This study further supports technical advantages, lighter impact of the surgical procedure on infants, lower incidence of complications, and better long-term outcome of the transanal pull-through compared to the Duhamel approaches.
Background
Solid pseudopapillary pancreatic tumors (SPPT) are an extremely rare entity in pediatric patients. Even if the role of radical surgical resection as primary treatment is well established, data about follow‐up after pancreatic resection in children are scant.
Methods
A retrospective review of data from the Italian Pediatric Rare Tumor Registry (TREP) was performed. Short‐term (<30 days) and long‐term complications of different surgical resections, as well as long‐term follow‐up were evaluated.
Results
From January 2000 to present, 43 patients (male:female = 8:35) were enrolled. The median age at diagnosis was 13.2 years (range, 7–18). Nine children had an incidental diagnosis, whereas 26 complained of abdominal pain and 4 of palpable mass. Tumors arose either from the head of pancreas (n = 14) or from body/tail (n = 29): only one patient presented with metastatic disease. Resection was complete in all patients (cephalic duodenopancreatectomy vs distal resection). At follow‐up (median, 8.4 years; range, 0–17 years), one recurrence occurred in a patient with intraoperative rupture. All patients are alive. Three pancreatic fistulas occurred in the body/tail group, whereas four complications occurred in the head group (one ileal ischemia, two stenosis of the pancreatic duct, and one chylous fistula).
Conclusion
Surgery is the best therapeutic option for these tumors; hence, complete resection is mandatory. Extensive resections, including cephalic duodenopancreatectomy, are safe when performed in specialized centers. Long‐term follow‐up should be aimed to detect tumor recurrence and to evaluate residual pancreatic function.
Background: Hypertension (HT) is rarely reported in patients affected by Neuroblastoma (NB), and management guidelines are lacking. Clinical features and perioperative medical treatment in such patients were reviewed to 1) ascertain whether a shared treatment strategy exists among centers and 2) if possible, propose some recommendations for the perioperative management of HT in NB patients. Methods: A retrospective multicenter survey was conducted on patients affected by NB who presented HT symptoms. Results: From 2006 to 2014, 1126 children were registered in the Italian Registry of Neuroblastoma (RINB). Of these, 21 with HT (1.8%) were included in our analysis. Pre-and intraoperative HT management was somewhat dissimilar among the participating centers, apart from a certain consistency in the intraoperative use of the alpha-1 blocker urapidil. Six of the 21 patients (28%) needed persistent antihypertensive treatment at a median follow-up of 36 months (range 4-96 months) despite tumor removal. Involvement of the renal pedicle was the only risk factor constantly associated to HT persistency following surgery. A correlation between the presence of HT and the secretion of specific catecholamines and/or compression of the renal vascular pedicle could not be demonstrated.
Survival rate for childhood cancer has increased in recent years, reaching as high as 70% in developed countries compared with 54% for all cancers diagnosed in the 1980s. In the remaining 30%, progression or metastatic disease leads to death and in this framework palliative care has an outstanding role though not well settled in all its facets. In this landscape, surgery has a supportive actor role integrated with other welfare aspects from which are not severable. The definition of surgical palliation has moved from the ancient definition of noncurative surgery to a group of practices performed not to cure but to alleviate an organ dysfunction offering the best quality of life possible in all the aspects of life (pain, dysfunctions, caregivers, psychosocial, etc.). To emphasize this aspect a more modern definition has been introduced: palliative therapy in whose context is comprised not only the care assistance but also the plans of care since the onset of illness, teaching the matter to surgeons in training and share paths. Literature is very poor regarding surgical aspects specifically dedicated and all researches (PubMed, Google Scholar, and Cochrane) with various meshing terms result in a more oncologic and psychosocial effort.
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