Pineal parenchymal tumors. Management with interstitial iodine-125 radiosurgery.

PURPOSE: To evaluate the efficacy of interstitial radiosurgery (IRS) for pineal parenchymal tumors (PPTs). PATIENTS AND METHODS: 18 consecutively admitted patients (twelve male and six female, age range 6-68 years, median age 34 years) with PPTs (eight pineocytomas, ten malignant PPTs) were treated at the authors' institution with IRS using stereotactically guided iodine-125 seed implantation ((125)I-IRS) as either primary or salvage therapy. The cumulative tumor surface dose ranged from 40 to 64 Gy. Adjuvant radiotherapy of the whole brain or the craniospine was done in patients with grade III and grade IV PPT. The median follow-up period was 57.4 months (range 6-134 months). RESULTS: Overall actuarial 5- and 8-year survival rates after IRS were 100% and 86% for pineocytomas, and the overall actuarial 5-year survival rate was 78% for high-grade PPTs. Follow-up magnetic resonance imaging showed complete remission in 72% (13/18) and partial remission in 28% (5/18) of the cases. One patient developed an out-of-field relapse 4 years after partial remission of a pineocytoma, which had already been treated with IRS. There was no treatment-related mortality. Treatment-related morbidity occurred in two patients only. CONCLUSION: This study indicates that stereotactic (125)I-IRS for the management of PPTs is quite efficient and safe. Due to the low rate of side effects, IRS may develop into an attractive alternative to microsurgery in de novo diagnosed pineocytomas. In malignant PPTs, IRS may be routinely applied in a multimodality treatment schedule supplementary to conventional irradiation.

[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.

Nonresective treatment of pineoblastoma: a case report.

BACKGROUND: Stereotactic biopsy followed by cytoreductive surgery and/or radiation are the standard treatment for pineoblastoma. We are reporting a patient with a relatively large pineoblastoma, who was treated with a combination of nonresective treatment modalities. METHOD: The patient is a 20-year-old woman who presented with signs and symptoms of raised intracranial pressure. Magnetic resonance imaging (MRI) showed a large tumor in the pineal region and hydrocephalus. She was emergently treated by placement of a ventriculoperitoneal shunt. Three days later she underwent a stereotactic biopsy, which showed the tumor to be pineoblastoma. She was then further treated with the following treatment modalities: permanent implantation of high activity I125 seeds, external radiation, and chemotherapy. RESULTS: At 10 months' follow-up, MRI showed complete disappearance of the tumor. At 18 months' follow-up, the patient is asymptomatic, neurologically intact, and her MRI scans show no evidence of intracranial or intraspinal tumor. CONCLUSIONS: Though the follow-up is relatively short, the results of the nonresective approach in this patient have been excellent.

Hypothalamic tumors in children. Their diagnosis and management.

17 intrinsic hypothalamic tumors in infancy and childhood occurring between 1963 and 1973 were analyzed in detail because of the consistent mode of approach to diagnosis, therapy and follow-up care. Careful examination of the symptom-time sequence and location provided anatomical-clinical correlations and was even suggestive of the probable histologic type of lesion present. The postoperative and long-term management problems have led to significant conclusions. The best results were obtained with those patients who underwent craniotomy and biopsy, followed by irradiation therapy.

Hypothalamic tumor presenting as anorexia nervosa.

The authors describe a patient with symptoms of anorexia nervosa who was initially treated on a psychiatric ward but was later found to have a hypothalamic tumor. They recommend repeated neurological and endocrine evaluations for patients with anorexia nervosa who are unresponsive or only partially responsive to psychiatric treatment. Initially negative medical evaluations, obvious psychopathology, and even some response to treatment may all prove insufficient to rule out organic disease.