1,721,037 research outputs found
Il ruolo dell'analisi morfologica del reticolo intercellulare (the "reticulin algorithm") nel distinguere le forme benigne dalle forme maligne di tumore adrenocorticale in età pediatrica
Full text linkABSTRACT
Background. The diagnosis of pediatric adrenocortical tumors (ACT) is sometimes difficult and it does not always allow to distingush beteween benigna and malignant tumors. Wieneke’s score is useful to classify ACT in benign, indeterminate and malignant tumors but it is based on the evaluation of nine different histological features. The Reticulin Algorithm (RA) has been proved to be reliable and easily reproducible in the classification of adult adrenocortical tumors. Aim of this study is to retrospectively validate the use of RA in a series of pediatric ACT.
Materials e methods. Thirty-seven pediatric ACT treated in 3 different european centers were evaluated: according the Wieneke’s score, they were classified as benign in 46% of cases (17), indeterminate in 22% (8) e malignant 32% (12). All cases were reclassified according the RA (malignant if necrosis, vascular invasion and high mitotic count were found associate to an altered reticulin framework). The mitotic index considered was both the adult one (>5/50 HPF; aRA) and the pediatric one (>15/20 HPF; pRA). The reticulin was considered altered with both qualitative and quantitative alterations. Five pathologists revised independently the sections stained for the reticulin framework and a second round was performed in order to obtain a consensus on discordant cases.
Results. Reticulin framework was intact in 8 cases (22%), all benign according Wieneke’s score as well. The remaining 29 cases (78%) showed an alteration (quantitative in 16 cases, qualitative in 9 and both in 4). Both Wieneke’s score (p<0.0001) and RA (pRA p=0.0005 e aRA p=0.015) showed an association with the overall outcome. All indeterminate cases according to Wieneke had a favourable clinical course, except for 2 who are alive with disease. aRA distributed these case as follows: 4 in the benign group e 4 in the malignant group; pRA classified 5 as benign and 3 as malignant.
Conclusions. RA is a reliable prognostic algorithm to diagnose and stratify pediatric ACT both in its version already described for adult ACT and in the version, described for the first time, here adapted for pediatric ACT
Could a careful clinical examination distinguish physiologic phimosis from balanitis xerotica obliterans in children?
Full text linkThe diagnosis of balanitis xerotica obliterans (BXO) in children may be challenging, since clinical examination only could lead to
an underestimation of its incidence. The aim of this retrospective and single-centre study is to assess the diagnostic performance
of clinical examination, together with clinical history, in identifying BXO. Ninety-seven children underwent circumcision for
phimosis from 2015 to 2019. Histology was routinely performed. Cohen’s kappa coefficient, sensitivity, specificity, predictive
values, likelihood ratios and accuracy of macroscopic appearance of the foreskin, steroid administration and past medical history
were estimated. Forty-eight patients (50%) were affected by BXO; 31 of them (69%) presented with suggestive clinical signs. A
strictured or whitish urethral meatus was detected during surgery in nine cases (19%); this was associated to allergic or immune
diseases (p = 0.046). Foreskin appearance alone mildly correlated with histology (k = 0.494; p < 0.001) and it showed a diagnostic
accuracy of 75%. The specificity and positive predictive value of abnormal macroscopic findings at examination, together
with a positive clinical history for other allergic or immune diseases, and/or for balanitis, were 100% and the positive likelihood
ratio was greater than 10. Conversely, sensitivity decreased to 4.5% (95% CI 0–11%).
Conclusion: Foreskin appearance together with clinical history could predict BXO with certainty. However, since the absence
of a positive medical history could not exclude the diagnosis, foreskin histology is still highly recommended
Inguinal hernia containing the uterus in the pediatric patient: a systematic review
No full textBackground
Inguinal hernia containing the uterus in pediatric patients with normal karyotype and phenotype is an extremely rare entity, and no consensus is available concerning diagnosis, surgical management and follow-up.
Methods
A systematic review according the Synthesis without Meta-analysis (SwIM) protocol was conducted. Studies including pediatric female patients with inguinal hernia containing the uterus were searched. Keywords and mesh term searches were conducted (Medline, Scopus and Web of Science). We additionally reviewed our Center's clinical records, finding one patient with inguinal uterus hernia that was included in the statistical analysis.
Results
36 articles and 73 patients were considered for this analysis. The median gestational age at birth was 36 weeks. The inguinal mass was first noticed at the median age of 1.5 months (0-18 months), on the left side in 61% (on the right in 16 39%). Sixty percent of patients had no associated symptoms nor signs; 37.5% showed symptoms and signs of an incarcerated hernia. Median age at surgery was 2 months (1-72 months). In all patients, one or both adnexa herniated with the uterus. An open approach was more frequently used (56.4%). Contralateral duct exploration and ligation was performed in 7 patients (24.1%). No post-operative complications nor recurrences have been described.
Conclusions
Data obtained do not allow us to draw univocal conclusions on whether which is the best management in these patients: it is still unclear if an elective approach in an asymptomatic patient may bear long term consequences on reproductive functions. No surgical technique proved to be superior to others, but both the open and laparoscopic approaches seem to be effective without postoperative complications nor recurrences
Long-term complications in patients with bladder-prostate rhabdomyosarcoma treated with brachytherapy: a systematic review
No full textIntroduction: Brachytherapy has been used for the multimodal treatment of pediatric bladder-prostate rhabdomyosarcoma in the last two decades. The aim of this systematic review is to gather the current evidence about this innovative technique with a special focus on long-term outcomes. Methods: According to PRISMA criteria, PubMed, Scopus, and Web of Science were searched for papers published between 2000 and 2022. Results: The search yielded 7338 papers but only seven were eligible, for a total of 196 children with a median age ranging from 23 to 32 months and a median follow-up ranging from eight to 64 months. The five-year overall survival was superior to 90%. However, at least one complication involving the urogenital apparatus was reported in 66 children (35%). Conclusion: Brachytherapy presented positive outcomes in terms of overall survival. On the other hand, further efforts should be made to decrease the risk of functional urogenital side effects
Newborn with incarcerated inguinal hernia and complete androgen insensitivity syndrome
Full text linkDisorders of sex development represent a large and heterogeneous group of diseases that have an important impact on physical and mental well-being of patients and their families. An early diagnosis and a multidisciplinary approach are fundamental to guarantee the highest standard of care.
We describe a case of complete androgen insensitivity syndrome (CAIS) in a six-days-old female newborn brought to our Emergency Department for intestinal occlusion due to an incarcerated inguinal hernia. During the operation, male-appearing gonads were identified in both inguinal canals and the histology confirmed the testicular features. Blood tests validated the diagnosis.
The diagnosis of CAIS is usually made during elective inguinal hernia repair or for delayed puberty. To our knowledge, this is the first case diagnosed in a neonatal urgent setting. Being able to immediately recognize this clinical picture, it allowed us to offer the best standard of care for the patient and the family
Metachronous Contralateral Cystadenoma After Ovariectomy for Juvenile Granulosa Cell Tumor in a Young Girl: An Uncommon Association
No full textBackground: A unique Case of metachronous contralateral cystadenoma diagnosed 2 years after a juvenile granulosa cell tumor (JGCT) of
the ovary is reported.
Case: One year after a left ovariectomy for JGCT, a 7 year-old girl was found to have, during her standard ultrasound follow-up, a 18-mm
cyst in the right ovary, which was initially considered to be a follicular cyst. Six months later, the mass appeared to be increased and
characterized by multivacuolar features, and inhibin A was mildly elevated. A laparoscopic-assisted cystectomy was performed, sparing
healthy ovarian tissue around. The pathology report showed a benign mucinous cystadenom
Approccio al bambino neuropatico con disfagia: l’alleanza necessaria tra anestesista e chirurgo.
No full textManagement of Symptomatic Mesenchymal Hamartoma of the Chest Wall: Surgical Resection Only in Symptomatic Cases
No full textMesenchymal hamartoma of the chest wall (MHCW) is a rare neonatal benign tumor with an estimated incidence of 1 in 3 000 among primary bone tumors, and 1 in one million in the general population. Traditionally, the treatment of choice was an "en bloc" resection, but surgery limited to symptomatic cases, is now suggested by most authors due to the numerous cases of spontaneous regressions. We report 2 patients of symptomatic MHCW, characterized by progressive respiratory distress, who underwent surgical treatment with prompt resolution of symptoms. Surgeons and neonatologists should be aware of this rare condition and its possible fatal or nearly-fatal complications
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