Carboplatin and doxorubicin (CARDOX) for nonmetastatic hepatoblastoma: a discouraging pilot study.
Explore the source record for details and available documents.
Adolescent↗
Biomedical subjects
M Guglielmi
Publications and source records attributed to M Guglielmi.
Lung volume reduction surgery in lieu of pneumonectomy in an infant with severe unilateral pulmonary interstitial emphysema.
A male infant with a prenatal diagnosis (at 20 weeks' gestation) of cystic adenomatoid malformation was delivered after 38 weeks' gestation (birth weight, 3 kg) and admitted to the neonatal intensive care unit. During the first few days of life, he developed mild respiratory distress; a chest radiograph and computed tomography scan showed multiple cystic areas in the left lower lobe with hyperinflation and herniation of the upper lobe across the midline. At 3 weeks of age, a left lower lobectomy was performed for presumed cystic malformation. To our surprise the pathology reports revealed pulmonary interstitial emphysema. The postoperative chest radiograph was unchanged, and mechanical ventilation was necessary and required progressively increasing ventilatory settings to provide adequate support. High-frequency oscillatory ventilation and selective right bronchus intubation failed to improve lung function. After 3 weeks, a left thoracotomy was repeated and lung volume reduction was performed with removal of 50' of the peripheral hyperinflated parenchyma. Postoperative recovery was rapid; the child was weaned from the ventilator after 3 days and discharged after 3 weeks. Follow-up chest X-rays showed a normally expanded right lung with mediastinal structures back to midline and a small left lung. Favorable results persisted at 3 years of follow-up. This first and successful experience with lung volume reduction in a neonate suggests that infants who need removal of a large portion of lung parenchyma to achieve adequate ventilation and gas exchange, lung volume reduction surgery should be considered as an alternative to pneumonectomy.
Cystic Adenomatoid Malformation of Lung, Congenita↗
Primary re-excision: the Italian experience in patients with localized soft-tissue sarcomas.
Primary re-excision (PRE) is a wide, non-mutilating procedure carried out in patients with soft-tissue sarcomas (STS) when microscopic residuals are left after initial excision or when there are insufficient data on its completeness. The aim of this study was to evaluate the role of PRE in patients enrolled in two consecutive Italian studies between January 1988 and September 1999. Of 126 patients with grade IIa tumors. 53 underwent PRE (23 rhabdomyosarcomas [RMS] and 30 non-RMS STS [NRSTS]). The primary sites were the extremities in 20, paratesticular 15, trunk 9, head-neck-non-parameningeal (HNnPM) 6, bladder 1, other sites in 2; the tumor (T) status was T1a in 30, T1b in 10, T2a in 9, and T2b in 4; the median interval between primary surgery and PRE was 36 days. Of the 53 patients, 45 had complete histologic excision of the tumor (residuals were found in 21/45 specimens) and subsequently received chemotherapy (CT) alone: 39/45 are in their first complete remission (CR) with a median follow-up of 53 months; 6/45 (3 RMS, 3 NRSTS) relapsed, 4 locally (2 extremities, 2 trunk), and 1 of these died of progressive disease, and 2 with metastatic spread died of their disease. In 8/53 cases (HNnPM 4. extremities 2, bladder 1, trunk 1) PRE did not achieve complete removal of the residuals (3 T1a, 2 Tlb, 2 T2a, 1 T2b); these patients were treated with CT and/or radiotherapy (RT); 1 also underwent further surgery. PRE was able to achieve or confirm complete excision in 45/53 patients, and 39 maintained the first CR without RT. The histologic types and the presence of residuals at PRE did not predict the failures; PRE was effective especially in extremity, trunk, and paratesticular sites, whereas its role was uncertain in large sarcomas over 5 cm in size.
Adolescent↗
Importance of local treatment in pediatric soft tissue sarcomas with microscopic residual after primary surgery: results of the Italian Cooperative Study RMS-88.
BACKGROUND: The goal of primary excision in soft tissue sarcomas is the complete removal of the tumor by a nonmutilating procedure. However, microscopic residuals may be left after a conservative procedure because of inadequate preoperative assessment or difficulties during the operation. The purpose of this report is to describe the treatment and the outcome in patients, enrolled in the Italian Cooperative Study RMS-88, with microscopic residuals after primary excision (IRS Group IIa). PROCEDURE: Microscopic residuals were evident at histology in 52 of 90 patients who had a macroscopic complete primary excision: 25 rhabdomyosarcomas (RMS) and 27 nonrhabdo-soft tissue sarcomas (NRSTS). Eighteen patients were treated with primary reexcision (PRE) and chemotherapy (CT) using VA or IVA regimens; 27 patients received radiation therapy (RT; 40 Gy) and IVA; 7 children in whom PRE was not feasible and RT could not be administered for age <3 years were treated with CT (IVA) alone. RESULTS: Of the 18 patients who underwent a successful PRE + CT, the local relapses were 3 (16.6%); of 27 cases who had RT + CT there were 4 local relapses (14.8%); 3 local relapses occurred in those 7 patients in whom CT alone was administered (43%). CONCLUSIONS: Microscopic residuals after primary surgery were difficult to manage because of the absence of a measurable target. PRE represented the treatment of choice for children <3 years of age who cannot receive RT and for paratesticular sites. PRE and RT showed similar results in achieving local control in extremity and trunk sites, but they could not always avoid local recurrence. In particular PRE was not effective in tumors larger than 5 cm. If microscopic residuals could not be avoided and PRE was not possible, adequate RT was effective both for RMS and for NRSTS.
Adolescent↗
Wilms tumor: does tumorectomy leave neoplastic tissue residual?
Explore the source record for details and available documents.
Child, Preschool↗
Congenital tracheobiliary fistula.
Congenital respiratory tract-biliary fistula, including tracheo- and broncho-biliary fistulae, are rare developmental anomalies. To date, only 18 cases have been reported. We present two additional cases that came to our attention after a long and difficult attempt to make a diagnosis. After surgical excision of the tract both children have remained symptom-free for 6 and 4 years, respectively. Bronchoscopy allows an early diagnosis, but patients also have to be investigated for associated biliary tree malformations.
Biliary Fistula↗
Is surgical treatment of lipoblastoma always necessary?
Lipoblastoma is an uncommon, benign mesenchymal tumor with an excellent prognosis despite its potential to local invasion and rapid growth. However, in the literature, a spontaneous resolution has never been reported, and, consequently, the need for a complete surgical excision has never been questioned. The authors report a case of a 2-day-old boy with congenital diffuse lipoblastoma in the left thigh, which forced us to withhold from surgical treatment to avoid the risk of mutilation in a patient so young. The lesion was followed-up by imaging, and a complete spontaneous resolution of the diffuse lipoblastoma was shown by magnetic resonance imaging (MRI) at 1-year follow-up. In the literature, a complete surgical excision is recommended. The results of this case suggest that a "wait and see" approach is justified at least in infants with huge invasive lesions requiring a mutilating excision.
Hip↗
Sclerosing treatment of lymphangiomas with OK-432.
Over a period of seven years, 15 patients (aged from birth to 15 years; median 22 months) with lymphangioma were treated with OK-432; they received a mean of three injections each. Ten received OK-432 as first line treatment; five were treated after surgery (three had a residual lymphangioma after incomplete removal and two had a late recurrence). OK-432 proved to be effective for primitive as well as for residual and recurrent lymphangioma. Seven cases were macrocystic; complete regression was obtained in all. Five cases were microcystic: two had more than 50% regression, and three less than 50%. Three cases were mixed, with both large and microscopic cysts: one had more than 50% regression, and two less than 50%. These last two cases underwent surgery after the sclerosing treatment. The results obtained were excellent in 100% of macrocystic cases; a shrinkage in size was obtained in all microcystic cases. OK-432 is therefore proposed as a first line option for treatment of lymphangiomas.
Adolescent↗
The chemotactic and mitogenic effects of platelet-derived growth factor-BB on rat aorta smooth muscle cells are inhibited by basic fibroblast growth factor.
In response to endovascular injury, platelet-derived growth factor-BB (PDGF-BB) and basic fibroblast growth factor (bFGF) are released locally and modulate vascular smooth muscle cells (SMC) proliferation and migration within the vascular wall. The aim of the present in vitro study was to determine how rat aorta SMC respond to the simultaneous exposure to PDGF-BB and bFGF. In a modified Boyden chamber assay bFGF exhibited a dose-dependent effect to inhibit the chemotactic action of PDGF-BB. A comparable result was observed in proliferation assays. In contrast, MIP-1 beta, epidermal growth factor (EGF), fibronectin and acidic FGF (aFGF) did not inhibit the chemotactic effect of PDGF-BB. Denatured bFGF did not exert an inhibitory effect and neutralizing antibodies either to bFGF or to bFGF-receptor abolished the inhibition observed in the presence of bFGF. The role played by PDGF receptor alpha (PDGF-Ralpha) was investigated in PDGF-Ralpha-dominant negative-transfected SMC, by selectively blocking PDGF-BB-binding to PDGF-Ralpha with neomycin, by neutralizing PDGF-Ralpha with a monoclonal antibody and by selectively stimulating PDGF-Ralpha with PDGF-AA; in all cases the effect of bFGF to inhibit PDGF-BB-directed SMC migration was abolished. These in vitro studies show that bFGF significantly inhibits PDGF-BB-induced SMC migration and proliferation and that this effect is mediated by both PDGF-Ralpha and bFGF receptor.
Animals↗
Selection, establishment and characterization of cell lines derived from a chemically-induced rat mammary heterogeneous tumor, by flow cytometry, transmission electron microscopy, and immunohistochemistry.
In order to isolate, characterize, and establish culture cell lines with different diagnostic and prognostic significance, derived from multiclonal neoplasms, a ductal infiltrating mammary tumor was induced in rats by 7,12-dimethylbenz[a]anthracene. Clones with different DNA/protein content, being the DI of 1.16, 1.30, and 1.60, respectively, were observed in the primary tumor. Biparametric flow cytometry suggested that the clone at 1.30 is made up of two subpopulations with different protein and slightly different DNA contents. The culture, after a few passages, exhibited the presence of aneuploid cells and the absence of diploid components, demonstrating that only tumor cells survived. The limiting dilution method gave rise to four lines with DI of 1.16, 1.25, 1.30, and 1.50; a mean chromosome number of 45, 46, 47, and 88, respectively; and different morphological and ultrastructural features. These characteristics were stable during the experimental procedure, that is, for about 20 passages. Conversely, the detection of cytoskeletal proteins indicated that the tumor epithelial cells underwent early dedifferentiation into sarcoma-like cells showing markers of stromal cell type and thus exhibiting phenotypic instability in vitro, a feature reported in many advanced human breast cancers in vivo. In conclusion, this cellular model represents the in vivo situation and appears suitable for in vitro studies of tumor cell characteristics and might be used to predict clinical behavior.
Animals↗
Inflammatory pseudotumors of the lung in children: conservative or aggressive approach?
BACKGROUND: Inflammatory pseudotumors (inflammatory myofibroblastic tumors) of the lung are myofibroblastic lesions of controversial nosology. The concept that these are benign lesions has been recently questioned given their capacity for local invasion and recurrence. PROCEDURE AND RESULTS: We observed five children with inflammatory pseudotumors of the lung in whom the tumor was resected using the most limited excision possible. Unusually, in all five cases the tumor was localized in the right upper lobe: Three were parenchymal, and the others involved the bronchus. The three parenchymal masses underwent wedge resection, whereas the two bronchial lesions required ex-cision followed by a bronchoplasty and an up-per lobectomy, respectively. In all, the pathology showed a complete resection, and the patients had uneventful recoveries. Follow-ups, including CT and bronchoscopies, ranged from 4 to 8 years (mean of 6.4 years) with no signs of recurrence. CONCLUSIONS: Our limited experience supports the idea that excisional surgery is the treatment of choice for inflammatory pseudotumors of the lung. Such excisions, both diagnostic and curative, carry minimal risks and avoid unnecessary mutilation. Closely monitored follow-up is mandatory, as the natural history of this lesion is not yet well understood.
Adolescent↗
Hepatoblastoma: DNA nuclear content, proliferative indices, and pathology.
Hepatoblastoma (HB) is the most frequent malignant liver tumor in infancy, and both its biological features and its prognostic behavior are still under investigation. DNA content and proliferative activity of the tumor have been considered as biological parameters related to the tumor's aggressiveness. The present study attempts to investigate the possible association between histologic subtype, DNA content, and proliferative indices in HB. DNA content and the proportion of cells in the S-phase were assessed by flow cytometry in 34 cases of HB (14 prior to chemotherapy, 20 after chemotherapy), using formalin-fixed, paraffin-embedded archival samples. The proliferative cell nuclear antigen (PCNA) labeling index was also evaluated by immunohistochemistry, and both the flow cytometry (FC) and the immunohistochemical data were correlated with tumor pathology. A significant association was found between histological type, DNA content and the percentage of cells in the S-phase, with aneuploidy and the highest proportions of S-phase cells significantly associated with embryonal tumors. The PCNA labeling index was found to be significantly higher in embryonal than in fetal phenotype. The biological heterogeneity of HB is Confirmed by the different nuclear content of the fetal (diploid) and embryonal (aneuploid) epithelial components of the tumor, also ruling out the likelihood of fetal (diploid) clones deriving from the embryonal (aneuploid) neoplastic cells. Since the highly proliferative neoplastic clones (i.e., embryonal) are thought to be more sensitive to antimitotic drugs, further studies are indicated to determine the relationship between ploidy, proliferative indices and chemoresponsiveness.
Child↗
Load compensation tasks evoke tremor in cerebellar patients: the possible role of long latency stretch reflexes.
'Tremor' is one of the clinical signs of cerebellar dysfunction. Its nature remains subject to debate, one hypothesis being that of a predominant role of peripheral afferences in its genesis. This study was designed to study whether load compensating tasks, evoking sudden stretch, and thus stimulation of peripheral afferences induced tremor in cerebellar patients. We study the kinematics and EMG pattern of a load compensating task which consists of maintaining a constant elbow position despite the onset and cessation of a 2 Nm torque loading the elbow flexors in eight cerebellar patients and six controls. Angular position and velocity, and EMG of the biceps and triceps are recorded at a sampling rate of 1 kHz. In normal subjects, trajectories are simple with little overshoot of the aimed position. EMG analysis shows a long latency stretch response (LLSR) which initiates a phasic and then tonic voluntary activity. In cerebellar patients, the two prominent cinematic features are hypermetria and tremor. The stretch response is of the same latency, but the EMG pattern is modified with bursts of activity related to the tremor. These results show severe perturbations of load compensating tasks in cerebellar patients. We discuss the possible role of the exaggeration of LLSR in both hypermetria and tremor.
Case-Control Studies↗
Endoscopic dilation of acquired tracheobronchial stenosis in infants.
Acquired tracheobronchial strictures in infants are associated with significant morbidity. Their treatment requires different types of therapeutic procedures depending on the severity and location of the stenosis. We successfully dilated five children (ages between 1 and 23 months) with acquired tracheobronchial (stenoses two carinal and three of the mainstem bronchi) using a new technique. Gruentzig balloon catheters of different caliber were introduced into the tracheal lumen of apneic children under endoscopic vision. Three infants required four dilations and in the other two cases only two procedures were necessary. One complication occurred during dilation and resulted in a pneumothorax caused by a partial-bronchial disruption. At follow-up (range, 18-36 months; mean, 23.4 months) all five children were clinically improved. In the four cases without complications the chest x-rays were normal and the last bronchoscopy showed tracheobronchial trees with normal calibers. The child who developed a pneumothorax during attempted dilation recovered. In this child, dilation of the right upper lobe bronchus was unsuccessful. Dilation was attempted without visualization of the bronchus and the stricture. Our limited experience confirms that pneumatic balloon catheters can successfully treat acquired tracheobronchial stenoses in infants. To avoid complications, strict criteria need to be applied in the selection of cases with tracheobronchial stenoses.
Bronchi↗
Is there a place for medical treatment in children with gallstones?
BACKGROUND: Medical treatment of gallstones with ursodeoxycholic acid (UDCA) or chenodeoxycholic acid (CDCA) has not been evaluated in children. AIM: The purpose of this study was to assess the effectiveness of UDCA in the treatment of gallstones in children. METHODS: UDCA was used to treat 15 patients, (7 boys and 8 girls; mean age, 7.8 years; range, 3 months to 15 years) for 1 year. All had radiolucent stones with a maximum diameter of 10 mm and a normally contractile gallbladder. RESULTS: The stones disappeared completely in two children but returned later. All symptomatic patients became symptom free. CONCLUSION: UDCA is ineffective in the treatment of gallstones in children except in terms of relieving symptoms while on treatment.
Adolescent↗
Nodular regenerative hyperplasia of the liver: description of two cases.
Nodular regenerative hyperplasia (NRH) of the liver is a condition of unknown origin, rarely occurring in children, usually accidentally discovered, described in association with a variety of clinical conditions and drugs. Confusion with other types of hepatic masses may pose a problem and for this reason NRH is considered a "tumor-like lesion." Histologically it consists of single or multiple regenerative foci. Hepatic failure and rupture of the liver have been rarely described as complications in adults, and not in children, and malignant transformation has not been demonstrated. Neither surgical removal nor other treatment is needed. These features are described as they were found in two patients we encountered.
Biopsy↗
Necrotizing enterocolitis: late surgical results after enterostomy without resection.
Between January 1985 and December 1992, 36 neonates were treated for necrotizing enterocolitis (NEC). 13/36 were treated conservatively and 2 (18%) died. 23/36 required surgical treatment at birth and 6/23 (26%) died; 14, out of 17 surviving initial surgery, underwent reintervention to restore intestinal continuity, 3 to 26 months later. In these 14 infants the acute necrotizing process involved the whole colon and ileum in 3 cases, the whole colon in 5, part of the colon in 3, part of the colon and ileum in 2 and was confined to the ileum in 1. 11 cases were perforated. During their first laparotomy all 14 cases had a decompressive enterostomy without resection. 21 reoperations were later needed in these 14 patients: simple enterostomy closure was performed in only 1 case, while resection of delayed strictures was required in 13. 12 cases underwent partial colectomy, 3 of these had multiple segmental resections for skip lesions and only 1 child had a total colectomy. The only postoperative complication was one anastomotic leak. Two children had short-gut syndrome but later outgrew their need for parenteral nutrition. Our experience does not support the need of resecting the gangrenous bowel in the acute phase. A simple diverting enterostomy seems to be effective treatment. Delaying the intestinal resection up to the time of recanalisation allows spontaneous healing and results in the sparing of a significant length of bowel.
Colectomy↗