Management of ACTH-secreting supradiaphragmatic adenomas.

Supradiaphragmatic adrenocorticotropic hormone (ACTH) secreting pituitary adenomas are exceptionally encountered (14 cases previously described) and raise issues concerning their nosology and management. If surgery is the treatment of choice, the nature of surgical approach (craniotomy, transsphenoidal approach) remains controversial. To illustrate this issue, we presented two cases of supradiaphragmatic ACTH secreting pituitary adenomas successfully excised via a subfrontal approach. Both patients were female (20 and 41 years) and had a typical Cushing's syndrome. MRI revealed, in both cases, a suprasellar mass in contact with the pars tuberalis of the pituitary. In the first case, the patient underwent initially a transsphenoidal approach with negative exploration and subsequent partial hypophysectomy. One year later, the patient was operated on again via a subfrontal approach, allowing excision of a supradiaphragmatic adenoma and a complete cure of Cushing's disease. In the second case, the patient underwent initially a subfrontal approach and was definitely cured. In both cases, the diaphragma sellae was found to be intact and the pituitary stalk could be preserved. Postoperative MRI demonstrated a clearly visible intact pituitary stalk in conjunction with normal aspect of the pituitary. Supradiaphragmatic pituitary adenomas are most likely adenomas of the pituitary stalk with extra-axial development. Surgery remains the treatment of choice. Should the superior approach be preferred, the transsphenoidal-transtuberculum sellae approach may represent a viable alternative when performed by a well-trained surgical team. Surgery may be difficult, and drawbacks are non negligible, particularly in elderly patients. In these cases, stereotactic radiosurgery should receive more consideration.

Natural course and prognosis of anaplastic gangliogliomas: a multicenter retrospective study of 43 cases from the French Brain Tumor Database.

Background: Anaplastic gangliogliomas (GGGs) are rare tumors whose natural history is poorly documented. We aimed to define their clinical and imaging features and to identify prognostic factors. Methods: Consecutive cases of anaplastic GGGs in adults prospectively entered into the French Brain Tumor Database between March 2004 and April 2014 were screened. After diagnosis was confirmed by pathological review, clinical, imaging, therapeutic, and outcome data were collected retrospectively. Results: Forty-three patients with anaplastic GGG (median age, 49.4 y) from 18 centers were included. Presenting symptoms were neurological deficit (37.2%), epileptic seizure (37.2%), or increased intracranial pressure (25.6%). Typical imaging findings were unifocal location (94.7%), contrast enhancement (88.1%), central necrosis (43.2%), and mass effect (47.6%). Therapeutic strategy included surgical resection (95.3%), adjuvant radiochemotherapy (48.8%), or radiotherapy alone (27.9%). Median progression-free survival (PFS) and overall survival (OS) were 8.0 and 24.7 months, respectively. Three- and 5-year tumor recurrence rates were 69% and 100%, respectively. The 5-year survival rate was 24.9%. Considering unadjusted significant prognostic factors, tumor midline crossing and frontal location were associated with shorter OS. Temporal and parietal locations were associated with longer and shorter PFS, respectively. None of these factors remained statistically significant in multivariate analysis. Conclusions: We report a large series providing clinical, imaging, therapeutic, and prognostic features of adult patients treated for an intracerebral anaplastic GGG. Our results show that pathological diagnosis is difficult, that survivals are only slightly better than for glioblastomas, and that complete surgical resection followed with adjuvant chemoradiotherapy offers longer survival.

Temozolomide treatment of an adult with a relapsing medulloblastoma.

Temozolomide is an oral cytotoxic agent that has demonstrated its interest in high grade glioma tumors. This drug can be used either concomitantly with radiotherapy or as chemotherapy. The prognosis of relapsing medulloblastoma is poor and treatment is often difficult, especially after radiotherapy. Here, we report the use of temozolomide in an adult presenting relapsing medulloblastoma. An initial partial response was observed for this previously heavily treated patient. This observation suggests this drug may be useful in medulloblastoma, either as conventional chemotherapy or for use together with radiotherapy.

Intrathecal morphine infusion for chronic non-malignant pain: a multiple center retrospective survey.

Intrathecal morphine (ITM) is commonly used for the treatment of cancer pain. There is reluctance for its use in France to treat chronic noncancer pain. In order to appreciate its popularity, efficacy, dose escalation with time, and long-term tolerance, we carried out a retrospective study in the neurosurgery departments of university teaching hospitals in France involved in intrathecal drug therapy. Only 44 patients with chronic noncancer pain used implanted pumps for ITM treatment. Nineteen patients were available for detailed analysis. This survey concerns these 19 patients. There were 13 women and six men. Their average age was 48.8 years (range: 30-69 y). The mean duration of pain before pump implantation was 100 months (range: 12-240 m). The mean follow-up since implantation was 54 m (range: 4-144 m). Thirteen patients were suffering from postsurgery lumbar and radicular pain. The average initial and final dose per day of morphine was 1.3 mg (range: 1-2 mg) and 2.5 mg (range: 1-6.7 mg) for patients with nociceptive pain and 1.2 mg (range: 0.7-2 mg) and 3 mg (range: 1-10 mg) for patients with mixed pain, respectively. Five (26.3%) of 19 patients returned to their initial jobs. Increase in activity level was reported as good in seven patients (36.8%). Patient satisfaction rate was 90%. The VAS rate was reduced to 49.2% of the initial values while the subjective pain relief was estimated at 67.8%. Two cases (10.5%) of late pump site infection and two patients (10.5%) with catheter displacements were recorded. Side effects imputable to morphine included; constipation, somnolence, decreased libido, weight gain, amenorrhoea, vomiting, nightmares, and itching. No development of tolerance or addiction were recorded. We conclude that in well selected cases ITM should be considered as a possible therapeutic option in the treatment of intractable chronic benign pain.