Combination chemotherapy using vinblastine and methotrexate for the treatment of progressive desmoid tumor in children.

PURPOSE: We report the treatment of 10 children for progressive desmoid tumor not amenable to standard surgical or radiation therapy with the use of vinblastine (VBL) and methotrexate (MTX). PATIENTS AND METHODS: Ten patients aged 6.4 to 18 years with primary (two patients) or recurrent (eight patients) desmoid tumor were treated with VBL and MTX for 2 to 35 months. Patients with recurrent tumors had been previously treated with surgical resection with (two patients) or without (five patients) radiation therapy or with radiation therapy alone (one patient). No patient had previously received cytotoxic chemotherapy. The tumor response was assessed at routine intervals by physical examination and magnetic resonance imaging (MRI). RESULTS: Five patients had clinical evidence of response to therapy with complete resolution (three patients) or partial resolution (two patients) of physical examination and radiographic abnormalities. Three patients had stable disease during 10 to 35 months of treatment. Two of these patients had progressive disease 9 and 37 months after treatment stopped; one patient had no progression 16 months after therapy. Two additional patients with stable disease had chemotherapy discontinued after 2 and 3 months. Common side effects included mild alopecia and myelosuppression and moderate nausea and vomiting. In patients with responding tumors, MRI showed decreased tumor size and, in two patients, changes consistent with fibrosis and decreased cellularity of the tumor. CONCLUSION: Combination chemotherapy with VBL and MTX appears to control desmoid tumor without significant acute or long-term morbidity in most children. This may allow for further growth and development in these patients, which may decrease the morbidity of subsequent definitive therapy.

Direct translation of a protracted irinotecan schedule from a xenograft model to a phase I trial in children.

PURPOSE: In a preclinical model of neuroblastoma, administration of irinotecan daily 5 days per week for 2 consecutive weeks ([qd x 5] x 2) resulted in greater antitumor activity than did a single 5-day course with the same total dose. We evaluated this protracted schedule in children. PATIENTS AND METHODS: Twenty-three children with refractory solid tumors were enrolled onto a phase I study. Cohorts received irinotecan by 1-hour intravenous infusion at 20, 24, or 29 mg/m(2) (qd x 5) x 2 every 21 days. RESULTS: The 23 children (median age, 14.1 years; median prior regimens, two) received 84 courses. Predominant diagnoses were neuroblastoma (n = 5), osteosarcoma (n = 5), and rhabdomyosarcoma (n = 4). The dose-limiting toxicity was grade 3/4 diarrhea and/or abdominal cramps in six of 12 patients treated at 24 mg/m(2), despite aggressive use of loperamide. The maximum-tolerated dose (MTD) on this schedule was 20 mg/m(2)/d. Five patients had partial responses and 16 had disease stabilization. On day 1, the median systemic exposure to SN-38 (the active metabolite of irinotecan) at the MTD was 106 ng-h/mL (range, 41 to 421 ng-h/mL). CONCLUSION: This protracted schedule is well tolerated in children. The absence of significant myelosuppression and encouraging clinical responses suggest compellingly that irinotecan be further evaluated in children using the (qd x 5) x 2 schedule, beginning at a dose of 20 mg/m(2). These results imply that data obtained from xenograft models can be effectively integrated into the design of clinical trials.

The "In-Training Examination" of the American Board of Pediatrics: does it test for "new" or "old" information?

The American Board of Pediatrics In-Training Examination or "Pre-Test" is not in widespread use as a means of evaluating the cognitive knowledge and problem-solving skills of hourse officers. The examination is modeled after the certifying examination of the American Board of Pediatrics. The 1977 Pre-Test was analyzed in an attempt to determine whether awareness of recent medical knowledge was required to pass this examination. The 7th through 10th editions of the Nelson Textbook of Pediatrics were used as a reference source. These editions represent information that is from 5 to at least 20 years of age. Analysis of the 190 items revealed that the correct answer was known for 148 or 78% of the questions at least 20 years ago. These results suggest that the Pre-Test, and presumably the American Board of Pediatrics certifying exam, measures the candidates' knowledge of long-standing core information.

Diagnosis and management of agenesis of the right lung and left pulmonary artery sling.

The diagnostic, developmental, and surgical aspects of left pulmonary artery sling associated with right lung agenesis in 3 patients are described. Cardiac catheterization may be avoided by the combined use of echocardiography and magnetic resonance imaging and/or computed tomography.

Estrogen deficiency in adolescents and young adults: impact on bone mineral content and effects of estrogen replacement therapy.

Because the long-term effects of estrogen replacement in adolescents with ovarian failure and hypothalamic amenorrhea have not been previously studied, we conducted a 2-year study of 35 patients to determine factors contributing to baseline bone density measures (bone density, bone mineral content, and bone width) and the response to estrogen therapy. Estrogen-deficient patients were often profoundly osteopenic by single-photon absorptiometry of the radius and dual-photon absorptiometry of the spine, despite estrogen replacement. Variables that were significant predictors of better initial single-photon absorptiometry measurements included increased age, increased body mass index, spontaneous pubertal development, lack of radiation therapy, and lower serum osteocalcin. Patients treated with estrogen/progestin had stable cortical bone mineral content and bone density at the distal one-third of the radius, a slight improvement in bone density at the distal one-tenth of the radius, and on encouraging, but marginal, improvement in the z score (standard deviation from the mean) of bone mineral content at the distal one-tenth. The z scores for cortical bone width and bone density decreased, suggesting a possible relative worsening over time. In untreated estrogen-deficient girls, bone mineral content and bone density decreased (but not significantly); the z score of cortical bone width showed a significant decrease. Using dual-photon absorptiometry, a history of radiation therapy was found to be a predictor of lower bone density compared with age-matched controls. Estrogen progestin therapy did not result in changes in serum levels of lipids and antithrombin III, weight, or blood pressure.(ABSTRACT TRUNCATED AT 250 WORDS)

Pitfalls in pediatric urinary sonography.

Our review of pediatric urinary tract ultrasonograms over a period of two and one-half years resulted in a catalog of pitfalls. Cases included normal scans mistaken for abnormal and vice versa. These erroneous diagnoses stemmed from the inappropriate selection of the primary imaging test, improper timing of the ultrasonogram, errors of commission or omission in performance of the scans, and improper interpretation of the findings. For ease of reference, the pitfalls are grouped under bladder, ureters, and kidney with emphasis on the first two which are common sources of error.

Interventional radiology in the acute pediatric abdomen.

This article addresses the most common types of interventional procedures performed in the pediatric abdomen. Nonvascular interventions are stressed because they are more common than vascular interventions.

Malignant and benign bone tumors.

The authors have intentionally included some confusing examples to demonstrate that the imaging findings of benign and malignant pediatric bone lesions are not always pathognomonic. The radiologist's role is to recognize malignant bone lesions and encourage the prompt referral of these patients to an appropriate institution for biopsy and treatment. The radiologist must also be able to identify benign lesions and help determine whether these lesions require biopsy and intervention or simply observation.

Improved MR imaging for patients with metallic implants.

Pediatric oncology patients with large metallic prostheses were imaged with one of two MR imaging techniques: 1) the "tilted view-angle" technique, 2) or a higher readout bandwidth technique. The tilted view-angle method uses an additional gradient in the slice selection direction during readout. The high bandwidth technique increases the readout bandwidth and shortens the echo time (TE). High bandwidth and short echo times were implemented in both T(1)-weighted (T(1)W) turbo spin echo and turbo short tau inversion recovery (STIR) sequences. Both imaging techniques reduced the size of metal-induced image artifacts. The tilted view-angle method reduced the artifact to a greater degree but had inherent shortcomings. The reformatted images were blurred and shifted. The area of interest was often moved outside of the field of view, unless parameters were adjusted on the basis of a pre-scan calculation. The high readout bandwidth, short echo technique required no special preparation and reduced metal artifacts without image blurring. The combination of high-bandwidth, shorter echo turbo STIR and T(1)W turbo spin echo sequences with subtraction of pre- from post-contrast images allowed effective fat suppression without local field inhomogeneity affects. This greatly improved our ability to evaluate suspected disease near metallic implants in pediatric cancer patients.

1D spectroscopic imaging with rf echo planar (SIRFEN) methods.

A recently developed rf echo planar imaging method has been modified to rapidly generate spectroscopic information along one in-plane axis and spatial information along the other. The method allows the production of one-dimensional chemical shift images (1D CSIs) in acquisition times of 18 sec or less. A specific phase-encode-reordering algorithm provides convenient manipulation of T2 weighting, yielding partial suppression of short T2 species like muscle water. The method is demonstrated in phantoms and in vivo with 1D CSIs of human brain and limbs. Abnormal fat distribution is demonstrated in the calf of a patient with aggressive fibromatosis. The advantages of short acquisition times obtainable with SIRFEN are offset by limited spectral resolution, suggesting that primary applications will be confined to rapid spatial mapping of major spectral components.

Therapeutic catheter procedures in pediatrics.

There is a large spectrum of interventional catheter procedures being performed presently in children. The procedures that offer a greater advantage than surgical techniques and that will continue to be established procedures include percutaneous angioplasty of the pulmonary valve, peripheral pulmonary artery and caval stenosis, embolizations of pulmonary collaterals and pulmonary arteriovenous malformations, angioplasty of vascular access shunt, embolization of bronchial arteries for hemoptysis, and percutaneous nephrostomy and abscess drainage procedures. Others that have been successful but not yet routinely established in children include thrombolysis, renal artery angioplasty, embolization of peripheral arteriovenous malformations, dilatation of the urinary and gastrointestinal tract stenosis, and percutaneous biopsy.

Localization of Tc-99m MDP in neuroblastoma metastases to the liver and lung.

Localization of skeletal tracer in a neuroblastoma primary is common but localization in extraskeletal metastatic sites has not received recognition. Tc-99m MDP concentration in hepatic or pulmonary metastases was noted in three of ten patients with such metastases. Lesion size appears to be important for demonstrating these metastases with Tc-99m MDP. This was particularly true for hepatic metastases, which were identified only when they were 5 cm or greater in diameter.

Liver biopsy methods for pediatric oncology patients.

BACKGROUND: Liver biopsy is a high-risk procedure in oncology patients, and optimal methods for children have not been established. OBJECTIVE: To assess the effectiveness and safety of two methods of performing liver biopsy in pediatric oncology patients. MATERIALS AND METHODS: Between May 1997 and July 1999, 51 liver biopsies (22 percutaneous and 29 transjugular) were performed. The 22 percutaneous biopsies (13 focal hepatic lesions and 9 general liver biopsies) were performed under sonographic guidance; 21 used a spring-loaded needle (usually 18 G). In 21 patients, a coaxial sheath was used to inject a slurry of microfibrillar collagen into the needle track. The 29 transjugular general liver biopsies were performed with a 19-G spring-loaded needle, under sonographic and fluoroscopic guidance. The transjugular technique was used for children with thrombocytopenia, coagulopathy, ascites, or recent bone-marrow transplantation. RESULTS: All biopsies yielded sufficient tissue for diagnostic studies. Bleeding occurred after 3 of 21 percutaneous biopsies, despite coaxial track embolization. No bleeding or other major complication occurred after transjugular biopsy. CONCLUSION: Coaxial percutaneous biopsy with track embolization was effective, but was not complication-free; it should be reserved for focal lesions or for patients at low risk of bleeding. Transjugular liver biopsy is safe and effective for use in high-risk pediatric oncology patients.

Percutaneous drainage of subperiosteal abscess: a potential treatment for osteomyelitis.

OBJECTIVE: Intravenous antibiotics and surgical drainage are the accepted methods of treating osteomyelitis complicated by abscess formation. The objective of this study was to determine whether percutaneous drainage of subperiosteal abscess is a potential treatment for osteomyelitis. MATERIALS AND METHODS: Three pediatric patients with subperiosteal abscesses from acute osteomyelitis had percutaneous drainage with sonographic and fluoroscopic guidance using a Seldinger technique and an 8-F catheter. RESULTS: Two patients required no further intervention and had the drainage catheter removed after 72 h. After completing a course of antibiotics they healed completely. One patient, after a week of purulent drainage, required open drainage including a bone debridement of an area of septic necrosis. CONCLUSION: Percutaneous drainage of subperiosteal abscess may be an alternative to surgical drainage when medical therapy alone is inadequate. Development of intraosseous abscess, necrosis or persistent drainage suggests further intervention may be necessary.

Intermittent hydronephrosis: a unique feature of ureteropelvic junction obstruction caused by a crossing renal vessel.

Obstruction at the ureteropelvic junction (UPJ) is usually intrinsic. Recently, however, 13 (11%) of approximately 120 children with UPJ obstruction were found to have extrinsic obstruction caused by a lower-pole renal vessel. In each case, the obstruction was confirmed at the time of surgery. Most of these patients had intermittent pain. Excretory urograms did not show obstruction between episodes of pain, but when a urogram was obtained during an episode, marked obstruction was noted. Contrast agent trapped in a segment of proximal ureter suggests the presence of an extrinsic obstruction. Properly timed renal imaging, therefore, can identify the urinary tract as the source of the symptoms.

Cartilaginous epiphysis and growth plate: normal and abnormal MR imaging findings.

The cartilaginous structures at the ends of growing bones constitute the "growth mechanism." These structures are not visible on radiographs, but they can be seen with MR imaging. Improved definition of cartilaginous abnormalities by MR imaging may permit earlier detection and treatment of these disorders and thus prevent bone deformity. This pictorial essay contains examples of normal and abnormal growth cartilage as seen with MR imaging. The indications for MR imaging in the evaluation of certain growth disorders are discussed, and the usefulness of MR imaging in certain clinical situations in which the role of this technique is still evolving is illustrated.

The cross-table lateral view in neonatal pneumothorax.

The cross-table lateral view of the chest can facilitate a more realistic appreciation of a pneumothorax than the supine frontal projection alone or in combination with the vertical-beam lateral view. This view also permits an optimal evaluation of the relative position of the lung, intrapleural air, and the tip of the chest tube in the supine newborn. The presence and size of a pneumothorax can usually be determined by locating the anterior lung edge on the cross-table lateral view. The anteroinferior corner of lung often aids in locating this anterior lung edge. The other structures that simulate the anterior lung edge on the cross-table lateral view include sternal retraction, rib ends, the axillary soft-tissue density, and posterior atelectasis. Because of the cause-effect relation of pneumothorax to intracranial bleeding in the premature infant, more widespread use of this technique to detect pneumothorax is recommended.

Malignant peritoneal mesothelioma in two pediatric patients: MR imaging findings.

Malignant mesothelioma is a rare tumor in childhood. We present two cases of malignant peritoneal mesothelioma in which contrast-enhanced, fat-saturated magnetic resonance (MR) imaging was used advantageously to detect peritoneal tumor involvement.