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. Herein, we describe an atypical case of MESTK diagnosed in a 12-year-old prepubertal boy who presented with hematuria. Pathology and immunohistochemistry revealed a typical MESTK. The child was free of disease at 2-year follow up after a partial nephrectomy and tumour excision.

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. Complications, histologic biopsy results, and outcomes were reviewed. RESULTS: One hundred thirty-nine procedures were performed in 134 children. There were 72 males and 62 females, with a mean age of 9.2 years (3 months to 17 years). The mean follow-up was 4.5 years. Thirty-eight primitive tumors were managed, 20 of which had a complete thorascopic resection, and nine biopsies were performed. In 9 additional cases, an open conversion was necessary. Among the 20 complete resections, 17 of the lesions were neurogenic tumors (i.e., neuroblastoma or ganglioneuroma). Three complications occurred: 2 chylothoraces and 1 with Horner syndrome. All three complications regressed. The mean follow-up was 3 years, and no recurrence has been noted. Fifty-three metastatic lesions were managed: 29 had a complete resection, 11 had a biopsy, and an open conversion was necessary in 13 cases. Among the complete resections, more than half were metastases from bone tumors. The mean follow-up was approximately 4 years and 5 pulmonary recurrences developed. Four deaths occurred, with 2 having had lung metastases. Forty-eight malignant hemopathies were managed; 9 biopsies for primitive tumors were performed. There were 36 secondary lesions, of which 5 were completely resected; 30 were biopsied and 1 required an open conversion. CONCLUSIONS: Thoracoscopy is a safe, effective approach for the evaluation and resection of solid lung tumors and for biopsy. Thoracoscopic resection of metastatic lesions is reasonable for nephroblastoma, but a thoracotomy is suggested for other metastases.

[Polyorchidism in child (a case report and review of literature)]. / Polyorchidie chez l'enfant (à propos d'un cas avec revue de la littérature).

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. The review of the literature finds cases of polyorchidism revealed by testis torsion and an increased risk of malignancy and infertility. We report the case of polyorchidism in a 14 year old child, at whom a painful testis mass indicate surgical exploration and documented the histological diagnosis of polyorchidism. Through this observation and review of the literature, authors describe pathological and managment findings of polyorchidism.

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%). The aim of this study was to evaluate the implementation of these guidelines in a single centre. DESIGN AND PATIENTS: Genetic diagnosis was proposed to the parents of all eight children born after 1992 from two RET 634 families. Total thyroidectomy was proposed before age 5 if the child carried a mutation. RESULTS: Genetic diagnosis was performed in all the children (aged 1-3) and thyroidectomy in the five who carried a mutation (aged 2-5). Cervical lymph node dissection varied from lymphadenectomy of central and lateral compartments in the eldest child to pickings in the youngest. There was no permanent hypoparathyroidism or recurrent nerve paralysis. C-cell hyperplasia, medullary thyroid carcinoma and lymph node metastasis were present in 5/5, 3/5 and 0/5, respectively. Undetectable pentagastrin-stimulated CT levels were achieved and maintained postoperatively in all five children (average follow-up 3.6 years). CONCLUSIONS: MEN 2 guidelines on thyroidectomy can be efficiently and safely implemented by a multidisciplinary team operating in a single centre. The lack of guidelines on cervical neck dissection remains a problem; this could be solved by determining an age under which this procedure would be deemed unnecessary.

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. The authors report a case of a baby girl with prenatal diagnosis of MP. Rapid worsening of clinical aspects at birth and the presence of a pneumoperitoneum on systematic abdominal plain radiographs led to urgent surgery on the 1st day of life. Laparotomy showed a perforated necrotizing tubular duplication of the transverse colon. Removal of the duplication followed by limited segmental colonic resection and double colostomy were carried out. Follow-up was uneventful.

[Testicular Leydig cell hyperplasia in children]. / Hyperplasie à cellules de Leydig du testicule chez l'enfant.

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. Scrotal ultrasound revealed an echogenic mass, 12 mm in diameter, with a solid appearance and several hypoechoic areas, without calcification, situated in the lower pole of the testis. Tumour markers and gonadotropin axis hormonal assessment were normal. On surgical exploration, the testis had a macroscopically normal appearance; opening of the tunica albuginea revealed the lesion and enucleation was performed. Histological examination confirmed the presence of LCH, 5 mm in diameter. The postoperative course was uneventful. Physical examination and scrotal ultrasound have remained normal with a follow-up of two years. DISCUSSION: The authors recall the characteristics of LCH, which usually presents, in children, in the form of signs of precocious puberty or more rarely by gynaecomastia. The lesion is rarely palpable. Scrotal ultrasound reveals a homogeneous mass with several hypoechoic nodules. An endocrine assessment must always be performed (frequent elevation of LH). Surgery should be as conservative as possible (enucleation-resection). Histological diagnosis may be difficult.