RESUMEN
Neonatal Cushing syndrome (CS) is most commonly caused by exogenous administration of glucocorticoids and rarely by endogenous hypercortisolemia. CS owing to adrenal lesions is the most common cause of endogenous CS in neonates and infants, and adrenocortical tumors (ACTs) represent most cases. Many ACTs develop in the context of a TP53 gene mutation, which causes Li-Fraumeni syndrome. More rarely, neonatal CS presents as part of other syndromes such as McCune-Albright syndrome or Beckwith-Wiedemann syndrome. Management usually includes resection of the primary tumor with or without additional medical treatment, but manifestations may persist after resolution of hypercortisolemia.
Asunto(s)
Neoplasias de la Corteza Suprarrenal/congénito , Adenoma Corticosuprarrenal/congénito , Carcinoma Corticosuprarrenal/congénito , Síndrome de Cushing/congénito , Corteza Suprarrenal/cirugía , Neoplasias de la Corteza Suprarrenal/diagnóstico , Neoplasias de la Corteza Suprarrenal/metabolismo , Neoplasias de la Corteza Suprarrenal/terapia , Adenoma Corticosuprarrenal/diagnóstico , Adenoma Corticosuprarrenal/metabolismo , Adenoma Corticosuprarrenal/terapia , Carcinoma Corticosuprarrenal/diagnóstico , Carcinoma Corticosuprarrenal/metabolismo , Carcinoma Corticosuprarrenal/terapia , Quimioterapia Adyuvante , Ritmo Circadiano , Síndrome de Cushing/diagnóstico , Síndrome de Cushing/metabolismo , Síndrome de Cushing/terapia , Humanos , Hidrocortisona/metabolismo , Recién NacidoRESUMEN
We describe a case of congenital non-functional adrenocortical carcinoma in a male infant who presented with recurrent pneumonia, paraparesis and sclerotic skeletal metastasis. To the best of our knowledge such presentation has never been reported.
Asunto(s)
Neoplasias de la Corteza Suprarrenal/congénito , Carcinoma/congénito , Osteosclerosis/etiología , Compresión de la Médula Espinal/etiología , Neoplasias de la Médula Espinal/secundario , Enfermedades de la Columna Vertebral/etiología , Tomografía Computarizada por Rayos X , Neoplasias de la Corteza Suprarrenal/diagnóstico por imagen , Neoplasias de la Corteza Suprarrenal/patología , Biopsia con Aguja Fina , Carcinoma/diagnóstico por imagen , Carcinoma/patología , Carcinoma/secundario , Diagnóstico Diferencial , Disnea/etiología , Humanos , Lactante , Masculino , Osteosclerosis/diagnóstico por imagen , Cuidados Paliativos , Paraparesia/etiología , Feocromocitoma/diagnóstico , Neumonía/etiología , Recurrencia , Neoplasias de la Médula Espinal/complicaciones , Neoplasias de la Médula Espinal/diagnóstico por imagen , Enfermedades de la Columna Vertebral/diagnóstico por imagen , Vértebras Torácicas/patologíaRESUMEN
The differential diagnosis of prenatally diagnosed adrenal masses includes neuroblastoma, adrenal hemorrhage, adrenal and cortical renal cysts, adrenal adenoma and carcinoma, subdiaphragmatic pulmonary sequestration, Beckwith-Wiedemann syndrome, duplication of the renal system, Wilms tumors, congenital mesoblastic nephroma, and mesenteric and enteric duplication cysts. The worldwide annual incidence of childhood adrenal cortical neoplasms ranges between 0.3 and 0.38 per 1 million children younger than 15 years. These neoplasms are even more unusual among infants, with only 23 cases reported in the literature.
Asunto(s)
Neoplasias de la Corteza Suprarrenal/congénito , Neoplasias de la Corteza Suprarrenal/diagnóstico , Adenoma Corticosuprarrenal/congénito , Adenoma Corticosuprarrenal/diagnóstico , Ultrasonografía Prenatal/métodos , Corteza Suprarrenal/diagnóstico por imagen , Corteza Suprarrenal/cirugía , Neoplasias de la Corteza Suprarrenal/cirugía , Adenoma Corticosuprarrenal/cirugía , Adulto , Diagnóstico Diferencial , Femenino , Humanos , Imagenología Tridimensional/métodos , Recién Nacido , Masculino , Embarazo , Radiografía Abdominal/métodos , Tomografía Computarizada por Rayos XRESUMEN
Congenital ardrenocortical neoplasms are exceedingly rare. Our review of the medical literature revealed 23 reported cases of adrenocortical neoplasm including this one. Eighteen of these cases were adrenocortical carcinoma and four were grouped as adrenocortical tumor. We have not found any reported case with a histological diagnosis of a congenital adrenocortical adenoma. We present this case of a congenital adrenocortical neoplasm with histological findings consistent with an adrenocortical adenoma in a premature infant aged 27 weeks and 4 days who had a prenatal sonogram showing a cystic right abdominal mass and a physical examination demonstrating a palpable mass.
Asunto(s)
Neoplasias de la Corteza Suprarrenal/congénito , Neoplasias de la Corteza Suprarrenal/diagnóstico , Adenoma Corticosuprarrenal/congénito , Adenoma Corticosuprarrenal/diagnóstico , Neoplasias de la Corteza Suprarrenal/diagnóstico por imagen , Adenoma Corticosuprarrenal/diagnóstico por imagen , Carcinoma Corticosuprarrenal/congénito , Carcinoma Corticosuprarrenal/diagnóstico , Diagnóstico Diferencial , Humanos , Recién Nacido , Masculino , Ultrasonografía Doppler en ColorAsunto(s)
Neoplasias de la Corteza Suprarrenal/patología , Carcinoma Corticosuprarrenal/patología , Neoplasias Hepáticas/secundario , Neoplasias de la Corteza Suprarrenal/congénito , Neoplasias de la Corteza Suprarrenal/diagnóstico , Carcinoma Corticosuprarrenal/congénito , Carcinoma Corticosuprarrenal/diagnóstico , Humanos , Recién Nacido , MasculinoRESUMEN
We describe a case of metastasizing congenital adrenocortical carcinoma and a follow-up of 3 1/2 yr. Treatment with surgery and mitotane was associated with multiple complications. The patient was in remission at 3 1/2 yr. Because of the rarity of this condition, we discuss step-by-step problems encountered during management.
Asunto(s)
Neoplasias de la Corteza Suprarrenal/cirugía , Neoplasias de la Corteza Suprarrenal/congénito , Neoplasias de la Corteza Suprarrenal/tratamiento farmacológico , Neoplasias de la Corteza Suprarrenal/patología , Antineoplásicos Hormonales/uso terapéutico , Terapia Combinada , Estudios de Seguimiento , Humanos , Hidrocortisona/uso terapéutico , Recién Nacido , Masculino , Mitotano/uso terapéutico , Metástasis de la Neoplasia , Complicaciones Posoperatorias , Factores de Tiempo , Tomografía Computarizada por Rayos XRESUMEN
We describe a very rare case of adrenocortical carcinoma (ACC) presenting with Cushing's virilising syndrome in a female child with congenital hemihypertrophy (CHh). CHh was of more value for early detection of ACC than Cushing's virilisation.
Asunto(s)
Neoplasias de la Corteza Suprarrenal/congénito , Síndrome de Cushing/congénito , Gigantismo/congénito , Adolescente , Neoplasias de la Corteza Suprarrenal/cirugía , Niño , Preescolar , Femenino , Estudios de Seguimiento , Humanos , Recurrencia Local de Neoplasia/cirugía , Neoplasias Primarias Múltiples/congénito , Neoplasias Primarias Múltiples/cirugía , Virilismo/congénitoRESUMEN
In the last 10 years, four children with adrenocortical tumors were treated in our hospital. Three of them had symptoms of adrenogenital virilizing syndrome, and another, nonfunctional, was found during a routine examination. A diagnosis of adrenocortical tumor was established given the symptoms, hormonal tests, and radiologic studies. Two of the tumors were located in the left side, and the histologic diagnosis was pleomorphic cortical adenoma; the ones that affected the right side were adenocarcinomas. All the patients were treated by surgery and none received chemotherapy. At both presurgical and postsurgical stages, the patients were treated with cortisol. All four children have had a favorable course, with normal growth and the disappearance of public hair and hirsutism; however, macrogenitals still persist.
Asunto(s)
Adenoma/congénito , Neoplasias de la Corteza Suprarrenal/congénito , Síndromes Paraneoplásicos Endocrinos/congénito , Adenoma/complicaciones , Adenoma/cirugía , Neoplasias de la Corteza Suprarrenal/complicaciones , Neoplasias de la Corteza Suprarrenal/cirugía , Niño , Preescolar , Femenino , Humanos , Masculino , Síndromes Paraneoplásicos Endocrinos/cirugía , Pubertad Precoz/etiología , UrografíaRESUMEN
Adrenal cortical carcinoma is an uncommon neoplasm in children. Only a handful of congenital adrenal cortical carcinoma cases have been described. A newborn who had metastatic adrenal cortical carcinoma (skin metastases and cerebral lesions) is described. This patient underwent surgical resection of the right adrenal primary, but no further treatment was given. Hemihypertrophy developed in this patient by 2 months of age, and at 4 months of age spontaneous regression of all skin nodules and central nervous system (CNS) lesions was observed. Follow-up at 1 year shows the patient to be alive, well, and disease-free. Evaluation of the tumor included DNA ploidy analysis that showed the tumor to be polyploid, a pattern recently associated with nonmetastasizing adrenal cortical neoplasm. The observation of apparent metastatic disease that regressed spontaneously highlights the prognostic value of DNA ploidy analysis and raises the possibility of an adrenal tumor with properties similar to those of Stage IV-S neuroblastoma.
Asunto(s)
Neoplasias de la Corteza Suprarrenal/congénito , Regresión Neoplásica Espontánea , Ploidias , Neoplasias de la Corteza Suprarrenal/genética , Neoplasias de la Corteza Suprarrenal/patología , ADN de Neoplasias/genética , Humanos , Recién Nacido , MasculinoRESUMEN
Masses involving the adrenal in the neonate are most commonly due to hemorrhage. The literature involving the neonatal adrenal reflects this propensity. Although there have been reports of newborns with neuroblastoma [1, 2] and other tumors [3], which are more common in older children, ultrasonographic descriptions of masses involving the adrenal secondary to such tumors are rare [1]. Within a 6-month span we have discovered a clinically unsuspected adrenal carcinoma and adrenal neuroblastoma.
Asunto(s)
Neoplasias de la Corteza Suprarrenal/congénito , Carcinoma/congénito , Neuroblastoma/congénito , Humanos , Recién Nacido , MasculinoRESUMEN
Sexual ambiguity at birth in a girl usually suggests congenital adrenal hyperplasia. After exclusion of this diagnosis, we thought of an isolated abnormal state. It was only when the girl was 14 months old that the diagnosis of adrenocortical tumor with signs of virilism was stated. The difficulties of this diagnosis and the therapeutical possibilities of such cases are discussed.