Ge Jia

The diagnosis and treatment of subependymal giant cell astrocytoma combined with tuberous sclerosis

BackgroundSubependymal giant cell astrocytoma develops in a small proportion of tuberous sclerosis patients. There are still several controversies on the early diagnosis of the tumor, treatment of hydrocephalus, and timing of operation, etc.MethodsFrom September 1996 to April 2006, 17 patients were admitted in neurosurgical department of “Beijing Tiantan Hospital”. The authors analyzed medical records and followed up every case.ResultsThere are 18 tumors out of 17 patients. One patient had double tumors. Sixteen patients except one underwent tumor resection 17 times. Fifteen tumors out of 17 were gross totally removed; two were partially removed. One patient died of brain infarction postoperatively. Three out of 13 patients with preoperative hydrocephalus still needed ventriculoperitoneal shunt after tumor resection. There was no recurrence after total resection.ConclusionDiagnosis of tumor should be made by clinical criteria. Serial follow-up is essential for a suspected perimonro lesion to find tumor growth earlier. When there is growth, tumor should be removed as soon as possible. Hydrocephalus will resolve in most cases after tumor resection, while external drainage is suitable for emergent cases. Transcallosal and transcortical approaches are both effective to resect the tumor. Tumor will not recur after total removal.

Microsurgical treatment for hypothalamic hamartoma in children with precocious puberty

BACKGROUND We review the surgical treatment of hypothalamic hamartoma causing precocious puberty. METHODS Six children (three girls and three boys) with precocious puberty secondary to hypothalamic hamartoma were recruited for our study. The mean age of the patients was 30 months old (range 13 months to 5 years), and the mean age of the onset of puberty was 7.3 months. All patients were treated by microsurgery. RESULTS All patients had higher then normal stature, body weight, bone growth, and serum levels of sexual hormones. The boys presented with mature external genitalia, pubic hair, frequent erection, and acne, while the girls presented with growth of breasts and menarche. Magnetic resonance image (MRI) revealed an isointense mass below the tuber cinereum extending into the supersellar and interpeduncular cistern, ranging from 4 to 12 mm in diameter, consistent with pedunculate hamartoma. The hamartoma was removed completely via a right pterional approach. The symptoms and signs of precocious puberty resolved completely, and sexual hormone levels decreased to the pre-pubertal range in all six patients without any postoperative complications. CONCLUSION We report a series of six children with hypothalamic hamartoma-induced precocious puberty who underwent microsurgical treatment. All of them recovered completely to their age-appropriate state. Microsurgery is a good choice of treatment for pedunculate hypothalamic hamartoma.

Transcallosal interforniceal approach to pineal region tumors in 150 children.

OBJECT Pediatric pineal region tumors are very difficult to cure surgically. The authors used the transcallosal interforniceal approach in patients with these lesions. METHODS One hundred fifty children, 98 boys and 52 girls, with pineal region tumors underwent tumor resection via the transcallosal interforniceal approach in the pediatric neurosurgery ward of TianTan Hospital. The patients ranged in age from 1 to 15 years. Fifty-eight patients had mature teratomas; 57, immature teratomas; 14, astrocytomas; 3, glioblastomas; 4, pineoblastomas; 2, pineocytomas; 4, choriocarcinomas; 4, cavernous hemangiomas; 2, germ cell tumors; and 2, epidermoid cysts. RESULTS One hundred twenty-nine tumors were totally removed, 15 were subtotally removed, and 6 were partly removed. There were no deaths and no subsequent instances of disconnection syndrome. Short-term memory deficits appeared in 94 patients but resolved within 6 months in most; only a few patients retained persistent deficits. There were 2 patients with mutism that resolved within 10 days. Parinaud syndrome was observed in 45 patients after surgery; 21 of these cases had appeared preoperatively. The syndrome resolved within 6 months in 31 patients, while it remained in the other 14. CONCLUSIONS The transcallosal interforniceal approach appears to be a safe route for pineal region tumors in children, and complete resection can be achieved in the majority of patients.

High expression levels of CXCL12 and CXCR4 predict recurrence of adamanti-nomatous craniopharyngiomas in children

BACKGROUND Adamantinomatous craniopharyngioma (ACP) is a benign but maldevelopmental tumor with a high recurrence rate. OBJECTIVE Theaim of this study was to investigate the dysregulated biological molecules that play important roles in the recurrence of ACP. METHODS We first performed microarray analysis on tumor samples from two pediatric patients with recurrent ACP and from two pediatric ACP patients without recurrence after a one-year follow-up. The expression of CXCL12 and CXCR4 in 45 specimens of pediatric ACP was further evaluated by immunohistochemistry. These results were correlated with the clinicopathological parameters and survival of the patients. RESULTS Four downregulated genes (APC, ITGA, MCAM, and TIMP4) and 16 upregulated genes (CST7, CTSK, CTSL1, CXCL12, CXCR4, FN1, FXYD5, ITGB3, MMP2, MMP3, MMP7, MMP9, NR4A3, PLAUR, TIMP2, and VEGFA) were found in the recurrent patients. CXCL12 and CXCR4 were highly expressed in 13 patients (28.9%) and 14 patients (31.1%), respectively. High levels of CXCL12 and CXCR4 expression were significantly associated with a poor recurrence-free survival and were the prognostic factors for ACP recurrence in pediatric patients. CONCLUSIONS High levels of CXCL12 and CXCR4 expression were associated with ACP recurrence. The role of CXCL12 and CXCR4 in the development of brain tumors requires further research.

Surgical treatment of hypothalamic hamartoma causing central precocious puberty: long-term follow-up

Hypothalamic hamartoma (HH) is a rare condition that often manifests as central precocious puberty (CPP). There is a lack of information available concerning the long-term effectiveness of surgery for the treatment of CPP due to HH. Here the authors describe 3 cases of CPP due to HH, with a follow-up ranging from 9 to 11 years after surgery. Three girls experienced breast growth and menses at 5-18 months of age and 5-36 months of age, respectively. Serum concentrations of luteinizing hormone, follicle-stimulating hormone, and estradiol concentrations ranged from 2.5 to 6.5 mIU/ml, 4.8-5.9 mIU/ml, and 47.9-133.0 pg/ml, respectively. Magnetic resonance imaging confirmed that CPP was caused by HH. Lesions were resected using a right pterional approach. After surgery, endocrine hormone concentrations were normalized, breasts shrunk, and menses ceased in each patient. Moreover, all of them subsequently developed normally and experienced age-appropriate onset of puberty. Each patients height and weight were normal at the most recent follow-up (9-11 years after surgery), and none had experienced learning difficulties. Central precocious puberty due to HH can be successfully treated with resection. In the 3 cases presented, this approach was associated with both short- and long-term efficacy.

Microsurgical treatment for hypothalamic hamartoma in children with precocious puberty. Commentary

BACKGROUND We review the surgical treatment of hypothalamic hamartoma causing precocious puberty. METHODS Six children (three girls and three boys) with precocious puberty secondary to hypothalamic hamartoma were recruited for our study. The mean age of the patients was 30 months old (range 13 months to 5 years), and the mean age of the onset of puberty was 7.3 months. All patients were treated by microsurgery. RESULTS All patients had higher then normal stature, body weight, bone growth, and serum levels of sexual hormones. The boys presented with mature external genitalia, pubic hair, frequent erection, and acne, while the girls presented with growth of breasts and menarche. Magnetic resonance image (MRI) revealed an isointense mass below the tuber cinereum extending into the supersellar and interpeduncular cistern, ranging from 4 to 12 mm in diameter, consistent with pedunculate hamartoma. The hamartoma was removed completely via a right pterional approach. The symptoms and signs of precocious puberty resolved completely, and sexual hormone levels decreased to the pre-pubertal range in all six patients without any postoperative complications. CONCLUSION We report a series of six children with hypothalamic hamartoma-induced precocious puberty who underwent microsurgical treatment. All of them recovered completely to their age-appropriate state. Microsurgery is a good choice of treatment for pedunculate hypothalamic hamartoma.