[Expanding mature pineal teratoma syndrome. Case report]. / Tératome mature évolutif pinéal. A propos d'un cas.

We present a case of growing teratoma syndrome of the pineal region. To our knowledge, this is the fourth case reported in the literature. A 13-year-old boy was referred for intracranial hypertension and bilateral papillary edema. CT scan showed a pineal region tumor with obstructive hydrocephalus. After CSF (cerebrospinal fluid) shunting, MRI showed that the tumor had a heterogenous signal enhancement. The tumor marker HCG (human chorionic gonadotrophin) was elevated in CSF and serum. After three cycles of chemotherapy, MRI showed an important increase in tumor size with morphologic modifications. However, HCG in CSF and serum returned to normal. Surgical resection was performed and histological examination of the whole specimen showed mature teratoma. On postoperative MRI, there was a small area of signal enhancement of the left thalamus. Radiotherapy was given. The child was in complete remission 15 months after the diagnosis. Growing teratoma syndrome is a mixed germ cell tumor with a secreting portion that responds to chemotherapy and a non secreting portion of mature teratoma that continues to grow under chemotherapy. The treatment should include chemotherapy for the malignant secreting portion and surgery for the mature teratoma.

[Tumour-induced precocious puberty. Discussion of the indications for neurosurgery (author's transl)]. / Les pubertés précoces d'origine tumorale. Discussion de l'indication neuro chirurgicale.

The tumour aetiology of precocious puberty is sometimes clinically evident when accompanied by visual disturbances or by raised intracranial pressure. It may be suspected in the presence of accelerated puberty but should also be feared in cases of apparently idiopathic precocious puberty. In the 13 cases studied, the tumour was studied in the posterior hypothalamus. Ten children underwent surgery (with additional radiotherapy in two cases). Two children died, the follow-up varying at present between 2 and 12 years. Amongst the various tumours, the hamartoma has a special nature by virtue of slow or absence of increase in tumour size. When the clinical and radiological signs suggest this type of tumour, abstention from surgery with close observation of the size of the tumour and medical slowing of puberty is advised.

Ectopic pinealoma: an unusual clinical presentation and a histochemical comparison with a seminoma of the testis.

A patient with ectopic pinealoma first presented with apparent anorexia nervosa and hypernatraemic coma. A history of diabetes insipidus two months previously was not known on admission to hospital. The diabetes insipidus was unmasked by the administration of steroids. Neuroendocrinal and neuropathological aspects of the case are discussed with reference to the march of symptoms due to the growth of the tumour. Histochemical evidence is presented supporting the similarity between ectopic pinealoma and seminoma which suggests that they may more properly be referred to as atypical teratomas.