1,720,974 research outputs found
Bronchiolitis obliterans organizing pneumonia (BOOP) in a child with mild-to-moderate asthma: evidence of mast cell and eosinophil recruitment in lung specimens
No full textAbstract
Bronchiolitis obliterans with organizing pneumonia (BOOP) is rarely described in children and little is known about its pathogenesis. This paper reports on an 11-year-old patient suffering from mild-to-moderate asthma. He presented with a retrocardiac density at chest computed tomography scan that was slow to resolve and failed to respond to antibiotic therapy. Open lung biopsy revealed a histological picture with buds of granulation tissue in respiratory bronchioles and alveolar ducts, with organized extensions into the alveoli. The use of monoclonal antibodies on biopsy specimens demonstrated the presence of an inflammatory process affecting not only the thickened alveolar walls, but also the remaining lung parenchyma, the pulmonary arteries, and the bronchioles. The inflammatory infiltrate consisted mainly of mast cells and eosinophils. The clinical condition improved with steroid therapy. To the best of the authors' knowledge, this is the first report of BOOP in an asthmatic child with recruitment of mast cells and eosinophils documented by using monoclonal antibodies
Intraoperative examination (IOE) in pediatric extracranial tumors
No full textBACKGROUND AND OBJECTIVES: Intraoperative evaluation of surgical specimens by frozen sections (IOE) is required to distinguish benign and malignant lesions, assess surgical margins, and determine sample adequacy of biopsies. In the last years, it has been used also for therapeutic decisions, particularly in children, who may need other ancillary procedures, in case of malignancies. Our purpose was the evaluation of diagnostic accuracy, limits, and different role of IOE in pediatric pathology.
PATIENTS AND METHODS: From 1990 to 2001, 416 IOEs were performed in 341 children, affected by lymph node pathology, soft tissue tumors, neuroblastic tumors, gonadal germ cell, and stromal tumors, hepatic lesions, renal tumors, and others; the technique was also used to assess surgical margins during major surgeries. IOEs were obtained from Tru-cut biopsies (<1 cm(3)), wide biopsies (>1 cm(3)), or from the whole lesions, and the subsequent final diagnoses were classified as conordant, discordant, and deferred.
RESULTS: Three hundred seventy cases (88.9%) were concordant, 10 (2.4%) discordant, and 36 (8.6%) deferred. The disagreement was found in two small lymph node samples, three soft tissue tumors, one hepatoblastoma, one metastasis, and three surgical margins. The deferred diagnoses were related to lymph node and soft tissue lesions.
CONCLUSIONS: IOE in pediatric oncology may integrate the diagnostic process and supports the therapeutic guidelines of different tumors. In our study, the diagnostic concordance was satisfactory. A rational use of the technique and the awareness of its limits are, however, prerequisites to avoid the risk of overtreatment
Renal angiomyolipoma in children: Diagnostic difficulty in 3 patients
No full textPURPOSE: Because angiomyolipoma is less common in children than in adults, its diagnosis can be difficult. We present 3 cases of pediatric angiomyolipoma in which diagnostic problems resulted due to the presenting characteristics.
MATERIALS AND METHODS: We report on 3 children with unilateral renal angiomyolipoma. Computerized tomography (CT) and ultrasonography revealed 3 large renal masses, 20, 7 and 8 cm. in diameter, respectively. A correct diagnosis was not made preoperatively in any case by CT, ultrasound or fine needle biopsy. Wilms tumor was suspected in the first patient who received preoperative chemotherapy. Imaging was inconclusive in the other 2 cases.
RESULTS: All patients underwent surgical exploration and subsequent nephrectomy due to the large size of the tumor. At followup 33, 23 and 13 months postoperatively all children were well without signs of recurrence.
CONCLUSIONS: It has been reported that the demonstration of fat on renal ultrasound and CT can diagnose angiomyolipoma in 95% of the cases. Most radiologists rely solely on CT demonstration of lipid density in the renal mass to diagnose angiomyolipoma but the identification at imaging of lipid tissue may be difficult in small tumors. In our cases the fat content of the tumors was less than 10% despite the large size. This low fat content results in misdiagnosis, since fatty tissue is also present in other renal tumors, such as lipoma, liposarcoma, teratoma and Wilms tumors. We recommend conservative surgery when tumor size permits in pediatric patients with angiomyolipoma to avoid chemotherapy
Expression of survivin, livin and caspase 3 in pediatric malignant perpheral nerve sheath tumors (MPNST)
No full textCytogenetic Analysis of Pediatric Anaplastic Large Cell Lymphoma
No full textBackground
Anaplastic large cell lymphoma (ALCL) constitutes approximately 15% of pediatric and 3% of adult non-Hodgkin lymphomas. Most pediatric cases harbor the reciprocal translocation t(2;5)(p23;q35), involving the alk gene. Cytogenetic studies of ALCL have mostly been published as case-reports. The aim of this study was to determine the cytogenetic profiles of a series of pediatric ALCL and to compare them with pediatric and adult ALCL from the literature.
Methods
Eighteen children treated at our Institution were studied by standard cytogenetic analysis and RT-PCR for the specific t(2;5) translocation product. Comparative analysis was performed on our findings and on the karyotypes of 48 pediatric and 39 adult ALCL reported in the literature.
Results
Karyotype was obtained in 16/18 ALCL: 9 showed translocation t(2;5) and 1 an alk variant form. Structural and numeric chromosomal abnormalities were identified in both pediatric and adult series. Trisomies were found preferentially in pediatric patients (P = 0.013) and monosomies in adults (P = 0.038). Trisomy 7 was found in 22% (13/59) of pediatric cases with abnormal karyotype and only in 5% (2/38) of adults; monosomy of chromosome 13 in 13% (5/38) of adults and only in 2% (1/59) of pediatric patients and monosomy of chromosome 15 in 16% (6/38) of adults and in none of the pediatric ALCL.
Conclusion
Our data suggest that pediatric and adult ALCL are characterized by different numerical chromosomal abnormalities. Larger prospective studies may elucidate their potential prognostic impact
Intraoperative examination (IOE) in pediatric extracranial tumors
No full textBACKGROUND AND OBJECTIVES: Intraoperative evaluation of surgical specimens by frozen sections (IOE) is required to distinguish benign and malignant lesions, assess surgical margins, and determine sample adequacy of biopsies. In the last years, it has been used also for therapeutic decisions, particularly in children, who may need other ancillary procedures, in case of malignancies. Our purpose was the evaluation of diagnostic accuracy, limits, and different role of IOE in pediatric pathology.
PATIENTS AND METHODS: From 1990 to 2001, 416 IOEs were performed in 341 children, affected by lymph node pathology, soft tissue tumors, neuroblastic tumors, gonadal germ cell, and stromal tumors, hepatic lesions, renal tumors, and others; the technique was also used to assess surgical margins during major surgeries. IOEs were obtained from Tru-cut biopsies (1 cm(3)), or from the whole lesions, and the subsequent final diagnoses were classified as conordant, discordant, and deferred.
RESULTS: Three hundred seventy cases (88.9%) were concordant, 10 (2.4%) discordant, and 36 (8.6%) deferred. The disagreement was found in two small lymph node samples, three soft tissue tumors, one hepatoblastoma, one metastasis, and three surgical margins. The deferred diagnoses were related to lymph node and soft tissue lesions.
CONCLUSIONS: IOE in pediatric oncology may integrate the diagnostic process and supports the therapeutic guidelines of different tumors. In our study, the diagnostic concordance was satisfactory. A rational use of the technique and the awareness of its limits are, however, prerequisites to avoid the risk of overtreatment
Going Beyond Counting First Authors in Author Co-citation Analysis
Full text linkThe present study examines one of the fundamental aspects of author co-citation analysis (ACA) - the way co-citation
counts are defined. Co-citation counting provides the data on which all subsequent statistical analyses and mappings
are based, and we compare ACA results based on two different types of co-citation counting - the traditional type that
only counts the first one among a cited work's authors on the one hand and a non-traditional type that takes into
account the first 5 authors of a cited work on the other hand. Results indicate that the picture produced through this non-traditional author co-citation counting contains more coherent author groups and is therefore considerably clearer. However, this picture represents fewer specialties in the research field being studied than that produced through the traditional first-author co-citation counting when the same number of top-ranked authors is selected and analyzed. Reasons for these effects are discussed
Inflammatory Myofibroblastic Tumors in Childhood. A Report From the Italian Cooperative Group Studies
No full textAbstract
BACKGROUND: Inflammatory myofibroblastic tumors (IMTs) are myofibroblastic lesions with unpredictable biologic behavior that occur at a young age. For this report, the authors investigated clinicopathologic features in a series of pediatric IMTs. The objective of the study was to identify morphologic or immunohistochemical prognostic markers and the possible pathogenic role of human herpes virus 8 (HHV-8).
METHODS: Twenty-six patients were observed over a period of 18 years. Clinical/histologic data were reviewed, and immunohistochemical/molecular studies were performed.
RESULTS: Patients ages 8-216 months (median age, 60 months) presented with tumors of the lung-bronchus (8 patients), abdomen (17 patients), and thoracic wall (1 patient). Twenty-one patients underwent complete excision, and microscopic or macroscopic residual disease was present in 5 of those patients. Chemotherapy was received by 5 patients. After a median follow-up of 6.6 years, 24 patients were in complete remission, and 2 patients had died of disease. Local recurrences were observed in 6 patients (including 4 recurrences that occurred after a complete excision). Cytologic atypia, low inflammatory infiltrate, and a rich myxoid pattern were detected in patients who had recurrent disease or a poor prognosis. Anaplastic lymphoma kinase (ALK) was positive in 7 patients (including 2 patients with recurrent disease). No correlation between clusterin expression and prognosis was demonstrated. HHV-8 was identified in 1 pulmonary IMT.
CONCLUSIONS: IMTs are locally aggressive lesions. In this series, the local recurrence rate was 23%, and the 5-year and 10-year event-free survival rates were 87.4% and 72.8%, respectively. The results indicated that the treatment of choice is a complete, nonmutilating excision; chemotherapy may be given to patients who have microscopic or macroscopic residual disease, although the results are controversial; cytologic atypia and positive ALK status are more frequent in aggressive tumors, whereas metastatic tumors are negative for ALK; and HHV8 is not involved in the pathogenesis of IMT
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