Cancer papilar de tiroides infanto-juvenil: re-evaluación de los factores pronósticos y nueva propuesta de estadificación / Papillary thyroid carcinoma in children and adolescents.A re-evaluation of prognostic factors

El cáncer capilar de tiroide(CaP)es el tumor tiroideo más frecuente en la población infanto-juvenil.Sin embargo no están definidos en este grupo etario los factores predictivos de agresividad.Estudiamos 24 pacientes con CaP entre 1988 y 1999,edades 4,2 a 20 años(19 mujeres,5 varones)En el mismo lapso se operaronn 20 pacientes con adenomas tiroideos,demostrando una altisíma frecuencia de malignidad del 55 por ciento dentro de las neoplasias tiroideas.Los pacientes fueron divididos en dos grupos según la edad al diagnóstico:G1<9 años,n=8 y G2>9 años,n=16.Evaluamos edad,sexo,tamaño del nódulo,adenopatías regionales,infiltración a tejidos vecinos,metástasis hematógenas a distancia,demora diagnóstica,función tiroidea,anticuerpos antitiroideos y tiroglobulina sérica.Conclusiones:la presencia de adenopatías regionales no modifica la evolución favorable del tumor.En cambio la infiltracíon a tejidos vecinos sería un dato clínico de gran peso como predictor de la presencia de metástasis a distancia.Nuestros datos apoyan la propuesta de una nueva estadificación del CaP unificando invasión local y metástasis pulmonares como estadío IV y establecen como los factores pronósticos adversos de mayor peso a la edad y a la invasión local.De esta forma,el exámen clínico permitiría la predicción de la metástasis pulmonares y adecuar el tratamiento a la agresividad del tumor

Cáncer papilar de tiroides infanto-juvenil: re-evaluación de los factores pronósticos y nueva propuesta de estadificación / Papillary thyroid carcinoma in children and adolescents.A re-evaluation of prognostic factors and new staging proposal

El cáncer capilar de tiroide(CaP)es el tumor tiroideo más frecuente en la población infanto-juvenil.Sin embargo no están definidos en este grupo etario los factores predictivos de agresividad.Estudiamos 24 pacientes con CaP entre 1988 y 1999,edades 4,2 a 20 años(19 mujeres,5 varones)En el mismo lapso se operaronn 20 pacientes con adenomas tiroideos,demostrando una altisíma frecuencia de malignidad del 55 por ciento dentro de las neoplasias tiroideas.Los pacientes fueron divididos en dos grupos según la edad al diagnóstico:G1<9 años,n=8 y G2>9 años,n=16.Evaluamos edad,sexo,tamaño del nódulo,adenopatías regionales,infiltración a tejidos vecinos,metástasis hematógenas a distancia,demora diagnóstica,función tiroidea,anticuerpos antitiroideos y tiroglobulina sérica.Conclusiones:la presencia de adenopatías regionales no modifica la evolución favorable del tumor.En cambio la infiltracíon a tejidos vecinos sería un dato clínico de gran peso como predictor de la presencia de metástasis a distancia.Nuestros datos apoyan la propuesta de una nueva estadificación del CaP unificando invasión local y metástasis pulmonares como estadío IV y establecen como los factores pronósticos adversos de mayor peso a la edad y a la invasión local.De esta forma,el exámen clínico permitiría la predicción de la metástasis pulmonares y adecuar el tratamiento a la agresividad del tumor

The association of Wilms' tumor, male pseudohermaphroditism and diffuse glomerular disease (Drash syndrome): report of eight cases with clinical and morphologic findings and review of the literature.

In 8 children (6 males) at least 2 of 3 disorders were found--male pseudohermaphroditism (MPH), Wilm's tumor, and glomerular disease. MPH was present in the 6 males; they had abdominal cryptorchidism (6/6), ambiguous genitalia (6/6), negative sex chromatin (5/5), and 46XY karyotype (2/2). The gonads examined microscopically in 3 patients were dysgenetic testes. The renal tumor present in 7 was nephroblastoma (NB) of the classical type without anaplasia or nephroblastomatosis, bilateral in 1, and unilateral but multinodular in 2. Five underwent nephrectomy for a renal mass, and in 2 NB was found at open biopsy or at autopsy. The mean age at diagnosis was 10 months. Glomerular disease in 6 patients began with onset of the nephrotic syndrome between 20 days and 39 months of age; it was resistant to steroid therapy and led to death from renal failure. Microscopically the glomerular process was a diffuse mesangial sclerosis (DMS). The 2 children with NB and MPH, but without DMS are healthy 2 1/2 and 9 years postnephrectomy. Neither familial incidence nor parental consanguinity was found. This syndrome has complete and partial forms, and its early recognition is important both for patient management and for assessment of prognosis.

Glandular schwannoma in a 17-month-old child.

Glandular schwannoma is an infrequent tumor, especially in the pediatric age group. We report a case in which the tumor developed in the retroperitoneum of a 17-month-old girl without stigmata of Von Recklinghausen's disease (VRD). She presented a local recurrence and bone and pulmonary metastases at 11, 23, and 30 months, respectively, after tumor resection. The pathologic findings in the original tumor did not permit us to predict its potential to metastasize. The ultrastructural characteristics of both glandular and schwannian elements are described. The rarity of this neoplasia in children and the absence of histological parameters of aggressiveness are emphasized.

[Association of Wilms' tumor, pseudohermaphroditism and glomerular nephropathy. Partial and complete forms of the syndrome]. / Asociación de tumor de Wilms, pseudohermafroditismo y nefropatía glomerular. Formas incompletas y completa del síndrome.

Four patients, followed at the Buenos Aires Cildren's Hospital are reported: one with a nephrotic syndrome and male pseudohermaphroditism, the second with nephrotic syndrome, male pseudohermaphroditism and Wilms' tumor, the third with pseudohermaphroditism and Wilms' tumor and the fourth with a nephrotic syndrome and Wilms' tumor. The cases presenting nephrotic syndrome, all had an early onset and a rapid and fatal course leading to death in renal failure or secondary to a related bacterial infection. The renal histology in all of the cases with nephrotic syndrome, was very similar: diffuse involvement of all glomeruli consisting in a severe increase of the mesangial matrix, with scarce mesengial proliferation.

Cystic nephroma.

The clinical and pathologic findings of four cases of cystic nephroma (so-called "renal multilocular cyst") in four infants nephrectomized with the diagnosis of Wilms' tumor are presented. Each lesion consisted of an encapsulated mass composed of cysts separated by thin septa. Histologically the cysts were lined by epithelium, and the septa contained foci of immature metanephric blastema (abundant in one case and sparse in another). rhabdomyocytes and mature tubules. Neither embryonic ducts nor cartilage were seen. One cystic lesion was adjacent to a nephroblastoma which formed a separate nodule. No recurrences of metastases developed, and the only death was due to postoperative sepsis. These lesions are considered by some authors to be dysplasia, while others interpret them as benign neoplasms. Our findings and a review of the literature probably represent the differentiated counterpart of nephroblastoma.