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Background: Hypertension (HT) is rarely reported in patients affected by Neuroblastoma (NB), and management guidelines are lacking. Clinical features and perioperativemedical treatment insuchpatientswere reviewed to1) 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 retrospectivemulticenter surveywas conducted on patients affected by NBwho 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. Conclusion: Based on this retrospective review of NB patients with HT, no definite therapeutic protocol can be recommended owing to heterogeneity of adopted treatments in different centers. A proposal of perioperative HT management in NB patients is however presented. Level of evidence: IV.

Congenital colonic stenosis is a rare pediatric condition. Since 1968, only 16 cases have been reported in the literature. To the authors’ knowledge, multiple congenital colonic stenosis has not been previously reported in the literature. We report the case of a 2-month-old male, presented at our Neonatal Intensive Care Unit with a suspicion of intestinal malrotation. Clinical examination revealed persistent abdominal distension. During the enema examination, the contrast medium appeared to fill the lumen of the colon up to three stenotic segments and could not proceed further. Intraoperatively we confirmed the presence of four types of colonic atresia, located in the ascending, transverse, and descending colon, respectively, plus appendix atresia. First surgical steps consisted in resection of proximal stenotic segment, appendix removal, proximal cecostomy, and distal colostomy on ascending colon in order to preserve colonic length. Histopathological examination confirmed the diagnosis of colonic stenosis. Final surgical step consisted in multiple colocolostomy and enteroplasty. A planned two-stage procedure, consisting of resection with colostomy for decompression as the first step and a later anastomosis, is recommended in order to allow bowel length preservation.

L'iniezione di polimeri di acido ialuronico/destranomero (Dx/HA) è un'opzione terapeutica efficace e mini-invasiva nel trattamento del reflusso vescico-ureterale.

We describe the results of a therapeutic approach with a pediatric BEP regimen (bleomycin, etoposide, cisplatin) from the Associazione Italiana Ematologia Oncologia Pediatrica (AIEOP) study on testicular germ cell tumors (TGCT).

Congenital malformations affecting the colon are rare pediatric conditions often presenting as obstruction. Colonic atresia accounts for 5-­‐10% of atresia in newborns, stenosis is even more rare.Since 1968, only 16 cases of CCS have been reported in literature (Table 1). Because of the rarity of the disease, little is known about this uncommon condition and management is still controversial. We present a case of multiple congenital colonic stenosis and review the literature with a special focus on management of CCS. To the authors' knowledge, multiple congenital colonic stenosis has not been reported previously in the literature.

Mature cystic teratoma is the most frequent benign ovarian neoplastic lesion in adolescents and is generally composed of fully differentiated tissue arising from multipotential three germinal layers. It accounts for approximately 50% of benign ovarian tumors in childhood. Rarely, a bilateral, synchronous, or metachronous presentation can be observed, supporting a conservative approach as the first surgical approach. We report a case of an ovarian mature cystic incarcerated in indirect inguinal hernia in a 15-year-old girl undergoing ovary-sparing surgery. To our knowledge this is the first case of such lesion incarcerated in an inguinal hernia reported in literature.

Visceral solitary myofibromas are uncommon in childhood. We report a case of a solitary asymptomatic visceral myofibroma of the bladder trigone occurring in a 3-month-old boy. Once malignancies were ruled out by cystoscopy, radical excision was performed in order to avoid any potential impairment of bladder dynamic. Postoperative course was uneventful and patient was discharged on day 3 after surgery. After 36 months of follow-up, the patient is toilet-trained and remains well; bladder function is normal.

SINDROME DI PALLISTER KILLIAN

Il Neuroblastoma (NB) con sintomatologia ipertensiva riveste un’esigua percentuale dei tumori neuroblastici trattati ogni anno, attualmente in letteratura esistono unicamente due studi (1,2), che riportano un’incidenza di sintomatologia ipertensiva del 10-19% dei casi. Il follow-up dei pazienti studiati risulta breve, inoltre il meccanismo fisiopatologico non è stato tuttora dimostrato, ipotizzando una combinazione tra la compressione del peduncolo vascolare renale e la secrezione di catecolamine urinarie. Per quanto riguarda il management intra-operatorio, che può risultare difficoltoso a causa di picchi iper\ipotensivi durante la manipolazione della massa, esistono unicamente case reports (3,4). Lo scopo dello studio è valutare l’approccio diagnostico e la gestione perioperatoria del bambino affetto da NB con sintomatologia ipertensiva

L’atresia intestinale tipo III B (apple peel) e la gastroschisi sono entrambe malformazioni congenite che necessitano di correzione chirurgica precoce in epoca neonatale. La loro associazione è un evento molto raro. Presentiamo il caso di un neonato a termine con l’associazione di atresia ileale apple-peel parziale e piccolo difetto della parete addominale anteriore, complicate con perforazione intestinale in utero e conseguente peritonite meconiale. Abbiamo riscontrato un’atresia parziale delle anse intestinali, coninteressamento dell’ileo terminale e risparmio del digiuno e di buona parte dell’ileo prossimale, difetto della parete addominale di piccole dimensioni e con poche anse erniate, malrotazione intestinale. L’ileo paralitico e le infezioni rappresentano le maggiori cause di morbidità e mortalità in età neonatale. Nel nostro caso, a dispetto del fenotipo “mild”, la prognosi è stata complicata dall’instaurarsi di un quadro di occlusione intestinale funzionale, determinata dalla peritonite chimica attribuibile al danno da contatto con fluido amniotico da un lato e meconio dall’altro. Intestinal atresia type III B (apple peel) and gastroschisis are both congenital malformations who require early surgical correction in neonatal age. Their association is very rare. We present the case of a full term infant with partial apple peel ileal atresia and a small defect of the anterior abdominal wall, complicated by in utero intestinal perforation and subsequent meconial peritonitis. We observed a partial atresia of small intestine, with involvement of terminal ileus savings of jejunum and a large part of the proximal ileum, small anterior abdominal wall defect with herniation of few bowel loops, intestinal malrotation. Paralytic ileus and infections are the main causes of morbidity and mortality at neonatal age. In our case, in spite of the mild phenotype, prognosis has been complicated by the onset of functional bowel obstruction, caused by chemical peritonitis resulting from contact with either amniotic fluid and meconium

Splenogonadal fusion is a rare congenital anomaly in which there is fusion between the spleen and gonad, epididymis or vas. We treated a patient with scrotal mass. A discontinuous fusion between the spleen and epididymis was found: after frozen section mass was excised sparing testis. Although rare, the splenogonadal fusion should be considered in the differential diagnosis of scrotal masses in children, and orchiectomy should be avoided. Moreover, examination of siblings showed a brother affected from accessory spleen. This finding, not reported previously to our knowledge, suggests a possible familiarity of this disorder.

Le neoplasie sono la seconda causa di morte in età pediatrica. Vengono prese in esame tutte le metodiche diagnostiche per una correta valutazione della malattia e gli attuali procedimenti terapeutici, dalla Chemioterapia alla chirurgia.

I tumori germinali maligni del testicolo rappresentano una evenienza rara. L'approccio chirurgico consiste nella orchiectomia radicale con eventuale esplorazione chirurgica dei linfonodi retroperitoneali doppo l'opportuna valutazione per imaging. vengono presentati i risultati di uno Studio Cooperativo Nazionale.

L'ipertensione intraaddominale (IAH) e la risultante sindrome da compartimento addominale (ACS), caratterizzata da incremento della pressione >20 mmHg e insufficienza d’organo o multiorgano, sono state descritte in neonati con patologie addominali chirurgiche. La gestione effettiva e preventiva dell'IAH è associata a minore morbidità. In uno studio retrospettivo abbiamo analizzato 20 neonati con distensione addominale persistente per individuare fattori predittivi di IAH ed ACS. Il Gold-Standard della misurazione dell'IAH è la misurazione pressoria intravescicale ancora non standardizzata c/o le UTIN. Per definire l'IAH abbiamo quindi utilizzato il monitoraggio della saturazione di ossigeno (SpO2) prossimale e distale rispetto all’addome e 2 segni di compromissione d’organo (oliguria, ipotensione, insufficienza respiratoria, acidosi metabolica). Abbiamo riscontrato un rischio tendenzialmente elevato di disfunzione multiorgano e decesso in neonati di età gestazionale maggiore (p=0,09) e con una causa congenita di ACS (p

Teratoma is the most common germ cell tumour in childhood; mature (MT) and immature teratomas (IT) are benign tumours, but if they recur, they can be in some cases malignant. The aim of this paper is to evaluate Italian patients with MT and IT enrolled from 1991 to 2001, in a prospective multicentric study. One hundred and eighty-three patients, observed in 15 Italian Centers of Paediatric Oncology and three Paediatric Surgical Units were enrolled. Clinical data, treatment and results were all analysed. Initial evaluation and subsequent follow up included clinical examination, tumour markers and imaging procedures. Surgical resection was recommended for all the tumours. Histology was centrally reviewed and IT was classified as grading 1-3. Chemotherapy (CT) with Vinblastine, D: -actinomycin and cyclophosphamide was indicated for extra-testicular IT grade 2 or 3. MT was diagnosed in 127 patients (93 F and 34 M, age 1-192 months, median 24): 58 patients had gonadic tumour (23 testicular, 35 ovaric), 69 extragonadic (45 sacrococcygeal, 11 mediastinic, 7 retroperitoneal, 6 in other sites). A complete resection was performed in 117 patients, a partial resection in eight patients and biopsy in one. IT was diagnosed in 56 patients (34 F, 22 M, age 1-168 months, median 7). The T grading was 1 in 14 cases, 2 in 26, 3 in 16; 28 had gonadic T (17 ovary, 11 testis), 28 extragonadic (sacrococcygeal 19, mediastinic 3, retroperitoneal 2, other sites 4). CT was administered in eight patients; 15/182 patients relapsed (1 in a metastatic site) and in 5/15 the relapse showed malignant histology. Seven MT (5.5%) relapsed (five sacrococcygeal, one retroperitoneal, one mediastinic): surgery at diagnosis had been complete in five and with residual in two; the relapse was malignant in two patients with sacrococcygeal (sc) tumours, who had a complete resection and a partial resection respectively. Eight IT (14.2%) relapsed (four ovary, three sc, one retroperitoneal). The initial surgical resection had been complete in one, with residual in six, and a biopsy had been performed in one. A malignant recurrence occurred in two patients with sc tumours (after partial resection in one and after biopsy + CT in one) and in one patient with ovarian IT after a partial resection. All the patients underwent surgical excision of the recurred mass; CT according to Protocol for Malignant GCT was administered to those who had malignant recurrence; 122/126 patients with MT and 53/56 with IT are alive without disease with a follow up of 8-144 months (median 56). Two patients with malignant relapse (one with sc MT, one with sc IT) died because of the progression of the disease. Another two died due to severe malformations (one MT, one IT) and three were lost to follow up (two MT, one IT). The overall survival (OS) at 10 years is 98% (95% CI 93.9-99.4); the event free survival (EFS) is 90.4% (95 CI 84.8-94.0). At Cox analysis no significant difference in EFS was found regarding age and site of the primary tumour, while females (P = 0.011), patients with grade 1-3 histology (P = 0.025) and patients with incomplete resection appeared at higher risk of death or relapse (P < 0.001), with a seven, three and eightfold increase in risk, respectively. Our data showed that incomplete resection and female gender are important risk factors for relapse or death, more so than IT histology. The number of patients treated with CT is not sufficient to evaluate the efficacy of CT in avoiding malignant relapse.

Gli Autori presentano i risultati di una ricerca nazionale multicentrica retrospettiva sulla Sindrome da Intestino Corto (SIC)