Abstract Gartner's duct cyst is a rare condition consequent to an unfinished disappearance of the mesonephric duct in females. In this report we present three cases of mesonephric duct remnants cysts: two of these were originated from its proximal part ,paraooforon, whereas in the third case the cyst was originated from its paraurethral distal end. These lesions are an uncommon and extremely rare manifestations in paediatric age and more in neonatal age: therefore we believe very interesting and useful to report these cases, describe their clinical data, debate the embryogenic origins, discuss diagnostic and therapeutic problems according to international literature.

BACKGROUND: In the pediatric age, mediastinal teratomas are an infrequent observation, accounting for only 7%-11% of extragonadal teratomas. Mainly located in the anterior mediastinum arising from the thymic gland, or exceptionally, from ectopic thyroid tissue, they may rarely be observed in the posterior mediastinum, sometimes in a paravertebral position, simulating a neuroblastoma. The Authors have extrapolated, from their entire experience of teratomas, 3 cases, mostly operated as emergencies; 1 of them was treated just after birth. Aim of this paper is to report the clinical and pathologic findings, to evaluate the surgical approach and the long-term biological behaviour in these cases, in the light of survival and current insights reported in the literature. MATERIALS AND METHODS: The Authors reviewed the most significant clinical, laboratory, radiologic and pathologic findings, surgical procedures, and early and long-term results in 3 children, 2 males and 1 female, suffering from extragonadal teratomas, located in the mediastinum, treated immediately after birth. In 1 of them the lesion was prenatally diagnosed by US scanning between the 2nd and 3rd trimester of pregnancy. All the infants were born by scheduled caesarean section in a tertiary care hospital and were then immediately referred to the N.I.C.U. because of a mostly acute clinical presentation. The 3 patients were referred to the surgical unit at different ages, namely 2 days, 10 years and 12.5 years, respectively. The initial clinical presentation was consistent with the site of the mass and/or its side-effects. The first patient, a female newborn, presented a worsening condition of respiratory distress, immediately after birth, that required mechanical ventilation and stabilization of the vital signs. Likewise, the presentation of case No. 2 was acute with dyspnea associated with an upper airways infection. Instead, the initial symptoms in case No. 3 were subacute and non-specific, characterized by worsening pain at the right shoulder extending to the neck and homolateral arm. The patients underwent laboratory and radiologic investigations that confirmed the clinical diagnosis of teratoma on the basis of elevated AFP values in 2 cases only (Case No. 1 and No. 2), while calcifications were lacking at imaging in all 3 patients. Emergency surgical management was required and, in accordance with recommended practice, the procedure was complete exeresis. RESULTS: All the patients underwent close long-term clinical, laboratory and imaging surveillance at shorter intervals during the first 5 years after the exeresis and annually thereafter. At the present time they are alive, disease-free and have not suffered any recurrence and/or distant metastases, with a follow-up of 7, 30 and 3 years respectively. CONCLUSIONS: Some extragonadal teratomas of childhood may rarely arise in the mediastinum. Being congenital tumors, prenatal diagnosis by US scan is extremely important in order to organize proper perinatal care in appropriate facilities where it is possible to define the diagnosis, and equipped with appropriate tools to carry out emergency surgery at minimal risk and to prevent severe complications after birth. An emergency procedure is frequently dictated both by complications related to the mass effect, and by the need to define the histology of the whole mass rather than just small biopsy specimens. Some teratomas can hide more or less extensive islands of immaturity or signs of malignant transformation that are clinically evident. It should be noted that calcifications and high levels of AFP and/or beta-HCG, usually pathognomonic elements for diagnosis, may not always be evident during the diagnostic work-up in mediastinal lesions. The prognosis is generally benign, although the AIEOP 2004 guidelines pointed out that high levels of circulating markers, including AFP, in children affected by mature or immature teratomas would indicate the

In a female child presenting with vaginal bleeding or a mass protruding through the vagina, it is mandatory to perform an endoscopic and laparoscopic examination and biopsy to establish the correct diagnosis and precise extent of the lesion. An endodermal sinus tumor (EST) of the vagina is a rare, malignant germ cell tumor that occurs primarily in infants. Clearly, the vagina is a critical site for treatment, and the surgeon should seek to preserve the reproductive and sexual function, if possible. We report our experience of a case of EST of the vagina in a 9-month-old female with a history of metrorrhagia. We employed minimally invasive procedures for diagnosis and then, after the administration of platinum- based chemotherapy (PEB regimen), removed a residual lesion during follow-up. These procedures resulted in tumor regression. At follow-up, after two years, the patient is alive and disease-free. KEY WORDS: Pediatric metrorrhagia, Minimally invasive surgery, Vaginal endodermal sinus tumor.

OBJECTIVES: Little is reported in literature regarding correct management of benign lesions of the kidney. The aim of our study is to present a series of total and partial nephrectomies performed in the last 5 years and to discuss indications. MATERIALS AND METHODS: Patients with benign lesions who underwent nephrectomy and partial nephrectomy at our institution in the period 2003-2008 were retrospectively included in the study. Notes were carefully reviewed and demographic data, symptoms onset, preoperative diagnosis, investigations, medical and/or surgical treatment, postoperative complications and definitive histological reports were collected. RESULTS: Forty procedures were performed. Twelve patients were preoperatively diagnosed of having multicystic dysplastic kidney (MCDK), which was confirmed in 10, whereas the remaining 28 patients of having severe dysplasia or hypodysplasia. Thirty-four patients underwent total nephrectomy, six underwent partial nephrectomy. Histopathological analysis confirmed segmental or complete abnormalities of the involved kidney in all cases. No malignancies were detected. DISCUSSION: Our study confirmed the extremely low malignancy rate of MCDK and hypodysplastic kidneys. The 20% mismatch of pre- and post-operative diagnosis suggests a common aetiology and shared therapeutic strategies for MCDK and hypodysplasia. At present, there is no consensus regarding correct indications for nephrectomy in paediatric age. As nephrectomy seems not to provide any advantage over preservation, but surgical and anesthesiological risks, we should be prudent in preserving every asymptomatic poorly or non-functioning kidneys maintaining a strict follow-up. Randomised controlled studies on larger multicentric series are strongly warranted to define this topic.

OBJECTIVE: VUR in patients with a duplex system (DS) is often treated by open surgery. The aim of this study was to evaluate the efficacy of subureteric polydimethylsiloxane (Macroplastique(®)) injection (SMING) in the management of VUR in duplex and single (SS) renal systems. PATIENTS AND METHODS: Fifteen children (24 refluxing renal units) with VUR in DS underwent SMING. VUR was more frequent in the lower moiety. VUR was graded moderate/severe in 88% of renal units. There was a history of urinary tract infections in 40% of cases. The outcome for DS patients was compared with 44 children (60 refluxing renal units) with moderate/severe VUR in SS. RESULTS: The VUR resolution/improvement rate was 88% in DS and 95% in SS patients. Ureteric reimplantation was required because of recurrent VUR in 13% and 7% of DS and SS groups, respectively. Transient ureteral obstruction was observed in 1/15 and 5/44 patients. Two required double-J ureteric stenting for 3 months. CONCLUSION: SMING seems an effective treatment for VUR in both DS and SS patients, even in severe cases. The complication rate does not significantly differ between the two groups. Copyright © 2010 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.

This report is aimed at describing our preliminary experience with the preperitoneoscopic approach to the bladder neck for rectus fascial sling suspension in neurogenic bladder. MATERIALS AND METHODS: A 13-year-old boy with spina bifida was admitted to our institution to treat bladder incontinence. A bladder neck suspension and bladder augmentation was planned. The preperitoneal space was progressively gained by blunt dissection under direct vision (laparoscopy with a single left subcostal trocar), and a total of three preperitoneal ports were inserted. Dissection of the bladder proved to be relatively easy, and the retropubic space as well as the deep pelvis were safely exposed. Owing to the excellent endoscopic visualization, the bladder was dissected downward from the rectum. Once complete mobilization of the bladder neck was achieved, an umbilical tape was passed around the proximal urethra to create an adequate passage for the fascial sling. RESULTS: The preperitoneoscopic procedure lasted 2 hours. No complications occurred. A good daytime dry period was achieved at the 4-month follow-up. DISCUSSION: Direct visualization of the bladder neck and minimal dissection to the deep pelvis are the key points of this approach. Provided certain technical details are considered, the minimally invasive preperitoneal access is a valuable alternative to the open approach for the rectus fascial sling procedure, particularly when dealing with male patients. Technical advances will, presumably, lead to a complete minimally invasive treatment of urinary incontinence in male patients with spina bifida.

BACKGROUND: Extragonadal teratomas are rare tumors in neonates and infants and can sometimes show unusual, distinctive feature such as an unusual location, a clinical sometimes acute, presentation and a "fetiform" histotype of the lesion. We have extrapolated, from our entire experience of teratomas, 4 unusual cases, mostly operated as emergencies; 2 of them were treated just after birth. Aim of this paper is to report the clinical and pathological findings, to evaluate the surgical approach and the long-term biological behaviour in these cases, in the light of survival and current insights reported in the literature. MATERIAL AND METHODS: The Authors reviewed the most significant (Tables I and II) clinical, laboratory, radiologic, and pathologic findings, surgical procedures, early and long-term results in 4 children, 1 male and 3 females (M/F ratio: 1/3), suffering from extragonadal teratomas, located in the temporo-zygomatic region of the head (Case n. 1, Fig. 1), retroperitoneal space (Case n. 2, Fig. 2) ,liver (Case n. 3, Figg. 3-5), kidney (Case n. 4, Fig. 6, 7), respectively. Of the 4 patients, 2 were treated neonatally (1 T. of the head, 1 retroperitoneal T.) A prenatal diagnosis had already been made in 2 of the 4 patients, between the 2nd and 3rd trimester of pregnancy, All the infants were born by scheduled caesarean section in a tertiary care hospital and were the immediately referred to thew N.I.C.Us. Because of a mostly acute clinical presentation, the 4 patients were then referred to the surgical unit at different ages: 7 days, 28 days, 7 months, and 4 years respectively. The initial clinical presentation (Table II) was consistent with the site of the mass and/or its side effects. The 2 newborns (Case 1 and 2) both with a prenatally diagnosed mass located at the temporozygomatic region and in the abdominal cavite respectively, already displayed, at birth a mass with a tendency to further growth. The symptoms and signs described to the primary care physician by the parents of the 2 patients suffering from intra-abdominal tumours (Cases n. 3, 4) were: swelling of the epigastrium and left hypochondrium due to a progressively growing hard mass, without impairment of the general, conditions in case n.3 (teratoma of the liver),while recurrent abdominal pain lasting for the 5 months was described in case n.4(retroperitoneal teratoma), followed by the development of an evident hard mass occupying the entire abdomen. In this case the symptoms suddenly worsened, with acute pain extending to the entire abdomen, high fever (>39° C), polypnea, anemia, deterioration of the general conditions and a rapid further enlargement of the mass. Antibiotic therapy was ansuccessful. The young child underwent a radiologic investigation (Fig. 6) that showed a large calcified mass in the left retroperitoneal space, associated with pleural effusion, In all the patients except for the Case n. 3, emergency surgical management was required and, in accordance with recommended practice, the procedure was complete exeresis. RESULTS (TABLE I): All the 4 patients had an uneventful postoperative course. Clinical surveillance and tests of AFP and other markers were scheduled every 6 months for the first years and annually thereafter. At the current date they are alive, disease-free and have not suffered any recurrence with a follow-up as reported in Table I, of 7 years in case n. 2; 23 years in case n. 1; 42 years in case n. 3 and 36 years in case n. 4. CONCLUSIONS: Some extragonadal teratomas of childhood of may rarely arise in the solid organs (liver, kidney), in the retroperitoneal space or the cranio-facial region, and also show unique histotype childhood characteristics ("fetiform") which distinguish them from more common cases. Being congenital tumours, prenatal diagnosis by US scan is extremely important in order to organize proper perinatal care in appropriate faciliti

Solid pseudopapillary tumors (SPTs) are classified among the rare malignant tumors but they usually appear as slowly growing and low-grade malignancy and also occur in children and adolescents. They are mostly detected incidentally, but may occasionally present with sudden pain or symptoms of compression of adjacent structures. We report the case of a 15-year-old girl having an SPT of the exocrine pancreas presenting with an upper abdominal mass following a slight trauma at the dancing school. Imaging investigation showed a fairly heterogeneous density consistent with an SPT arising from the body and the tail of the pancreas, adherent to the splenic vein. The patient underwent complete enucleation. The aim of this paper is to report and discuss this recent experience and also review the literature focusing some questions still debated on this intriguing topic.

BACKGROUND: Sacrococcygeal teratomas are the most common and best known extragonadal teratomas in neonates and infants, but they sometimes present unique, distinctive features unlike those commonly described, that can be considered exceptional and noteworthy. MATERIAL AND METHODS: The Authors reviewed the most significant (Table I, II) clinical, laboratory, radiological and pathologic findings, surgical procedure, and early and long-term results in 5 children, 2 males and 3 females, suffering from sacrococcygeal teratomas. Four of 5 patients were observed and managed in the neonatal age. A prenatal diagnosis had already been made in 2 of them between the 2nd and 3rd trimester of pregnancy. Two patients were also suffering from the Currarino syndrome associated with Hirschsprung's disease and other, multiple malformations and a cloacal anomaly with anal imperforation, respectively. This last developmental anomaly had been prenatally suspected at US scanning, which had demonstrated a severe sacral anomaly and a large abdominal mass with perineal extension and dilated bowel loops. All the infants were born by scheduled caesarean section in a tertiary care hospital and were then referred to the N.I.C.Us. because of a mostly acute clinical presentation, except for case N°4, who was referred at the age of 3.3 years. Laboratory and radiologic investigations confirmed the clinical diagnosis of teratoma on the basis of elevated AFP values and imaging findings. All patients underwent emergency surgical management, in accordance with recommended practice, consisting of complete exeresis of the tumor, including coccygectomy, in 3 of the 5 children. RESULTS: Of the 5 patients, the female newborn affected by the Currarino syndrome, associated with persistence of the common cloacal canal and anal imperforation, died two days after surgery, of cardiovascular and respiratory complications. All the other patients had an uneventful postoperative course. Two years after the first exeresis at birth, relapse was observed in case N° 3, with a malignant component, YST, anticipated by elevated AFP values but negative physical signs and a benign cystic imaging pattern. After adjuvant chemotherapy she underwent a second complete exeresis. So far, 3 years after the second surgery and 5 years after the diagnosis and first treatment, no local recurrence or distant metastases have occurred. The other 3 patients are also all alive, disease-free and with no signs of relapse or distant metastasis, after a follow-up ranging from 2 years to 28 years. None of the 3 cases treated for sacrococcygeal teratoma, operated in 1985, 1984 and 2006, have ever developed functional sequelae such as ano-rectal and/or bladder dysfunction or hypotonia of the lower limbs. CONCLUSIONS: From this review of our selected cases and in the light of literature reports, we can draw the following conclusions. Some sacrococcygeal teratomas may have unique characteristics distinguishing them from more common cases, especially those included in the Currarino Syndrome, with or without an association with other malformations such as Hirschsprung's Disease and Cloacal anomalies. Being congenital tumors, prenatal diagnosis by US scan is extremely important in order to decide either for an anticipated delivery or to perform, in critical fetuses, prenatal treatment within highly specialized facilities, or to organize proper perinatal care, always in appropriate facilities where it is possible to define the diagnosis and carry out emergency surgery. An emergency procedure is frequently dictated both by complications related to the mass effect, and by the need to define the histology of the whole mass rather than just small biopsy specimens. Some sacrococcygeal teratomas can hide more or less extensive islands of immaturity or signs of malignant transformation that are clinically evident. The prognosis is generally benign, a