaluate the endocrine sequelae in children with CNST according to the type of tumour and treatment received. PATIENTS AND METHODS: A retrospective review of the clinical features, auxology, hormone determinations and imaging findings of 38 patients (36.8% females, 63.2% males) with CNST, with a minimum of 5 years follow-up, was performed. RESULTS: The mean age at diagnosis was 5.34±3.07 years, with 76.3% of the patients having at least one hormone deficiency, of which growth hormone (GH) (73.7% of all patients) was the most prevalent, followed by thyrotropin (TSH) (68.4%), corticotropin (31.6%), antidiuretichormone (28.9%), and gonadotropin (LH/FSH) (21.1%) deficiency. Precocious puberty was found in 21.1% of patients. After 5 years of follow-up, 28.9% were obese. Craniopharyngioma had more hormone deficiencies, obesity and recurrence rates. The most frequently administered treatment was surgery + chemotherapy + radiotherapy, in 47.4% of the patients. Mean final height (20 patients) was−1.2 1.6 SDS, with a mean difference of−0.53 SDS regarding their target height. CONCLUSIONS: 1) The type of tumour and treatment received influence the endocrinological sequelae. 2) The most frequent hormone deficiencies in all types of CNST, regardless of the treatment received, were GH and TSH. 3) Early diagnosis and prompt intervention of endocrine dysfunction can reduce the morbidity and improve quality of life over the long term

[Endocrinological outcome in children and adolescents survivors of central nervous system tumours after a 5 year follow-up]. / Secuelas endocrinológicas en niños y adolescentes supervivientes de tumores del sistema nervioso central tras 5 años de seguimiento.

INTRODUCTION: Given the successful increase in survival rates with the current treatments for central nervous system tumours (CNST), survivors are at high risk for late adverse effects. PURPOSE: To evaluate the endocrine sequelae in children with CNST according to the type of tumour and treatment received. PATIENTS AND METHODS: A retrospective review of the clinical features, auxology, hormone determinations and imaging findings of 38 patients (36.8% females, 63.2% males) with CNST, with a minimum of 5 years follow-up, was performed. RESULTS: The mean age at diagnosis was 5.34 ± 3.07 years, with 76.3% of the patients having at least one hormone deficiency, of which growth hormone (GH) (73.7% of all patients) was the most prevalent, followed by thyrotropin (TSH) (68.4%), corticotropin (31.6%), antidiuretic hormone (28.9%), and gonadotropin (LH/FSH) (21.1%) deficiency. Precocious puberty was found in 21.1% of patients. After 5 years of follow-up, 28.9% were obese. Craniopharyngioma had more hormone deficiencies, obesity and recurrence rates. The most frequently administered treatment was surgery + chemotherapy + radiotherapy, in 47.4% of the patients. Mean final height (20 patients) was -1.2 1.6 SDS, with a mean difference of -0.53 SDS regarding their target height. CONCLUSIONS: 1) The type of tumour and treatment received influence the endocrinological sequelae. 2) The most frequent hormone deficiencies in all types of CNST, regardless of the treatment received, were GH and TSH. 3) Early diagnosis and prompt intervention of endocrine dysfunction can reduce the morbidity and improve quality of life over the long term.

Nefroma mesoblástico congénito con hiperecogenicidad medular que simula nefrocalcinosis / Congenital mesoblastic nephroma with medullary hyperechogenicity simulating nephrocalcinosis

El nefroma mesoblástico congénito (NMC) es un tumor raro, siendo el más frecuente a nivel renal en los pacientes menores de 2 meses. Su origen histológico es la estroma renal inmadura, y se distinguen los subtipos clásico, mixto y celular. El tratamiento de elección es quirúrgico y su pronóstico es excelente. Se han descrito casos de NMC asociado a nefrocalcinosis en relación con la hipercalcemia paraneoplásica. Exponemos el caso de un recién nacido que presenta en la ecografía imágenes de hiperecogenicidad medular renal bilateral, similar a una nefrocalcinosis, en el contexto clínico de un NMC(AU)

Congenital mesoblastic nephroma (CMN) is a rare tumour which is the most frequent in the first 2 months of life. Its histological origin is the immature renal stromal cells. There are three histological subtypes: clasic, mixte and cellular. The treatment of choice is surgical and the prognosis is excellent. CMN has been reported associated with nephrocalcinosis in relation to paraneoplasic hypercalcaemia. We report a case of a new born with ultrasound imagen of renal medullary hyperechogenicity simulating nephrocalcinosis in the clinical setting of CNM(AU)