Antônio Gonçalves de Oliveira-Filho

Adrenocortical tumors in Brazilian children : Immunohistochemical markers and prognostic factors

CONTEXT The behavior of adrenocortical tumors (ACTs) is usually difficult to establish in childhood, and the role of immunomarkers in predicting outcome has not yet been elucidated. OBJECTIVE To investigate the relationship between clinical, pathologic, and immunohistochemical findings and prognosis in a series of children with ACTs. PATIENTS AND METHODS Clinical data were evaluated retrospectively in 33 children with ACTs, including age at diagnosis, sex, time between first symptoms and diagnosis, clinical signs and symptoms, tumor position, and follow-up. Histologic sections were reviewed, each tumor was classified, and staging was performed according to previously published criteria. Immunohistochemical analysis of p53, Ki-67, c-Erb-B2, and Bcl-2 was performed according to previously published techniques. RESULTS Sixty-four percent (n = 21) of the patients were female, and the age at diagnosis in the cohort ranged from 2 to 96 months. Virilization alone affected 70% (n = 23) of the patients, and 18 patients had stage 1 disease, 9 had stage 2 disease, and 3 each had stage 3 and stage 4 disease. Female sex and stage 1 and stage 2 disease were associated with good outcome. None of the histopathologic criteria evaluated correctly predicted outcome. Only tumors with a volume exceeding 200 mL were associated with malignant behavior. Because only a small number of tumors expressed the antigens, results of these immunohistochemical tests were considered inconclusive. CONCLUSION In this sample of pediatric ACTs, the clinical and surgical parameters are the most important prognostic factors, while the immunohistochemical markers evaluated were not predictive of outcome.

Laparoscopic upper-pole nephroureterectomy in infants

OBJECTIVE Report the results of laparoscopic upper-pole nephroureterectomy in infants. MATERIALS AND METHODS Six consecutive infants underwent 7 laparoscopic upper-pole nephroureterectomy. Pre and postoperative evaluation included renal sonography, voiding cystourethrogram and renal scintigraphy. All infants showed upper-pole exclusion. Surgery was performed through a transperitoneal approach with full flank position in all infants. Three or 4 ports were used according to the necessity of retracting the liver. The distal ureter was ligated close to the bladder whenever reflux was present and the dysplastic upper-pole was divided with the help of an electrocautery. Data regarding operative time, postoperative use of analgesics, time to resume oral feeding, hospital stay and tubular function were collected and analyzed. RESULTS All procedures were concluded as planned. Mean operative time was 135 min. One patient underwent staged bilateral upper-pole nephrectomy. There were no complications and the postoperative hospital stay was 48 hours in 5 procedures and 24 hours in 2 procedures. Pain medication was required only in the first day. Renal tubular function showed improvement in half of the cases. CONCLUSION Laparoscopic partial nephrectomy is a safe and feasible procedure in infants. Due to the magnification provided by the lenses, a better vision of the structures is achieved, facilitating selective dissection of vascular upper-pole, renal parenchyma and distal ureter. This approach is less damaging to the lower pole, and is associated to low morbidity and a short hospital stay.

WILMS TUMOR IN A PRENATALLY DIAGNOSED MULTICYSTIC KIDNEY

Although conservative treatment of multicystic dysplastic kidney is generally accepted, there are reports of complications when such a kidney is retained, particularly malignant degeneration.1.2 We report on a neonate with Wilms tumor in a prenatally diagnosed multicystic dysplastic kidney. To our knowledge this is the fifth case of Wilms tumor arising in a multicystic dysplastic kidney but only the second diagnosed prenatally.

Epignathus: Report of a case with successful outcome

Epignathus is an extremely rare form of teratoma that arises from the palate or pharynx in the region of the basisphenoid (Rathkes pouch). This condition is associated with a high mortality rate caused by severe airway obstruction in the neonatal period, thus requiring prenatal planning and prompt surgical treatment after birth. The authors describe a case of a giant epignathus that was successfully resected followed by an uneventful recovery.

Evolution of critically ill patients with gastroschisis from three tertiary centers

OBJECTIVES AND INTRODUCTION: Gastroschisis is a congenital abdominal wall defect with increasing occurrence worldwide over the past 20–30 years. Our aim was to analyze the morbidity of newborns after gastroschisis closure, with emphasis on metabolic and hydroelectrolyte disturbances in patients at three tertiary university centers. METHODS: From January 2003 to June 2009, the following patient data were collected retrospectively: (A) Background maternal and neonatal data: maternal age, prenatal diagnosis, type of delivery, Apgar scores, birth weight, gestational age and sex; (B) Surgical modalities: primary or staged closure; and (C) Hospital course: levels of serum sodium and levels of serum albumin in the two first postoperative days, number of ventilation days, other postoperative variables and survival. Statistical analyses were used to examine the associations between some variables. RESULTS: 163 newborns were included in the study. Primary closure of the abdominal defect was performed in 111 cases (68.1%). The mean serum sodium level was 127.4±6.7 mEq/L, and the mean serum albumin level was 2.35±0.5 g/dL. Among the correlations between variables, it was verified that hyponatremia and hypoalbuminemia correlated with the number of days on the ventilator but not with the number of days on total parenteral nutrition (TPN); mortality rate correlated with infection. The final survival rate was 85.9%. CONCLUSION: In newborns with gastroschisis, more aggressive attention to hyponatremia and hypoalbuminemia would improve the outcome.

Chondromatous Hamartoma of the Chest Wall: Description of 3 New Cases and Literature Review

Chondromatous hamartoma of the chest wall is an extremely rare, benign lesion that usually occurs in early infancy. It typically arises in the rib cage and produces a large mass. It is composed of a varying admixture of hyaline cartilage that has features resembling growth plate cartilage, fascicles of spindle cells, woven bone, and hemorrhagic cysts. Treatment consists mainly of surgical excision, which is usually curative. We present 3 new cases, which demonstrated interesting findings, including multicentricity, involvement of the sternum, and local recurrence, and we discuss these findings in the context of a literature review.

Alpha-fetoprotein following neonatal resection of sacrococcygeal teratoma.

The sacrococcygeal teratoma (SCT) is the most frequent tumor in the neonatal period. Alpha-fetoprotein (AFP) levels fall after tumoral resection, although the time required for level normalization has not been established. The authors evaluate the time required for AFP levels to reach normal levels in 6 infants with prenatal diagnosis of SCT who underwent surgery for resection. The mean time required for AFP level normalization was 9 months. All patients were alive and 1 had neurogenic bladder. AFP has a progressive decrease, which may last 9 months in average and should not be confused with tumoral relapse.

Monitoring intravesical pressure during gastroschisis closure. Does it help to decide between delayed primary or staged closure

Introduction and objective: Correction of gastroschisis may be accomplished by either primary or staged closure or even delayed primary closure after the use of a preformed silo. However, there is neither a consensus on the best approach nor established criteria to favor one method over the other. The aim of this paper was to investigate the role of intravesical pressure (IVP) as a tool to prevent abdominal compartment syndrome in newborns undergoing correction of abdominal wall defects. Methods: We retrospectively analyzed 45 newborns with gastroschisis in whom trans-operative intravesical pressure was used to choose between primary or staged closure. A threshold of 20 cm H2O was used and the outcomes between the two methods were compared. Results: In 24 children delayed primary closure was achieved while the remaining 21 underwent staged reduction and closure. There was no difference in the frequency of complications, time to begin oral feeding, length of parenteral nutrition or length of hospital stay between the children of the two groups. The incidence of temporary oliguria or anuria, averaged 33% and it was similar in both groups of children. Conclusion: The data here presented suggests that monitoring intraoperative IVP during correction of gastroschisis may help to select children in whom staged closure is necessary, keeping their complication rate and overall outcome similar to that of children undergoing delayed primary closure. Further prospective studies should investigate more deeply the correlation between type of closure and the development of a compartment syndrome.

Selective surgical indication in the management of neutropenic children presenting with acute abdomen.

As the treatment of pediatric malignancies improves and survival increases, the diagnosis of acute abdomen in these patients also becomes more common. Nevertheless, the management of this a condition is still controversial. The authors report their experience in treating 12 neutropenic children with acute abdomen. The charts of 12 neutropenic patients with a diagnosis of acute abdomen treated at Boldrini Childrens Cancer Center in Campinas, Brazil, between 1991 and 1996, were reviewed. Therapeutic strategy included an initial period of bowel rest, general supportive measures, and broadspectrum antibiotics while waiting for the neutrophil count to rise. Three patients recovered completely without surgery, 8 under went late surgery without complications, and 1 died due to uncontrolled sepsis before surgery. The treatment of acute abdomen in neutropenic children remains controversial. As shown in the present series, an initial nonoperative approach with selective surgical indication appears to be safe and to yield good results. Supportive treatment, until the neutrophil count rises, followed by surgery, if necessary, appears to be a sound therapeutic approach for neutropenic children with acute abdomen.

A unique case of synchronous functional adrenocortical adenoma and myelolipoma within the ectopic adrenal cortex in a child with Beckwith–Wiedemann syndrome

We report a unique case of synchronous functional adrenocortical adenoma and an incidental myelolipoma within ectopic cortical adrenal tissue located in the renal hilum in a child with Beckwith-Wiedemann syndrome and review the association between adrenal gland disorders and myelolipomas. To the best of our knowledge, this is the first documented case of a simultaneous occurrence of these three conditions. A 17-month-old child with Beckwith-Wiedemann syndrome was diagnosed with a left adrenal tumor during complementary radiologic studies. Biochemical investigation before surgery showed elevated blood levels of cortisol and dehydroepiandrosterone hormones. The patient underwent a left adrenalectomy with ipsilateral renal hilar and intercaval-aortic lymph node dissection. Pathology findings revealed a left adrenocortical adenoma and an incidental myelolipoma growing within ectopic cortical adrenal tissue in the renal hilum. The patient is doing well and does not have any current health issues. Patients with adrenal cortex disorders, such as hyperplasias and neoplasms, particularly when associated with hormonal imbalances, may have an increased risk of developing myelolipomas. Whether Beckwith-Wiedemann syndrome may, by itself, contribute to simultaneous occurrence of adrenocortical adenomas and myelolipomas remains to be clarified.