Very rare tumors are usually divided into two groups: One includes tumors that are rare among both children and adults; the other one encompasses tumors that frequently occur in adults but are rarely observed in children. In this review, we focus on adrenocortical tumors, neuroendocrine tumors of the appendix, pheochromocytoma and paraganglioma, pancreatoblastoma and solid pseudopapillary tumors of the pancreas, with special attention to the role of surgery as main curative intervention or as part of the multimodal treatment.
View Article and Find Full Text PDFBackground: Invasive fungal disease (IFD) is typically aggressive and related with high mortality in children with a hematological malignancy. The association of medical and surgical treatment may ameliorate the outcome. The aim of this study was to analyze the surgical treatment of fungal infections in pediatric oncological populations.
View Article and Find Full Text PDFGerm cell tumors (GCTs) are a heterogeneous group of pediatric cancers. In up to one-third of male patients, a primary mediastinal location is associated with the presence of Klinefelter syndrome (KS). We describe a case of mediastinal GCT in a patient, with unacknowledged KS, that presented a relapse 7 years from diagnosis, that is, 2 years after the end of the follow-up program usually recommended for patients with GCT.
View Article and Find Full Text PDFBackground: To assess the outcomes of pediatric patients with undifferentiated embryonal sarcoma of the liver (UESL) and treatment including at least surgery and systemic chemotherapy.
Methods: This study included patients aged up to 21 years with a pathological diagnosis of UESL prospectively enrolled from 1995 to 2016 in three European trials focusing on the effects of surgical margins, preoperative chemotherapy, use of radiotherapy (RT), and chemotherapy.
Results: Out of 65 patients with a median age at diagnosis of 8.
Although survival after rhabdosarcoma treatment has improved over the years, one third of patients still develop locoregional relapse. This review aims to highlight developments pertaining to staging and local treatment of specific RMS tumor sites, including head and neck, chest/trunk, bladder-prostate, female genito-urinary, perianal, and extremity sites.
View Article and Find Full Text PDFIntroduction: Vascular complications are severe complications of pediatric kidney transplantation (KT). We aimed to investigate whether a complex bench surgery (BS) affects the outcomes.
Methods: All pediatric KT performed at the University Hospital of Padua from 2015 to 2019 were analyzed, comparing those in which a standard BS was possible to those that necessitated a complex BS.
Background: End stage renal disease (ESRD) requiring kidney transplant (KT) remains an important cause of morbidity in anorectal malformations (ARM) patients. Current literature is scarce on defining the risk factors for ESRD and the outcomes of KT in ARM patients.
Objective: This study aimed to identify predisposing factors to ESRD in ARM patients and verify if the long term outcome of KT in these patients differs from pure urological anomalies (UA).
Background/objectives: Rhabdomyosarcoma of the perianal/perineal region (PRMS) is rare, with poor survival and limited understanding of the functional consequences of treatment.
Design/methods: International Society of Pediatric Oncology (SIOP) malignant mesenchymal tumor (MMT) 95, Italian RMS 96, and European paediatric Soft tissue sarcoma Study Group (EpSSG) RMS 2005 studies were interrogated to identify factors that impact survival; in RMS 2005, functional outcomes were analyzed.
Results: Fifty patients (nonmetastatic) were identified, median age 6.
Myositis ossificans circumscripta (MOC) is a benign disease characterized by localized heterotopic bone formation within muscles or soft tissue, usually interesting great muscles of extremities. We report a rare case of unusual location in the neck not associated with previous trauma, mimicking a solid tumor, with well-documented diagnostic imaging features. During COVID-19 pandemic outbreak in Italy, in May 2020, a 14-year-old boy developed a progressive and persistent neck pain on the right side, without known history of trauma.
View Article and Find Full Text PDFBackground: A prompt detection of graft venous thrombosis might preserve the transplanted organ. A real-time near-infrared spectroscopy (NIRS) monitoring of the allograft perfusion could fulfill this goal. The aim of this work was to report the trend of allograft perfusion (rSO2) after pediatric kidney transplantation (KT) complicated by graft venous thrombosis.
View Article and Find Full Text PDFBackground: Infants (<12 months) with rhabdomyosarcoma have historically had poorer outcome than the older age groups. We present outcomes for infants and young children aged 12-36 months with localised rhabdomyosarcoma with a particular emphasis on infants.
Patients And Methods: All children less than 36 months of age enrolled on the EpSSG RMS 2005 study for localised disease are included.
Pediatric abdominal surgery is constantly evolving, alongside the advent of new surgical technologies. A combined use of new tools and traditional surgical approaches can be useful in the management of complex cases, allowing less invasive procedures and sometimes even avoiding multiple interventions. This combination of techniques has implications even from the anesthetic point of view, especially in post-operative pain control.
View Article and Find Full Text PDFExtraperitoneal approach is sometimes recommended for kidney transplantation (KT) in children weighting <15 kg. We hypothesized that this approach might be as successful as in patients with normal weight. Data of all consecutive KTs performed between 2013 and 2019 were retrospectively reviewed.
View Article and Find Full Text PDFProcedure: The survival of children with rhabdomyosarcoma (RMS) has gradually improved as a result of the adoption of multidisciplinary treatments. Dedicated skills and facilities are indispensable and more readily available at reference centers. In this study, we examined the role of centers' experience (based on the number of patients treated) in their management of patients with RMS.
View Article and Find Full Text PDFBackground: Allograft venous thrombosis is a severe complication after kidney transplantation (KT). Early diagnosis and prompt treatment are crucial in preserving the survival of the allograft. In this study, we aimed to describe an emergent strategy for the management of acute allograft venous thrombosis.
View Article and Find Full Text PDFNeuroblastoma (NB) is the most common extracranial, solid, pediatric malignancy and, despite the constant progress of treatment and development of innovative therapies, remains a complex, challenging disease causing major morbidity and mortality in children. There is significant variability in the management of neuroblastoma, partially due to the heterogeneity of the clinical and biological behavior, and partially secondary to the different approaches between treating institutions. Anesthesia takes an integral part in the multidisciplinary care of patients with NB, from diagnosis to surgery and pain control.
View Article and Find Full Text PDFBackground: Irinotecan is a drug active against pediatric sarcomas with a toxicity profile that theoretically allows for its association with more myelotoxic drugs. We examined the feasibility of a dose-density strategy integrating irinotecan in standard chemotherapy regimens for patients with high-risk sarcomas.
Methods: Between November 2013 and January 2020, 23 patients ≤25 years old were included in the study.
Background: Rhabdomyosarcoma (RMS) is the most common form of soft tissue sarcoma in children. We report the results of the European paediatric Soft tissue sarcoma Study Group (EpSSG) RMS 2005 study, which prospectively evaluated the reduction of chemotherapy in patients with embryonal RMS (ERMS) after initial surgery.
Methods: Between October 2005 and December 2016, all patients with localised ERMS with an initial microscopically complete resection (IRS group I) with lymph node-negative (N0) were prospectively enrolled in the low-risk (n = 70, subgroup A; age < 10 years and tumour size ≤ 5 cm) or standard-risk group (n = 108, subgroup B; age ≥ 10 years or tumour size > 5 cm.
Pediatric adrenocortical tumors (ACT) are rare and sometimes aggressive malignancies, but there is no consensus on the outcome predictors in children. A systematic search of MEDLINE, SCOPUS, Web of Science, and the Cochrane Library for studies from 1994 to 2020 about pediatric ACT was performed. In 42 studies, 1006 patients, aged 0-18 years, were included.
View Article and Find Full Text PDFBackground: 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.
Pediatr Blood Cancer
September 2020
Background: Paratesticular rhabdomyosarcoma (PT RMS) is rare compared to benign scrotal pathology. Inappropriate first surgery (InFS) required supplementary treatment to maintain excellent outcomes. Initial staging of regional lymph nodes is important.
View Article and Find Full Text PDFPediatr Blood Cancer
July 2019
Objectives: To evaluate the impact of local therapies on the outcome of patients with liver-bile duct rhabdomyosarcoma (LBDRMS).
Methods: Data of 30 patients included in the EpSSG-RMS 2005 study were analyzed.
Results: The median age at diagnosis was 3 years (11 months-8 years).