Approccio chirurgico iniziale al Rabdomiosarcoma (RMS): dati degli Studi del Gruppo Cooperativo Italiano

CHIRURGIA LAPAROSCOPICA DEL VARICOCELE: STATO DELL'ARTE

Laparoscopic management of an isolated left fallopian tube torsion due to twisted Morgagni hydatid in a pre-menarcheal girl.

Isolated torsion of fallopian tube, meanwhile uncommon, should be considered in diagnosis of pelvic and lower abdominal pain. US investigation is an useful diagnostic tool. A prompt diagnosis could avoid salpingectomy and preserve fertility. Laparoscopy, as the first approach, should be preferred. We report a case of isolated tubal torsion occurring in a premenarcheal girl successfully managed by laparoscopy.

Outcome dei pazienti con Sindrome da Intestino Corto. Valutazione da una casistica nazionale multicentrica

Salvage treatment for children with relapsed/refractory germ cell tumors: The Associazione Italiana Ematologia Oncologia Pediatrica (AIEOP) experience.

Background Malignant germ cell tumors (GCTs) are a heterogeneous group of rare neoplasms in children. Optimal outcome is achieved with multimodal therapies for patients with both localized and advanced disease, especially after the introduction of platinum-based chemotherapy regimens. In this respect, data on salvage treatment for children with relapsed or platinum-refractory disease are still limited. Methods Retrospective analysis of data regarding patients affected by malignant GCTs with platinum-refractory or relapsed disease after first-line treatment according to AIEOP TCGM 2004 protocol was conducted. Results Twenty-one patients, 15 females and 6 males, were considered for the analys…

PEDIATRIC TESTICULAR GERM CELL TUMORS: the ASSOCIAZIONE ITALIANA ONCOLOGIA PEDIATRICA (AIEOP) study

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).

Fetal unilateral urinoma in upper urinary tract obstruction: our experience and review of literature. Does it protect renal function?

Predictive Factors of Abdominal Compartment Syndrome in Neonatal Age

In the pediatric population, abdominal compartment syndrome (ACS) is a known complication of abdominal wall defect repair. However, there are only few reports on ACS in newborns and only a proposal of critical intra-abdominal pressure value (IAP) in term newborns, absent in preterm newborns. Although the prevalent clinical sign is tense abdominal distension, it may be difficult to distinguish ACS from pathologies that will not require decompression. The purpose of this study was to identify predictors for ACS and therefore morbidity or mortality indicators. We reviewed newborns presenting with tense abdominal distension and end organ failure. Anamnestic, clinical, laboratory, and instrument…

Malignant germ cell tumors in childhood: results of the first Italian cooperative study "TCG 91".

Background and Aims About 20% of patients with germ cell tumor (GCT) are still resistant to therapy. To investigate which features are present in resistant patients, a multicenter study on GCT in children was undertaken to correlate clinical and laboratory parameters with the outcome. Methods Patients aged less than 16 years, with histologically proven extracranial GCT were included. Results Ninety-five patients (median age 33 months, 45 males) were eligible. The site of the primary tumor was gonadal in 59, extragonadal in 36. The stage was I in 39; II in 5; IIIa (microscopic residue) in 7; IIIb (macroscopic residue) in 16; IIIc (unresectable) in 13; IV in 15. The treatment was surgery alon…

SINDROME DI PALLISTER KILLIAN. DESCRIZIONE DI UN CASO E CORRELAZIONE CLINICO GENETICA

SINDROME DI PALLISTER KILLIAN

Meconial peritonitis in a rare association of partial ileal apple-peel atresia with small abdominal wall defect.

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 morbidi…

Testicular tumors in childhood: a national report

The authors report preliminary data of an Italian multicentric, retrospective study of primary testicular tumors (PTT) in childhood involving 20 Italian departments of pediatric surgery. Forty cases of PTT were observed during a 10-year period (1977–1987), with yolk-sac tumors being the most frequent (45%). Most testicular tumors were first perceived as a unilateral testicular mass. Scrotal ultrasonography represents the main diagnostic investigation, together with serum marker levels.

Malignant ovarian germ cell tumors in pediatric patients: The AIEOP (Associazione Italiana Ematologia Oncologia Pediatrica) study.

Objective Malignant ovarian germ cell tumors (MOGCT) carry an excellent prognosis, and the treatment aims to achieve results with the least possible treatment-related morbidity. The aim of this study was to assess the outcomes of pediatric patients with MOGCT. Methods Patients were treated according to their stage: surgery and surveillance for stage I; a modified bleomycin–etoposide–cisplatin (BEP) regimen for stages II (three cycles), III, and IV (three cycles) with surgery on residual disease. Results Seventy-seven patients were enrolled (median age 11.8 years), 26 with dysgerminoma (Dysg), 13 with immature teratoma and elevated serum alpha-fetoprotein levels (IT + AFP), and 38 with nondy…

Perioperative management of hypertensive neuroblastoma: A study from the Italian Group of Pediatric Surgical Oncologists (GICOP)

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 manageme…

UNESPECTED COMPLICATION OF TIP URETHROPLASTY: THE DIVERTICULUM OF NEO-URETHRA

MONITORAGGIO ECOGRAFICO NEL TRATTAMENTO ENDOSCOPICO PER REFLUSSO VESCICO-URETERALE: QUANDO LA CISTOURETROGRAFIA PUO' ESSERE EVITATA

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

STAGE-I GOMADAL GERMS CELL TUMORS (GGCT):OUTCOME AFTER THE SURVEILLANCE STRATEGY AS APPLIED IN THE ASSOCIAZIONE ITALIANA EMATOLOGIA ONCOLOGIA PEDIATRICA (AIEOP) 2004 PROTOCOL

Outcome dei pazienti con sindrome da intestino corto. valutazione da una casistica nazionale multicentrica

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

Approccio laparoscopico al dolore pelvico in età puberale

GESTIONE PERIOPERATORIA DEI BAMBINI AFFETTI DA NEUROBLASTOMA E IPERTENSIONE TUMORE-DIPENDENTE: STUDIO DEL GRUPPO ITALIANO DI CHIRURGIA ONCOLOGICA PEDIATRICA (GICOP).

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…

Surgery in Malignant Germ Cell Tumours of Chilhood. Results of the Second Italian Cooperative Study

La nutrizione parenterale e l’intestino residuo: quale relazione nel decorso dei pazienti con sindrome da intestino corto? Valutazione da una casistica nazionale multicentrica.

Reverse Rotation of the Kidney associated with vascular anomaly producing ipsilateral ureteropelvic junction obstrution

Multiple Congenital Colonic Stenosis: Case Report and Review of the Literature

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.

Il dosaggio del lattato in neonati con distensione addominale come fattore prognostico di sindrome da compartimento addominale

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…

Solitary Myofibroma of the Bladder Trigone in a 3-Month-Old Patient: First Case Report

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.

Teratoma with a malignant somatic component in pediatric patients: The Associazione Italiana Ematologia Oncologia Pediatrica (AIEOP) experience

Background Teratoma with a malignant somatic component (TMSC) is rare but described in adults, whereas information on pediatric presentation is sparse. Procedure The Associazione Italiana Ematologia Oncologia Pediatrica identified 14 cases of TMSC. Clinical files and pathology specimens were reviewed. Results The series (9 female, 5 male) showed the following disease: testis (2), sacrococcygeal (3), ovary (3), retroperitoneum (3), mediastinum (2), and foot soft tissue (1). Distribution of the somatic component was: carcinoma (4), pancreatic neuroendocrine tumor (1), neuroblastoma (3), rhabdomyosarcoma (3), rhabdomyosarcoma plus liposarcoma, chondrosarcoma, neurogenic sarcoma (1), chondrosar…

Fibrosarcoma in pediatric patients: Results of the Italian Cooperative Group Studies (1979-1995)

Background and Objectives Fibrosarcoma is a rare soft tissue sarcoma (STS) that has two peaks of incidence in pediatric patients: one in infants and young childern (infantile fibrosarcoma), another in older children (“adult type” fibrosarcoma). The purpose of this study was to describe the clinical features and the treatment results in patients affected by fibrosarcoma enrolled in two consecutive studies run by the STS-Italian Cooperative Group (ICG) between 1979 and 1995. Patients and Methods Twenty-five evaluable patients were grouped according the intergroup rhabdomyosarcoma staging (IRS) system: 12 Gr I, 5 Gr II, 8 Gr III. The cut-off point between the two forms was considered the age o…

Peritonite meconiale in una rara associazione di atresia ileale apple-peel con difetto di parete addominale

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,…

Congenital hepatic mesenchymal hamartoma associated with mesenchymal stem villous hyperplasia of the placenta: case report.

A newborn with an unusual association of hepatic mesenchymal hamartoma and mesenchymal stem villous hyperplasia of the placenta is presented. At birth, the large hepatic mass caused severe respiratory distress necessitating early surgical intervention. This report on the association of hepatic mesenchymal hamartoma and mesenchymal stem villous hyperplasia of the placenta strongly suggests a common pathogenetic origin of the 2 lesions.

Fibrous hamartoma of infancy: an Italian multi-institutional 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 4…

Rationale and results of the international society of pediatric oncology (SIOP) Italian pilot study on childhood hepatoma: Surgical resectionD'Emblée or after primary chemotherapy?

Il bambino neoplastico

Trattamento laparoscopico del varicocele di tipo misto (di 3° tipo sec.coolsaet)

SPLENOGONADAL FUSION: A Genetic disorder? Report of a Case and Review of the Literature

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.

MATURE AND IMMATURE TERATOMA: RESULTS OF THE FIRST PEDIATRIC ITALIAN STUDY ITALIAN

Abstract 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 w…

Unexpected complication of TIP urethroplasty: the diverticulum of neo-urethra

An Uncommon Case of Adolescent Ovarian Teratoma Incarcerated in Inguinal Hernia

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. Mature cystic teratoma …

LA NUTRIZIONE PARENTERALE E L’INTESTINO RESIDUO: QUALE RELAZIONE NEL DECORSO DI PAZIENTI CON SINDROME DA INTESTINO CORTO? VALUTAZIONE DA UNA CASISTICA NAZIONALE MULTICENTRICA

Splenogonadal Fusion: A Genetic Disorder?—Report of a Case and Review of the Literature

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. Keywords: Splenogonadal fusion, Testis, Accessory spleen

Reverse rotation of the right kidney associated with vascular anomaly producing ipsilateral ureteropelvic junction obstruction.

Fattori di rischio chirurgico nella chirurgia primaria del Neuroblastoma localizzato. Dati dal primo Studio Europeo (LNESG 1)

LA CHIRURGIA NEI PAZIENTI AFFETTI DA TUMORI A CELLULE GERMINALI MALIGNI (tgcm) TESTICOLARI: COMPLIANCE ALLE LINEE GUIDA CHIRURGICHE E RISULTATI DELLO STUDIO COOPERATIVO ITALIANO

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.