Fibrous hamartoma of Infancy: An Italian multi-istitutional experience.

Fibrous hamartoma of infancy: an Italian multi-institutional experience. Carretto E, Dall'Igna P, Alaggio R, Siracusa F, Granata C, Ferrari A, Cecchetto G. Source Division of Paediatric Surgery, Department of Paediatrics, University of Padua, Padua, Italy. Abstract BACKGROUND: Fibrous hamartoma (FH) of infancy is a benign mesenchymal tumor, occurring as a superficial mass. Complete excision is curative. OBJECTIVE AND METHODS: The clinical features and treatment results of 18 children with FH are described. RESULTS: Local excision was the most common procedure. Surgery was radical in 10 patients, with microscopic residual disease in 6; all of them are alive with no evidence of disease 2 to 49 months after diagnosis. One patient, treated with a local reexcision for macroscopic residual disease (and chemotherapy for a synchronous desmoid fibromatosis) is well 83 months after diagnosis; the last patient, with a lesion of the labia majora, only underwent biopsy and is doing well, awaiting plastic surgery. LIMITATIONS: The results did not reach statistical significance due to difficulties in collecting cases. CONCLUSIONS: FH should be treated by complete excision; in our experience a nonradical excision was also able to achieve the cure. An aggressive approach should be avoided, as the overall prognosis is excellent. PMID: 16635660 [PubMed - indexed for MEDLINE

Salvage rates and prognostic factors after relapse in children and adolescents with initially localised synovial sarcoma

Background: Previous studies have reported a poor outcome for synovial sarcoma patients whose tumours relapse. Methods: This study analysed 44 relapsing cases in a series of 118 consecutive patients<21 yr of age with non-metastatic synovial sarcoma prospectively enrolled in Italian paediatric protocols between 1979 and 2006. In an effort to identify a possible risk-adapted stratification enabling a better planning of second-line treatment, the relapsing patients' outcome was analysed vis-à-vis their clinical picture at onset, first-line treatments, clinical findings at the time of first relapse and second-line treatment modalities. Results: The first event was a local recurrence in only 15 cases, and metastatic in 29 (associated with local relapse too in 7 cases). The time to relapse ranged from 4 to 108 months (median 20 months). Overall survival was 29.7% and 21.0% five and ten years after relapsing, respectively. The variables influencing survival were the timing and type of relapse (combined) and the chances of a secondary remission, which correlated strongly with the feasibility of complete surgery. Conclusions: Our study confirmed a largely unsatisfactory prognosis after recurrences in children and adolescents with synovial sarcoma: the chances of survival can be estimated on the basis of several variables for the purposes of planning risk-adapted salvage protocols. An aggressive surgical approach should be recommended. New effective systemic agents are warranted, and experimental therapies can be offered to patients with little chance of salvage. © 2012 Elsevier Ltd. All rights reserved

Obstructive jaundice secondary to pancreatic head adenocarcinoma in a young teenage boy: a case report

<p>Abstract</p> <p>Introduction</p> <p>Pancreatic adenocarcinoma is extremely rare in childhood. We report a case of metastatic pancreatic adenocarcinoma in a 13-year-old boy, revealed by jaundice.</p> <p>Case presentation</p> <p>A 13-year-old Moroccan boy was admitted with obstructive jaundice to the children's Hospital of Rabat, Department of Pediatric Oncology. Laboratory study results showed a high level of total and conjugated bilirubin. Computerized tomography of the abdomen showed a dilatation of the intra-hepatic and extra-hepatic bile ducts with a tissular heterogeneous tumor of the head of the pancreas and five hepatic lesions. Biopsy of a liver lesion was performed, and a histopathological examination of the sample confirmed the diagnosis of metastatic ductal adenocarcinoma of the pancreas. Our patient underwent a palliative biliary derivation. After that, chemotherapy was administered (5-fluorouracil and epirubicin), however no significant response to treatment was noted and our patient died six months after diagnosis.</p> <p>Conclusion</p> <p>Malignant pancreatic tumors, especially ductal carcinomas, are exceedingly rare in the pediatric age group and their clinical features and treatment usually go unappreciated by most pediatric oncologists and surgeons.</p

Percutaneous Trans-Thoracic Procedures in Children With Tumors of Thoracic Wall, Mediastinum and Lung. The Experience of a Single Institution

Background While percutaneous trans-thoracic procedures (PTTP) are commonly performed in adults with tumors of thoracic wall, mediastinum and lung, the experience is limited in children, in whom however less invasive methods should be the choice for the diagnosis or the identification of small pulmonary nodules that need to be removed, sparing lung tissue. The results of the PTTP performed by the interventional radiologists in our Pediatric Surgery Department are analyzed. Methods CT-guided biopsies, utilizing a 64-slice CTscanner, with low-radiation dose, were performed applying the coaxial technique with 16-18G needles with a single tissue path. For localization of lung nodules before surgery, two 20G-hook wires were positioned beyond the nodule. CT images after each manipulation of the needles were obtained. US-guided biopsies were performed either with or without coaxial technique through a needle bracket. Younger patients required sedation. All patients underwent a chest radiogram two hours after the procedure and remained under observation for 24 hours. Results From January 2015 to March 2019, 23 procedures were performed in 22 patients (Age:16M- 19Y): 6 patients underwent CT-guided biopsy (4 lung nodules, 2 mediastinal mass); 3 underwent 4 CT-guided hook-wire localization of pulmonary nodules, just before surgery; 13 underwent US-guided biopsy (posterior mediastinum 2; anterior mediastinum 5, thoracic/intrathoracic mass 5). Adequate core biopsies were obtained in all patients, except three, who underwent thoracoscopy/thoracotomy. The hook-wires were successfully positioned in all cases, as confirmed by histology. After the procedure, two patients presented perilesional hemorrhage and one pneumothorax, but they did not required treatment. Conclusion PTTP were successful in most patients, without significant complications. These techniques should be encouraged to avoid diagnostic aggressive surgical approaches in children with cancer. For all cases a multidisciplinary team is essential to discuss the indications and planning the procedures

Long-term prognosis of patients with pediatric pheochromocytoma

A third of patients with paraganglial tumors, pheochromocytoma, and paraganglioma, carry germline mutations in one of the susceptibility genes, RET, VHL, NF1, SDHAF2, SDHA, SDHB, SDHC, SDHD, TMEM127, and MAX. Despite increasing importance, data for long-term prognosis are scarce in pediatric presentations. The European-American-Pheochromocytoma-Paraganglioma-Registry, with a total of 2001 patients with confirmed paraganglial tumors, was the platform for this study. Molecular genetic and phenotypic classification and assessment of gene-specific long-term outcome with second and/or malignant paraganglial tumors and life expectancy were performed in patients diagnosed at <18 years. Of 177 eligible registrants, 80% had mutations, 49% VHL, 15% SDHB, 10% SDHD, 4%NF1, and one patient each in RET, SDHA, and SDHC. A second primary paraganglial tumor developed in 38% with increasing frequency over time, reaching 50% at 30 years after initial diagnosis. Their prevalence was associated with hereditary disease (P=0.001), particularly in VHL and SDHD mutation carriers (VHL vs others, P=0.001 and SDHD vs others, P=0.042). A total of 16 (9%) patients with hereditary disease had malignant tumors, ten at initial diagnosis and another six during follow-up. The highest prevalence was associated with SDHB (SDHB vs others, P<0.001). Eight patients died (5%), all of whom had germline mutations. Mean life expectancy was 62 years with hereditary disease. Hereditary disease and the underlying germline mutation define the long-term prognosis of pediatric patients in terms of prevalence and time of second primaries, malignant transformation, and survival. Based on these data, gene-adjusted, specific surveillance guidelines can help effective preventive medicine.publishersversionPeer reviewe