Deborah L. Day

Primary pulmonary rhabdomyosarcoma of the lung in children: report of two cases presenting with spontaneous pneumothorax

This report describes two cases of pulmonary rhabdomyosarcoma occurring in children, ages 21 and 30 months at diagnosis. Both patients presented with spontaneous pneumothorax and had cystic changes in the affected lung by chest x‐ray. Ultrastructural and immunohistochemical studies supported the myogenic phenotype of these neoplasms. The authors were unable to confirm that either of these tumors had unequivocally originated in a congenital cyst although previous authors have reported this association. An apparent morphologic spectrum of embryonic appearing neoplasms including pulmonary blastoma and a malignant mesenchymoma have been observed in the lungs of children. Cancer 59:1005‐1011, 1987.

Hereditary tyrosinemia type I (chronic form): Pathologic findings in the liver

Hereditary tyrosinemia type I presents with either acute hepatic failure in the neonatal period or later in infancy with progressive liver dysfunction secondary to cirrhosis. The inevitably fatal outcome in those children with the chronic form has been transformed with the advent of liver transplantation. Native livers from five children who received allografts were studied pathologically and compared with earlier hepatic biopsies in two of these patients that had been performed several years before transplantation. Our findings support the conclusion that a sequence of morphologic changes from the initial micronodular cirrhosis through an intermediate mixed cirrhotic pattern to macronodular cirrhosis occurs. The micronodular phase is transitory, over a period of only a few months, since mixed micronodular macronodular cirrhosis was already present in the livers of children who received transplants by 11 months of age. Focal hepatocellular dysplasia was present in one of the livers with mixed cirrhosis but was not identified in the other two cases. Macronodular cirrhosis accompanied two cases of hepatocellular carcinoma in this study. In order to preclude the latter complication, liver replacement is necessary before the age of 2 years.

CT findings in left paraduodenal herniae

Computed tomography (CT) was used to evaluate mass effect on the greater curvature of the stomach in two children with histories of intermittent, recurrent vomiting. The paraduodenal herniae were identified on CT in both of these patients as small bowel interposed between the stomach and the body of the pancreas.

Metastasizing Chordoma in Early Childhood

A 2 1/2-year-old female with a sphenooccipital-vertebral chordoma presented with neck pain, torticollis, fever, a lytic lesion of C2 vertebra, and bilateral nodular infiltrates in the lung. The lung biopsy revealed multiple tumor emboli by an enigmatic epithelioid-appearing neoplasm with immunohistochemical staining for vimentin, cytokeratin, and epithelial membrane antigen. A thorough roentgenographic evaluation disclosed a destructive, prepontine mass in the region of the clivus, erosion of the odontoid process, and compression of the cervical spinal cord. The patient died after a clinical course of 3 months. We identified 16 additional cases of metastasizing chordomas in the pediatric-age population; this case is the first to our knowledge with pathologically documented pulmonary metastasis at presentation.

Post-operative abdominal CT scanning in extrahepatic biliary atresia

A retrospective review of the abdominal CT scans of 26 children with extrahepatic biliary atresia was performed, and the results were correlated with available surgical and pathologic data. Associated congenital anomalies or acquired abnormalities were identified in these patients. Congenital anomalies included polysplenia, venous anomalies and bowel stenosis. Acquired abnormalities developed secondary to cirrhosis, portal hypertension, intrahepatic biliary duct dilatation, and hepatic ischemia. Despite frequent episodes of ascending cholangitis in these children, no hepatic abscesses were identified by CT or by pathologic examination. In conclusion, abdominal CT scanning of children with extrahepatic biliary atresia can define congenital and acquired abnormalities and provide important anatomic data for the surgeons before liver transplantation.

Kidney size at diagnosis of childhood acute lymphocytic leukemia: lack of prognostic significance for outcome.

The prognostic significance of kidney size at diagnosis of acute lymphoblastic leukemia (ALL) was assessed in a population of 142 children. Kidney size was determined using three different methodologies, and its significance was determined by univariate and multivariate life-table methods. Enlarged kidney size (as determined by any of the three methods used) was not associated with an overall poorer survival. These findings were consistent when kidney size at diagnosis was analyzed as a singled variable and when it was considered after adjustment for the known prognostic factors of age, sex, and initial WBC count. Assessment of renal size at the time of diagnosis of childhood ALL is not indicated for the purpose of predicting subsequent prognosis.

CT appearance of antibiotic-induced colitis

Eleven abdominal computed tomographic (CT) examinations were performed in 10 patients with antibiotic-induced colitis. The clinical, endoscopic, and CT findings are presented. The most common CT findings were colonic dilatation and bowel wall thickening. However, small bowel dilatation was commonly seen. Intramural gas and ascites were less frequent findings. The diagnosis of antibiotic-induced colitis was rarely made before CT examination. Consequently, attentiveness to these CT abnormalities can expedite specific treatment. When these findings are noted on CT they represent the equivalent of toxic megacolon and administration of rectal contrast is contraindicated.

Hodgkin's disease in children: correlation of clinical characteristics, staging procedures, and treatment at the University of Minnesota.

The cases of 63 children treated for Hodgkins disease were retrospectively evaluated according to clinical and laboratory characteristics at initial appearance, clinical and pathologic staging, and treatment for their effects on survival and disease-free survival. An initial erythrocyte sedimentation rate over 50 mm/h was common in patients who ultimately had a relapse. There was no correlation between the size of the mediastinal mass at diagnosis and occurrence of relapse. A residual mediastinal mass was found in 22% of patients after 1 year of treatment regardless of its size at initial appearance. With a median follow-up time of 10.5 years, the overall survival rate is 89%, and disease-free survival rate is 71%. The disease-free survival rates for patients with stages I-IV disease are 92,81, 78, and 40%, respectively. Relapses occurred in 7 of 22 (36%) patients with positive staging laparotomy despite radiotherapy for three with stage IA and IIA disease, chemotherapy alone for two with stage IIIB disease, or chemotherapy for one with stage IIIB and one with stage IVB disease. Of patients who had no evidence of abdominal Hodgkins disease at a staging laparotomy, 6 of 34 (19%) had a relapse. These included one with stage IA and five stage IIA disease, all treated with radiotherapy alone. Treatment of stage III and IV disease with regimens including CCNU (lomustine) or cyclophosphamide, plus vinblastine sulfate or vincristine sulfate, prednisone, and procarbazine hydrochloride with or without radiation therapy yielded poor results, with 6/7 having a relapse.