C. Everett Koop

SPECIAL PATTERN OF WIDESPREAD NEUROBLASTOMA WITH A FAVOURABLE PROGNOSIS

Abstract There is a group of children with disseminated neuroblastoma with a surprisingly good prognosis. Patients who fit the syndrome can have widespread disease in the liver, skin, and bone-marrow, or any combination of these. The primary tumour in some may be relatively small. 21 of 25 such patients (84%) survived for two years or longer. Radiation therapy and chemotherapy may not be necessary in the management of certain children with this syndrome.

SURGICAL MANAGEMENT OF DUPLICATIONS OF THE ALIMENTARY TRACT.

Abstract Duplications may be found along any portion of the alimentary tract. They may or may not communicate and share a common wall with adjacent bowel; they may be cystic, tubular or multiple, may involve several body cavities, may be associated with other anomalies and may lead to secondary complications. Proper surgical management requires a thorough knowledge of the anatomic varieties and various surgical technics that have been successfully used.

Neuroblastoma: An assessment of therapy in reference to staging

Abstract A total of 134 patients with neuroblastoma have been treated in one clinic with a relatively consistent method of management. When these patients are staged according to the system of Evans et al., the results in reference to survival would seem to validate the staging system and warrant its use by others. If the stage of the tumor is accurately assessed, patients with Stage I and II tumors require no radiation therapy whereas those with Stage III tumors benefit from postoperative irradiation.

Diagnosis and Treatment of Neuroblastoma

This article focuses on signs and symptoms, diagnostic and radiological studies, differential diagnosis, and a staging system of neuroblastoma. The roles of surgical management, radiation therapy, and chemotherapy are assessed.

Colon interposition for the short bowel syndrome

A 5-mo-old male had disabling diarrhea and malabsorption following massive small bowel resection. His transit time was 10 min. After 9 mo of conservative treatment, a 24-cm isoperistaltic segment of colon was interposed 6.5 cm from the ligament of Treitz. Transit time was increased to 105 min, and he was eventually able to be maintained on an enteric diet without significant diarrhea. Colon interposition requires little manipulation of the remaining small intestine and does not rely on active intestinal obstruction to effect an increased transit time.

Mesenchymal hamartoma of the liver in infants

Mesenchymal hamartoma of the liver is a rare benign tumor of childhood which has been confused with various other benign liver lesions, particularly vascular hamartomas which are mesodermal but not mesenchymal. The mass, frequently cystic, is characterized by an admixture of epithelial structures in a loose connective tissue stroma with fluid accumulation suggestive of lymphangiomatous channels. Four patients are presented and reviewed with 25 previously reported cases. The patients, usually asymptomatic, present during the first two years of life with progressive abdominal distention, which may be rapid because of increasing fluid content in the connective tissue stroma and cysts. Exploration and biopsy may be necessary before definitive excision. The present cases include a newborn, the youngest known, and a case in which radiation resulted in hyalinization of the mesenchyme, decrease in fluid content, and easlier resection. Respiratory distress and signs of vena caval obstruction due to intra‐abdominal pressure were noted. Prognosis after extirpation is very good. Cancer 42:2483–2489, 1978.

The role of multimodal therapy in patients with local and regional neuroblastoma.

The records of 49 patients with local and regional neuroblastoma treated at The Childrens Hospital of Philadelphia between 1972-1981 were reviewed to determine the contribution of radiation therapy and chemotherapy to their management. All 11 state I patients were treated with surgery alone and 10 (91%) survived for periods ranging from 3 to 10 years. Half of the 24 stage II patients received radiation therapy with or without chemotherapy and 8/12 (67%) survive. Ten of the 12 remaining stage II patients survive (83%) following surgery alone. All stage II patients had residual disease and 13 had involvement in lymph nodes. 7 in the combined treatment group and 6 in the surgery alone group. All 14 stage III patients received postoperative chemotherapy and radiation therapy (RT) and 7 or 50% remain disease free. We conclude that surgery alone is sufficient for patients with stage I NBL, that RT and chemotherapy do not appear to alter the outcome in stage II patients, and better methods of therapy are needed for patients with stage III disease.

Percutaneous subclavian venous catheters in neonates and children

There were 191 central venous catheters placed through the subclavian vein in 135 neonates and children over a 20-mo period, providing central venous access for a total of 4525 patient days. There were 132 (69.2%) catheters inserted in patients who were in their first year of life and 60 (31.4%) weighed less than 2.5 kg. The procedure was associated with a low technical complication rate. The greatest potential intermediate or long-term complication was the development of primary catheter sepsis that occurred in 11 (5.8%) of the catheters placed.

The management of abdominal neuroblastoma

Although survival of children with abdominal neuroblastoma seems at this time to be unrelated to the therapeutic effort, a method of management according to the stage of disease has been developed. Seventy‐three percent of patients have metastases at the time of diagnosis. Survival is best in children under 1 year of age. Survival by stage of disease drops progressively from 90% in Stage I disease to 2.4% in Stage IV disease. Irradiation therapy seems unrelated to survival in Stage I, II, and IV disease, while chemotherapy has not been shown to alter survival rates in any stage. Surgical removal of the tumor is still the primary therapy; irradiation is of significant benefit in patients with Stage III disease.

Silastic abdominal patch for temporary hepatomegaly in stage IV-S neuroblastoma

Of our 174 neuroblastoma patients, 11 were classified as Stage IV-S. The primary tumor is usually small and there is widespread disease in the liver, skin, bone marrow, or any combination of these. The prognosis is favorable and radiation and chemotherapy should be avoided if possible. In some of these children the rapidly enlarging liver may cause respiratory distress and vena caval compression. The creation of a ventral hernia by means of a Silastic patch is a useful, temporary procedure during the life-threatening period of rapid growth and increased intra-abdominal pressure. Closure is simple once the liver has returned to normal.