Resection of primary tumor at diagnosis in stage IV-S neuroblastoma: does it affect the clinical course?

PURPOSE: To determine whether resection of primary tumor has a favorable influence on outcome of infants (age 0 to 11 months) with stage IV-S neuroblastoma. PATIENTS AND METHODS: Between March 1976 and December 1993, 97 infants with previously untreated neuroblastoma diagnosed in 21 Italian institutions were classified as having stage IV-S disease. Seventy percent were younger than 4 months. Adrenal was the primary tumor site in 64 of 85 patients with a recognizable primary tumor. Liver was the organ most often infiltrated by the tumor (82 patients), followed by bone marrow and skin. RESULTS: The overall survival (OS) rate at 5 years in 80% and event-free survival (EFS) rate 68%. In 24 infants, the effect of resection of primary tumor could not be evaluated because of rapidly fatal disease progression (n = 8), absence of a primary tumor (n = 12), or partial resection (n = 4). Of 73 assessable patients, 26 underwent primary tumor resection at diagnosis: one died of surgical complications, one relapsed locally and died, and two others relapsed (one of these two locally) and survived, for a 5-year OS rate of 92% and EFS rate of 84%. Of the remaining 47 patients who did not undergo primary tumor resection at diagnosis 11 suffered unfavorable events, of whom five died, for an OS rate of 89% and EFS rate of 75% (no significant difference from previous group). Disease recurred at the primary tumor site in only one five who died, and in only one of six survivors of progression or relapse; in these patients, the primary tumor, located in the mediastinum, was successfully resected. CONCLUSION: Infants who underwent resection of the primary tumor at diagnosis had no better outcome than those in whom the decision was made not to operate.

Ectopic neuroblastoma of the thoracic wall: a case report.

A case of thoracic neuroblastoma arising at the anterior mammillary line from the inner surface of the second and third right rib in a 17-month-old male child is reported. Primary surgery allowed excision of the mass. According to the histologic, histochemical, and molecular features of the tumor, all consistent with the diagnosis of neuroblastoma stage 2, the patient underwent adjuvant chemotherapy and, after 2 years, he is alive and disease free at follow-up. In view of different clinical and therapeutical approaches, only a strict adherence to histopathologic criteria allows differentiation of neuroblastoma, even in a very unusual anatomical site, from other small-cell, peripheral neoplasms presumably arising from the embryonic neural crest, such as Askin's tumor, neuroepithelioma, and ectomesenchymoma. The site of origin of these tumors is an important diagnostic clue but can be misleading: only careful microscopic evaluation and molecular analysis lead to incontrovertible diagnosis and yield correct clinical management.

Feeding troubles following delayed primary repair of esophageal atresia.

Severe feeding troubles were recorded in five babies with long-gap esophageal atresia who underwent, between 1985 and 1990, a delayed primary anastomosis after spontaneous growth of their esophageal stumps. A comparison with 20 cases of direct esophageal anastomosis, operated on in the same period, was carried out by means of recorded esophagrams, pH monitoring and questionnaires charting the growth pattern and feeding habits of the patients. Bottle feeding, and, later on, the introduction of semi-solid foods was significantly retarded in the group of children with delayed primary anastomosis (labeled as group B) as well as height and weight parameters. Failure to complete feeds, dysphagia, vomiting, coughing, choking and recurrent respiratory symptoms were also significantly more common in this group than in the primary anastomosis group (labeled as group A) even in the absence of stricture. Variable degrees of disordered esophageal motility were present in all patients but pooling of the contrast medium, retrograde flow and delayed clearing of the esophagus were more frequent in group B. No patient was shown to have associated hiatal hernia. A 24 hour pH recording showed severe gastroesophageal reflux in 4 out of 13 cases of group A and in 3 out of 5 cases of group B. Clearing times were significantly delayed in all refluxing children. Our data suggest that the retarded start of oral feeding and swallowing coordination in patients with delayed primary anastomosis add further negative factors to their congenitally impaired esophageal motility, causing protracted dysphagia which represents a major problem for both family and hospital staff.

[Unusual case of ano-rectal malformation]. / Inusuale caso di malformazione ano rettale.

The authors present an unusual case of anorectal malformation: it's an intermediate from with a cutaneous fistulous tract. This case is similar to that described in 1982 by the Japan study group of anorectal anomalies.

[Omphalocele: 10-year-experience]. / Onfalocele: dieci anni di esperienza.

We review our experience of 32 cases of omphalocele treated in the last ten years. Improved neonatal intensive care unit, as well as other support care measures, allowed to extend even to the large defects the indication to radical primary repair. Our experience has supported that this is the method of choice in almost all cases. At present the surgical success depend on associated anomalies, cardiac especially (25% in our series), and on development of sepsis. Analysis of the results shows an overall mortality rate of 41% in the period 1973/1982, significantly decreased to 33% in the last five years.

[Intestinal occlusion caused by remnants of the vitelline artery]. / Occlusione intestinal da residui dei vasi vitellini.

The authors present the unusual occurrence of intestinal occlusion due to vitelline artery remnants. They emphasize this situation is very rare in medical literature.

[Orchiopexy by the Stephens-Fowler method (thermographic evaluation of the results)]. / Orchiopessia secondo Stephens e Fowler (Valutazione termagrafica dei risultati).

The authors present 12 cases of orchiopexy second Stephens and Fowler. They discuss the surgical technique and focus their attention on the evaluation of the functional result: they stress the utility of the thermography in this evaluation.

Regina Margherita Children's Hospital, Turin, Paediatric Surgery Division.

Atresia of the colon was first described in the literature. The authors present 4 cases seen at the paediatric surgery division of the Regina Margherita Children's Hospital in Turin. After a brief discussion of the embryology, attention is focused on the treatment of this malformation.

[Diagnosis of megacolon in the neonatal period]. / Diagnostica degli stati megacolici nel periodo neonatale.

The importance of diagnosis in megacolon is underscored and an account is given of its radiological and histochemical aspects. If correctly performed, radiological examination enables the condition to be recognised. This was always the case in a personal series of 17 cases. Histochemical examination according to Meyer-Ruge is also useful. This is a simple method and demands minimal withdrawals from the submucosa. The findings are also more readily interpreted than those offered by conventional histological examination. Stress is laid on the possibility of false negatives in the newborn as described in the recent literature.