Journal of Neurosurgery
Ji Hoon Phi, Seung-Ki Kim, Sung-Hye Park, Seok Ho Hong, Kyu-Chang Wang and Byung-Kyu Cho
Immature teratomas of the central nervous system (CNS) are rare neoplasms. Although adjuvant therapy is generally recommended after resection, the exact role of each therapeutic modality is not yet established. The purpose of this study was to analyze the clinicopathological correlation and the role of resection to define the optimal treatment modalities for immature teratomas of the CNS.
Between 1987 and 2002, eight patients underwent radical surgery for a lesion diagnosed as a CNS immature teratoma at the authors' institution. The clinical courses of these patients and the pathological features of their tumors were retrospectively reviewed.
Gross-total resection (GTR) was achieved in six patients at the initial operation. The mean follow-up period was 75 months. Two patients received postoperative adjuvant therapies and two patients did not, against medical advice. None of the four patients experienced recurrence after long-term follow up. Another four patients, all of whom underwent GTR of the tumor, did not receive adjuvant therapy as part of a prospective treatment scheme. One of them exhibited early recurrence and metastasis. The tumor had pathological features denoting a high-grade (Norris Grade III) lesion and neurocytomatous differentiation.
Aggressive resection seems to be of utmost importance in the treatment of immature teratomas of the CNS. Adjuvant chemotherapy and radiotherapy can be deferred if GTR is achieved in low-grade, immature teratomas, but adjuvant therapies may be warranted for high-grade ones.
Chang Sub Lee, Seok Ho Hong, Kyu-Chang Wang, Seung-Ki Kim, Joong Shin Park, Jong-Kwan Jun, Bo Hyun Yoon, Young-Ho Lee, Son Moon Shin, Yeon Kyung Lee and Byung-Kyu Cho
The prognosis of fetal ventriculomegaly (FVM) varies because of the disease’s heterogeneity and the diversity of accompanying anomalies. Moreover, the cases that are referred to neurosurgeons may have different clinical features from those typically encountered by obstetricians. The object of this study was to delineate the prognosis of FVM in cases for which neurosurgical consultation was sought.
Forty-four cases of FVM that were diagnosed before birth and referred to neurosurgeons for prenatal consultation were analyzed retrospectively. Twenty-five of the 44 patients had accompanying anomalies, but in only three (12%) of the cases were they detected prenatally. Postnatal imaging studies revealed that agenesis of the corpus callosum (nine cases) was the most common associated anomaly. Neuronal migration disorders, periventricular leukomalacia, and arachnoid cysts were present in four cases each, and aqueductal stenosis was present in three cases.
Thirty-three patients were followed up longer than 11 months; in 15 (45%) delayed cognitive and/or motor development was documented, and all had accompanying anomalies. All 10 of the patients with isolated FVM exhibited normal development during the follow-up period. Eleven (25%) of the 44 patients underwent neurosurgical interventions for ventriculomegaly, which included ventriculoperitoneal shunt placement in seven cases. Four patients (9%) died.
The authors conclude that delayed development and disturbed functional status in patients in whom FVM was diagnosed prenatally are closely related to the presence of certain accompanying anomalies. On postnatal examination, more than half of the patients in whom the diagnosis of FVM was based on ultrasonography findings and whose parents were offered prenatal neurosurgical consultation were found to have additional anomalies that were not detected prenatally. Because of the possibility of additional undiagnosed anomalies, consulting neurosurgeons should be cautious in giving a prognosis in cases of FVM, even when prenatal ultrasonography reveals isolated ventriculomegaly and tests for intrauterine infection and chromosomal abnormality yield negative results.