Ovarian germ cell tumors in children. Management, survival and ovarian prognosis. A report of 75 cases.

Background/purpose: The aims of this study were to evaluate survival and ovarian prognosis in patients treated for ovarian germ cell tumor (OGCT) and to propose a decision-making protocol.

Methods: Charts of girls operated on for OGCT from 1976 up to 2009 were reviewed retrospectively. Tumor characteristics were assessed by tumor markers, imaging, and pathology.

Ovarian torsion. Management and ovarian prognosis: a report of 45 cases.

Background/purpose: Ovarian torsion in childhood and adolescence is a rare entity. Traditionally, treatment is oophorectomy. The aim of this study was to evaluate ovarian outcome and to propose a decision-making protocol for suspected ovarian torsion.

An N-terminal WT1 mutation (P181S) in an XY patient with ambiguous genitalia, normal testosterone production, absence of kidney disease and associated heart defect: enlarging the phenotypic spectrum of WT1 defects.

Objective: This study reports the clinical and molecular data of an XY patient with a very unusual phenotype due to a Wilms' tumor-suppressor (WT1) gene mutation. The genotype-phenotype relationship of different WT1 mutations is then discussed.

Patient: The patient presented at birth with micropenis, severe hypospadias and cryptorchidism.

Large cell neuroendocrine carcinoma of the lung: pathological study and clinical outcome of 18 resected cases.

Large cell neuroendocrine carcinoma of the lung (LCNEC) has been recently redefined by the World Health Organisation (WHO) classification but the appropriate treatment remains unclear. We reviewed 18 consecutive resected cases of LCNEC. Two pathologists assessed diagnosis by applying rigorously the last WHO criteria.