[Unusual variation of the splenic artery]. / Variante originale de l'artère splénique.

The authors describe an exceptional variation of the splenic artery found on a preoperative angiogram and confirmed after surgery on splenopancreatectomy specimens. Total duplication of the splenic artery is herein reported for the first time and suggest the need for a new classification of digestive arteries anomalies.

CT and MR findings of a retrorectal cystic hamartoma confused with an adnexal mass on ultrasound.

We describe the imaging features of a tailgut cyst mistaken for an adnexal mass. A pelvic ultrasound in a 28-year-old woman showed a 10-cm hypoechoic left pelvic mass. Having not seen the left ovary, the radiologist concluded that the mass was an endometrioma. CT disclosed a retrorectal cystic lesion with wall calcifications and internal septa. MR confirmed the extra-ovarian location of the tumor, which was hyperintense on T2-weighted images and had an intermediate signal on T1-weighted images. Surgery revealed a retrorectal cystic hamartoma. Radiological diagnosis of a tailgut cyst requires first correct localization of the tumor and then differentiation from other retrorectal masses.

[Central neurocytoma. Four patients]. / Neurocytome central. A propos de quatre cas.

We report four cases of central neurocytoma we operated on at our institution, between 1994 and 2002. This uncommon lesion occurred in young patients, was located in the lateral ventricules and was revealed by signs of intracranial hypertension. The tumor was totally removed in three cases and subtotally in one. Immunohistochemistry provided useful information to distinguish neurocytoma from other nervous system tumors. Prognosis was excellent in two patients. One patient developed tumor recurrence seven years after surgery, without any progression on further follow up evaluation. In the last patient, the tumor showed a more aggressive progression requiring the adjunction of radiosurgery.

Malignant struma ovarii: an unusual presentation.

Malignant struma ovarii is a rare disease; only a few cases are well documented in the literature. Thus, the overall prognosis and modalities of treatment are still somewhat controversial. In this article, the authors report a case of malignant struma ovarii discovered 4 years after ovariectomy after metastasis to the lungs and bones. Review of the pathology of the ovarian struma did not reveal the classic criteria of malignancy, there were, however, many features considered to be atypical and thus suspicious. The patient was treated by total thyroidectomy followed by repetitive doses of 131I. However, because of difficulties in increasing the level of endogenous thyrotropin (TSH) because of functional thyroid metastases in such an advanced disease, recombinant human thyrotropin (rhTSH; Thyrogen, thyrotropin alpha, Genzyme Corporation, Cambridge, MA) was used before administration of radioiodine. With this therapeutic protocol, the patient is still clinically stable 2 years after diagnosis.

[Progressive myoclonic epilepsy type Lafora]. / Epilepsie myoclonique progressive type Lafora.

Lafora's disease is a form of progressive myoclonic epilepsy characterized by seizures, myoclonus and dementia. We present the case of a 12-year-old girl who is complaining of epilepsy and myoclonic jerks starting a year ago, with deterioration of school performance, and abnormal EEG. The axillary skin biopsy showed PAS-positive inclusions in the cells of sweat glands, typical of Lafora's disease.