Anaplastic astroblastoma of childhood: aggressive behavior.

OBJECTIVE: Astroblastoma is an unusual brain tumor in childhood. Tumor usually arises from cerebral hemispheres. This large tumor is well-demarcated, lobulated, solid or cystic, and therefore, may resemble glioblastoma. Histopathologically, there are two types of astroblastoma: well-differentiated (low-grade) or anaplastic (high-grade). In low-grade astroblastoma, treatment of choice is complete excision. This type of tumor usually does not recur. However, anaplastic tumors can recur despite surgery, radiation, and chemotherapy and may be problematic for clinician. CASE REPORT: A 7-year-old female patient presented with an acute onset of vomiting and seizure. Magnetic resonance imaging study revealed a large mass in the left parieto-occipital region. She underwent total excision of the tumor. Histopathologically, the tumor was an anaplastic astroblastoma. Her adjuvant treatment was planned to consist of radiation therapy and cisplatin-based chemotherapy. However, the tumor recurred early in the course, and she died 18 months after diagnosis. CONCLUSION: High-grade astroblastomas behave like glioblastoma, as emphasized in this case report. Local control of this type of tumor seems difficult despite surgery, radiation therapy, and cisplatin-based chemotherapy.

Role of the constructive interference in steady-state sequence in the classification of sacral meningeal cysts.

PURPOSE: To investigate the role of the CISS (constructive interference in steady state) sequence in sacral meningeal cysts. MATERIAL AND METHODS: Fourteen patients with sacral meningeal cysts were included. Conventional T1W and T2W sequences and the CISS sequence (TR/TE = 12.25/5.90) were obtained at 1.5-T. The 1-mm-thick base images and multiplanar reformatted images of the CISS sequence were studied. The sacral meningeal cysts were classified by the CISS sequence in accordance with the previously described surgical and histopathological criteria. RESULTS: A total of 25 sacral meningeal cysts were identified in the 14 patients. The cysts and their contents were visualized by the CISS sequence, and the CISS sequence was superior to the T1W and T2W images. Fifteen of the cysts were consistent with type I lesions (extradural meningeal cysts without nerve fibers inside) and 10 cysts with type II lesions (extradural meningeal cysts with nerve fibers inside). There were no type III lesions (intradural meningeal cysts) in the sacral region. CONCLUSION: Previous studies have indicated that conventional MRI as well as magnetic resonance myelography are inconsistent for a classification of sacral meningeal cysts. The CISS sequence with its capability to obtain T2W thin slice acquisitions is superior in showing the nerve root fibers contained in the cysts, which is essential in the differentiation of type I and II cysts. Application of the CISS sequence is recommended in the diagnosis of sacral meningeal cysts.

Neuroendoscopic biopsy for intraventricular tumors.

Neuroendoscopy has been shown to be an effective and minimally invasive method in the management of intraventricular tumors. Endoscopic tumor biopsy with or without additional endoscopic procedures such as third ventriculostomy and septostomy can be performed at the same session. Neuroendoscopic tumor biopsy was performed in 18 patients in our department. Their ages ranged from 2 to 65 years (median 12 years); only two of them were adult. Location of the tumors were as follows: pineal region in 7, hypothalamus and 3rd ventricle in 4, lateral ventricle in 4, thalamus in 2, and tectal in 1 patient. All procedures were performed under general anesthesia using rigid neuroendoscopes. Cerebrospinal fluid (CSF) was collected at the beginning of the procedure for cytological analysis and for pineal tumor markers. Biopsy forceps were used to obtain tissue from the lesion. The third ventriculostomy was performed in all patients with a pineal tumor, in addition to the tumor biopsy. The pathological examinations revealed a low-grade astrocytoma in 6 patients, anaplastic astrocytoma in 3, germinoma in 3, pineocytoma in 1, pineoblastoma in 2, glioblastoma multiforme in 1 and granulomatous lesion in 1. Subsequent mode of treatment such as radiation therapy, chemotherapy or radical surgery was determined on the basis of pathological diagnosis. Neuroendoscopic tumor biopsy is a less invasive method than open surgery and has some advantages such as treating the hydrocephalus at the same surgical session and the availability of CSF cytology. Neuroendoscopic techniques should be considered in selected patients.

Cervical spondyloptosis: a case report.

A 5-year-old male child who had undergone orthopedic surgery for foot deformities three years prior presented to the outpatient clinic with a complaint of flexion restriction in both hands. The radiological evaluation of the patient showed C3-4 spondyloptosis. Halo traction was placed and posterior and anterior spinal fusions were performed respectively to achieve realignment of the spine. The postoperative period was uneventful and the patient has been followed for 24 months. The etiology, diagnostic features and treatment options of childhood cervical spondyloptosis have been briefly reviewed and the pertinent literature discussed.

Is meningocele really an isolated lesion?

OBJECT: We designed this study to elucidate the associated occult spinal lesions in patients with simple dorsal meningocele. METHODS: The study population was comprised of two groups. Group I comprised newly diagnosed patients with dorsal spinal meningocele, and group II comprised patients who had had surgery for meningocele and presented with progressive neurological deficits. Magnetic resonance imaging (MRI) scans of the whole spinal column were done. The associated spinal cord malformations were also treated at the same operation. There were 14 boys and 8 girls, with an age range from birth to 4 years (mean 3.9 months), in group I. Of 20 patients (90%) with associated spinal lesions, 6 had more than one lesion, excluding hydromyelia. Group II was made up of 6 patients who had been previously operated on for a meningocele and who presented with tethered cord syndrome. These were 4 boys and 2 girls, who ranged in age from 4 to 10 years (mean 6 years). RESULTS: The level of the conus terminalis was lower than L3 in all patients. The other findings on MRI, besides low conus, were as follows: tight filum, split cord malformation, epidermoid, dorsal lipoma and hydromyelia. CONCLUSIONS: Meningocele frequently camouflages a second, occult, spinal lesion. MRI of the whole spinal column should be performed. An intradural exploration performed with a microneurosurgical technique is needed to detect the fibrous bands that may lead to spinal cord tethering and to release the entrapped nerve roots. The other associated spinal anomalies should be operated on during the same operation.

Child abuse in Turkey: an experience in overcoming denial and a description of 50 cases.

OBJECTIVE: To share the experiences of the first clinical multidisciplinary child abuse and neglect (CAN) team in Turkey with international child abuse community. METHODS: The authors established the first Turkish CAN follow-up team at Dr. Behcet Uz Children's Hospital. Following a training program in five teaching hospitals in Izmir, the authors kept a record of every case diagnosed with CAN from these hospitals between 1996 and 1998. The demographic, epidemiologic, and medical features of this case series are summarized. RESULTS: Fifty cases were diagnosed and followed-up. Seventy-six percent of patients were reported from Dr. Behcet Uz Children's Hospital. Age and sex distribution was 9.2 +/- 6.7 years and 46% male, 54% female, respectively. The offenders were only father in 38%, only mother in 28%, and multiple in 34%. More than three CAN risk factors were present in 94%. Of the children reported 44% survived, 14% died, and 42% were lost to follow-up. Sixteen percent were free of reabuse, and 42% survived with handicaps. CONCLUSIONS: Child abuse and neglect is a reality in Turkey. The team's work increased pediatricians' awareness of CAN. Reporting these cases to Social Affairs Bureau was established for the first time in Turkey. Physicians in Turkey need training to diagnose and properly report CAN. The implemen tation of a national CAN prevention program is an urgent need for Turkey.

Tethered cord syndrome in adults.

BACKGROUND: The tethered cord syndrome (TCS) consists of an abnormally low conus medullaris tethered by a thickened filum terminale or various forms of spinal dysraphism. The adult variant of the syndrome seems not to be as rare as once thought. METHOD: This study includes 11 patients with a TCS. Clinical, radiological findings and outcome were reviewed in these adult patients with TCS. FINDINGS: The patients ranged in age from 18 to 34 years (mean 24.09 years). There were 7 men and 4 women. The presenting symptoms in order of frequency were as follows: low back pain radiating to legs, urinary complaints, weakness in legs and impotence. All patients had magnetic resonance imaging scans. Physical exercise in 8, birth delivery in 1 and carrying heavy objects in 1 patient were determined as the precipitating factors. No precipitating factor could be defined in one of the patients. All patients were operated on, except for one. None of the patients worsened after surgery. INTERPRETATION: The patients presenting with low back pain and sciatica responded to surgery better than those with sphincter problems.

Growing skull fractures (craniocerebral erosion).

The incidence of growing skull fractures ranges from less than 0.05% to 1.6%. We reviewed 22 growing skull fracture patients retrospectively. There were 15 boys and seven girls ranging in age from newborn to 6 years (mean: 12.4 months) at the time of injury. Falling was the most frequent cause of injury. In total, 17 patients presented with a scalp mass. The scalp was sunken over the bone defect in three patients. Other symptoms and signs were seizure in five patients, hemiparesis in four, recurrent meningitis in one, and pulsatile exophthalmus in one. The most common location was the parietal region. The extent of dural defect was always greater than that of bony defect, except in one case that had been previously shunted for hydrocephalus. In another patient with a growing fracture in the posterior cranial fossa, the dural edges could not be exposed, although a wide craniotomy was performed. Therefore, a cystoperitoneal shunt was inserted. Gliotic tissue was present in all the patients. Cyst or cystic lesions were observed in only nine patients, duraplasty was performed in 21, 16 were neurologically intact, and six had minor deficits. All patients under the age of 3 years with a diastatic skull fracture should be closely followed up. A sustaining diastatic fracture and brain herniation through the skull defect shown on CT or MRI imply a growing skull fracture.

Complications of subduroperitoneal shunting.

OBJECTS: Subduroperitoneal (SP) shunts have been widely used in the management of pediatric subdural fluid collections. METHODS: We retrospectively reviewed the complications of SP shunting in 73 boys and 24 girls, who ranged in age from 1 to 180 months (median 7 months). Subdural fluid collection was bilateral in 75 and unilateral in 22 patients. The most common complication was shunt obstruction (13 patients). Shunt migration was seen in 8 patients. Migration occurred only with unishunts without a reservoir and with peritoneal catheters. However, the shunts with a reservoir or flushing valve led to skin necrosis in 4 patients (P=0.003). Unilateral drainage though bilateral collections were present, infection, bowel perforation, and ileus occurred in 5, 4, 1 and 1 patients respectively. CONCLUSIONS: These SP shunt complications, some of which are avoidable, should be kept in mind.

Intraosseous neurinoma of the parietal bone.

Intraosseous neurinoma is very uncommon tumor. Its location in the skull is extremely rare. A 4-year-old boy presented with a lump in the right occipital region. Computed tomography revealed a soft tissue mass with bony erosion in the right occipital bone. The solid, nontender, and immobile mass was totally removed. The pathological examination showed that it was an intraosseous neurinoma. Total resection of intraosseous neurinomas is sufficient treatment and is not followed by recurrence.

Intracranial meningeal tumours in childhood: a clinicopathologic study including MIB-1 immunohistochemistry.

Primary tumours of the meninges with a relatively high tendency for malignant behaviour are uncommon in childhood. This study concerns 18 cases of meningeal tumours in children under the age of 16, of which 13 were meningiomas and five were other tumours arising in the meninges. Meningiomas showed a preponderance in females as in adult series, and the majority were supratentorial in localisation. The percentage of meningeal tumours and meningiomas among all brain tumours in our centre were 3.72% and 2.69%, respectively. Four out of 13 meningiomas were fibroblastic, four were transitional, one was meningothelial, two were psammomatous and two were papillary meningiomas. Seven (38.8%) out of 18 tumours showed anaplastic features, including two papillary meningiomas, two hemangiopericytomas, one mesenchymal chondrosarcoma, one pleomorphic sarcoma and one anaplastic meningeal tumour. Papillary meningiomas with hemangiopericytoma-like solid areas were seen frequently in our cases (15.3%). Meningoangiomatosis was associated with two meningeal tumours. MIB1 (Ki-67) labelling indices (LIs) ranged between 0% and 13.6% (mean 1.83%) in benign, and between 1% and 20% (mean 7.2%) in malignant tumour, including papillary meningiomas. Mean MIB-1 LIs were 5.61% and 1.14% in non-recurrent and recurrent cases, respectively. MIB-1 LIs showed significant differences between benign and malignant meningeal tumours but no significant correlation either with prognosis or recurrence. Despite the fact that brain tumours are among the most common neoplasms of childhood, meningeal tumours are rare lesions, accounting for less than 2% of published series of intracranial neoplasms in childhood [5, 8, 18, 24, 30, 32]. It has been suggested that the clinical and pathological characteristics of meningiomas in this age group differ from those of adults [14, 18, 24, 45]. Besides meningiomas, there are a few reports of other meningeal tumours in childhood and difficulties in differential diagnosis may arise within this group, especially in anaplastic tumours [11, 13, 32, 44, 46]. One of the major problems in meningiomas and some tumours arising in the meninges is the discordance that arises between the histologic appearance of the tumour and behaviour [4]. Several studies have attempted to determine the proliferation potential of meningiomas, including immunohistochemical labelling with monoclonal antibodies to Ki-67, proliferating cell nuclear antigen (PCNA), and bromodeoxyuridine (BUdR); flow cytometric DNA analysis; or argyrophilic nucleolar organizer regions (AgNORs) counting [9, 10, 15, 19, 22, 26, 31, 35, 53]. The studies concerning proliferation markers have contradictory results [9, 10, 15, 26, 31, 42, 53]. MIB-1 detects the same or a similar epitope as the original antibody Ki-67 and reacts with a proliferation associated antigen expressed in all active parts of the cell cycle, G1, S, G2 and M (mitosis), but not in the G0 or quiescent phases [7]. In this study we examined the clinicopathological characteristics and MIB1 values of 18 meningeal tumours in children under the age of 16 years within the last 25 years (from 1970 to 1995).