Biomedical subjects

C Boglino

Publications and source records attributed to C Boglino.

At least 19 recordsLinked to original sources

PubMed2001

111 renal neoplasms of childhood: a clinicopathologic study.

PURPOSE: The aim of this study was to perform a clinicopathologic evaluation of a single pediatric institution renal tumor series. Most patients were treated within the frame of 3 consecutive SIOP trials, which included preoperative chemotherapy as their main feature. METHODS: Medical records and diagnoses of 111 patients were reviewed. The association of pathologic features with outcome was investigated by means of the Kaplan-Meier method, the Cox model, and a logistic multivariate analysis. Comparison among different trial results was carried out. RESULTS: In 98 patients (88%), nephroblastoma was diagnosed, followed by 6 adult-type renal tumors, 3 cystic nephromas, 2 mesoblastic nephromas, and 2 clear cell sarcomas. For nephroblastoma, a statistically significant correlation between grade and both disease-free survival rate and 5-year survival rate, and between stage and overall survival rate was shown. Lymph node involvement, local relapse, nephrogenic rests, and older age at presentation appeared to be less important prognostic factors. Tumor spillage was very sensitive to chemo or radiotherapy. No significant difference in outcome was observed among different trials. CONCLUSIONS: Wilms' tumor was the most frequent neoplasm and resulted in a 5-year cure rate of 90%. Clinical course was influenced mainly by diffuse anaplasia and, to a minor extent, by lymph node involvement. Because some tumors followed an unpredictable course, it is likely that also other biological factors played a significant role.

Adolescent↗

PubMed2001

Glutathione transferase P1 polymorphism in neuroblastoma studied by endonuclease restriction mapping.

Several members of the different glutathione transferase (GST) gene classes are polymorphic. Particular interest has been focused on the GSTP class because this gene class is up-regulated during the early stage of oncogenesis and is significantly overexpressed in many human tumors. It has also been shown that high levels of GSTP1 expression are associated directly with tumor drug resistance and with poor patient survival. Our aim was to understand the possible association between GSTP1 polymorphism and cellular response to chemotherapeutic drugs in neuroblastoma. In fact, several antineoplastic drugs used in the neuroblastoma high-risk chemotherapeutic protocol are potential substrates of GSTP1-1 (etoposide, adriamycin and carboplatin). The GSTP1 genotype homozygote *A/*A was identified in 11 patients independent of their response to the chemotherapeutic treatment. Only four patients had a heterozygote genotype A*/B*. Therefore, based on our preliminary data, we were not able to conclude that GSTP1 polymorphism had an impact on patient response to treatment in neuroblastoma.

Adolescent↗

PubMed2000

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↗

PubMed2000

Features and outcome of neuroblastoma detected before birth.

BACKGROUND/PURPOSE: The growing use of routine ultrasonography during pregnancy is leading to an increasing number of prenatally diagnosed neuroblastomas. Optimal strategy has not yet been defined for these patients, because knowledge on this particular neuroblastoma (NB) population is still limited. However, definite guidelines are needed to avoid inadequate treatment. The authors analyzed the cases of antenatally detected NB (ADNB) reported in the Italian Neuroblastoma Registry during the past 6 years to elucidate the features of this subset of NB. METHODS: The Italian Neuroblastoma Registry was reviewed for the period January 1993 to December 1998 to collect clinical, radiographic, surgical, and histopathological data on ADNB cases. NB stage was evaluated according to INSS criteria. All patients had undergone imaging (computed tomography or magnetic resonance imaging) of the primary tumor and bone marrow biopsy before surgical resection. RESULTS: Seventeen patients were identified. Primary tumour site was adrenal glands in 16 cases and retroperitoneal ganglia in 1. Stage distribution was stage I, 13 cases; stage II-A, 1 case; stage II-B, 1 case; stage IV-S, 2 cases. All cases underwent primary tumour resection. Mean age at surgery was 4 weeks. Resection of primary tumor was radical in 16 cases, partial in 1. All tumors were characterised by favourable histology according to Shimada classification. N-myc gene amplification was studied in 14 patients. N-myc amplification was detected only in a newborn with stage II-A NB, who died of massive bleeding 2 days after tumor resection. DNA index and 1p deletion were studied in 11 and 8 patients, respectively. Both diploidy and deletion of 1p were observed in a newborn who subsequently died of disease progression despite surgery, chemotherapy, and radiation therapy. Fourteen of 17 patients currently are alive and free of disease, and one with IV-S NB and short follow-up is alive with disease. CONCLUSIONS: Our data give evidence that in most cases infants with ADNB represent a subset of patients with excellent outcome. Aggressive treatment may not always be necessary. Infants with ADNB with unfavorable features should undergo early surgical excision, whereas patients with favourable features could be observed awaiting spontaneous regression of the mass, reserving delayed surgery for tumors that increase in size or do not regress.

Adrenal Gland Neoplasms↗

PubMed1999

Spinal cord vascular injuries following surgery of advanced thoracic neuroblastoma: an unusual catastrophic complication.

BACKGROUND: Spinal cord injury is a possible complication associated with removal of thoracic dumbbell neuroblastomas. Our experience with two children whose postsurgical course was complicated by midthoracic spinal cord ischemia is reported there. Permanent paraplegia resulted in both. PROCEDURE AND RESULTS: Preoperative awareness of the origin and distribution of the Adamkiewicz artery (arteria radiculomedullaris magna, ARMM) and of the possible collateral pathways for spinal cord blood supply may be helpful in the planning of operations that involve dissection in the midthoracic posterior mediastinum. Otherwise, a flaccid paraplegia may result. CONCLUSIONS: The syndrome is presumed to be triggered by a spasm, an embolism, or a iatrogenic interruption of the ARMM.

Child, Preschool↗

PubMed1999

Myofibroblastic tumours: neoplasias with divergent behaviour. Ultrastructural and flow cytometric analysis.

Myofibroblasts are spindle cells having ultrastructural features in common with smooth muscle cells and fibroblasts. In the last few years, tumours have been described in which myofibroblasts represent not only a reactive mechanism but also a true neoplastic component. They constitute new nosologic entities which might be termed "myofibroblastic tumours". Tumours with benign and, rarely, malignant behaviour are reported to belong to this group of lesions. Recently, a third tumour type with borderline biological course, named "inflammatory myofibroblastic tumour" (IMT), has been identified, a condition that has been regarded as a benign and reactive disorder for a long time. Only in recent reports has been demonstrated that, in spite of an apparently benign morphological pattern, some cases of IMT have a malignant course. In this connection, DNA analysis by flow cytometry is a valuable diagnostic tool, because it allows identification of the ploidy status, a procedure that is often useful for predicting the nature and the biological behaviour of the lesion. In this study, 11 cases of myofibroblastic tumours were examined retrospectively by evaluating clinicopathological features and DNA ploidy status by flow cytometry. The diagnosis of myofibroblastic tumour was confirmed by performing histology, immunohistochemistry, and electron microscopy in all patients. In detail, these 11 cases were composed of 1 benign myofibroblastoma, 1 myofibrosarcoma and 9 IMTs. Among these myofibroblastic tumours, all those with local recurrence or distant metastases (one myofibrosarcoma and three IMT) showed an aneuploid cell population demonstrable by flow cytometric analysis, whereas the other cases with benign course (one benign myofibroblastoma and six IMT) exhibited an euploid DNA content. These data suggest the following: a) Besides the rare myofibroblastomas and myofibrosarcomas, IMTs represent a larger group of lesions with potentially different biological and clinical course. b) DNA flow cytometric analysis is a reliable tool that support histopathological examination in characterizing those cases of IMT that, though being malignant, mimic benign lesions. Consequently, it establishes the basis for a different therapeutic approach according to the euploid or aneuploid DNA content.

Adult↗

PubMed1998

[Recurrent abdominal pain caused by nodular focal hyperplasia of the liver. A case report].

A case of focal nodular hyperplasia of the liver in childhood (FNH or NRC) is reported; a conservative approach is usually preferred in this disease when the diagnosis is surely identified, and a follow-up for some years is recommended. Despite the increased accuracy of modern imaging methods none of them, singly or together, is able to differentiate a malignant lesion with certainty and histological confirmation is mandatory. In the case described a laparotomy frozen section was performed but it was not possible to make a diagnosis and the liver mass was removed. The patient had a complete relief of symptoms with no evidence of recurrence in the follow-up. A review of the literature has been made, trying to point out this disease and its treatment.

Abdominal Pain↗

PubMed1998

[Peripheral neuroectodermal tumors of the chest wall. Description of a case].

The authors discuss a case of malignant small round cell tumor of the thoracopulmonary region (Askin tumor) in which the initial pathological diagnosis was "sarcoma". Only a review of the histological specimens and the clinical features and evolution revealed the correct diagnosis of "Askin tumor". A 15 year old girl with fever, dyspnoea, cough underwent thoracentesis, which revealed hemothorax. At surgery, a large mass not involving the lung, with pleural thickening, was found in the left hemithorax and excised, without rib resection. No metastasis was observed at that time. Histological findings revealed features of malignant small round cell tumors, with occasional pseudo-rosettes. Chemotherapy was performed. 16 months after clinical presentation the girl died with abdomino-thoracic metastatic disease.

Adolescent↗

PubMed1996

Resection of primary tumor at diagnosis in stage IV-S neuroblastoma: does it affect the clinical course?

PURPOSE: To determine whether resection of primary tumor has a favorable influence on outcome of infants (age 0 to 11 months) with stage IV-S neuroblastoma. PATIENTS AND METHODS: Between March 1976 and December 1993, 97 infants with previously untreated neuroblastoma diagnosed in 21 Italian institutions were classified as having stage IV-S disease. Seventy percent were younger than 4 months. Adrenal was the primary tumor site in 64 of 85 patients with a recognizable primary tumor. Liver was the organ most often infiltrated by the tumor (82 patients), followed by bone marrow and skin. RESULTS: The overall survival (OS) rate at 5 years in 80% and event-free survival (EFS) rate 68%. In 24 infants, the effect of resection of primary tumor could not be evaluated because of rapidly fatal disease progression (n = 8), absence of a primary tumor (n = 12), or partial resection (n = 4). Of 73 assessable patients, 26 underwent primary tumor resection at diagnosis: one died of surgical complications, one relapsed locally and died, and two others relapsed (one of these two locally) and survived, for a 5-year OS rate of 92% and EFS rate of 84%. Of the remaining 47 patients who did not undergo primary tumor resection at diagnosis 11 suffered unfavorable events, of whom five died, for an OS rate of 89% and EFS rate of 75% (no significant difference from previous group). Disease recurred at the primary tumor site in only one five who died, and in only one of six survivors of progression or relapse; in these patients, the primary tumor, located in the mediastinum, was successfully resected. CONCLUSION: Infants who underwent resection of the primary tumor at diagnosis had no better outcome than those in whom the decision was made not to operate.

Adrenal Gland Neoplasms↗

PubMed1994

[Echinococcosis/hydatidosis in children: combined medical and surgical treatment].

The outcome of 5 children with visceral hydatid cysts treated with Albendazole and surgical therapy is reviewed. The diagnoses were confirmed by immunological tests, X-ray, ultrasound scanner (US) and computer tomography. The therapeutic approach was correlated to the type of infection pictures. Immunological tests, X-ray and US were performed with the aim to evaluate the response to the therapeutic protocol. Combination of pharmacological and surgical treatment resulted in a complete resolution for all patients without any recurrence and further dissemination of infection.

Albendazole↗

PubMed1993

[Surgery of thyroglossal duct in childhood].

A retrospective review of 177 neck masses in childhood treated between January 1975 and December 1990 at the "Bambino Gesù" Pediatric Hospital of Rome is presented, with particular emphasis on recurrences and their causes. 105, object of our study, were thyroglossal duct cysts (TDC) (58.7%); in the remaining 72 cases there were dermoid cysts (23.1%), granulomas (7.9%), lateral neck masses (1.69%) and other histological patterns. The aim was to identify the variables that could change postoperative hospitalization or cause the recurrence. The TDC's group was separated in two: 92 primary patients, while 13 received a preliminary operation in other institutions. In the first group we applied 41 Sistrunk, 43 Schlange, 8 cystectomies; 5 recurrences occurred (5.43%), 3 of which in patients operated on with modified Sistrunk's technique (Schlange) (60%). In the second group (13 patients) a standard Sistrunk procedure was always performed. We had only one recurrence (7.69%) due to a remnant of multiple proximal thyroglossal duct. In 11 cases (10.47%) there were postoperative complications due to an existing inflammatory condition of the TDC (8 patients -72.72%). In only one case there was a recurrence after 27 months. Histological study of the specimens showed that thyroglossal ducts were frequently multiple and widely spaced and may also be present in the distal portion of the duct; in our series in 16.19% we found the distal portion of the duct during the operation. Our overall recurrence frequency is 5.7%. Every lesion referred to a TDC must be treated with a Sistrunk procedure for the better results achieved with this surgical approach but, in our experience, we prefer to complete the Sistrunk's technique with the research and exeresis of the distal portion of the duct by a "muscular sparing procedure" that preserves little patients from pharyngodynia. At the follow-up, we have not recurrence in the 17 cases operated on by this procedure.

Adolescent↗

PubMed1993

[Treatment of parapneumonic empyema in children].

A 31 case series of pleuropneumonia is presented, in a 7 year period. All patients were treated following same standards consisting of a pleural catheter insertion. Results support conservative treatment: short hospitalization and absence of psychological or physical traumas was achieved. Follow up is very good for all patients and none had invalidating consequences.

Child↗

PubMed1993

D-CECaT: a breakthrough for patients with neuroblastoma.

In view of the high relapse rate following chemotherapy for patients with advanced neuroblastoma (NB) and primitive neuroectodermal tumors (PNET), we designed a novel chemotherapy program which incorporated the iron chelator deferoxamine. The purpose of the deferoxamine was to sensitize the cells to standard chemotherapy. The D-CECaT regimen contained (in mg/m2): deferoxamine 4500 during days 1-5; cyclophosphamide 600 mg over days 6 and 7; etoposide 300 mg over days 7 and 8; carboplatin 100 mg over days 7 and 8; and thiotepa 30 mg over days 6-8. Between October 1989 and May 1992 we entered 23 advanced NB and two PNET patients. Sepsis occurred in four courses, nausea and vomiting in 30 courses, and 50 courses required blood and platelets. Responses observed in previously untreated patients with stage III NB: six out of six CR (17+ to 41+ months), with stage IV NB, nine out of 11 CR (14+ to 28+ months), two out of 11 VGPR (22+ months), with stage IV PNET two out of two CR (1+ to 35+ months). With previously treated and failed stage IV NG, two out of six VGPR for 19+ and 20 months, and four out of six PR 1, 8, 9 and 11 months. Median survival for 19 new patients was 22+ months (6 to 41+ months; two patients in CR died at 7 months during adjuvant autologous marrow transplant). In conclusion, D-CECaT is an effective initial cytoreductive regimen for advanced stage NB/PNET patients. Additional patients and studies are required to determine its use as an alternative to autologous bone marrow transplantation.

Antineoplastic Combined Chemotherapy Protocols↗

PubMed1992

[Persistent multiple urachal complex. Echographic-surgical correlations].

Urachus is a tubular structure lined between foetal bladder and the umbilicus and is susceptible to complete or partial involution after birth. Persistence of the urachus results in a wide spectrum of anomalies: patent urachus, vesicourachal diverticulum, urachal sinus and cysts are more frequently seen than rare multiple urachal remnants. This kind of pathology focuses the problem of differential diagnosis (tumours, omental and ovarian cysts, vesical diverticulum or duplication) and may be complicated by a superinfection. The Authors discuss a bizarre multiple urachal remnant, presenting with urinary tract symptoms, which may be clinically confused with acute appendicitis or Meckel's diverticulitis. Contribution of sonography for a complete diagnosis is stressed, such as the precise correlation with surgical findings.

Adolescent↗

PubMed1992

Neurotensin localization in adenomatoid cystic malformation versus normal lung: preliminary report of six consecutive cases.

Neuropeptides are considered a new class of neurotransmitters, several of which interact with the immune system as well as the macrophagic activity. Among these, neurotensin (NT) enhances the phagocitic response of macrophages and is the only neuropeptide that can enhance the cytolytic effects of activated macrophages. In this way, it may play a role as an inflammatory mediator. In order to investigate the possible relationship between NT and the defence mechanisms of the lung, we started to localize the presence of NT in pulmonary adenomatoid cystic malformation (CCAM). This series consists of 6 children affected by CCAM. In every case, at operation, we obtained specimens of both normal and pathological lung. Tissue sections from the pathological lung showed a significant increase of NT-like immunoreactivity in respect to sections of normal lung. NT influences and activates the macrophages, thus suggesting that it could represent a defence mechanism in children's lung activated in some malformative conditions. Finally, the increasing evidence of NT immunoreactivity in CCAM could explicate an in utero infectious pathogenesis of this malformation.

Child↗

PubMed1991

[Antibiotic prophylaxis with ceftazidime in pediatric surgery].

The authors report their experience with Ceftazidime in surgical prophylaxis in childhood. 50 patients have been treated, suffering from surgical diseases, with 100 mg/kg of Ceftazidime in 3 doses (1 before, 1 after 6 hrs and 1 after 12 hrs from surgery). No side effects were observed and in no case was it necessary to discontinue treatment. Both bacterial and clinical results indicate good tolerability and very rapid effectiveness of the drug. Complications were present in 3 cases (6%) (in 2 cases wound infiltration and in 1 case a Douglas abscess) but no modification in treatment was necessary. All complications were present in so-called "dirty surgery" with a relative percentage of 18.7, that is far below values reported in the literature for this kind of surgery.

Adolescent↗

PubMed1991

[Interstitial pulmonary emphysema. Combined therapeutic approach in a retrospective multidisciplinary study].

Pulmonary interstitial emphysema (PIE) may occur spontaneously, as a complication of endotracheal tube displacement, intrauterine viral pneumonia or massive aspiration of formula. Nevertheless PIE occurs more frequently in neonates requiring mechanical ventilation for RDS. Untoward effects of large air collections in the extra-alveolar spaces are based on decreased perfusion and ventilation of the affected lung tissues, compression of adjacent pulmonary parenchyma and mediastinum, possible air embolism. Sometimes PIE spontaneously regress, but in some instances the process is self perpetuating, leading to an "air block syndrome". Fourteen (19.4%) of 72 infants ventilated for RDS in a three-year period had radiological evidence of PIE during the first few days of life. All 5 bilateral, 7 diffuse and 2 localized cases were treated with "vigorous pulmonary therapy". In addition to these procedures, 4 neonates were submitted to a selective bronchial intubation (SBI) and 5 different infants improved after HFV. No one of our patients underwent a surgical procedure. Infants with fine linear hyperlucencies improved sooner. All pneumothoraces (7 of 14) were preceded by X-ray appearances of PIE. Three neonates died. Mortality was observed in newborns with bilateral PIE, because of an intraventricular hemorrhage in two and an intractable under tension pneumothorax in one patient. Plain chest roentgenograms, histological pictures and treatment modalities of PIE remain separated from these considered for congenital lobar emphysema, congenital cystic adenomatoid malformation, bronchogenic cyst or lung sequestration. Surgical treatment of PIE is not as universally accepted as in congenital cystic lesions of the lung, in which it's mandatory.

Acetylcysteine↗

PubMed1990

[Experience in the prophylactic use of ceftriaxone in pediatric surgery].

Authors report on their experience about ceftriaxone in surgical prophylaxis in childhood. 30 patients have been treated, suffering from surgical diseases, either thoracic or abdominal ones, including malignancies. Ceftriaxone was administered once a day; patients received ceftriaxone only once preoperatively: Results have been very satisfactory, the only two complications having been found in two severely immunocompromised patients.

Abdomen↗