Mixed epithelial and stromal renal tumour in a 12-year-old boy.

Mixed epithelial and stromal tumour of the kidney (MESTK) is a rare kidney neoplasm that occurs almost exclusively in perimenopausal women. Long-term oestrogen replacement appears to play a major role in its pathogenesis. Around 70 cases have been described in the international literature, none of which involve male children.

Thoracoscopy and solid tumors in children: a multicenter study.

Purpose: The aim of this study was to evaluate the efficacy and safety of thoracoscopy in the treatment of solid thoracic tumors in children.

Methods: A retrospective, multicenter review of 139 thoracoscopies was performed. The procedures were either resection of pulmonary lesions or biopsy.

[Polyorchidism in child (a case report and review of literature)].

Polyorchidism is an urogenital curiosity defined by the presence of more than two testes confirmed by histology. This anomaly is extremely rare and only a hundred world cases were described in the literature. Although it can remain asymptomatic, polyorchidism is often associated to processus vaginalis anomalies in childhood (hernia, hydrocele) and undescended testis.

[Pseudo-"isolated" intestinal perforation in a very low birth weight infant: exceptional presentation of Hirschsprung's disease].

Intestinal perforations in preterm newborn are characterized with high morbidity and mortality rates. They often are associated with necrotizing enterocolitis and seldom correspond to idiopathic spontaneous intestinal perforation. Perforations upstream of an intestinal organic obstruction (atresia), or of a functionnal obstruction (meconium-ileus, Hirschsprung disease) have been considered to be rare in preterm newborns.

Very early prophylactic thyroid surgery for infants with a mutation of the RET proto-oncogene at codon 634: evaluation of the implementation of international guidelines for MEN type 2 in a single centre.

Objective: Genetic diagnosis available since 1993 established germinal mutations of the RET proto-oncogene at codon 634 as the main cause of inherited medullary thyroid carcinoma (MTC). International guidelines established in 1999 recommend that children with such mutations undergo a total thyroidectomy before age 5, with unspecified cervical neck dissection. Since 1993, only 41 of 275 thyroidectomies reported in RET 634 children were performed before age 5 (15%).

Perforated tubular duplication of the transverse colon: a rare cause of meconium peritonitis with prenatal diagnosis.

The transverse colon is an exceptional location of intestinal duplication. Perforated duplications are rarely described in neonates. Meconium peritonitis (MP) can originate from prenatal perforated intestinal duplication.

Successful use of recombinant factor VIIa for severe surgical liver bleeding in a 5 month-old baby.

A 5 month-old baby developed non-ceasing intra-peritoneal bleeding after extensive surgical biopsy for an hepatoblastoma. A single recombinant activated factor VII injection following enlarged hepatectomy helped to resolve quickly this life-threatening haemorrhagic syndrome.

[Surgical aspects of intussusception due to lymphoma in children].

Background: Intussusception due to lymphoma is a challenging condition for pediatric surgeons. The aim of this study is to report seven cases of this entity and to discuss its management.

Case Report: Six boys and one girl, 3-15-years-old, were admitted for intussusception secondary to a lymphoma.

[Testicular Leydig cell hyperplasia in children].

Introduction: Leydig cell hyperplasia (LCH) of the testis is rarely described in children. The authors report a case of incidental discovery of LCH on ultrasound examination.

Case Report: The authors report the case of a 9-year-old boy presenting with isolated and painless increased volume of the left testis, with no clinically palpable mass.

[First-intention surgical treatment of thyroglossal duct cysts in children: apropos of 99 cases].

Introduction: Thyroglossal duct cyst (TGDcs) is the most common malformation of the neck. The risk of infection and malignant transformation impose its treatment.

Objectives: The purpose of our study were: 1) to specify some points about the symptomatology and preoperative evaluation necessary for TGDcs diagnosis; 2) to analyse the factors who can explain an unsuccessful surgical treatment.

Anal canal duplication in infants and children--a series of 6 cases.

The authors present a series of six anal canal duplications (ACD), duplications of the alimentary tract located along the posterior side of the anal canal, with a perineal opening just behind the anus. Five asymptomatic duplications were diagnosed before the age of one year, by simple perineal inspection. A twelve-year-old girl presented with perineal and anal pains and diarrhoea.

[An unusual care of intestinal invagination: jejunojejunal invagination].

Background: Jejunal intussusception is uncommon in comparison with ileocolic form. It is more frequent in children over 2 years of age and has an atypical subacute presentation. An underlying anatomical cause is usually found.

Pheochromocytoma and paraganglioma in children: a report of 24 cases of the French Society of Pediatric Oncology.

Pheochromocytoma and paraganglioma of childhood are rare neuroendocrine tumors. Urinary catecholamine measurements, metaiobenzylguanidine (MIBG) scanning, computed tomographic scanning, and magnetic resonance imaging have greatly facilitated diagnosis. Prognosis after surgical resection is excellent.

Mesenteric adenitis caused by Yersinia pseudotuberculosis presenting as an abdominal mass.

Infection by Yersinia pseudotuberculosis has become of increasing pathological importance. Patients normally present with symptoms similar to those of appendicitis, due to mesenteric adenitis. We present the case of 3 patients infected by Yersinia pseudotuberculosis who in addition to fever and abdominal pain had a palpable abdominal mass, so great was the enlargement of the mesenteric nodes.

Malignant peripheral nerve sheath tumor arising in a "de novo" ganglioneuroma: a case report and review of the literature.

A case of a "de novo" ganglioneuroma showing a large area of malignant peripheral nerve sheath tumor (MPNST) is described. The tumor arose in an 11.5-year-old girl with neither stigmata nor family history of von Recklinghausen's neurofibromatosis.

Feasibility of retroperitoneal pelvic lymph node exploration in cervical carcinoma. Assessment of morbidity in 33 cases treated by combined radio-surgery or definitive radiotherapy.

Background And Purpose: Laparoscopic modalities by selective biopsies permit a better assessment of the pelvic lymph nodes status than imaging procedures in cervix carcinoma. They could enable the radiation oncologist to adapt the target volume of external irradiation, provided the feasibility of such procedures is good and the toxicity reduced as much as possible.

Material And Methods: From June 1980 to May 1993, 52 women with a mean age of 49, underwent a retroperitoneal pelvic lymph node laparoscopic procedure for cervix carcinoma classified according to FIGO as stages IA2 (14), IB (12), IIA (6), IIB (14), IIIB (3) and IVA (3).

[Granulosa cell tumors of the ovary in children and adolescents. Multicenter retrospective study in 40 patients aged 7 months to 22 years].

Background: Juvenile granulosa cell tumors (JGCT) of the ovary are rare in children. The over-all outcome after surgery is relatively good, but the indication and type of complementary treatment for severe forms are still unclear.

Population: A retrospective survey of the majority of patients with JGCT of the ovary admitted between 1965 and 1990 to 11 French oncologic pediatric centers was carried out.

[Mediastinoscopy].

Mediastinoscopy was initiated by E. Carlens in 1959 and enabled the exploration of the mediastinal tissues, above all the lymphatics with both a diagnostic and a prognostic aim. Surgical intervention was performed most often under general anaesthesia and cervical mediastinoscopy was carried out under direct visual inspection thanks to a thoracoscope with its own light source so that the mediastinal axis, the thymic area and also the peripheral mediastinum could be examined.

[Iron salt poisoning complicated by gastric stenosis].

Iron salt poisoning has been reported in a 7-year-old child. Desferioxamine was used for 6 days; systemic toxicity remained moderate. The clinical course was marked by gastro-intestinal symptoms and gastric stricture.

[Parameningeal cervical rhabdomyosarcoma in the neonatal period].

A case of parameningeal cervical rhabdomyosarcoma with severe bone destruction is reported in a 3 month-old infant; symptoms were present at birth. The treatment consisted of exclusive intensive chemotherapy. The outcome was favourable with complete tumor regression and vertebral bone reconstruction.

Single-stage correction of posterior hypospadias (178 cases). Comparison of three techniques: free skin graft, free bladder mucosal graft, transverse pedicle preputial graft.

Three techniques have been developed for single-stage repair of posterior hypospadias: free skin grafts, free bladder mucosal grafts, and pedicle preputial grafts. This multicenter retrospective study of 178 children who underwent surgery for posterior hypospadias was designed to compare the results achieved with these procedures. Free skin grafts (15 cases) resulted in the most frequent complications, and in particular the most severe strictures; in our opinion this technique should be abandoned.

[A rare case of bladder exstrophy with a completely malformed complex in a girl].

Report of the observation of a female newborn presenting a complex malformative set: distal anorectal malformation without fistula; bladder exstrophy without epispadias, with cutaneous bridge; congenital urethrovaginal fistula; malformative sexual ambiguity with bulky genital tubercle, whole urethra, urethral meatus on the apex of the genital tubercle, associated with a mid labial genital joint and a high genital orifice; unilateral multicystic kidney; vertebral abnormalities with supernumerary thoracolumbar vertebrae; no karyotype abnormalities and no familial pathological history; no endocrine abnormalities. The therapeutic multistage program permitted a good reconstruction of the different lesion, particularly with bladder functioning witch in hope a good continence with two years of follow-up. No similar case is reviewed in the literature.