Tumors of Lateral Ventricles in Cases with Tuberous Sclerosis: Diagnosis and Surgical Treatment

Gorelishev S.K.*, Satalin L.A.*, Huhlaeva E.A.*, Korshunov A.G.*, Ozerova V.I.*, Semenova Zh.B.**, Dorofeeva M.Yu.***

* Burdenko Research Institute of Neurosurgery, Moscow, Russia
** Scientific Research Institute of Emergent Pediatric Surgery and Traumatology, Moscow, Russia
*** Scientific Research Institute of Pediatrics and Pediatric surgery, Moscow, Russia

 

Tuberous sclerosis (TS) is a neurodermal disease, causing development of tumors of the CNS and internal organs.

Material and Methods. There were 42 cases with verified TS, operated for subependymal giant cell astrocytomas (SGCA) at the Research Institute of Neurosurgery in 1989-2006. Their mean age was 14.2 years. TS was unquestionable diagnosis. Tumors were giant in 37% of cases (a diameter of more than 5 cm); its size was less than 3 cm only in 10.5% of patients.

Direct tumor removal was performed in 41 cases; 1 patient underwent shunting operation. Anterior transcallous (72.9%), transcortical (21.6%) or combined (5.4%) accesses were used. Total, subtotal and partial tumor removal was performed in 44.8%, 31.6% and 23.6% of cases, respectively. Operation efficacy was conditioned by a tumor size. A histological picture was almost the same. Immunohistochemical examination revealed insignificant changes of an index of labeling by Ki-67. As for p27 and p14 epiproteins, they were present in the majority of tumors. A rate of apoptosis did not exceed 0.01%. Samples of all the tumors were of the same immunophenotype. SGCA removal had no effect on a structure and severity of paroxysmal symptoms and intellect. A follow-up period was 2.5 years. Further tumor growth was diagnosed in 6 cases 2-7 years after operation. Initial treatment of all of them consisted in only partial removal.

Conclusion. Cases with TS demand annual CT- or MR-examination for diagnosing SGCA at an early stage. SGCA removal is a method of choice in cases with TS. Total tumor removal is most probable, when its size is smaller than 5 cm.