High-dose induction chemoradiotherapy followed by autologous bone marrow transplantation as consolidation therapy in rhabdomyosarcoma, extraosseous Ewing's sarcoma, and undifferentiated sarcoma.

PURPOSE: To improve response and survival rates in patients with high-risk rhabdomyosarcoma (RMS), extraosseous Ewing's sarcoma, and undifferentiated sarcoma, we used a short course of induction with multi-agent chemotherapy, hyperfractionated radiotherapy, and surgery when possible. Consolidation was with intensive chemotherapy and autologous bone marrow transplantation (ABMT). PATIENTS AND METHODS: Twenty-six patients (21 with RMS, three with undifferentiated sarcoma, and two with extraosseous Ewing's sarcoma) were entered onto the protocol between June 1990 and March 1994. Induction consisted of ifosfamide, etoposide, doxorubicin, dactinomycin, cyclophosphomide, and vincristine, and a split course of hyperfractionated radiotherapy. Patients who attained a complete response (CR) or good partial response (GPR) received consolidation with high-dose melphalan and etoposide followed by ABMT. RESULTS: Of 26 previously untreated patients 19 (73%) achieved a CR (n=13) or GPR (n=6) at the completion of induction and underwent ABMT. Two-year overall survival (OS) was 56% (95% confidence interval [CI], 36% to 76%) and progression-free survival (PFS) was 53% for the whole group (95% CI, 33% to 73%). CONCLUSION: Consolidation of response by myeloablative chemotherapy was well tolerated. Split-course hyperfractionated radiotherapy did not increase the rate of local control. The results of this short-course therapy were comparable to previous therapies of 1 to 2 years' duration. Induction and consolidation chemotherapy, as well as radiation dose, could be further intensified, since no death due to toxicity occurred among these patients.

Adrenal neoplasms in children.

Neuroblastoma is the most common extracranial neoplasm of childhood. Although it most commonly occurs in the adrenal gland, it may be found anywhere along the sympathetic chain. The characteristic clinical and imaging features of neuroblastoma are discussed in this article. Less common neoplasms of the adrenal gland, adenoma, adrenocortical carcinoma, and pheochromocytoma are also discussed.

Pediatric HIV infection in its second decade--the changing pattern of lung involvement. Clinical, plain film, and computed tomographic findings.

The changing pattern of pediatric HIV infection is illustrated in this article with plain films and computed tomographic scans. Today, vertical transmission from infected mothers results in HIV infection of exposed infants in about one third of pregnancies. Although the high mortality with catastrophic illnesses such as Pneumocystis carinii pneumonia seen in early life appears to be decreasing, chronic illness with pulmonary involvement due to diffuse lymphocytic infiltrative syndromes continues. Lymphocytic interstitial pneumonitis (LIP) has evolved in some patients to cystic lung disease and bronchiectasis. There are increasing numbers of patients infected with Mycobacterium. Masses seemingly of smooth muscle origin, thought to be leiomyosarcoma are appearing; they may be pseudotumors related to concomitant Mycobacterium avium intracellulare infection.

Neuroimaging of brain injury in neonates treated with extracorporeal membrane oxygenation: lessons learned from serial examinations.

The head ultrasound (US) is used extensively at most extracorporeal membrane oxygenation (ECMO) centers to screen for intracranial pathology in the ECMO candidate. Daily head US examinations are obtained in patients on ECMO to detect the emergence of intracranial hemorrhage (ICH). The authors asked whether these serial studies could be correlated with more definitive diagnostic studies, such as computed tomography (CT) and magnetic resonance imaging (MRI) scans, autopsy data, or the long-term neurodevelopmental status, to discern the predictive value of these daily examinations. Seventy-four consecutively treated neonates with reversible respiratory failure refractory to conventional support met institutional criteria for placement on ECMO. In addition to a pre-ECMO US, daily real-time portable head US images were evaluated for changes in echotexture, ventricular configuration, and extraaxial fluid. Follow-up CT and MRI scans were evaluated for the presence of hemorrhagic or ischemic lesions. Autopsy data were obtained from nonsurvivors. Survivors were examined by a neurodevelopmental specialist at regular intervals and classified as normal or delayed for chronological age. In this series of 74 patients, CT/MRI scanning and autopsy data demonstrated structural injury in 19 patients; there were 16 ischemic infarctions and three hemorrhages. The incidence of hemorrhage in this series was considerably lower than that previously reported. Ten of the 19 patients had serial head US findings demonstrating a progression from focal increases in echotexture to diffuse effacement of cerebral architecture. In the remaining nine, serial head US examinations did not show injury. An additional 10 children had a clear delay in neurological development despite no evidence of anatomic injury on serial head US examinations or CT/MRI scanning.(ABSTRACT TRUNCATED AT 250 WORDS)

Testicular-sparing surgery for benign testicular tumors.

BACKGROUND/PURPOSE: Although there has been a precedent of testicular-sparing surgery in some centers, the authors find it is still not general practice among pediatric surgeons. To address this and emphasize the role of testicular-sparing surgery in children, four patients with testicular masses are presented who underwent this procedure. METHODS: Four patients who underwent testicular-sparing surgery between the years 1993 and 1998 were reviewed. Demographic data, histopathology, and follow-up data were obtained from office charts. The period of follow-up ranged from 1 to 5 years. RESULTS: Four patients whose ages at diagnosis were 1, 2, 4, and 17 years presented with unilateral testicular masses. The alpha-fetoprotein and beta-human chorionic gonadotropin levels were within normal limits. Testicular ultrasonography was carried out on all patients, and groin exploration with spermatic cord isolation was performed in each case. After enucleation, frozen sections to confirm benignity was carried out before repair of the testis. Follow-up of 6 months to 5 years has shown no recurrence, and on examination, testicular volume is normal in all cases. CONCLUSIONS: Testicular-sparing surgery preserves testicular volume, which is important for both cosmetic and functional purposes. It is a viable and useful method in the management of benign testicular tumors in children.

Real time ultrasonographic evaluation of the infant hip.

Although the technique for ultrasonographic evaluation of the infant hip is difficult to learn, it is a valuable diagnostic tool. In many institutions, hip ultrasonography expands upon and complements the clinical examination and minimizes radiographs.

Posterior mediastinal cystic teratoma surrounded by fat in a 13 month old boy. Value of magnetic resonance imaging.

A 13 month old boy had a large left sided posterior cystic mass initially thought to be in lung. MRI showed the cystic mass to be surrounded by fat. A benign mediastinal teratoma was removed.

The infant with possible biliary atresia: evaluation by ultrasound and nuclear medicine.

Twenty-eight infants with jaundice were evaluated with ultrasound and radionuclide scans. Comparison of these studies with pathologic, surgical and clinical examinations demonstrated good correlation between ultrasonic and radionuclide studies. Ultrasound is an important preliminary study in the workup of such patients. Combined imaging provides the most information in a patient with suspected biliary atresia.

Benign vascular tumors of the liver in infants: sonographic appearance.

The distinctive sonographic appearance of benign vascular tumors of the liver (one hemangioendothelioma and two mesenchymal hamartomas) in three infants is presented. Each child had hepatomegaly and congestive heart failure, and all had confirmatory arteriography. The sonographic pattern of a complex mass, dilated proximal aorta, and enlarged nutritive and draining vascular structures should alert the radiologist and clinician to this diagnosis.

Childhood typhlitis: its increasing association with acute myelogenous leukemia. Report of five cases.

Five children with acute myelogenous leukemia (AML) are described in whom typhlitis developed early in the course of the disease. The plain and barium-enema radiographic findings of mass effect and bowel edema ("thumbprinting") were similar among the cases, although nonspecific. The increasing association of typhlitis with AML and its earlier appearance in the course of the disease should be recognized.

Transient focal liver scan defects in children receiving chemotherapy (pseudometastases). Work in progress.

Three pediatric patients with tumors (two rhabdomyosarcoma, one Wilms tumor) had significant focal defects on Tc-99m sulfur colloid scans while receiving chemotherapy (all three had received chemotherapy, including actinomycin D, within ten days prior to scanning). In all three, the defects resolved spontaneously; one biopsy showed fibrosis of a mild degree. The finding of defects on liver scans of pediatric patients receiving chemotherapy must not be automatically assumed to be metastatic disease; the changes may relate to hepatic response to recently administered chemotherapy.

The diagnosis of abdominal neuroblastoma: relative roles of ultrasonography, CT, and MRI.

Abdominal neuroblastoma is reviewed in terms of its diagnosis, including in utero, at birth, and through infancy into childhood. Age at diagnosis remains the best predictor of survival, with infants under 1 year of age having almost 100% cure. Ultrasonography and magnetic resonance imaging (MRI) are the recommended diagnostic modalities to stage the abdominal primary, although computed tomography (CT) (including myelography) is still widely and efficiently used. Examples are given of each stage.

Splenic lymphangiomatosis in children.

PURPOSE: To study the clinical and imaging features of splenic lymphangiomatosis. MATERIALS AND METHODS: The clinical and abdominal imaging data of 10 children with splenic lymphangiomatosis were retrospectively reviewed. The modalities used in the study included computed tomography (CT) (10 patients), sonography (five patients), and magnetic resonance (MR) imaging (two patients). Pathologic confirmation of lymphangiomatosis was obtained in nine patients. RESULTS: Splenic lymphangiomatosis was discovered incidentally in all cases and was a key finding in enabling the correct diagnosis in six children with extrasplenic disease as well. Only two children had clinical splenomegaly. Sonograms and MR images showed multiple, wEll-defined cysts. Multiple, low-attenuation lesions that did not enhance with intravenous administration of contrast material (n = 8) or a mottled spleen (n = 2) were seen at CT. One of the mottled spleens had target lesions on an early (arterial) image obtained after administration of a bolus of contrast material. One child underwent a splenectomy; one child underwent therapeutic embolization. Eight patients remained asymptomatic with respect to the spleen 1-20 years later. CONCLUSION: Splenic lymphangiomatosis is often an incidental imaging finding that frequently has a characteristic imaging appearance. The recognition of this appearance helps in diagnosis of this disease and may prevent the need for further invasive procedures. Splenic changes can be isolated or can coexist with bone or soft-tissue lymphangiomas.

MR imaging of diffuse bone marrow replacement in pediatric patients with cancer.

In the magnetic resonance (MR) imaging examinations of three children with tumors (two neuroblastoma, one rhabdomyosarcoma) and three with leukemia, the marrow demonstrated a diffuse, uniform pattern of hypointensity on T1-weighted images and hyperintensity on T2-weighted images. The authors observed that this reversal ("flip-flop") of the usual MR characteristics of fatty marrow was seen in the epiphyses, metaphyses, and diaphyses. The purpose of this study was to establish the radiographic and clinicopathologic correlates of this MR finding on the basis of findings from plain radiographs, bone scans, and bone marrow aspirates. Plain radiographs and bone scans demonstrated either normal findings or changes limited to the metaphyses. In all patients, analysis of bone marrow aspirates demonstrated metastases. The authors concluded that even in the absence of evidence of discrete bone metastases on a plain radiograph or a bone scan, this diffuse and uniform "flip-flop" pattern reflects diffuse marrow replacement by tumor cells.

Stage IVN neuroblastoma: MRI diagnosis of left supraclavicular "Virchow's" nodal spread.

Stage IV neuroblastoma is associated with high mortality; an exception are patients whose stage IV status includes distant positive nodes, but no skeletal metastases-stage IVN neuroblastoma. We describe our experience with preoperative MRI in three patients with extensive abdominal neuroblastoma without cortical bony involvement but with unsuspected metastatic involvement to the left supraclavicular (Virchow's) node. We review findings of left supraclavicular nodal spread in five earlier cases of IVN neuroblastoma.

Cervical neuroblastoma in eleven infants--a tumor with favorable prognosis. Clinical and radiologic (US, CT, MRI) findings.

Cervical neuroblastoma, a disease primarily of infants, has a favorable prognosis. Eleven patients are reported. Clinical presentations (other than mass) included stridor and swallowing problems. Masses when felt were commonly mistaken for infectious adenitis. Imaging studies (US, CT, MRI) showed solid masses with vascular displacement and narrowing; intraspinal extension was absent though extension into the adjacent sites of mediastinum and skull occurred. Horner syndrome was seen in five patients with accompanying heterochromia iridis in one. Five tumors had calcification. A high index of suspicion will lead to biopsy and less delay in diagnosis once a mass is felt or imaged.

Etiologic factors in rickets of very low-birth-weight infants.

The incidence of rickets was found to be 32% (39/125) in a retrospective review of consecutive survivors of very low birth weight in whom serial radiographic and biochemical data were obtained. A higher proportion of these infants were black, had a greater initial weight loss, and had a longer hospitalization; there was a prevalence of births in the spring. Soy formula, supplemented with calcium and vitamin D but not phosphorus, was used predominantly in both groups; cumulative calcium, phosphorus, vitamin D, and caloric intakes were the same. We believe that the etiology of rickets in VLBW infants is multifactorial; however, nutritional deficiency is of central importance. Soy isolate formula, as well as human milk and many other commercially available formulas, do not provide sufficient calcium and phosphorus to keep pace with rates of intrauterine accretion. Supplementation with calcium, phosphorus, and vitamin D, beginning as soon as possible after birth, is indicated.

Biliary atresia and noncardiac polysplenic syndrome: US and surgical considerations.

Ten percent of children with biliary atresia have an associated complex of anomalies, including polysplenia, azygous continuation of the inferior vena cava, preduodenal portal vein, hepatic arterial anomalies, and bilaterally bilobed lungs. These abnormalities will not be detected if the preoperative workup is limited to hepatobiliary nuclear scanning. Ultrasonography is important in the preoperative evaluation of patients suspected of having biliary atresia. It is important to identify the associated abnormalities preoperatively because they have an impact on the initial portoenterostomy and may preclude subsequent orthotopic liver transplantation.