Wilms tumor is the most common primary renal tumor in childhood. Children with Wilms tumor typically present with an asymptomatic abdominal mass, usually detected on a routine medical checkup or discovered coincidentally by parents. The initial differential diagnosis is with extrarenal abdominal masses; once a tumor of renal origin is established, distinguishing between Wilms tumor and other primary renal neoplasms such as congenital mesoblastic nephroma, clear cell sarcoma, malignant rhabdoid tumor and renal cell carcinoma may not be easy. However, in many cases imaging findings in conjunction with the patient's clinical and epidemiological data, allow the diagnosis of Wilms tumor. Wilms tumor care offers one of the most striking examples of success of pediatric oncology. Over the last decades the European SIOP studies have been the key to developing standardized diagnostic procedures, improved risk stratification, and adjusted treatment recommendations for children with Wilms tumor and this has resulted rate of overall survival is currently greater than 90%. As in previous SIOP trials and studies, the new protocol for the diagnosis and treatment of childhood renal tumors, the UMBRELLA SIOP–RTSG 2016, mandates preoperative chemotherapy without preceding mandatory histological assessment. Therefore, imaging studies are essential to obtain a presumptive diagnosis of WT, to provide disease staging information and to measure the tumor volume after neoadjuvant chemotherapy for the purposes of postoperative treatment stratification. This review describes role of imaging in the management of children with Wilms tumor, according to the current recommendations of the UMBRELLA protocol.
In Italia il linfoma di Hodgkin (LH) costituisce il 6% dei tumori ed il 43% di tutti i linfomi nella fascia di età compresa tra 0-14 anni ed è più frequente nei maschi. I pazienti con LH nella maggior parte dei casi presentano al momento della diagnosi linfoadenopatie prevalentemente latero-cervicali e/o in sede toracica; invece, sono infrequenti una presentazione addominale e/o sintomi sistemici quali febbre, calo ponderale e sudorazioni notturne. Raramente il LH si manifesta con il quadro clinico di sindrome nefrosica (SN). In età pediatrica il tasso di incidenza annuale della SN è di 2-5 casi su 100.000 bambini e nei piccoli pazienti con meno di 8 anni è più frequente nei ma-schi; è solitamente idiopatica, ma esistono forme congenite o secondarie a glomerulonefriti e pa-tologie sistemiche. La SN si associa al LH solo nell’1% dei casi. La nefropatia a lesioni minime è la patologia renale più comunemente riscontrata nei bambini con LH. La lesione glomerulare pro-voca l’incremento della permeabilità della parete dei capillari glomerulari e conseguenzialmente la SN si manifesta con proteinuria, ipoalbuminemia, diminuzione della pressione oncotica pla-smatica e edema. Nel presente articolo descriviamo il caso raro di una bambina di 7 anni, giunta alla nostra osservazione con SN, che risultava resistente al trattamento con corticosteroidi. Dopo circa due mesi veniva diagnosticato un LH in addome.
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